Hearing Health Fall 2018

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Fall 2018  A Publication of Hearing Health Foundation  hhf.org

The Military & Seniors Issue Recognizing the service of our military, and the need for healthy aging and hearing for all


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letter from the chair

DEAR READERS & SUPPORTERS, it’s a real privilege this past year to have joined Hearing Health Foundation’s Board of Directors, all of whom have a strong personal connection to our precious human sense of hearing. I’ll be endeavoring to fill the shoes of Elizabeth (Betsy) Keithley, Ph.D., as chair of the board, as we transition into 2019. We owe Betsy an immeasurable debt of thanks for her many contributions to Hearing Health Foundation (HHF) and to the field of otolaryngology research. She has generously dedicated her entire career to the study of hearing and its preservation and restoration. This issue is devoted to our American military, many of whom return from theaters of combat in Iraq and Afghanistan with significant hearing loss and tinnitus, often accompanied by post-traumatic stress disorder as well. Year after year, the military tops the annual Gallup Poll on confidence in American institutions. We revere these men and women for their willingness to go into harm’s way to preserve our freedom and who, even in peacetime, are exposed to dangerous noise levels as part of their extreme environments. I am proud to have served alongside so many who selflessly performed their duties. As careful as I was as a U.S. Army officer during peacetime years, I still became a “casualty” to noise-induced hearing loss from large-caliber gunfire and the high-pitched whine of many different aircraft engines. Our cover story is by Bruce Douglas, D.D.S., a World War II and Korean War veteran, and a professor of health and aging at the University of Illinois in Chicago. He tells his poignant story of helping others while himself suffering hearing disabilities (page 6). James Henry, Ph.D., of the National Center for Rehabilitative

Auditory Research in Portland, Oregon, describes the current standard of care for tinnitus, a condition he has been researching for the past quarter century (page 10). While all of the U.S. Armed Forces now try to better equip service members with hearing protection, a new U.S. Air Force program is aimed at better measurement of noise exposure around the clock (page 14). HHF medical director David Haynes, M.D., FACS, and his colleague René Gifford, Ph.D., CCC-A, remind us of the advantages of cochlear implants for cases of severe or profound hearing loss (page 18). We also stress the importance of addressing age-related hearing loss for both health and financial reasons (page 22). As always, we are truly grateful to your donations to fund research in a broad range of hearing and balance issues that affect so many lives. Every research grant approved by HHF is carefully scrutinized for the greatest likelihood of innovation and impact, and we are pleased to have recently gained approval by the Combined Federal Campaign, the workplace giving program for the U.S. federal government. Thank you for your continued support.

Colonel John T. Dillard, U.S. Army, Retired Chair-Elect, Hearing Health Foundation’s Board of Directors

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HEARING HEALTH The Military & Seniors Issue Fall 2018, Volume 34, Number 4

As we salute military service members who risk their lives and health to preserve our freedom, we also recognize the importance of healthy aging—including hearing—for all.

Publisher Nadine Dehgan CEO, Hearing Health Foundation Editor Yishane

Art Director Robin Senior Editors

Features

10 Veterans Tackling Tinnitus. James A. Henry, Ph.D. 14 Military Total Noise Exposure. Richard Hartman, Ph.D. 16 Veterans Blue Skies Above. E. Bryan Crenshaw III, Ph.D. 18 Cochlear Implants Your Concerns About Cochlear Implants, Answered. René H. Gifford, Ph.D., CCC-A, and David S. Haynes, M.D., FACS 20 Seniors The Miracle of the Cochlear Implant. Barbara Sinclair 22 S eniors How Hearing Loss Affects Everything Between Your Ears. Barbara E. Weinstein, Ph.D., and Elizabeth Crofts

Amy Gross, Lauren McGrath

03 Letter From the Chair 26 Planned Giving Heinrich Medicus, Ph.D. 27 Meet the Fundraisers Holly dee Gadow; Beth and Jeremy Hochheiser

Staff Writers

36 Research Presenting the 2018 Emerging Research Grantees.

advertising@hhf.org, 212.257.6140

40 Research Toward Customized Hearing. Elizabeth Crofts 42 Research Recent Research by Hearing Health Foundation Scientists, Explained. 44 Research The Hearing Restoration Project: Update on the Seattle Plan and More. Peter G. Barr-Gillespie, Ph.D. 46 Research Accomplishments by ERG Alumni.

50 Meet the Researcher A. Catalina Vélez-Ortega, Ph.D.

Sponsored 48 Advertisement Gift Guide. 49 Marketplace

Hearing Health Foundation and Hearing Health magazine do not endorse any product or service shown as paid advertisements. While we make every effort to publish accurate information, we are not responsible for the correctness of information herein.

Cover Bruce L. Douglas, D.D.S., shown around the time his hearing was damaged from rifle practice on Parris Island, South Carolina, in 1951.

Visit hhf.org/subscribe to receive a FREE subscription to this quarterly magazine. hhf.org

S. Haynes, M.D.

32 Hearing Health A Fast Track to Hearing Damage. Andrew J. Guralnick. Noise: Exposed. Nadine Dehgan

Departments

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Medical Director David

06 Veterans Here’s to Healthy Aging. Bruce L. Douglas, D.D.S.

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Barbara Jenkins, Au.D. Emily L. Martinson, Au.D., Ph.D. Kathi Mestayer Advertising

Editorial Committee

Peter G. Barr-Gillespie, Ph.D. Robert A. Dobie, M.D. Judy R. Dubno, Ph.D. Melissa E. Heche, Au.D. Anil K. Lalwani, M.D. Joscelyn R.K. Martin, Au.D. Board of Directors

Elizabeth Keithley, Ph.D., Chair Sophia Boccard Robert Boucai Noel L. Cohen, M.D. Col. John T. Dillard, U.S. Army (Ret.) Judy R. Dubno, Ph.D. Ruth Anne Eatock, Ph.D. Jason Frank Roger M. Harris David S. Haynes, M.D. Anil K. Lalwani, M.D. Michael C. Nolan Paul E. Orlin Robert V. Shannon, Ph.D. 363 Seventh Avenue, 10th Floor New York, NY 10001-3904 Phone: 212.257.6140 TTY: 888.435.6104 Email: info@hhf.org Web: hhf.org Hearing Health Foundation is a tax-exempt, charitable organization and is eligible to receive tax-deductible contributions under the IRS Code 501 (c)(3). Federal Tax ID: 13-1882107 Hearing Health magazine (ISSN: 0888-2517) is published four times annually by Hearing Health Foundation. Copyright 2018, Hearing Health Foundation. All rights reserved. Articles may not be reproduced without written permission from Hearing Health Foundation. USPS/Automatable Poly To learn more or to subscribe or unsubscribe, call 212.257.6140 (TTY: 888.435.6104) or email info@hhf.org.


5 Ways to Make a Long-Term Impact You can make a meaningful impact on Hearing Health Foundation’s future. Cash, check, or credit card gifts by mail, phone, or online are easy—especially as a recurring monthly donation—but you may also want to consider these longer-term, alternative options.

Charitable bequest

If you would like to make a substantial gift to HHF, but do not have the means to do so today, please consider a charitable bequest. An example of the language used in wills is: “I give to Hearing Health Foundation the sum of $__________ (or _____ percent of my estate) to be used by HHF to further its mission.”

Charitable remainder trust This provides for annual payments to the designated beneficiary(ies) at a specified percentage—at least 5 percent of the value of the trust as it is valued each year. Since the value may vary year to year, the payments may also vary.

Stock donation

A donation of appreciated stock can reduce your tax bill, because you receive a charitable tax deduction for the full value of the stock today and will avoid paying capital gains tax on the stock as it appreciates.

Gift of retirement plan at death

Retirement-plan benefits left to heirs are often more highly taxed than other assets. Consider making a meaningful gift to HHF instead, and leave other lower-taxed assets to heirs.

Life insurance beneficiary

If you have a life insurance policy you no longer need, you can designate HHF as the beneficiary and make a considerably larger gift than you thought possible—larger than if you were to donate the cash equivalent of the policy’s premiums.

Thank you for your support of HHF’s mission to prevent and cure hearing loss and tinnitus through groundbreaking research and to promote hearing health. Please learn more at hhf.org/how-to-help, by emailing development@hhf.org, or by calling 212.257.6140.

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HEALTHY AGING A veteran, oral surgeon, professor, legislator, and activist harnesses decades of experience to boost our collective and individual health equity. By Bruce L. Douglas, D.D.S.

The port of Sasebo, Japan (top), is where I was transferred so I could be closer to the front in Korea (just 90 miles away). I was certified to administer anesthesia for surgery and used a wire recorder (foreground, bottom) with earphones for patients to listen to relaxing music as they fell asleep.

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i am a healthy 93-year-old adult with a severe hearing loss. Many parts of my body don’t work as I would like them to anymore, but my hearing loss has been my biggest physical challenge. People with hearing loss often cite the example of Helen Keller who, when comparing blindness with deafness, observed that it was much easier adjusting to sightlessness than total hearing loss because, while blindness obstructed her contact with things, hearing loss interfered with her relationships with people. Those of us who know how it feels to sit amid family and friends and have no idea what is being discussed have a responsibility to coach our unknowing friends, colleagues, and family members to understand that fact, and to accept willingly our requests to sit in certain parts of the room, turn off background music, not to try to communicate with us from another room, or, when our backs are turned, delay conversation until we see each other face to face. Hearing loss is an obstruction to human communication. It is, as yet, not completely correctable with hearing devices, although slowly but surely we appear to be getting to the point where with science and technology we may someday be able to reverse hearing loss. I wore hearing aids for decades, and in 2016 received a cochlear implant, soon after I turned 91. After more than six decades working as a dentist, oral surgeon, service member, legislator, and activist, both in the U.S. and overseas, my current role as a professor of health and aging at the University of Illinois at Chicago (UIC) School of Public Health is to research how aging affects quality of life. Call it the logical culmination to a long career: I want to detail the importance of the ability to communicate, especially in the face of hearing loss, for healthy aging.

Front of the Room

As a boy, I remember always taking a seat at the front of the room, when most other kids would vie for one in the back, so they could cheat on exams without being seen by the teacher. I was the “goody-goody” up front. I didn’t realize how poor my hearing was until I was reprimanded for inconspicuously (I thought) using a nail clipper under my desk in class. I had no idea because I couldn’t hear it! I was more aware of my hearing limitations as a teen but didn’t know where to turn. Our family doctor even had a pair of hearing aids on his desk during one visit, but when I asked about them he told me to get hearing aids when I was older.


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To compensate for my hearing loss, I kept sitting at the front, even placing a “reserved” sign on a chair so I could be sure to get a seat, and stared intently at people as they spoke, subconsciously learning to lip-read (now called speech-reading). I tended to monopolize conversations—not realizing till later that so long as I did all the talking I didn’t have to strain to hear other speakers (or their opinions). All are antisocial behaviors that helped me but surely annoyed others. My father, with whom I share many similar characteristics, had a “favorite” word—“Huh?”—which is how he invariably responded to my mother’s questions. They argued about his inability to hear everything and her inability to see everything, and this discord got under my skin as a child. Not infrequently I would leave the room. While this kind of social interchange might have been humorous in banter between Archie and Edith Bunker, I realized that untreated hearing loss was a cause of family dysfunction when I was growing up. The same is true in families with returning warriors today, where hearing loss and tinnitus are the major exiting maladies veterans have to learn to live with in civilian life. In recent years science has confirmed the various health conditions associated with untreated hearing loss: higher risks for cardiovascular disease, cognitive decline and dementia, depression, diabetes, and falls.

Service Overseas

I grew up in Brooklyn, during the Great Depression, but was fortunate to attend an international scholarship camp in New York for several summers that created a lifelong interest in world cultures and serving others. One exercise at Camp Rising Sun was to write your 21-year-old self a letter. Mine included the prediction I would live into the 21st century and devote myself to serving humanity beginning with the year 2000. I enlisted in the U.S. Navy while in my first year of college. It was 1943 and two years into America’s involvement in World War II, but the Navy allowed me to remain at Princeton. The war ended while I was in graduate school for dentistry and oral and maxillofacial surgery, but I had incurred an obligation to return for active duty during the Korean War. It was during training on Parris Island, South Carolina, in 1951, when my life changed forever: A sergeant made us­—75 Navy dentists—endure rifle practice to fulfill his dream of exacting revenge on pain-causing dentists. A drill for a drill, he called it. On what was my 26th birthday, after pulling the trigger on the M1 rifle with no protection (none of us had any) multiple times, I was left with tendonitis in both knees—and worse, permanent, chronic tinnitus due to acoustic trauma. (The photo above is from that fateful day.) My hearing went downhill ever after, and every imaginable kind of sound and sensation has resulted from my tinnitus.

On what was my 26th birthday, after pulling the trigger on the M1 rifle with no protection (none of us had any) multiple times, I was left with tendonitis in both knees—and worse, permanent, chronic tinnitus due to acoustic trauma. My hearing went downhill ever after, and every imaginable kind of sound and sensation has resulted from my tinnitus.

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I will speak up for anyone with a hearing condition in asking others to be patient and accept the reality that we have an invisible condition that wove its way into our nervous system, most often beyond our control. We do all we can to listen to you and respond to the best of our ability.

On his first trip to the Korean war zone, Navy Surgeon General Admiral Lamont Pugh visited my oral surgery operating room to see my musical analgesia machine in use. The Navy had publicized this novel, humane way of treatment for extracting wisdom teeth.

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I was stationed in the Japanese port of Sasebo, where U.S. Marines took off for the Korean battleground, and I joined them periodically under M.A.S.H. tents to assist with wounds from tripping landmines. I had another life-changing acoustic event during that time. In 1952, in an accident while it was being serviced, the USS Ashtabula blew up less than a football field away from where I was standing. I was one of the first responders and didn’t realize the effect on my hearing until bedtime. I was deaf for a week afterward, but gradually I regained enough hearing to function. Back in the U.S., I realized that practicing in a private clinic wasn’t for me. I returned to Asia with my wife and 1-year-old son on a Fulbright grant, to be a professor of oral surgery and anesthesiology at Okayama University in western Japan. Okayama is an hour by train from Hiroshima, and in 1959 Japan was just over a decade from the end of the war. Some of my students were the children of victims of the Hiroshima atomic bomb blast, and we were all still healing, literally and figuratively, from the world war our countries fought.

Progress in Audiology

In the ensuing decades I became an outspoken critic of the Vietnam War and a proponent of a national healthcare system, an advocate for community health worldwide, and an expert on the older worker, as well as a newspaper editor, textbook writer, and the founding first faculty member of the UIC School of Public Health (UICSPH). But I did not get my first hearing aids until 1980—made, coincidentally, by my former dental student George Osborne, Ph.D., DDS (who studied dentistry before returning to audiology). He was frank about those early devices, telling me they would merely amplify, not clarify. Thanks to rapid advances in technology, my most recent hearing aids worked very well, and while I knew about cochlear implants (and, having practiced maxillofacial surgery for 66 years had no problem understanding its being a minor surgery), I assumed as a nonagenarian I could not be a candidate. But audiologists at the Edward Hines, Jr. Department of Veterans Affairs (VA) Hospital convinced me otherwise—and I cannot begin to express my gratitude. I had surgery on my nearly deaf right ear, and went through an almost two-year period of adjustment. My brain jumped to the challenge of teaching my two ears to talk to each other, through the implant and a hearing aid, so I was able to remain active with both family and work. In fact, my latest faculty appointment at UIC is my sixth (since 1962), believe it or not! It took many years for me to try to come to terms with my untreated hearing loss. I refused to hide in corners of rooms and restaurants, and dealt with my problem largely by disclosing my hearing loss to people and asking for their cooperation in our verbal relationships. I will speak up for anyone with a hearing condition in asking others to be patient and


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accept the reality that we have an invisible condition that wove its way into our nervous system, most often beyond our control. We do all we can to listen to you and respond to the best of our ability.

The Study of Senescence

I’m very lucky, so far, to have lived into my 90s. And I’m especially fortunate that the few downsides to my health are not issues that are likely to affect my longevity. So, onward I go to fulfill my pledge of devoting the years gifted to me since the turn of the century (when I turned 75) to serve humanity. As I entered what turned out to be my first retirement, I received an unexpected call from the Illinois Chapter of the AARP inviting me to serve on the Illinois lobbying team for older Americans. Since I had already qualified both for Medicare and senior citizenship, in cooperation with the AARP I started my research into the issue of the older worker at UICSPH’s department of environmental and occupational health sciences. My wife’s career in the world of human capital management and her books on absence management, workers’ compensation, and integrated disability management—all areas with direct application to the aging workforce—set me off on an advocacy pathway on behalf of a rapidly aging world population. To this day, I have not wavered in my support of older citizens and the processes—biological, social, and psychological—that impact the quality of our lives as we enter our later years. In 2013, I was invited by the

Senescence refers to the biological and physiological changes that occur in all human beings as they age. Aging is simply a measure of the number of years since birth. Everyone ages at the same chronological rate; senescence, on the other hand, is highly variable and proceeds at different rates in different individuals in the same species.

dean of UICSPH to join a team to lay the groundwork for a broadened and pragmatic approach to senescence, a process that affects all human cellular life and is, in essence, the ultimate cause of the end of that life. Senescence refers to the biological and physiological changes that occur in all human beings as they age. Aging is simply a measure of the number of years since birth. Everyone ages at the same chronological rate; senescence, on the other hand, is highly variable and proceeds at different rates in different individuals in the same species. The senescent differences basically relate to genetic makeup and a number of lifestyle factors that influence the rate at which our bodies and their many organs change with advancing age. To a significant degree, aside from chance, successful aging is dependent on how diligently an individual self-manages their own well-being throughout life, including treating any hearing condition. Those of us who have merged into a common community called “public health” bring views from individual professions that emphasize the importance of health equity, which relates not only to race and gender but also to different categories in the aging spectrum, and the approaches, based on evidence-based research, to each of them, from the cradle to the grave. I look forward to yet more discoveries and treatments in science, medicine, and technology for hearing loss and senescence, in time for my 100th birthday in 2025.

Bruce L. Douglas, D.D.S., M.A., M.P.H., is a professor of health and aging at the University of Illinois at Chicago School of Public Health. He is a participant in HHF’s Faces of Hearing Loss campaign, hhf.org/faces.

Share your story: Is your hearing condition connected to your military service? Tell us at editor@hhf.org. Support our research: hhf.org/donate

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TACKLING TINNITUS The National Center for Rehabilitative Auditory Research has been quantifying, assessing, and managing tinnitus, the number one disability among veterans—and finding success. A leading researcher who has focused on the hearing condition for the past 25 years shares the progress made. By James A. Henry, Ph.D.

What Is the NCRAR?

One of the statutory missions of the U.S. Department of Veterans Affairs (VA) Veterans Health Administration is to fund healthcare research. The research arm of the VA is the Office of Research and Development. One of its four branches is the Rehabilitation Research & Development Service, which funds 13 centers, each of which focuses on an area of healthcare relevant to veterans.

the veterans benefits administration reported tinnitus as the second most prevalent service-connected disability for veterans annually from 1994 to 2006. In 2007, tinnitus became the most prevalent service-connected disability, and has remained so every year since. As of 2016, over 1.6 million veterans had a service-connected disability award for tinnitus. The National Center for Rehabilitative Auditory Research (NCRAR) at the VA Portland Health Care System in Oregon is dedicated to addressing the rehabilitation needs of veterans with hearing and balance conditions. Since it was founded in 1997, one major area of research has been tinnitus, or the experience of hearing sound (such as ringing or buzzing) without an external sound source. Primary tinnitus, or tinnitus with an unknown cause, of a duration of at least six months is usually a permanent condition with no cure currently available. Individuals whose tinnitus is bothersome must rely on methods designed to reduce emotional reactions and functional limitations associated with tinnitus. The NCRAR has focused on developing such methods, with the overall goal to develop and validate all procedures necessary for assessing the tinnitus condition and providing evidence-based intervention.

Standardizing Assessment The National Center for Rehabilitative Auditory Research (NCRAR), located at the VA Portland Health Care System in Oregon, is one such center, focusing on the rehabilitation needs of veterans with auditory-system impairments and balance dysfunction.

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The NCRAR’s first tinnitus project focused on tinnitus psychoacoustic assessment. Like any real sound, tinnitus has different acoustic qualities— loudness, pitch, and “spectrum.” The NCRAR team developed computerautomated methods to vary these qualities of real sounds to match a person’s perception of tinnitus. This work has been near-continuous since 1997. Most individuals who experience tinnitus do not require tinnitusspecific clinical services. Determining where a patient should be placed on the continuum from non-bothersome to debilitating tinnitus requires the appropriate instruments for measuring tinnitus reactions and disability. The NCRAR team has focused on the development and validation of such instruments. In collaboration with the late Mary Meikle, Ph.D., the Tinnitus Functional Index (TFI) was developed as the first tinnitus questionnaire validated for “responsiveness” (sensitivity to changes in tinnitus effects resulting from intervention).


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First published in 2012, the TFI has garnered considerable international attention and has been translated into more than 15 languages. The TFI is attaining recognition as a “standard” tinnitus questionnaire, which will enable outcomes of tinnitus services to be directly compared across different clinics and research studies. Additional instruments developed by the NCRAR team are: (1) the Tinnitus and Hearing Survey, which addresses the problem of distinguishing between tinnitus and hearing difficulties as the source of patients’ disabilities; and (2) the Tinnitus Screener, an algorithmic instrument used to differentiate temporal categories of tinnitus (constant, intermittent, occasional, temporary, or spontaneous). These instruments have been shown to be valid and reliable tools for both clinical and research applications.

Validation of a Management Program

In 1999, the NCRAR tinnitus research team was funded for the first of a series of 13 clinical trials (12 complete; one underway) to evaluate different methods of tinnitus clinical management. These trials have identified procedures that are the most efficacious, and efficient, for VA clinical application. The culmination of these efforts is the development of Progressive Tinnitus Management (PTM), an interdisciplinary, stepped-care program that combines elements of acoustic therapy and cognitive-behavioral therapy. The five levels of PTM are: Level 1 Referral; Level 2 Audiologic Evaluation; Level 3 Skills Education; Level 4 Interdisciplinary Evaluation; and Level 5 Individualized Support. With PTM’s stepwise approach and validation efforts through clinical trials, so far a very small number of patients have required Level 4 or Level 5 services. Level 1 Referral consists of referral guidelines for any provider encountering patients who complain of tinnitus. Level 2 Audiologic Evaluation involves audiologic testing, brief tinnitus assessment, hearing aids if needed, and brief tinnitus counseling. The NCRAR research team has completed two randomized controlled trials to evaluate the use of hearing aids and combination instruments (hearing aids with built-in sound generators) for tinnitus management. These clinical trials validated both types of ear-level devices as efficacious for tinnitus management—findings that support Level 2 Audiologic Evaluation as the gateway of any PTM program. Patients requiring tinnitus services beyond Level 2 Audiologic Evaluation are advised to attend Level 3 Skills Education, involving five sessions (individual or group) of educational counseling to learn self-care skills for bothersome tinnitus. An audiologist who teaches the principles of sound-based therapy conducts two of the sessions, and a mental health provider who administers components of cognitive-behavioral therapy teaches the remaining three. In a VA-funded project, the NCRAR conducted a “clinically embedded” randomized controlled trial at two VA medical centers—Memphis, Tennessee; and West Haven, Connecticut—revealing the efficacy of Level 3 Skills Education. A second randomized controlled trial, evaluating a telephone-administered version of Level 3 Skills Education (“Tele-PTM”), demonstrated the efficacy of remote counseling for bothersome tinnitus. These NCRAR clinical trials have provided evidence for delivering effective services for tinnitus, either in the clinic or via telehealth, that address the tinnitus needs for the great majority of veterans suffering from this invisible condition. In 2009, the VA National Audiology and Speech Pathology Service has endorsed PTM as the VA standard of care for tinnitus, and since then the NCRAR tinnitus team has conducted numerous trainings and individual consultations to hundreds of clinicians providing tinnitus services to veterans.

Like any real sound, tinnitus has different acoustic qualities: loudness, pitch, and “spectrum.” The NCRAR team developed computer-automated methods to vary these qualities of real sounds to match a person’s perception of tinnitus.

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Epidemiological Research

NCRAR clinical trials have provided evidence for delivering effective services for tinnitus, either in the clinic or via telehealth, that address the tinnitus needs for the great majority of veterans suffering from this invisible condition.

The NCRAR continues to increase its collaboration with the U.S. Department of Defense Hearing Center of Excellence (HCE). Most notably, we are conducting a collaborative epidemiology study. Informally referred to as the Noise Outcomes in Servicemembers Epidemiology Study (“NOISE Study”), the project was designed to examine the prevalence of, and risk factors for, tinnitus and hearing loss among military service members and veterans. This study was originally funded by Congressionally Directed Medical Research Programs for three years. The current program is a four-year continuation, with the intent to sustain the study for 20 to 30 years. A multisite study, it includes the NCRAR as the main site and the HCE in San Antonio, Texas, which has established two testing sites. To date, over 630 veterans and active service members have been enrolled. Focused intently on “restoring veterans to their family, job, and life,” NCRAR tinnitus research has resulted in clinical methodology that has been endorsed by the VA Central Office for use throughout the VA system. Both clinically and during its development, PTM has provided relief to innumerable veterans. Continued research will ensure the maximum reach of this evidencebased service to veterans in need. (And it should be noted NCRAR tinnitus research extends beyond that of my work, which is what is covered here.) It is clear that the NCRAR’s research on tinnitus, PTM development, and understanding the epidemiology of tinnitus and hearing loss will continue to generate knowledge that reduces suffering and improves the quality of life for veterans who experience tinnitus.

James A. Henry, Ph.D., is a VA Rehabilitation Research & Development (RR&D) Research Career Scientist at the RR&D National Center for Rehabilitative Auditory Research (NCRAR), located at the VA Portland Health Care System in Oregon. He is also a research professor in the department of otolaryngology–head and neck surgery, Oregon Health & Science University. He received the 2017 Jerger Career Award for Research in Audiology from the American Academy of Audiology, and the RR&D 2016 Paul B. Magnuson Award for innovative work on the treatment and management of tinnitus. To read Henry’s article about Progressive Tinnitus Management, “A Progressive Approach,” see the Summer 2013 issue at hhf.org. For references, see hhf.org/fall2018-references.

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military

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“exposomics” is the measurement of all of an individual’s exposure to diseasecausing environmental factors over the course of their lifetime, while taking into account the individual’s unique genetic makeup (genotype) and how that affects their health. It is known that people with different genotypes have diverse reactions to the same environmental factor. Put another way, how a person is affected by an outside health risk factor is a combination of your genes and your environment. Noise is one such environmental health factor. Left untreated, hearing loss is associated with certain chronic diseases, dementia, depression, and falls. According to a 2017 report (using 2012 population data) by the Centers for Disease Control and Prevention, 40 million American adults show signs of noiseinduced hearing loss (NIHL), yet more than half A novel U.S. Air Force program reported no captures an individual’s 24/7 exposure to loud noise exposure to noise along at work, the with genomics data to better usual source. protect against hearing loss. While By Richard Hartman, Ph.D. hearing loss does not discriminate by age, race, gender, and/or socioeconomic status, the condition is more prevalent in military populations. More than one million active service members and veterans have hearing conditions, and hearing loss and tinnitus are consistently the top medical complaints among service members and veterans. Hearing health expenditures range from $1.5 billion to $3 billion annually for the U.S. Department of Veterans Affairs (VA) in benefits and medical costs. What’s more, the military in particular demands excellent hearing: It is critical for mission readiness—for communication and awareness. Ten published studies, with small to modest sample sizes, have established a link between multiple genetic variants associated with NIHL.

TOTAL

NOISE EXPOSURE

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The effect is surprisingly large, with odds ratios of 5.2 to 22.36 indicating an elevated risk. So the question is: What if a person’s cumulative, daily exposure to noise could be measured, and this data then combined with their genetic predisposition for NIHL, in order to optimally protect against it? More broadly speaking, what if—by aggregating big data and genomics—a person’s susceptibility to external health risk factors could be predicted and then alleviated using individualized health protocols?

Capturing Noise Levels

To test this concept, the U.S. Air Force (USAF) Medical Service launched a bold initiative to institute true primary prevention called Total Exposure Health (TEH). The first focus was on noise exposure, with the idea to expand TEH to other physical risks such as radiation and chemical exposure from the workplace and environment as well as lifestyle risks from recreational activities and diet. Given the cost of NIHL to the military in terms of health, safety, and dollars, this project aimed to eliminate any decisions being made using incomplete data that could lead to permanent hearing loss that may be preventable, and that also affect mission readiness. In addition, effective hearing protection options are available, thanks to extensive U.S. Department of Defense research and development investments into such devices as triple-hearing protection (form-fitting earplugs plus sound-canceling earmuffs). The environmental risk factor, the population to test, and the solution at hand were all in place. Also known is that among the USAF population, 17 percent show a gene variant, rs7598759, indicating they may be at a substantially increased risk for a hearing threshold shift—that is, of a hearing loss susceptibility due to noise. The study used sensor technology to collect total noise exposure 24 hours a day, seven days a week. Unique to these noise monitoring devices, the USAF wearable sensor collects sound events from both ambient noise in the workplace and environment as well as in-ear sounds from ear buds used with smartphones and other devices to capture digital media (such as music). A smartphone app recorded those decibel (dB) readings. These readings were combined with medical information gathered via the Defense Occupational and Environmental Health Readiness System, and specific genetic data associated with NIHL.


military

Given the cost of NIHL to the military in terms of health, safety, and dollars, this project aimed to eliminate any decisions being made using incomplete data that could lead to permanent hearing loss that may be preventable, and that also affect mission readiness. Twenty-two subjects participated in this study at Moody Air Force Base, Georgia, over 10 days in June 2018. Captured were 12,680 noise events of 70 dBA or above; of these, 2,968 events, or 23 percent, were 95 dBA or above. (A-weighted decibels are adjusted for human hearing.) The average daily decibel dose was about 75 dBA. About 10 percent of the subjects had total daily noise exposures under 70 dB, and 10 percent over 80 dB, with the majority in the middle. The study found high noise exposure both at the USAF workplace (46 percent of users) and while offduty (52 percent), and significant, cumulative, high noise exposure on an individual basis (three to 27 hours over the 10-day study period). It also identified geospatial “hotspot” locations of high noise exposure. Participants were provided with form-fitting hearing protection, but despite sensor and app warnings when volume levels reached over 90 dBA, the study found participants opted to use the protection in just 1.4 percent of high noise events.

Analyzing Data Points

Sound intensity exposure is dose dependent. The Occupational Safety and Health Administration (OSHA) noise guideline is a maximum 90 dBA

for the eight-hour workday—but what happens if, after clocking in a day’s work on the airfield, a service member has dinner in a noisy restaurant? When a person’s 90 dBA noise exposure exceeds eight hours, this pushes them over the regulated OSHA level. And if they are genetically predisposed to hearing damage, the risk of NIHL is even greater. Overall, the TEH noise study, as the demonstration project, met its objectives of developing a low-cost noise dosimeter/sensor that monitors external/environmental as well as internal/smartphone volume levels around the clock; using advanced analytics to collate multiple sensor devices, with geospatial layering; and managing participant compliance. The next steps are to expand this demonstration project to better understand and improve user compliance with the monitoring devices; account for hearing protection use; and survey a larger sample size to effectively train base families on the dangers of noise and to raise awareness. Boosting the consistent use of hearing protection when the sensors send loud noise alerts is another goal. This noise study validates the application of TEH, demonstrating how public health professionals can

leverage exposure measurements combined with genomic data and medical information to create individualized intervention strategies. Through the TEH, the hope is to create a movement where people can take control of their health and make informed decisions with transparent, accurate, and understandable health information.

Richard Hartman, Ph.D., is an independent consultant. HHF intern Elizabeth Crofts contributed to this article. For references, see hhf.org/fall2018-references.

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veterans

h ear i n g health foundation

BLUE SKIES ABOVE A hearing researcher shares his love for nature with

military service members. By E. Bryan Crenshaw III, Ph.D.

Fellowship with other vets allows for unfettered time to swap “war stories” with peers. Being able to find common ground through these shared experiences is often cathartic.

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camp elk tannery is a place in the idyllic northeast Pennsylvania countryside where military veterans can find solace. Several times a year, veterans gather at the camp, 110 largely untouched acres nestled in the valley between the North and Central Mountains just below the beautiful Ricketts Glen State Park, to share experiences with fellow active and former service members. The weekend outings, largely spent outdoors, are filled with camaraderie with other veterans. Research has shown that time spent in nature is good for the soul, resulting in reduced stress and a renewed sense of awe about our natural world. Fellowship with other vets also allows for unfettered time to swap “war stories” with peers. Being able to find common ground through these shared experiences is often cathartic. These veterans outings came to be as a result of the combined efforts of the national Sierra Club, local Sierra Club chapters, and several individuals who understood the deep value in enjoying the outdoors together. It started when the national club began to build a broad-based coalition to fight climate change and environmental injustice, embarking on outreach programs to people who did not fit into the typical Sierra Club member mold, including conservative groups such as hunters, fishermen, and veterans. The national group incorporated an outside organization that led outings for veterans and formed the Sierra Club Military Outdoors (SCMO) program. SCMO then made the connection with Monte Kapec, a deputy chief of staff in the U.S. Army Medical Command. An army veteran himself, Monte wanted to open up his family property in rural Pennsylvania as a venue for vets outings. At the same time, our local Southeast Pennsylvania Group of the Sierra Club


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had reached out to the Veterans Multi-Service Center in Philadelphia. When asked for ideas for outings that we could take with the vets in Philly, I had many, because I had hiked and biked around the city and the surrounding area with my son ever since he was a toddler. Once the SCMO connected with Monte, it was a natural fit for our local Philly group to organize a trip to his property, Camp Elk Tannery, about three hours from the city. The first outing at “the Tannery” in the spring of 2015 brought in our group plus vets from New York City and elsewhere in Pennsylvania. Spending time with veterans (including my own relatives), many of whom deal with lifelong consequences from their service, from mental trauma to physical disabilities, also helps ground me in my day job as a hearing researcher at The Children’s Hospital of Philadelphia. Tinnitus and hearing loss are the top two service-connected disabilities among all veterans. Although my research is mainly with children, from working in the hearing loss field I know firsthand how untreated hearing loss is associated with other health consequences, including depression and cognitive decline. As with most things Sierra Club, a largely volunteer-based grassroots organization, the format for the outings grew organically. Monte, who had a clear idea of what he wanted the camp to become, had contacts with local vets, one of whom led an archery program at the nearby Wilkes-Barre VA Medical Center. The outings evolved to include bonfires, card games, and hikes, all of which seem to flow naturally from being outdoors in the camp’s bucolic setting. Monte founded the Vets in the Valley Foundation, an organization whose mission is to support the vets outings at the Tannery. This year we were one of six awardees of the 21st Annual Huplits Wildlife Grants Competition, administered by another local Sierra Club chapter, the Allegheny Group of the Pennsylvania Sierra Club. The grant recognizes the Tannery’s veterans outreach and forest and wildlife conservation efforts. Often, the service members I bring from Philly are Vietnam War–era vets from highly urbanized parts of the city, a few of whom told us that they had not been in the forest since their time in Vietnam. Many of them really appreciate the opportunity to be in nature again, but some are quite apprehensive. I remember one hike along a stream during which a vet looked very nervous, so I began a conversation with him, trying to alleviate any anxiety that he may have about the outdoors, such as bears or rattlesnakes. As it turned out, the experience reminded him of his days in ’Nam, where many patrols were deployed along waterways. Eventually, he seemed more comfortable with the hike, but this anecdote reminds me of the debt of gratitude that we owe our vets for the burdens that they still carry with them, even after so many years.

A former member of Hearing Health Foundation’s Scientific Advisory Board, E. Bryan Crenshaw III, Ph.D., is the director of basic science research in the division of otolaryngology and a scientist at the Center for Childhood Communication at The Children’s Hospital of Philadelphia. For more about Camp Elk Tannery, see campelktannery.org.

Tinnitus and hearing loss are the top two service-connected disabilities among all veterans. From working in the hearing loss field, I know firsthand how untreated hearing loss is associated with other health conditions, including depression and cognitive decline.

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cochlear implants

hearing health foundation

CI

Your Concerns About Cochlear Implants, Answered

By René H. Gifford, Ph.D, CCC-A, and David S. Haynes, M.D., FACS

“If you qualify for a cochlear implant (CI) but do not have one, please indicate reasons you have not pursued.” This question was one of three dozen on hearing conditions posed in Hearing Health Foundation’s 2017 Reader Survey, which was administered through the pages of this magazine, online, and also with Hearing Loss Association of America, through their magazine and online. More than 2,300 people responded to the survey. Those who responded to the CI question above gave the following answers, and they were able to check off as many as applied. In order of popularity, the responses were: 1) not convinced of improvement, 2) surgery complications, 3) waiting for biological cure, 4) concerns about sound quality, 5) cosmetic, and 6) fear of discomfort.

Although “other” with a fill-in option was offered, age did not appear to be a primary concern. In fact, many older adults with severe to profound hearing loss whose hearing aids are no longer are beneficial have found success with CIs, as demonstrated by Barbara Sinclair (page 20), who received an implant 17 years ago at age 72, and our cover story author Bruce Douglas (page 6), who had implantation surgery at age 91. Part of the survey’s purpose is to better understand the needs of our community of readers and supporters, and so, as cochlear implant surgeons, we wanted to address these concerns.

on data and experience, answers this question with an incredible degree of accuracy. Our goal is to reach a level of hearing that dramatically outperforms the best hearing aid outcomes for a given individual. Expectations are much higher than this, however, and it is extremely rare for a patient who is wearing their implant full-time not to experience much better preoperative hearing performance. The benefit has been so pronounced that Vanderbilt and other CI centers are working to expand implantation criteria so that this technology reaches people with milder forms of hearing loss.

Not convinced of improvement

Surgical complications

All CI centers, including ours at Vanderbilt University Medical Center, perform extensive presurgical testing to determine if a CI is the right option for a patient, versus the continued use of hearing aids. The testing, based 18

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Cochlear implantation has one of the most favorable risk–benefit ratios of any surgical procedure in the U.S., offering significant communicative benefit while incurring little risk. Our center performs nearly 300 implants

per year, and we monitor and track all procedures, outcomes, and complications. As with any operation your surgical team will provide a list of potential complications in order to be comprehensive, but the actual incidence of CI surgery complications ranges from under 1 percent to 3 percent. If any do occur, they are considered minor and temporary, such as postoperative taste disturbances and dizziness. At most CI centers, implantation is completed as an outpatient procedure and generally performed in 1 to 1.5 hours. We recently completed cochlear implantation on a 96-yearold patient who went home on the same day of surgery.

Waiting on a biological cure

The field of hearing restoration through hair cell regeneration—some of which is being conducted by HHF scientists, through the Hearing


cochlear implants

has never heard). If this occurs, it typically dissipates with continued use of the CI and the stimulation of auditory pathways. Signal processing technology also continues to advance at a rapid rate, allowing for personalized programming for the best hearing outcomes, and— especially with any neural changes with age—programming is important to do at regular intervals.

At most CI centers, implantation is completed as an outpatient procedure and generally performed in 1 to 1.5 hours. We recently completed cochlear Cosmetic The thin internal portion of the CI implantation on a is designed to sit flush with the skull and is not visible. The visible 96-year-old patient external components (the battery, who went home sound processor, microphone, and transmitting coil) mostly fit behind on the same day the ear, not much larger than a standard behind-the-ear hearing of surgery. Restoration Project—is still in its earliest phases. While there have been exciting advances in gene therapy, current technology via cochlear implants can provide people with severe to profound hearing loss immediate access to sound, and all the benefits that this brings. In addition, improved success with CIs is linked to implantation that occurs closer to the onset of hearing loss, as auditory pathways in the brain need to be stimulated or they weaken. Otherwise the resulting permanent changes in the brain’s auditory centers may limit the ability of a patient to hear, even with a perfectly intact cochlea.

Concern about sound quality

Despite CIs being a mechanical device, the voice sound quality has the potential to be no less electronic sounding than that from a telephone, computer, or television. Often the abnormal sound is due to the stimulation of an ear that hasn't heard for many years (or an ear that

aid. The latest sound processors are self-contained in a single unit about the size of a half dollar coin. These “off-the-ear” processors do not have an over-the-ear component, but rest directly over the magnet that is behind the ear and within the hairline. Eventually we expect that all implanted systems will be compatible with these smaller, off-the-ear processors, and nanotechnology and battery miniaturization will further reduce processor size. (And, the boom in wearable consumer technology makes visible devices even more mainstream.)

Fear of discomfort

Implantation incisions behind the ear heal quickly, and the drilling of the bone required to place the implant is a simple mastoidectomy. It is a component of most ear procedures and is not painful. Our center performs over 1,200 mastoidectomies per year across various different ear procedures. Postoperative discomfort is a rare complication and easily managed with over-the-counter medications such as acetaminophen (Tylenol).

Do You Qualify? If you have a hearing loss that prevents you from talking on the phone without visual cues (such as needing video calls or caption calling); are unable to understand television programs without closed captioning; and/or are actively avoiding large group gatherings for fear of conversational difficulty, talk to your hearing healthcare professional to see if you may be a CI candidate. CIs are the most successful sensory restoration prostheses to date and have been successfully placed in more than half a million individuals worldwide. The wonders of this technology vastly improve hearing, speech understanding, and overall quality of life.

René H. Gifford, Ph.D, CCC-A, is a professor in the department of hearing and speech sciences with a joint appointment in the department of otolaryngology at Vanderbilt University, Tennessee. She and HHF medical director David S. Haynes, M.D., FACS, direct the Cochlear Implant Program at the Vanderbilt Bill Wilkerson Center. For references, see hhf.org/fall2018-references.

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seniors

h ear i n g h ealth foundation

The

Miracle of the Cochlear Implant Surgery 17 years ago for the writer, then age 72, opens up a world of new sounds. By Barbara Sinclair

my mother didn’t realize i couldn’t hear until one afternoon when I was about 3 years old. I was happily playing on the floor with my younger brother. Suddenly I glanced up and saw Tex looking at Mother, who had clapped her hands. Not hearing any noise, I didn’t respond. Frantic with worry, Mother called Daddy telling him I couldn’t hear. She then took me to the doctor. No wonder I was slow in talking! Sound meant nothing to me. I didn’t know that I needed to talk to express feelings. A teacher trained at St. Louis’s Central Institute for the Deaf taught me how to speak and read lips at home in Cleveland. I never heard a sound until I got my first hearing aid around age 9. The earliest audiometric evaluation I still have is from 1984, when I was 55, and it showed a profound hearing loss in my right ear. My left ear was even worse, termed “dead.” With training in speaking and lip-reading (speechreading), I stayed in a mainstream school with the help of a hearing aid, although I really depended on lipreading to get by. The hearing aid gave me a sense of being 20

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able to communicate, but it didn’t help much when it came to understanding speech. I imagine anyone born with a hearing loss doesn’t always understand why they can’t hear. Many times I had wondered this myself. My doctors are also unaware of the cause. I speak a little differently, with a metallic sound and slight accent, sometimes accenting the wrong spots. However, this did not impede school or, later, work. After I graduated from Arizona State University, I held jobs in bookkeeping, the library, and human resources.

Sounds such as shouting, banging, ringing, and clanging all sound the same to me. What does a ticking clock sound like? Tap-tap, or click-click? Or running water? To me, these sound the same. I can’t hear the wind rattling the window. I feel it. I can’t hear the fury of a rainstorm. I feel it. It’s hard for me to detect changes in speech tones or pitch, or to tell a low voice from a higher one. I read that a child’s laughter is like the delightful rippling of a water stream. I can’t identify that sound. But even though I can’t enjoy music or follow group conversations, there are some

I imagine anyone born with a hearing loss doesn’t always understand why they can’t hear. Many times I had wondered this myself. My doctors are also unaware of the cause. I speak a little differently, with a metallic sound and slight accent, sometimes accenting the wrong spots.


seniors

For 30 days I agonized whether I’d be able to hear. The incision behind the ear must heal for that period before the bionic ear can be activated. Then, finally, activation day came—and was successful! There were beeps and squawks, but I could hear! Relief enveloped me as I progressed through the programming of the device.

advantages to not hearing—I sleep without any interfering noises. In 2001, our audiologist mentioned cochlear implants to my husband Charles, then age 72, who also has a hearing loss as well as being blind from retinitis pigmentosa. I researched cochlear implants and found this description from ABC News. It dates from 2001 but is still accurate today: “For those with normal hearing, sound enters the ear, triggering hair cells in the cochlea, a spiral tube filled with fluid. Those excited hair cells send information to the hearing nerve, which sends signals to the brain, allowing us to hear. “But, if deaf people have damaged hair cells in their cochlea, an implant can also do the same work. With an implant, sound is picked up by a tiny microphone connected by a cord to a small box outside the ear. The box turns sound into a signal— transmitting it through the skin, straight into the skull. Electronics in the skull send the signals straight to the hearing nerve, bypassing the cochlear hair cells that don’t work.” We went to see an otolaryngologist

in San Antonio named Wesley Krueger, M.D., . After a series of tests, Dr. Krueger told Charles that his hearing wasn’t actually severe enough to be a candidate for an implant. He was stunned for a minute, and then asked the doctor if there was a possibility for me to get an implant. Then it was my turn to be stunned. Weeks later, following my own series of tests, Dr. Krueger came into the room, grinning, and announced, “You are a candidate for a cochlear implant!” I was speechless as he showed me the components of “the bionic ear”: the external hearing aidlike processor and transmitter; the receiver under the skin that connects to electrodes; the magnet that holds the implant in place on the skull. I had the surgery a month later, when I was 72 years old. A week after the bandage from my right ear was removed, I felt dizzy, but there was almost no pain at all. I then realized that the implant made me unable to hear with my hearing aid. Whatever hearing I had was gone. For 30 days I agonized whether I’d be able to hear. The incision behind the ear must heal for that period

before the bionic ear can be activated. Then, finally, activation day came— and was successful! There were beeps and squawks, but I could hear! Relief enveloped me as I progressed through the programming of the device. Sounds were distorted and muddled, but they were all new to me. It has been 17 years since the implant. My device has been reprogrammed again and again until clarity reached its peak. I still don’t understand speech perfectly, but I do hear sounds I had not heard before: a ticking clock, running water, a humming car motor—and yes, the laughter of our four grandchildren.

Barbara Sinclair lives in Texas. Share your story: Have you received cochlear implants at an older age? Tell us at editor@hhf.org. Support our research: hhf.org/donate

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seniors

h ear i n g h ealth foundation

HOW HEARING LOSS AFFECTS

everything ears BETWEEN YOUR

By Barbara E. Weinstein, Ph.D., and Elizabeth Crofts

Barbara E. Weinstein, Ph.D., is a professor of audiology and an adjunct professor of medicine at the City University of New York’s Graduate Center. She has been investigating age-related hearing loss (ARHL) and its effects, primarily dementia and social isolation, as well as screenings of ARHL and the quantification of patient outcomes. A recent presentation Weinstein made to the Friends of the Congressional Hearing Caucus in Washington, D.C., showed lawmakers why it is imperative to address hearing loss in older Americans. Hearing Health Foundation CEO Nadine Dehgan also attended the talk. She, along with HHF, is committed to sharing this important information to benefit the health and welfare of our aging population.

AN AGING DEMOGRAPHIC BRINGS RISING HEALTHCARE COSTS the u.s. and the world’s population will be rapidly aging over the next several decades. By 2050, the number of people who will be 65 years old and older is expected to more than double. By 2061, 74 million U.S. adults are projected to have a hearing loss, compared with 44 million today. According to a 2013 report in the International Journal of Environmental Research and Public Health, healthy aging is multifactorial, requiring social participation, autonomy, quality of life, and minimal functional or cognitive impairments. Since untreated hearing loss is associated with social isolation, cognitive decline, falls, and other 22

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health conditions, Weinstein argues that healthy aging can be achieved by preventing or treating hearing loss and that by preemptively treating hearing loss, hundreds of millions can be saved in future healthcare costs. According to a 2015 report in The Lancet, hearing loss is a threat to overall health and well-being and ranks fourth among chronic conditions worldwide as a leading contributor to years living with a disability. Research from 2014 in the Journals of Gerontology shows hearing loss is associated with a 20 percent higher risk of mortality, independent of demographic factors. Here, Weinstein reviews the scientific literature connecting

untreated hearing loss to various health conditions and the human and financial costs they bring.

Social Isolation

Effective communication is the most important of all life skills, and the inability to do so can be emotionally devastating. For people with untreated hearing loss, feeling left out of the conversation can lead to depression, anxiety, or anger, prompting them to withdraw from social situations and become isolated. According to a 2015 meta-analysis in the journal Perspectives on Psychological Science, social isolation, loneliness, and living alone can boost the risk of premature death by 26 to 32 percent.


seniors

Preliminary studies have demonstrated that the use of hearing aids to treat hearing loss can improve brain function and working memory.

Double Growth in the number of people worldwide ages

65 and older by 2050.

4th Where hearing loss ranks among chronic conditions affecting healthy aging.

20 percent

Elevated risk from hearing loss for mortality, independent of demographic factors.

Falls

Hearing and balance share the same neural network, and inner ear hair cells help sense both sound and stability. Untreated hearing loss reduces your awareness of surroundings and increases cognitive load, which may affect the brain’s ability to maintain gait and balance. A 2016 meta-analysis in The Laryngoscope found the odds of falling increase by 2.39 times—making it more than twice as likely—among older adults with untreated hearing loss, compared with adults who have typical hearing. According to the Centers for Disease Control and Prevention, in 2015 falls in older adults cost the United States $50 billion in medical bills. If hearing loss were treated and prevented, eliminating it as a risk factor for falls, an estimated $29 billion would be saved.

Cognitive Decline

In 2017, 50 million people were estimated to be living with dementia worldwide, according to Alzheimer’s Disease International. With the number nearly doubling every 20 years, by 2050 the population with dementia will increase to 131.5 million. Otologist and epidemiologist Frank R. Lin, M.D., Ph.D., of Johns Hopkins University found links between untreated hearing loss and cognitive decline. In his 2013 JAMA Internal Medicine study, people with hearing loss showed a 30 to 40 percent accelerated rate of cognitive decline over six years, compared with individuals with typical hearing. According to a 2017 dementia

study in The Lancet, more than a third of dementia cases are preventable through lifestyle changes. Treating hearing loss was recognized as the most modifiable risk factor, contributing to 9 percent of dementia risk. In order to put the costs of dementia in perspective, Weinstein points out that if dementia care was a country, it would have the 18th largest world economy. If hearing loss as a modifiable dementia risk factor were completely eradicated, and 9 percent of dementia cases did not occur, $74 billion would be saved.

Dependency

According to Alzheimer’s Disease International, dementia is the leading cause of dependency and disability among the elderly. An Age and Aging report says people with untreated hearing loss are twice as likely to depend on community and informal supports as those without hearing loss. In addition to this greater cost burden, untreated hearing loss contributes to reduced independence and quality of life.

BURDEN OF HEARING LOSS Financial Burden

Due to these related health conditions, adults ages 70 years and older with untreated hearing loss are 1.27 times more likely to experience frequent hospitalizations, compared with those who have typical hearing, according to a 2013 JAMA research letter. A 2018 University of Michigan

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percent Increased risk by social isolation, loneliness, and living alone for premature death.

1.27x

Rate at which adults ages 70 years and older with untreated hearing loss are more likely than those with typical hearing to experience

frequent hospitalizations.

$51.4B Estimated national cost for the

first year of hearing loss treatment

for adults ages 65 and older in 2030, up from $8.2 billion in 2002.

paper in JAMA Otolaryngology–Head & Neck Surgery showed that adults with hearing loss who used hearing aids experienced reduced emergency room visits, hospitalizations, and inpatient stays, compared with those who did not use hearing aids. Hearing aid users reported more office visits, but these are less expensive than emergency care and hospitalizations.

A 2018 University of Michigan paper in JAMA Otolaryngology–Head & Neck Surgery showed that adults with hearing loss who used hearing aids experienced reduced emergency room visits, hospitalizations, and inpatient stays, compared with those who did not use hearing aids. Hearing aid users reported more office visits, but these are less expensive than emergency care and hospitalizations. While it may be a financial burden to pay for hearing aids when they can cost thousands of dollars, proactively treating hearing loss may save even more by preventing or reducing other medical issues associated with hearing loss. The Michigan study authors theorize that the reduced healthcare costs among hearing aid users may be due in part to better communication with medical professionals informing a greater awareness of overall health. Indeed, a 2011 report in the Journal of Community Health found that older adults with untreated hearing loss were 1.85 times more likely to report difficulty or delay in obtaining healthcare.

Lost Productivity

Not only does untreated hearing loss place a financial strain on an 24

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individual, it can affect society as a whole as well. The International Journal of Technology Assessment in Healthcare executed a study to determine just how much an individual with untreated hearing loss “costs” the nation. The subjects of the study were people with severe to profound hearing loss. It was found that over their lifetime, each adult

with untreated hearing loss would cost society $297,000 (and this was in 2001 dollars using data from the 1990s). The majority of this cost was due to reduced workplace productivity. The researchers concluded that early identification and medical intervention may substantially defray these costs. Weinstein cites a 2010 Journal of the American Geriatric Society report estimating the financial burden of hearing loss on individuals ages 65 and older. In 2002, the cost was $8.2 billion nationally for the first year of treatment, and the lost productivity cost attributable to hearing loss was $1.4 billion nationally. In 2030, the first year of treatment is estimated to jump to $51.4 billion, while lost productivity is estimated to increase to $9 billion nationally.


seniors

Cost-Effective Interventions

200 percent

Increased risk that people with untreated hearing loss

depend on community and informal supports,

compared with those without hearing loss, creating a financial burden while also reducing independence and quality of life.

$9B

Estimated national cost for

lost productivity

attributable to hearing loss among adults ages 65 and older in 2030, up from $1.4 billion in 2002.

A 2017 World Health Organization report, “Global Costs of Unaddressed Hearing Loss and Cost-Effectiveness of Intervention,” evaluated the cost of untreated hearing loss for society, and found that unaddressed hearing loss poses substantial costs to the healthcare system and economy as a whole. It also concluded that public health interventions for prevention and early identification of hearing loss are cost effective. In a 2017 JAMA Otolaryngology– Head & Neck Surgery report, 2016 Emerging Research Grants (ERG) recipient Harrison Lin, M.D., of the University of California, Irvine, Medical Center, found attention to age-related hearing loss was woefully insufficient compared with its prevalence. “We found that the healthcare field is not doing a good job identifying and educating the public about hearing loss and ways to address hearing loss,” he says. By being informed about how to prevent and treat age-related hearing loss, adults are better equipped to maintain their quality of life through preserving independence and their overall health, and to save money for themselves and their community. Samira Anderson, Ph.D., a 2014

ERG recipient at the University of Maryland, has been researching the importance of treating hearing loss. “Preliminary studies have demonstrated the use of hearing aids to treat hearing loss can improve brain function and working memory,” she says. “These results underscore the need to seek help for hearing difficulties as soon as hearing problems are suspected.”

Shared Goals

Weinstein and HHF agree on three main goals: reduce the incidence of hearing loss through prevention; reduce the progression of hearing loss; and treat hearing loss to prevent related health conditions. In parallel, the WHO recommends hearing loss be addressed as a public health issue globally, urging that policy makers allocate needed resources for access to hearing care. Public health strategies need to address prevention, screening, and early intervention of hearing loss to target people at every stage of hearing loss, in every community, and among developing as well as developed nations. Please join us in raising awareness, taking action, and advocating for and supporting people with hearing loss.

Barbara Weinstein, Ph.D. (far left), is a professor of audiology and an adjunct professor of medicine at the City University of New York’s Graduate Center. A 2018 HHF intern, Elizabeth Crofts is studying biomedical engineering at Boston University. For references, see hhf.org/fall2018-references.

Share your story: Tell us your family’s hearing loss journey at editor@hhf.org. Support our research: hhf.org/donate

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planned giving

h earing health foundation

IN MEMORY OF, AND WITH SINCERE THANKS TO, HEINRICH MEDICUS, PH.D. heinrich medicus, ph.d., was a nuclear physicist who generously remembered Hearing Health Foundation (HHF) in his will, for which HHF is deeply grateful. Medicus died at age 98 in 2017 after a long career at Rensselaer Polytechnic Institute (RPI) in Troy, New York. By all accounts, the Swiss-born scientist was something of a Renaissance man, with wide-ranging interests in not only science and research, but also classical music and ancient cultures. He was a collector of Egyptian antiquities; taught a popular course on “The Science, Technology, and Gastronomy of Alcoholic Beverages”; and was a downhill skier into his eighth decade. As a philanthropist, Medicus supported various educational, scientific, medical, and cultural institutions. Known for the enthusiasm he invested in all of his varied interests, Medicus still holds the world record for the long-distance flight of a Champagne cork, which he set in 1988. The Guinness Book of World Records lists Medicus’s achievement at 177 feet, 9 inches—the effort, according to his official obituary, was to teach RPI’s “future captains of industry [that] they should know something about the finer things in life.” It was actually Medicus’s second world record. While a doctoral student at the Swiss

A nuclear physicist and Egyptian antiquities collector, Medicus was known for the enthusiasm he invested in all of his varied interests. He still holds the World Record for longest flight of a Champagne cork. Federal Institute of Technology in Zurich, he discovered "the then-smallest known nuclear energy level transition" that proved important in nuclear medicine. HHF is truly grateful to Heinrich Medicus, Ph.D., and his family for remembering us in his will. We pledge to use every dollar of his gift to support our mission to prevent, research, and cure hearing loss and other hearing and balance conditions. (For references, see hhf.org/fall2018-references.)

Create a Legacy to Fund the Most Promising Science By naming HHF in your will, you create a legacy to fund innovative hearing and balance research, making a true impact for years to come. Through the generous gifts of individual donors, as well as family and corporate foundations, key findings by HHF-funded researchers have helped pave the way for major scientific advances over the past several decades, including cochlear implant technology, outer hair cell motility leading to otoacoustic emissions as a hearing measurement, and the discovery that inner ear sensory hair cells spontaneously regenerate in chicks. The researchers we support work tirelessly toward our goal of achieving hair cell regeneration, and therefore hearing restoration, in humans. A recent article in The New York Times says most people make charitable giving a part of their final will. If you have questions, or would like to notify Hearing Health Foundation that you have remembered us in your will, please contact us at info@hhf.org or 212.257.6140. Thank you for your generosity.

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meet the fundraiser

ANGER then ACCEPTANCE By Holly dee Gadow

while some people never know the exact cause of their hearing loss, I do. My hearing loss was the result of an attack. My ears were boxed from behind, and the next thing I knew, my ears were searing with pain. They rang for months. Within the first week I saw a doctor but he just said that “hopefully the ringing would stop.” A hearing test eventually confirmed a severe hearing loss. I could not hear higher frequencies, such as rain or birdsong, and now my hearing is very limited to hearing only lower frequency sounds. It wasn’t until five years after the attack that I got a hearing aid for my left ear; my right ear is considered deaf. I try to upgrade the device as much as possible every few years to take advantage of the latest technology. After the incident, I was initially extremely angry, because my hearing had actually been excellent. Anger was followed by deep sadness, depression, and embarrassment. I tried to hide it. I tried to make excuses, and then finally I had to own up to the fact that I could not hear well. In my job working in finance, I am still coping with the best way to use a phone and have conversations in noisy environments; in my personal life, I’m trying to figure out how to talk to loved ones. Often people do not have the patience to repeat and rephrase what they are saying so I can fully understand, and I am still getting used to asking for accommodations. To compensate, I developed some lip-reading (speech-reading) techniques on my own. Throughout, I have tried to put on a brave face and make the best of it, and not let it defeat me. I do this every single day. This is why I am so excited about the regeneration of hair cells and curing hearing loss itself. I’ve known for

years about the ability chickens have to regrow their hair cells, and have been patiently waiting for this cure. I get emotional thinking about what a cure would bring. I would be able to hear everything my granddaughter and grandson are saying; I would be able to call my mother and have a conversation about nothing and everything, like I did back when I could hear. I could whisper silly jokes and hear when jokes are whispered back to me.

I have tried to put on a brave face and make the best of it, and not let my hearing loss defeat me. I do this every single day. And this is why I donated to Operation Regrow, Hearing Health Foundation’s fundraiser in support of its Hearing Restoration Project in June 2018 (I was thrilled that all the donations from that two-week period were matched by an anonymous donor). I also encouraged family and friends and coworkers to donate. I truly believe that, with the best scientific minds and resources available, a cure is possible. I am proud to be a donor.

Holly dee Gadow lives in California. More than a dozen people donated in her name to Operation Regrow, with a company match; please email development@hhf.org for ideas on how you can do the same. HHF sincerely thanks Gadow and her supporters for their generosity.

Share your story: Tell us your hearing loss journey at editor@hhf.org. Support our research: hhf.org/donate

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meet the fundraisers

hearing health foundatio n

A BIRTHDAY WISH FOR A CURE By Beth and Jeremy Hochheiser

In Beth’s words our son jeremy recently turned 29, and for his birthday on his Facebook page he asked family and friends to donate to Hearing Health Foundation (HHF) to help reach his fundraising goal, which he exceeded. Jeremy introduced us to HHF when he discovered its commitment to hair cell regeneration. He has a profound hearing loss and has been using hearing aids successfully since childhood. We did not realize there was a problem with his hearing until he was 14 months old because he had been making sounds like a typical baby. Even as a baby, Jeremy had an infectious belly laugh and was always very attentive to what was going on around him.

But when we discussed a potential hearing issue with our pediatrician, he didn’t seem concerned. It was only after we went to see an audiologist that we finally got a diagnosis of profound congenital bilateral sensorineural hearing loss. Throughout Jeremy’s childhood we visited audiologists and doctors regularly. We found an otolaryngologist in New York City who specialized in hearing disabilities, and we kept up with the latest technology in hearing aids to understand options for Jeremy. As he got older, we always encouraged him to ask people to repeat themselves if he couldn’t hear or understand them. Since Jeremy was diagnosed with quite a bit of residual hearing, the audiologist suggested an unconventional

As a kid Jeremy liked to place one of his hearing aids on his tummy and say, “Mom, I’m so hungry, I can hear my stomach growling!”

 Host a Fundraiser Get started at hhf.org/how-to-help, where you can also see examples of past events and get ideas for creating your own—such as golf outings, bake sales, birthdays, weddings, marathons, and triathlons. Let your talents and interests guide your ideas; no event is too large or small. Questions? We’re here to help! Email development@hhf.org. 28

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means of learning speech, the auditory-verbal approach introduced by Helen Beebe. Children learn to use the hearing they still have by being bombarded with speech consistently. I read books and introduced pictures by sitting next to Jeremy, not facing him, so that he could learn how to repeat words and speech sounds by hearing them rather than watching my face and lip-reading (speech-reading). As a result, Jeremy’s speech developed as a typical hearing child’s would—by listening and repeating. We as his parents knew that developing Jeremy’s hearing to its fullest potential was imperative—the “if you don’t use it, you’ll lose it” theory—and that lip-reading could come later, as a supplement. With his hearing aids Jeremy thrived, learned to play piano at age 7, and was even presented with an award at Carnegie Hall. He excelled at school and showed us that his hearing was not an issue or a factor that would get in the way of his education or competitive spirit. The natural belly laugh he had as an infant translated into a great sense of humor and positive outlook. As a kid Jeremy liked to place one of his hearing aids on his tummy and say, “Mom, I’m so hungry, I can hear my


meet the fundraisers

Jeremy and Lauren on their wedding day in 2017 with his parents Beth and Sandor (far left); playing chess is just one of Jeremy’s many interests.

stomach growling!” We’re so proud of him and his studies, the many activities he has thrown himself into, his thriving career, and his own family and baby to come.

In Jeremy’s words Be Bold My advice to anyone with hearing loss (and their loved ones) is to be bold, brave, and up front about your hearing. Accept it and wear it proudly, otherwise others may misinterpret who you really are, or even bully you. Hearing loss is a part of you, but doesn’t define who you are. I also tell those new to hearing loss to never stop using your mind. Your brain is your most powerful tool. I play chess and compete in Brazilian jiu jitsu, plus I love being a software engineer and doing math, exploring art, and enjoying nature. I love to learn. Being fully engaged keeps your brain active and fights off feeling down from hearing challenges. Your Voice Matters I remember being afraid to ask others to repeat themselves, but as I got older I learned to ask, even if I had to do it more than once. In this way, I show I am involved and can contribute meaningfully to the conversation. I earn respect for that. Your voice and opinions really do matter. What definitely won’t work is to hold back. Sometimes when I ask

someone to repeat themselves it can break the flow of the conversation, or cause frustration in a new acquaintance who doesn’t understand. But it is infinitely more frustrating if you can’t fully participate. New Challenges My wife Lauren has typical hearing and sees me as a typical hearing person. But when I am tired and my hearing is down, I have to ask her to repeat herself or let me see her lips. Then when I am less tired and my hearing is better, I get frustrated again if Lauren is still making accommodations for me that I don’t think I need—and that can frustrate her, constantly having to switch! I love my wife for going with the flow and understanding what I need to hear. Managing Hearing Loss I find that there are days where I can understand what people are saying without looking, and then sometimes I have to rely on speech-reading. People have said to me, “You can hear better with your glasses on!” The company I work at now has wonderful benefits and accommodations for their employees with needs. They hold meetings throughout the year among those who have requested accommodations in order to foster an inclusive environment, and they are proactive about making sure I have everything I need. It’s been very welcoming.

How I Discovered HHF When I was in middle school, my parents and I went to a support group, and I met a couple of kids my age who also have a hearing loss. One of them happened to end up working for HHF, Laura Friedman (HHF’s former communications and programs manager). I also followed news on the development of hair cell regeneration in the inner ear. I am encouraged about HHF’s Hearing Restoration Project consortium bringing together multiple labs and scientists. All of this is why I wanted to do my part to raise money for research toward the cure.

Beth Hochheiser lives in New Jersey, and Jeremy Hochheiser lives in Pennsylvania. HHF sincerely thanks the Hochheisers and their family and friends for their support.

Share your story: Tell us your family’s hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate

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A FAST TRACK TO HEARING DAMAGE An analysis of 120 noise samples shows the New York City subway system can derail your hearing health. By Andrew J. Guralnick millions of commuters using the new york city subway system know it can be noisy, but just how loud is it? As a 2018 Hearing Health Foundation (HHF) intern, I set out to assess the potential for danger that the NYC Metropolitan Transit Authority (MTA) subway system presents to riders and employees. Not surprisingly, I found that the system significantly breaches the threshold of what is safe for our ears. To protect hearing, both the U.S. Environmental Protection Agency and the World Health Organization recommend an average exposure limit of 70 decibels (dB) over the course of 24 hours. But what my colleagues and I measured exceeds that limit: Samples show the average noise levels on all subway platforms and on all subway rides (inside subway trains) is between 72.5 and 76.5 dB and between 74.1 and 75.8 dB, respectively. And with maximum readings actually as high as 119 dB on platforms and 120 dB on rides—based on actual recorded data within the sample—the NYC subway is an auditory minefield. (See hhf.org/subway for full data.) Using our data’s sample averages, we determined ranges as to what the actual averages are on all subway platforms and rides through the MTA system. Results are presented with a 99 percent confidence level.

Collecting and Analyzing

From January to August 2018, three data collectors used Decibel Meter Pro, an app installed on Apple iPhones and an Apple iPad, to collect 120 samples from platforms and rides. All 60 platform samples were equally represented at five minutes each. The 60 ride samples were assigned random recording lengths from 10 to 30 minutes. Samples on Saturday and Sunday or between 11 p.m. and 4:45 a.m. on any day were excluded. Random sampling was utilized as much as possible to help ensure generalizability on behalf of all platforms and rides. The analysis examined potential harm to hearing from loud noises on subway platforms and during subway rides. For platform noise, the main variable is the number of trains that pass; for subway ride noise, the main variable is the number of local stations the train passes. We also investigated the number of seconds the subway noise level reached 75 dB or higher. When measuring subway rides, we noted train travels between Manhattan and another borough or vice versa; whether a train runs above ground; whether the sample 32

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With maximum readings actually as high as 119 dB on platforms and 120 dB on rides— based on actual recorded data within the sample—the NYC subway is an auditory minefield. was collected during rush hour; and whether a local train ever becomes an express train, with fewer stops. The statistical method of multiple regression was used to predict dangerous noise exposure on both platforms and rides. We can predict that each train that enters or leaves a platform will expose a rider’s ears to 16.53 seconds of noise at 75 dB or higher. For example, if a rider waits at a platform where two trains come and go before their train arrives, that would be a predicted exposure of 82.65 additional seconds of noise at 75 dB or higher. We can also predict that each subway stop that is passed will expose a rider’s ears to 36.06 seconds of noise of 75 dB or higher. For example, if a rider passes 10 local train stops on their trip, the predicted exposure of noise at 75 dB or higher is 360.60 additional seconds—or just over 6 additional minutes.

Cumulative Effect

HHF’s recommendation for commuters, MTA staff, and platform retailers such as newsstand operators is simple: Wear ear protection. MTA staff and platform retailers are


hearing health

at elevated risk given the hours they spend underground and on the trains. The tendency for many commuters to block noise by raising the volume of their headphones is not a helpful approach and could in fact damage hearing even more. The subway is merely one of many sources of daily noise. “Noise-induced hearing loss can result from a single, sudden noise event and from constant exposure to loud noises that has a cumulative effect, not unlike sun exposure,” says Nadine Dehgan, HHF’s CEO. “This noise exposure can lead to related negative health effects when unknown and untreated.” The MTA appears aware of the issue of subway noise. The newly built Second Avenue subway line uses effective noise-reduction measures such as low vibration tracks and sound absorbing panels. We hope the MTA will continue to use these quieter, low vibration tracks when making subway and station upgrades, especially since they are more cost-effective than traditional wooden tracks.

Variables Defined (see hhf.org/subway for details) Train Platform Variables (SX2–SX5 are controls) Number of Times Trains Passed (Enter and Exit Separate) (SX1) Above Ground = 1, Below Ground = 0 (SX2) Day of Week (Wednesday = 1, Other = 0) (SX3) Location (1 = Back, 0 = middle/front) (SX4) Rush Hour, 1 = Yes, 0 = No (6:30–9:30 a.m. and 3:30–8 p.m.) (SX5)

»» »» »» »» »»

Train Ride Variables (TX2–TX5 are controls) Number of Local Stations Met (TX1) Interborough (Between Manhattan and a different borough or vice versa), Yes = 1, No = 0 (TX2) Above Ground = 1, Below Ground = 0 (TX3) Rush Hour, 1 = Yes, 0 = No (6:30–9:30 a.m. and 3:30–8 p.m.) (TX4) Express at Any Point = 1, Local = 0 (TX5)

»» »» »» »» »»

2018 HHF intern Andrew J. Guralnick is pursuing a master’s in public administration at Baruch College in New York City. For the full report, see hhf.org/subway. For references, see hhf.org/fall2018-references.

Support our research: hhf.org/donate

NOISE:

P X E

D E OS

aboard my noisy flight to the Hearing Loss Association of America convention in June, I couldn’t help but reflect upon loud sounds—and what can be done to reduce our exposure to noise. I’d recently learned that the word “noise” is derived from “sea sickness” or “nausea” in Latin. Noise has been associated with poor health outcomes for literally thousands of years. Synonyms for “loud” include “earsplitting” and “deafening.” In fact, vibrations from loud noises travel through the eardrum to reach our inner ear, where sensory hair cells change them into electrical signals to be interpreted by the brain. Hair

When a radio station promotes “ear-popping music” and a dance studio tops 105 decibels, we need to do more to undo the illusion that loud means good—for our health and that of older as well as younger generations. By Nadine Dehgan

cells, however, come in limited supply. Humans are typically born with thousands—and when these hair cells are damaged by noise, age, ototoxic drugs, or other factors, the brain’s ability to communicate with the ears is significantly weakened, resulting in permanent hearing loss. Concerned about my fellow plane passengers’ hair cells, I opened my phone’s decibel (dB) measuring app, which indicated the maximum noise level after takeoff was 92 dB, while the average was 83 dB. The app also pointed out that this dB level is equivalent to that of alarm clocks. While this doesn’t seem uncomfortable, it’s actually not

recommended for periods over two hours. I’d come prepared with both earplugs and noise-canceling headphones—which I limit to 60 percent of maximum volume in accordance with the World Health Organization’s recommendation. But not everyone flying comes prepared for the dangerous levels of noise inside the plane. (Nor may subway riders realize their hearing can be damaged during their daily commute; see our story on the opposite page.) The National Institutes of Health states noise greater than 75 dB can harm hearing, and in 1974, the Environmental Protection Agency recommended that sound exposure

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How can we be okay with hearing loss and ear damage advertised as a positive experience? No one would advertise skin cancer from excessive sun exposure as a perk of a beach vacation. Nor would a beverage manufacturer tout soda’s negative impact on dental health or diet. should remain at or below 70 dB to prevent noise-induced hearing loss. Sudden loud noises—like those from blasts, gunfire, firecrackers, and bullhorns—can also cause hearing loss with levels reaching 165 dB! This is why so many military veterans return with hearing loss and tinnitus. Tragically, these are the two most common disabilities for those who serve. And yet our society glorifies noise. Two confessions explain my frustration. The first: I love to listen to songs from the ’90s (my children think these songs are current hits). The second: When my kids are not in my car I often listen to classical music, but once in awhile I listen to current hits. One station’s tagline is actually “Ear-Popping Music.” I couldn’t believe that damaging eardrums was being advertised as a good thing! My youngest daughter, Emmy, had many eardrum ruptures—from infections, not noise—and she truly suffered. My anguish as a parent watching my young child in pain was nothing compared to the pain she endured with no understanding of why. How can we be okay with hearing loss and ear damage advertised as a positive experience? No one would advertise skin cancer from excessive sun exposure as a perk of a beach vacation. Nor would a beverage manufacturer tout soda’s negative impact on dental health or diet. It is my wish that one day we take the real risk of hearing loss seriously and recognize it for the epidemic that it is. Experts say approximately one in five American children will have permanent hearing 34

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loss (largely noise-induced) before reaching adulthood. University of Ohio scientists report that even mild hearing losses in children can cause cognitive damage that would typically not occur until at least age 50. This is horrifying. Still, we surround our children with damaging noise. Birthday parties, movie theaters, weddings, and family celebrations can blast noise exceeding 115 dB. Football stadiums, hockey arenas, exercise classes, and music concerts have clocked in at over 140 dB, which can cause irreversible hearing loss—whether sudden or progressive damage—in minutes. Recently, a friend told me she complained of high noise levels (105 dB!) to her daughter’s dance studio. Instead of offering to turn down the volume, management told her that she could leave the class. While her daughter can no longer attend dance class, my friend has the consolation of knowing her child is safer. My thoughts go to the employees of fitness centers, stadiums, restaurants, bars, and other commercial establishments whose ears are constantly assaulted. Before becoming CEO of Hearing Health Foundation (HHF), I didn’t appreciate the dangers and consequences of loud sound. I now know that even a mild untreated hearing loss can lead to social issues including isolation, depression, and poor academic performance in children. In adults the stakes are also high, with untreated hearing loss bringing the risks of mental decline, falls, and premature death. Hearing loss can be mitigated

by technology including hearing aids and cochlear implants. While these treatments are beneficial and life-changing, HHF is funding research toward permanent cures. Birds, fish, and reptiles are all able to restore their inner ear hair cells once damaged—but mammals including humans cannot. Through our Hearing Restoration Project, HHF funds a consortium of top hearing scientists who study how other species are able to regenerate their hearing in order to apply this knowledge to humans through a biological cure. As the plane descended toward Minneapolis, my ears popped, but I know the minor discomfort can’t compete with what Emmy experiences. As the mother, sister, daughter, and granddaughter of individuals with hearing loss, I repeat my two biggest wishes: for society to place a greater value on hearing protection, and for HHF to continue to support researchers on their quest to treat and cure hearing loss and related conditions.

Nadine Dehgan is the CEO of Hearing Health Foundation. For references, see hhf.org/fall2018references.

Share your story: Tell us about loud environments you encounter at editor@hhf.org.


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FEDERAL LAW PROHIBITS ANYONE BUT REGISTERED USERS WITH HEARING LOSS FROM USING INTERNET PROTOCOL (IP) CAPTIONED TELEPHONES WITH THE CAPTIONS TURNED ON. IP Captioned Telephone Service may use a live operator. The operator generates captions of what the other party to the call says. These captions are then sent to your phone. There is a cost for each minute of captions generated, paid from a federally administered fund. No cost is passed on to the CapTel user for using the service. CapTel captioning service is intended exclusively for individuals with hearing loss. CapTel® is a registered trademark of Ultratec, Inc. The Bluetooth® word mark and logos are registered trademarks owned by Bluetooth SIG, Inc. (v2.5 7-18)


research

EMERGING RESEARCH GRANTS

Presenting the 2018 Emerging Research Grantees

Since 1958, Hearing Health Foundation has awarded more than 2,000 grants that fund hearing and balance research. We are proud to announce these 15 Emerging Research Grants scientists who are working in the areas of central auditory processing disorder, hearing health, hearing loss in children, hyperacusis, tinnitus, and Usher syndrome. HEARING LOSS IN CHILDREN One grant was awarded for research on congenital and acquired childhood hearing loss and its etiology, assessment, diagnosis, and treatment. This grant was generously funded by The Children’s Hearing Institute.

HYPERACUSIS One grant was awarded focused on research that will increase our understanding of the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance. This grant was generously funded by Hyperacusis Research Ltd.

USHER SYNDROME One grant was awarded for research to increase our understanding of the mechanisms, causes, diagnosis, and treatments of Usher syndrome, the most common cause of combined blindness and deafness. This grant was supported by donors who designated their gifts to fund Usher syndrome research.

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Babak Vazifehkhahghaffari, Ph.D.

Washington University in St. Louis Project: Enhancing cochlear implant performance through development of improved auditory nerve fiber biophysical models with a combined wet lab and dry lab approach Goal: To improve success with cochlear implants, such as understanding of speech in noise and perceiving pitch and music, by combining models of electric field potential with biophysical models of auditory nerve fibers, and to better understand hearing loss mechanisms by detailing the anatomy and electrophysiology of inner ear sensory hair cells.

Kelly Radziwon, Ph.D.

University at Buffalo, the State University of New York Project: Noise-induced hyperacusis in rats with and without hearing loss Goal: To broaden our understanding of the neural mechanisms underlying loudness perception in order to find a potential therapeutic target to correct or mitigate bothersome hyperacusis. By comparing electrophysiological recordings with behavioral performances of individual animals trained to react to sounds of varying intensities, this project aims to characterize the relationship between changes in neural activity and loudness perception in animals with and without noise-induced hearing loss.

Clive Morgan, Ph.D.

Oregon Health & Science University Project: Characterization of Usher syndrome 1F protein complexes Goal: To understand the molecular architecture of the hair bundle, which extends from the surface of inner ear sensory hair cells and carries out mechanotransduction (how sound wave vibrations are converted into electrical signals). This project will analyze individual protein complexes using a new hair bundle isolation strategy, allowing for the comparative analysis of the makeup of all Usher syndrome protein complexes, shedding new light on the proteins directly involved in mechanotransduction, and uncovering the molecular basis for genetic abnormalities leading to deafness.


research

CENTRAL AUDITORY PROCESSING DISORDER Four grants were awarded for research that will increase our understanding of the causes, diagnosis, and treatment of central auditory processing disorder (CAPD), an umbrella term for a variety of disorders that affect the way the brain processes auditory information. All four CAPD grantees are generously funded by the General Grand Chapter Royal Arch Masons International.

Alisha Lambeth Jones, Au.D., Ph.D.

Auburn University Project: Evaluating central auditory processing, language, and cognition skills in adolescents born prematurely Goal: To determine if there are significant differences in auditory processing, language, and cognition skills among adolescents with a preterm birth history when compared with adolescents with a full-term birth history. Once patterns of deficits are identified, early intervention treatment as well as a rehabilitation program will be developed to either prevent or treat the deficit areas.

Elliott Kozin, M.D.

Massachusetts Eye and Ear, Harvard University Project: Evaluation of hearing loss and quality of life in patients with mild traumatic brain injury Goal: To better diagnose and treat patients with auditory dysfunction following mild traumatic brain injury, thereby minimizing hearing loss, tinnitus, and hyperacusis. Findings will be applied to clinical guidelines that address at-risk patients and the need for monitoring via audiometric testing, with implications for a broad range of individuals with isolated and/or repetitive head injuries, such as athletes and military personnel.

Khaleel Razak, Ph.D.

University of California, Riverside Project: Age-related hearing loss and cortical processing Goal: To better understand the relative contributions of peripheral hearing loss and brain aging to auditory processing declines related to presbycusis (age-related hearing loss), and to systematically examine the optimal combination of hearing aids and behavioral and pharmacological approaches to delay or prevent these declines.

Joseph Toscano, Ph.D.

Villanova University Project: Cortical EEG measure of speech sound encoding for hearing assessment Goal: To use electroencephalogram (EEG) techniques to measure brain responses to specific acoustic cues in speech, which will be compared with other audiometric measures in order to develop a clinical test for auditory neuropathy, a problem with the way the brain processes sounds, and other types of hearing loss. Diagnosing auditory neuropathy in newborns and infants is particularly challenging, as it is often difficult to distinguish it from sensorineural hearing loss using current measurement approaches.

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Rachael R. Baiduc, Ph.D., MPH

HEARING HEALTH HHF awarded six grants for the best overall hearing research proposals. These grants were funded by HHF’s Board of Directors and donors who designated their gifts to fund the most promising hearing research. Evelyn Davies-Venn, Au.D., Ph.D.’s grant was partially funded by an anonymous donor; David Ehrlich, Ph.D.’s grant was partially funded by the Meringoff Family Foundation; and A. Catalina Vélez-Ortega, Ph.D.’s grant was generously funded by Cochlear Americas, the cochlear implant manufacturer.

“We are particularly excited to invest in cutting-edge research taking place in New York City,” says Meringoff Family Foundation executive director Stuart Post. “We’re eager to see where this work will lead.”

University of Colorado Boulder Project: Hearing loss and cardiovascular disease risk burden: epidemiological and physiological data Goal: To explore the link between cardiovascular disease (CVD) and hearing loss using specific measures of auditory status via a large, cross-sectional study. Through cost-effective, clinically available techniques in conjunction with epidemiological data, a greater understanding of CVD risk factors that contribute to hearing loss will help develop clinical tools for prevention, early identification, and treatment for at-risk individuals.

Evelyn Davies-Venn, Au.D., Ph.D.

University of Minnesota Project: Behavioral and neural correlates of amplification outcomes Goal: To better understand basic auditory mechanisms that are affected by background noise in order to improve hearing aid algorithm design and hearing loss treatment outcomes. Behavioral, speech, and non-speech measures will be used to determine how spectral auditory processing interacts with high intensity sounds and influences amplification. This will help us determine factors that contribute to diminished speech perception in noisy environments for individuals with hearing loss and how their perception of amplified speech can be enhanced in noisy environments.

David Ehrlich, Ph.D.

New York University School of Medicine Project: Neural computations for vestibular control of movement initiation Goal: To inform therapeutic strategies to treat balance deficits by defining how cells in the brainstem act collectively to produce rapid responses to sensations of instability, revealing general principles of how brain cells encode sensations from the inner ear and how the brain initiates responses when stability is lost. This project will measure balance responses in cell populations deep in the brain using microscopy of the zebrafish, which swim to remain balanced, providing a model for balance control and brainstem function in general.

Soumen Roy, Ph.D.

National Cancer Institute Project: High-dimensional analysis of cochlear immunity and cisplatin-induced inflammation Goal: To identify a key pathway or leukocyte (white blood cell) subset that regulates ototoxicity or hearing protection in order to prevent or cure ototoxicity, which may be applicable for hearing loss generally. Cisplatin is a life-saving chemotherapy drug but causes hearing loss in 40 to 80 percent of cancer patients. Cisplatin enters the cochlea through systemic circulation and gains access to inner ear hair cells after disrupting the protective blood-labyrinth-barrier. This project builds on the hypothesis that a defined subset of innate immune cells regulates hair cell death by controlling cisplatin-induced inflammatory pathways within the cochlea. Support our research: hhf.org/donate

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A. Catalina Vélez-Ortega, Ph.D.

University of Kentucky Project: TRPA1 activation in the cochlea as an intrinsic mechanism of protection against noise-induced hearing loss Goal: To explore whether, after noise exposure, activation of TRPA1 (an ion channel known for its role as an “irritant sensor” in pain-sensing neurons) contributes to the temporary shift in hearing thresholds to allow the cochlea time to repair or recover from noise-induced tissue damage. By helping us better understand the protective effects of TRPA1 activation after noise exposure, and the specific cell types within the inner ear that are involved in this process, this project will explore how TRPA1 activation modifies cochlear mechanics and hearing sensitivity in order to eventually uncover new therapeutic targets to prevent hearing loss or tinnitus.

Philippe Vincent, Ph.D.

Johns Hopkins University Project: Investigating mechanisms of degeneration of ribbon synapses between auditory inner hair cells and type 1 afferent nerve fibers after noise trauma in mammals Goal: To understand the molecular mechanisms of synaptic transmission between hair cells and auditory nerve fibers and how they are affected after noise trauma, in order to find methods to protect hair cell synaptic function and prevent hearing loss. This project will examine the underlying mechanisms for the excitotoxicity of auditory nerve fibers by what may be an excessive influx of calcium in reaction to noise exposure.

TINNITUS Two grants were awarded for innovative research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus. David Jung, M.D., Ph.D.’s grant is supported by HHF donors who designated their gifts to fund promising tinnitus research, while Tenzin Ngodup, Ph.D., received the Les Paul Foundation Award for Tinnitus Research.

David Jung, M.D., Ph.D.

Massachusetts Eye and Ear, Harvard Medical School Project: Mechanisms and development of novel small molecule treatments for cochlear synaptopathy Goal: To develop therapies for hearing loss, tinnitus, and hyperacusis by reestablishing synaptic connections between inner ear sensory hair cells and neurons (nerve cells). The loss of these connections results in what has been termed “hidden” hearing loss because traditional auditory measures may not detect it. This project stems from the development of a novel way to anchor special molecules that promote synaptic regeneration into the bone of the inner ear, which maximizes the stimulation of inner ear neurons.

Tenzin Ngodup, Ph.D.

Oregon Health & Science University Project: Discovery of novel inhibitory cell types in the cochlear nucleus Goal: To discover and study the functional significance of novel inhibitory neurons in the brainstem region called the ventral cochlear nucleus (VCN), whose inhibitory action, if compromised, may lead to the hyperactivity and auditory dysfunction that is believed to underlie tinnitus. The diversity of inhibitory cell types and circuitry within the VCN is far richer than previously described, and understanding their excitatory and inhibitory neuronal connections (synapses) may eventually help prevent and treat tinnitus.

For more details about the 2018 ERG scientists and their research projects, please see hhf.org/erg-2018-grantees and hhf.org/mtr. a publication of hearing health foundation

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Toward

CUSTOMIZED HEARING Evelyn Davies-Venn, Au.D., Ph.D., a 2018 Emerging Research Grants scientist, is exploring individual variances in hearing aid outcomes in quiet and noisy environments. By Elizabeth Crofts

EMERGING RESEARCH GRANTS

One project to improve hearing aid success focuses on an emerging technology called the “cognitive control of a hearing aid.” It will analyze multiple sounds, complete an acoustic scene analysis, and separate sounds into individual streams.

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more than 460 million people worldwide live with some form of hearing loss. For most, hearing aids are the primary rehabilitation tool, yet there is no one-size-fits-all approach. As a result, many hearing aid users are frustrated by their listening experiences, especially understanding speech in noise. Evelyn Davies-Venn, Au.D., Ph.D., of the University of Minnesota, is focusing on two projects—one of which is funded by Hearing Health Foundation (HHF) through its Emerging Research Grants (ERG) program—that will enhance the customization of hearing aids. She presented the two projects at the Hearing Loss Association of America convention in June. Davies-Venn explains that some of the factors dictating individual variances in hearing aid outcomes in noisy environments include audibility, spectral resolution, and cognitive ability. Audibility changes—how much of the speech spectrum is available to the hearing aid user—is the biggest factor. “Speech must be audible before it is intelligible,” Davies-Venn says. Another primary factor is spectral resolution, or your ear’s ability to make use of the spectrum or frequency changes in sounds. This also directly affects listening outcomes. Secondary factors include the user’s working memory and the volume of the amplified speech. These impact how well someone can handle making sense of distortions (from ambient noise as well as from signal processing) in an incoming speech signal. Working memory is needed to provide context in the event of missing speech fragments, for instance. Needless to say, it is a challenge for conventional hearing aid technology to address all of these complex variables. In light of this challenge for conventional hearing aids, Davies-Venn highlights two projects that are meant to improve hearing aid success. The first focuses on an emerging technology called the “cognitive control of a hearing aid,” or COCOHA. It is an improved hearing aid that will analyze multiple sounds, complete an acoustic scene analysis, and separate the sounds into individual streams, she says. Then, based on the cognitive/electrophysiological recordings from the individual, the COCOHA will select the specific stream that the person is interested in listening to and amplify it—such as a particular speaker’s voice. The cognitive recording is captured with a noninvasive, far-field measure of electrical signals emitted from the brain in response to sound stimuli (similar to how an electroencephalogram, or EEG, captures signals). Davies-Venn’s ERG grant will support research on the use of electrophysiology—far-field or distant (recorded at the scalp) electrical signals from the brain—to design hearing aid algorithms that can control individual variances due to level-induced (high intensity) distortions from hearing aids.


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The second project involves sensory substitution. This project explores the conversion of speech to another sense—for example, touch—through a mobile processing device or a “skin hearing aid.” For the device to function, a vibration is relayed to the brain for speech interpretation. This technology seems cutting edge, but is believed to have been invented in the 1960s by Paul Bach-y-Rita, M.D., of the Smith-Kettlewell Institute of Visual Sciences in San Francisco. Even though it has not yet been incorporated into hearing aid technology intended for mass production, David Eagleman, Ph.D., of Stanford University, and others are hoping to make this a reality. Davies-Venn’s research motives are inspired by a personal connection to her work. “I have a conductive hearing loss myself,” she says. “I had persistent/chronic ear infections as a child that left me a bit delayed in developing speech. I still get ear infections as an adult and have grown accustomed to the low-frequency hearing loss that results until they resolve.” Davies-Venn notes that she also has family members with hearing loss, which makes developing advanced hearing assistance technology even more important to her. Davies-Venn’s projects are in the early stages, and it may take as long as a decade for them to reach the market from the concept. “The goal is to develop individualized

HHF Launches New Website About Ménière's Disease

hearing health foundation recently unveiled a new website dedicated to Ménière's disease, a chronic vestibular (inner ear) condition with no known cure. Launched in September 2018, the website, menieresdisease.org, is designed to build awareness and

hearing aid signal processing to improve treatment outcomes in noisy soundscapes,” she says. “We want to say, this is the most optimal treatment protocol, and it’s different from this person’s, even though you have the same hearing threshold.” Solving hearing aid variances in a precise, individual manner that accounts for variables such as age and cognitive ability will improve communication and quality of life for the millions with hearing loss who use hearing technology.

A 2018 ERG scientist, Evelyn Davies-Venn, Au.D., Ph.D. (far left), is an assistant professor in the department of speech-language and hearing sciences at the University of Minnesota; see more about her ERG project on page 38. 2018 HHF intern Elizabeth Crofts is a junior at Boston University studying biomedical engineering. Support our research: hhf.org/donate

provide information using simple, easy-to-understand pages. The goals are to inspire hope among people who live with Ménière's disease and their loved ones, share updates about Ménière's disease from HHF-funded scientists, and provide opportunities to donate toward Ménière's research. HHF is fortunate to have all administrative expenses covered by its board of trustees and endowment, so 100 percent of gifts directly support life-changing scientific research. Ménière's disease affects about 600,000 people in the U.S. Symptoms include a sensation of ear fullness, dizziness, vertigo, nausea, fluctuating hearing loss, and tinnitus. Some patients benefit from treatments, but many find the disease debilitating, preventing work. Episodes of vertigo can strike anytime without warning, triggering related conditions of

anxiety and depression. Ménière's disease has long been an area of interest in HHF’s research initiatives, including its Emerging Research Grants program. Inspired by supporters interested in Ménière's disease and to further research in the condition, HHF created the Ménière's Disease Grants (MDG) program in 2017. MDG is exclusively focused on the etiology, diagnosis, and treatment of Ménière's disease. The program, which awards scientists up to $125,000 for a two-year grant period, is governed by the Ménière's Disease Advisory Board, comprised of senior researchers and physicians throughout the country who review each application for scientific merit and program relevance. Learn more at menieresdisease.org. —Lauren McGrath

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Recent Research by Hearing Health Foundation Scientists, Explained

A Review of Advancements in the Regeneration of Auditory Hair Cells and Hearing Restoration recent technological advancements have opened up several promising avenues to combat hearing loss. However, many challenges still exist that make it extremely difficult to properly regenerate hair cells, the sensory cells in the inner ear that, when they are damaged or die, result in permanent hearing loss. Proper orientation, tonotopic arrangement (the spatial organization of where sounds of varying frequency are processed in the brain), appropriate integration, and adequate innervation of the cells all have to be considered to provide a functional solution to hearing loss. Of the different strategies outlined, gene therapy will require considerable improvement in order to better control gene targeting, editing, and expression. Adeno-associated viruses are currently the popular choice in delivering these gene therapies, but with the advent of other more sophisticated gene editing technologies like the CRISPR/Cas9 system, it is probable that these newer strategies will also become extremely useful in sensory regeneration research. While the technical capability of these methods has been explored in supporting cells and inner hair cells, there has not been substantial understanding of pathologic mechanisms to advance these strategies into effective therapies. At most, inner ear gene therapy has largely focused on gene replacement or amplification, but

This is a schematic representation of the development of gene therapy in the inner ear. Gene therapy begins with transfection of adenovirus vectors (A) or drug applications (B) to cell cultures. Modified functions are evaluated in in vitro models, specifically inner and/or outer hair cells (C). The results with the best outcomes will move on to be tested in in vivo systems (D) to validate therapeutic benefits before being tested in human clinical trials (E).

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these tactics are only effective in recessive deafness. Since some dominant forms of deafness result from single-point mutations, gene editing technologies including CRISPR/Cas9 will become a valuable tool to deliver therapy in the near future. Due to the limited ability of mammalian hair cells to regenerate, this aspect of hearing loss will continue to receive the most attention to develop novel treatment modalities. The main therapeutic strategies will tend to implicate resetting the course of the reprogramming of supporting cells to a proliferative progenitor state followed by a differentiation phase where both new hair cells and supporting cells are produced. One other stem cell option may in the future be the ex vivo expansion of a patient’s own stem cells and then reintroduction into a damaged inner ear site such as the spiral ganglion. The field’s comprehension of the underlying mechanisms is not yet complete enough to progress into tangible, working treatments. However, the auditory research community working in regenerative medicine is confident that with the evolution of new technologies, treatments for inner ear sensory disorders are sure to emerge. —Rahul Mittal, Ph.D. A 2016 ERG scientist, Rahul Mittal, Ph.D., is an assistant scientist at the University of Miami’s Miller School of Medicine.

illustration credit: adapted from rousset et al., 2015.

EMERGING RESEARCH GRANTS


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Support for a Theory Explaining Otoacoustic Emissions it’s a remarkable feature of the ear that it not only hears sound but also generates it. These sounds, called otoacoustic emissions (OAEs), were discovered in 1978. Thanks in part to ERG research, measuring OAEs has become a common, noninvasive hearing test, especially among infants too young to respond to sound prompts. There are two theories about how the ear produces its own sound emanating from the interior of the cochlea out toward its base. The traditional one is the backward traveling wave theory, in which sound emissions travel slowly as a transverse wave along the basilar membrane, which divides the cochlea into two fluid-filled cavities. In a transverse wave, the wave particles move perpendicular to the wave direction. But this theory does not explain some anomalies, leading to a second hypothesis: the fast compression wave theory, which holds that the emissions travel as a longitudinal wave via lymph fluids around the basilar membrane. In a longitudinal wave, the wave particles travel in the same direction as the wave motion. Figuring out how the emissions are created will promote greater accuracy of the OAE hearing test and a better understanding of cochlear mechanics. Fangyi Chen, Ph.D., started investigating the issue at Oregon Health & Science University as a 2010 ERG recipient and is now at China’s Southern University of Science and Technology. His team’s paper, published in the journal Neural Plasticity

The Importance of Combining Information Across Multiple Frequencies understanding speech in noisy environments is a crucial ability for communication, although many individuals with and without hearing loss suffer from dysfunctions in that ability, including those with auditory processing disorders. This study finds that how well you combine information across multiple frequencies is a critical factor for good speech-in-noise understanding. Our team performed tests using a pitch-fusion task in “hybrid” cochlear implant users who receive both low-frequency acoustic and high-frequency electric stimulation within the same ear. In the pitch-fusion task, subjects heard either a tone consisting of many frequencies in a simple mathematical relationship or a tone with more irregular spacing between frequencies. Subjects had to say whether the tone sounded “natural” or “unnatural” to them, given the fact that a tone consisting

in July 2018, for the first time experimentally validates the backward traveling wave theory. Chen and his coauthors—including 1989–90 ERG recipients Allyn Hubbard, Ph.D., and Alfred Nuttall, Ph.D.—directly measured the basilar membrane vibration in order to determine the wave propagation mechanism of the emissions. The team stimulated the membrane at a specific location, allowing for the vibration source that initiates the backward wave to be pinpointed. Then the resulting vibrations along the membrane were measured at multiple locations in vivo (in guinea pigs), showing a consistent lag as distance increased from the vibration source. The researchers also measured the waves at speeds in the order of tens of meters per second, much slower than would be the speed of a compression wave in water. The results were confirmed using a computer simulation. In addition to the wave propagation study, a mathematical model of the cochlea based on an acoustic electrical analogy was created and simulated. This was used to interpret why no peak frequency-to-place map was observed in the backward traveling wave, explaining some of the previous anomalies associated with this OAE theory. —Yishane Lee A 2010 ERG scientist, Fangyi Chen, Ph.D., is an associate professor in the department of biology at Southern University of Science and Technology in China.

of frequencies in a simple mathematical relationship sounds much more natural to us. We are now studying how we can improve the sensitivity to this “naturalness” in listeners with hearing loss, expecting to provide individualized therapeutic options to address the difficulties in speech-in-noise understanding. —Inyong Choi, Ph.D. A 2017 ERG scientist generously funded by the General Grand Chapter Royal Arch Masons International, Inyong Choi, Ph.D., is an assistant professor in the department of communication sciences and disorders at the University of Iowa. For references, see hhf.org/fall2018references. To read more about ongoing work by ERG alumni, see hhf.org/erg-alumni.

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THE HEARING RESTORATION PROJECT: UPDATE ON THE SEATTLE PLAN AND MORE By Peter G. Barr-Gillespie, Ph.D. hearing health foundation launched the Hearing Restoration Project (HRP) to understand how to regenerate inner ear sensory cells in humans to restore hearing. These sensory hair cells detect and turn sound waves into electrical impulses that are sent to the brain for decoding. Once hair cells are damaged or die, hearing is impaired, but in most species, such as birds and fish, hair cells spontaneously regrow and hearing is restored. The overarching principle of the HRP consortium is cross-discipline collaboration: open sharing of data and ideas. By having almost immediate access to one another’s data, HRP scientists are able to perform follow-up experiments much faster, rather than having to wait years until data are published. You may remember that two years ago, we changed how we develop our projects. We decided together on a group of four projects—the “Seattle Plan”—that are the most fundamental to the consortium’s progress. These projects, which grew out of previous HRP projects, have now been funded for two years, and considerable progress has been made. We have also funded several other projects that have bubbled up out of new observations and capabilities, and they have added considerably to our knowledge base. With this in mind, I am pleased to share with you the latest updates for our 2018–19 projects.

SEATTLE PLAN PROJECTS

Transcriptome changes in single chick cells Stefan Heller, Ph.D. • Found that all “tall” hair cells are exclusively regenerated mitotically in this animal model. • Compiled evidence for different supporting cell subtypes. • Obtained good quality single cell RNA sequencing (scRNA-seq) data and are in the process of evolving an analysis strategy for the baseline cell types (control group). Identified about 50 novel marker genes for hair cells, supporting cells, and homogene cells, including subgroups. • Developed a strategy to finish all scRNA-seq using a novel peeling technique and latest generation library construction methods. • Established two methods for multi-color in situ hybridization (PLISH, proximity ligation in situ 44

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hybridization) and SGA (sequential genomic analysis) for spatial and temporal mRNA expression validation. Epigenetics of the mouse inner ear Michael Lovett, Ph.D., David Raible, Ph.D., Neil Segil, Ph,D., Jennifer Stone, Ph.D. • Completed epigenetic, chromatin structure, and RNAseq datasets for FACS-purified cochlear hair cells and supporting cells from postnatal day 1 and postnatal day 6 mice, and provision of these data sets to the gEAR (gene Expression Analysis Resource portal) for mounting on their webpage through EpiViz for access by the HRP consortium. • Established a webpage (EarCode) so that HRP consortium members can access the current data directly through a University of California, Santa Cruz, genome browser. • Discovered maintenance of the transcriptionally silent state of the hair cell gene regulatory network in perinatal supporting cells is dependent on a combination of H3K27me3 and active H3k27deacetylation, and that during transdifferentiation, these epigenetic marks are modified to an active state. Mouse functional testing John Brigande, Ph.D. • Defined in vitro and in vivo model systems to interrogate genome editing efficacy using CRISPR/Cas9. Implementing the gEAR for data sharing within the HRP Ronna Hertzano, M.D., Ph.D. • Added scRNA-seq workbench for easy sharing and viewing of scRNA-seq data. Such data, which are now driving the field forward, have been particularly difficult to share. • Created additional public datasets to improve data sharing. • Completely rewrote the gEAR backbone to be updated to the latest technologies, allowing the portal to now handle a much larger number of datasets and users. • Performed hands-on gEAR workshops at the Association for Research in Otolaryngology and the Gordon Research Conference, increasing the number of users with accounts to greater than 300.


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Single Cell RNA-seq of homeostatic neuromasts Tatjana Piotrowski, Ph.D. • Optimized protocols for fluorescent-activated cell sorting and scRNA-seq; obtained high quality scRNA-seq transcriptome results from 1,400 neuromast cells; clustered all cells into seven groups; and performed analyses to align the cells along developmental time, providing a temporal readout of gene expressions during hair cell development.

OTHER PROJECTS

Integrated systems biology of hearing restoration Seth Ament, Ph.D. • Discovered 29 novel risk loci for age-related hearing difficulty through new analyses of genome-wide association studies of multiple hearing-related traits in the U.K. Biobank (comprising 330,000 people), and predicted the causal genes and variants at these loci through integration with transcriptomics and epigenomics data from HRP consortium members. • Generated scRNA-seq of 9,472 cells in the neonatal mouse cochlea and utricle (postnatal days 2 and 7). • Conducted systems biology analyses that integrate multiple HRP datasets to characterize gene regulatory networks and predict driver genes associated with the development and regeneration of hair cells. These analyses utilize scRNA-seq of sensory epithelial cells in mouse, chicken, and zebrafish hearing and vestibular organs, as well as epigenomic data (ATAC-seq) from hair cells, support cells, and non-epithelial cells in the mouse cochlea. Comparison of three reprogramming cocktails Andy Groves, Ph.D. • Created and validated transgenic mouse lines expressing three different combinations of reprogramming transcription factors. • Demonstrated these lines can produce new hair cell–like cells in the undamaged and damaged cochlea of the immature mouse. • Compiled preliminary data showing Atoh1 and Gfi1 genes can create ectopic hair cells in the adult mouse cochlea.

Signaling molecules controlling avian auditory hair cell regeneration Jennifer Stone, Ph.D. • Identified four molecular pathways (FGF, BMP, VEGF, and Wnt) that control hair cell regeneration in the bird auditory organ. These pathways were identified in Phase I (gene discovery) as being transcriptionally dynamic in birds, fish, and mice during regeneration, which indicated they may be universal regulators of hair cell regeneration. • Determined that the Notch signaling pathway (a powerful inhibitor of stem cells) also blocks supporting cell division in the chicken auditory organ after damage. This discovery shows that Notch is a negative regulator of regeneration, conserved in birds, fish, and mice. • Identified signaling molecules in birds that are correlated with either mitotic or non-mitotic modes of hair cell regeneration, and are now exploring how these signaling molecules interact to determine which mode of regeneration occurs. Since mammals only exhibit non-mitotic regeneration, we are particularly interested in determining how this mode is controlled.

Up Next

We look forward to our annual meeting, which will be held in Seattle in November. There we will discuss and integrate these data to develop our plans for our 2019–20 projects. As always we are very grateful for the donations we receive to fund this groundbreaking research to find better treatments for hearing loss and related conditions. Every dollar counts, and we sincerely thank our supporters.

The Hearing Restoration Project’s scientific director, Peter G. Barr-Gillespie, Ph.D., is a professor of otolaryngology at the Oregon Hearing Research Center, a senior scientist at the Vollum Institute, and the interim senior vice president for research, all at Oregon Health & Science University. For more, see hhf.org/hrp.

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AND ONE CORRECTION

In the Summer 2018 issue of Hearing Health, we inadvertently left off an investigator’s name and photo in one of the Seattle Plan projects. Here it is in full:

Epigenetics of the mouse inner ear

Michael Lovett, Ph.D., Imperial College London; David Raible, Ph.D., University of Washington; Neil Segil, Ph.D., University of Southern California; Jennifer Stone, Ph.D., University of Washington

Inner ear supporting cells from newborn mice harbor a latent capacity for some regenerative responses, but these disappear within the first few weeks of life. This observation provides an experimental window that this proposal will exploit to address fundamental questions about the failure of hair cell regeneration in mammals. Specifically, we propose experiments to identify those changes in the genetic material, the chromatin, that are responsible for orchestrating the differentiation of new hair cells within the perinatal organ of Corti; and investigating the changes in the chromatin, the epigenome, that lead to the failure of regeneration in the adult inner ear. Support our research: hhf.org/donate

ACCOMPLISHMENTS BY ERG ALUMNI Hearing Health Foundation’s E M E R G I N G cornerstone for six decades has R E S E A R C H been funding innovative, earlyGRANTS career hearing and balance researchers through its Emerging Research Grants (ERG) program. Here is a recent study by two ERG alumni investigating aging effects on speech recognition; see the ever-expanding list of published papers by ERG alumni at hhf.org/erg-alumni. Age Effects on Speech Recognition age-related changes in perceptual organization have received less attention than other potential sources of decline in hearing ability. Perceptual organization is the process by which the auditory system interprets acoustic input from multiple sources to create an auditory scene. In daily life this is essential, because speech communication occurs in environments in which background sounds fluctuate and can mask the intended message. Perceptual organization includes three interrelated auditory processes: glimpsing, speech segregation, and phonemic restoration. Glimpsing is the process of identifying recognizable fragments of speech and connecting them across gaps to create a coherent stream. Speech segregation refers to the process where 46

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the glimpses (speech fragments) are separated from background speech, to focus on a single target when the background includes multiple talkers. Phonemic restoration refers to the process of filling in missing information using prior knowledge of language, conversational context, and acoustic cues. A July 2018 study in The Journal of the Acoustical Society of America by William J. Bologna, Au.D., Ph.D., Kenneth I. Vaden, Jr., Ph.D., Jayne B. Ahlstrom, M.S., and Judy R. Dubno, Ph.D., investigated these components to determine how their declines may contribute to increased speech recognition difficulty with age. As expected, older adults performed more poorly than younger adults. Older adults were less able to make use of limited speech information and reduced continuity. A competing talker created hearing challenges regardless of age. The study concludes, “Taken together, these results suggest that age-related declines in speech recognition may be partially explained by difficulty grouping short glimpses of speech into a coherent message.” —Elizabeth Crofts A 2015 ERG scientist, Kenneth I. Vaden, Jr., Ph.D., is a research assistant professor at the Medical University of South Carolina; 1986–88 ERG scientist Judy R. Dubno, Ph.D., is a member of HHF’s Board of Directors. For references, see hhf.org/fall2018-references. For a continually updated list of publications by ERG alumni, see hhf.org/erg-alumni.


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Can’t Hear Voices On TV? This smartphone app provides captioning for incoming and outgoing calls within one to two seconds with 95 percent accuracy, using stenographers, not voicerecognition technology. FCC-funded, InnoCaption is free to use for those with hearing loss. innocaption.com 703.865.5553

The Les Paul Foundation inspires innovative and creative thinking by sharing the legacy of Les Paul through support of music education, recording, innovation, and medical research related to hearing.

lespaulfoundation.org 212.687.2929

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medel.com 888.MEDEL.CI (888.633.3524) Can’t hear dialogue on TV? Flat-screen TVs have tiny speakers with weak sound. Our AccuVoice® Speaker uses digital algorithms to lift pageDialogue 52 is incredibly clear, even at voices out of background sounds. low volumes. Only 17" wide, it fits anywhere – with just one connecting cord. Room-filling sound, with the clearest voices we’ve heard. Read our 1800+ 4.5-star reviews on amazon.com.

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“Great for folks who have trouble hearing the TV.”

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Hear and be heard, loud and clear. Panasonic’s amplified cordless phone systems are ideal for everyone affected by hearing loss. Among many features, the Volume Booster amplifies the call volume up to 50 decibels.

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800-561-5020 ReSound offers hearing aids for any listening environment and lifestyle. Find the perfect hearing aid for your needs from one of the world’s largest manufacturers.

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fall 2018

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meet the researcher

hearing health foundation

EMERGING RESEARCH GRANTS

Emerging Research Grants (ERG) As one of the only funding sources available for innovative research, HHF’s ERG program is critical. Without our support, scientists would not have the needed resources for cutting-edge approaches toward understanding, preventing, and treating hearing and balance disorders.

Meet the Researcher A. Catalina Vélez-Ortega, Ph.D. University of Kentucky

Vélez-Ortega received a master’s in biology from the University of Antioquia, Colombia, and a doctorate in physiology from the University of Kentucky, where she completed postdoctoral training and is now an assistant professor in the department of physiology. Vélez-Ortega’s 2018 Emerging Research Grant was generously funded by Cochlear Americas.

In Her Words trpa1 is an ion channel known for its role as an “irritant sensor” in pain-sensing neurons (nerve cells). Noise exposure leads to the production of some cellular “irritants” that activate TRPA1 channels in the inner ear. The role of TRPA1 channels has been a puzzling project, with most experiments leaving more questions to pursue. My current project seeks to uncover how TRPA1 activation modifies cochlear mechanics and hearing sensitivity, in order to find new therapeutic targets to prevent hearing loss or tinnitus.

i take dance lessons and participate in flash mobs and other dance performances. But I used to be extremely shy. As a child I simply could not look anyone in the eye when talking to them. I was also terrified of being onstage. It was only after college that I decided to finally correct the problem. Interestingly, taking sign language lessons was very helpful. Sign language forced me to stare at people to be able to communicate. It was terrifying at first, but it started to feel very natural after just a few months.

my father, our town’s surgeon, fueled my desire to learn. When I asked him how the human heart works, he called the butcher, got a pig’s heart, and we dissected it together. I was about 5 when I learned how the heart’s chambers are connected and how valves work. He also set up an astronomy class at home with a flashlight, globe, and ball when I asked, “Why does the moon change shape?” My father’s excitement kept my curiosity from fading as I grew older. That eager-to-learn personality now drives my career in science and teaching.

A. Catalina Vélez-Ortega, Ph.D.’s grant was generously funded by Cochlear Americas. We thank the cochlear implant manufacturer for supporting innovative research investigating noise-induced hearing loss and are grateful for its support of our work.

my training in biomedical engineering guided my interest into hearing science. The field of inner ear research mixes physics and mechanics with molecular biology and genetics in a way I find extremely attractive. Analytics also intrigues me. People who work with me know how complex my calendar and spreadsheets can get. I absolutely love logging all kinds of data and looking for correlations. I also like to plan ahead—passport renewal 10 years from now? Already in my calendar! 50

hearing health

hhf.org

We need your help funding the exciting work of hearing and balance scientists. Please consider donating today to Hearing Health Foundation to support groundbreaking research. Visit hhf.org/how-to-help.


Hamilton® CapTel® 2400i

When it comes to hearing on the phone, seeing really is believing If you haven’t experienced the clear difference a Hamilton® CapTel® phone can make, now is the time to see for yourself. Reliable, accurate and word-for-word captions of everything said to you over the phone ensures clarity on every call – eliminating the frustration even a simple phone call can make! See for yourself – you won’t believe your eyes! If you or someone you care about has difficulty hearing on the phone, see how easy it is to get a life-changing Hamilton CapTel phone at no cost* – just in time for the Holidays!

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The Hamilton CapTel phone requires telephone service and high-speed Internet access. Wi-Fi capable.

091218

FEDERAL LAW PROHIBITS ANYONE BUT REGISTERED USERS WITH HEARING LOSS FROM USING INTERNET PROTOCOL (IP) CAPTIONED TELEPHONES WITH THE CAPTIONS TURNED ON. IP Captioned Telephone Service may use a live operator. The operator generates captions of what the other party to the call says. These captions are then sent to your phone. There is a cost for each minute of captions generated, paid from a federally administered fund. To learn more, visit fcc.gov. Third-party trademarks mentioned are the property of their respective owners. Copyright © 2018 Hamilton Relay. Hamilton is a registered trademark of Nedelco, Inc. d/b/a/ Hamilton Telecommunications. CapTel is a registered trademark of Ultratec, Inc.


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