Fall 2017
A Publication of Hearing Health Foundation
hhf.org
The Veterans & Seniors Issue A salute to our troops, past and present, whose top health concerns are hearing loss and tinnitus
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letter from the chair
DEAR READERS & SUPPORTERS, it is my pleasure to write the introduction for the Fall issue of Hearing Health. Perhaps it is because I am now a full-fledged “senior”—one of the themes of this issue, along with veterans. As I see my friends and relatives begin to lose their hearing, it drives home the lessons of my research in age-related hearing loss, a topic I began investigating in the 1970s. Hearing loss affects the majority of people over the age of 60, with men more severely affected than women, especially those who served in the military. A good friend of mine who used to laugh and tell stories at parties now sits quietly because he does not know what is being said, and he has grown tired of asking, “What?” It is my hope that you, as a reader of Hearing Health, have learned from us and our community about the many options and resources available to keep you, as well as friends and family, from feeling and becoming isolated. But even with existing technology, creating or restoring previously damaged hearing is difficult. For that reason, I am committed to Hearing Health Foundation’s (HHF) mission of funding critical hearing and balance research—in addition to announcing our 2017 Emerging Research Grants scientists (page 32), we have seven pages of published research (page 36)—and providing vital resources for those with hearing loss through our education and awareness programs. In the 1990s, HHF was instrumental in working toward universal newborn hearing screening legislation. Today, 97 percent of newborns are screened for hearing loss, up from 5 percent in 1993. But this accomplishment was at risk of being undone: The proposed 2018 federal budget had called for a complete elimination of all funding—$18 million— toward newborn hearing screenings. In reaction, this past summer HHF immediately joined with other organizations to tell Congress how important this screening legislation is to the most vulnerable Americans. In addition, HHF CEO Nadine Dehgan reached out to all members of the U.S. Senate and the U.S. House of Representatives by phone, email, letters, and in-person
meetings to remind them that detecting hearing loss early is not only the right thing to do because it benefits the child—it is also prudent because it saves money in the long run. I am so very happy to report that 2018 funding for infant hearing screenings and intervention has been reinstated in the federal budget. I also want to give special thanks to Frank and Chris De Francesco, who have made a very generous financial commitment toward that goal by creating a bequest in their will. I have known Frank and Chris for about 15 years through Ralph Strahm, formerly our organization’s tireless development director, who is now in his 80s and still residing in the San Diego area. Frank and Chris are wonderful, outgoing, and welcoming people whose story you can read on page 18. I thank them for the time we have spent together and their confidence in HHF. I think they would agree that all of us as older adults find we have new freedom from the constraints of youth and the time to focus on what we believe in most. Please be assured that HHF continues to earn high marks for our fiscal responsibility, most recently moving up to an A+ rating by CharityWatch, and earning a 4-star rating for the second consecutive year from Charity Navigator, an achievement attained by only 28 percent of the charities it rates. If you would like to support HHF’s research by making a bequest or other donation, we would be most grateful. Please contact us at info@hhf.org for more information. Sincerely,
Elizabeth (Betsy) Keithley, Ph.D. Chair of HHF’s Board of Directors; Professor Emeritus, Division of Otolaryngology, Head and Neck Surgery, University of California, San Diego
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HEARING HEALTH The Veterans & Seniors Issue Fall 2017, Volume 33, Number 4
Publisher Nadine
The top two disabilities for service members and veterans are hearing loss and tinnitus. Here’s how veterans and seniors are coping with hearing challenges, including taking the first steps toward addressing a hearing loss.
Features
12 Seniors Our Half-Century Journey Through Hearing Loss. Merv Daniels, with Bev Daniels 14 Managing Hearing Loss How to Buy Hearing Aids. Barbara Jenkins, Au.D., BCABA 16 Managing Hearing Loss ...And Now From a Wearer’s Perspective. Kathi Mestayer 18 Planned Giving Why We Believe in Hearing Health Foundation. Frank and Chris De Francesco 20 Veterans Guitars for Vets Bernie Kampf
22 Seniors 4 Decades of Coping With Ménière’s Disease + 2 Cochlear Implants = 6 Insights Into Hearing. Robert W. Pope 24 Seniors A Grandfather’s Lesson. Janice Beetle 28 Hearing Health Your Health, Your Hearing. 32 Research Presenting the 2017 Emerging Research Grantees. 36 Research Recent Research by Hearing Health Foundation Scientists, Explained. 50 Research Meet the Researcher: Timothy Balmer, Ph.D.
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03 Letter From the Chair
26 Advertisement Being Able to Hear Is Priceless. Jim Ryun
06 Letters to the Editor 48 Marketplace
44 Advertisement Gift Guide
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 John Dillard in the Mojave Desert, Fort Irwin, California, in December 1977, when he was a first lieutenant in the U.S. Army.
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Editor Yishane
Lee
Art Director Robin Senior Editors
Kidder
Laura Friedman, Amy Gross
Medical Director David
S. Haynes, M.D.
Staff Writers
08 Veterans One Man’s Military Perspective. John T. Dillard
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Dehgan CEO, Hearing Health Foundation
a publication of hearing health foundation
Barbara Jenkins, Au.D.; Emily L. Martinson, Au.D., Ph.D.; Kathi Mestayer; Pranav Parikh; Kathleen Wallace Advertising
Laura Friedman advertising@hhf.org, 212.257.6143 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.; 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 2017, Hearing Health Foundation. All rights reserved. Articles may not be reproduced without written permission from Hearing Health Foundation. USPS/Automatable Poly To subscribe, unsubscribe, or for more information, please call 212.257.6140 or email info@hhf.org.
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letters to the editor
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@editor
dear editor: Teri L. Rocco’s Summer 2017 cover story, “My Own Personal Amusement Ride” (at hhf.org), really hit home for me. I, too, suffer from Ménière's disease, luckily without the “drop attacks” that Rocco experienced. Symptoms for me also include severe tinnitus and significant hearing loss in one ear. When I went to the audiologist, it became apparent that due to nerve damage, a hearing aid was not the answer; sounds were merely amplified, with no improvement in clarity or speech recognition. What has been a lifesaver for me is a Phonak BiCROS system with a wireless transmitter in the side with hearing loss and a wireless receiver in my normal ear. Now I can hear someone speaking on my left side! To my surprise, the ability to determine directionality of sound is regained when I'm wearing the BiCROS. This has been truly lifechanging for me. A low-salt diet, adequate sleep, and lowering stress levels have minimized severe episodes of vertigo, although I carry Transderm Scop patches in case of emergency. The Vestibular Disorders Association (VEDA) has also been a wonderful resource for suggestions for living with balance issues. Thank you for your terrific, relevant magazine and the support for hearing loss research. Keep up the great work! Sharon Leaf, Pharm.D., California
dear editor: Over the past year or longer, I have frequently experienced deafeningly loud TTY/TDD type blasts on VCO (Voice Carry Over) calls. These ear-piercing blasts may occur as the Relay operator starts to place a VCO call or may happen during the conversation. One day in May, I experienced the blasts three times during the same conversation. Over the many many months I have suffered from these blasts, I have been transferred to a Relay supervisor, but it appears that nothing is being done. Though one supervisor offered a suggestion, it had no effect.
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Technical support at my telephone company told me that the problem is called “foreign voltage.” My hearing specialist told me that the blasts are damaging my hearing. Indeed, I am noticing increasing hearing loss. Other hearing persons must be using VCO to converse with oral-deaf individuals, too. Can I be the only person experiencing and trying to eliminate a blast problem? Joyce Gammon, California from the editor: We reached out to staff writer Kathi Mestayer for input, since she has written about assistive listening technology. We were happy to receive this response from Hamilton Relay to help resolve the situation: “We have been in contact with Joyce regarding the loud noises that she has been hearing on VCO calls through California Relay. We have determined that setting up a Customer Profile for the person Joyce is calling should alleviate the noises she hears. Our Customer Care department will implement this. Thank you for forwarding this message to us so that we could resolve Joyce’s concerns.”
dear editor: Many thanks for publishing Ruth Bernstein's tips on traveling with hearing loss (Summer 2017’s “In Transit,” at hhf.org). She draws on many years of experience, and those of us with hearing loss are fortunate to be able to benefit from her expertise. Katherine Bouton, President, Hearing Loss Association of America, New York City Chapter
Share your story: Send us your feedback and stories at editor@hearinghealthmag.com.
I WANT YOU TO HEAR. Every day, I want you to hear the love in my voice. The music box singing to you. The lullabies as you fall asleep. I want you to hear your name called at graduation. The words “You’re hired.” The words “I do.” The first words from your own child. I want you to hear the world today, tomorrow, forever.
Call 1 866 922 9211, or visit www.IWantYoutoHear.com for more information.
©2016 Cochlear Limited. All rights reserved. Trademarks and registered trademarks are the property of Cochlear Limited. CAM-MK-PR-273 ISS1 MAR16
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ONE MAN’S MILITARY PERSPECTIVE A retired colonel who continues to teach and research tinnitus treatments on behalf of the military shares how he has managed his tinnitus, and his hopes for a cure. By Colonel John T. Dillard, U.S. Army (Ret.)
The author stands on the far left with his “Band of Brothers” from the 2nd Battalion, 504th Parachute Infantry Regiment, 82nd Airborne Division, in the Sinai Desert in 1981.
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the top two disabilities for our returning veterans from Iraq and Afghanistan are hearing loss and tinnitus, or ringing of the ears (which is actually a sound inside the brain). Both conditions became a problem for me and for many of my friends in the service. A lifetime spent in the U.S. Army, starting in the 1970s, meant frequent exposure to gunfire and proximity to screaming jets and helicopter engines. Even during a peacetime career in the military, our soldiers, sailors, airmen, and marines are subject to a barrage of auditory insults from the weapons and equipment they operate. It all stacks up to a gradual, although sometimes very abrupt, loss of hearing, usually starting at the higher frequencies. For those in the service, any age-related decline in hearing gets accelerated, to the extreme, by repeated exposure to noise at unsafe levels. For me, tinnitus began faintly and increased with more hearing loss, reaching a crescendo with one big acoustic trauma—a gunshot right next to me in 2009. I immediately began searching for any kind of treatment that would alleviate the loud ringing in my head, which was actually measured in a laboratory at being around a constant 70 decibels. That is roughly equivalent to the noise inside a fairly strong shower, and I soon discovered that people would use long showers to find a bit of relief by masking their tinnitus. (However, I take short showers!) Armed with a background in biology and technology, I began to review all the research I could find. As it turns out, the typical tinnitus condition consists of several brain components: auditory (hearing it); attentional (your awareness of it); memory (persistence); and emotional (how it affects your mood). After many hours on the web, I spent thousands of dollars on things that didn't work, undergoing treatments in all areas of pharmacology, sound therapy, acupuncture, hyperbaric oxygen, and even transcranial magnetic stimulation.
veterans
Tinnitus as a condition is not considered psychological, even though the phantom sound you hear is a result of something occurring in the brain, and not from an external sound source. However, in psychology, attitudes can exert a powerful influence over behaviors.
None of these had any effect for me whatsoever. And despite some incredible recent advances in neuroscience to better understand all of the brain’s complexities, there is still no proven cure or even a viable treatment for tinnitus or to reverse hearing loss. I eventually realized I would have to tackle my tinnitus with the only things out there that to me were credible for managing tinnitus. I eventually found an audiologist who would fit me with hearing aids that provided a built-in tinnitus masking sound. Without a doubt, this became the best purchase decision of my life. As I continued my research, I came across and absorbed an important principle from Pawel Jastreboff, Ph.D., of Emory University. His decades of research led to this tinnitus paradigm: that once the emotional component of how one reacts to the noise is conquered (called “the habituation of reaction”), then a phenomenon called “the habituation of perception” can actually occur. This means it is possible to subconsciously shift your “brain state” so that your own perception of the tinnitus sound can be reduced. It has not been a universal outcome for all patients but was backed up substantially in the accepted research literature, so I gave it a try. I started using Jastreboff’s concepts of Tinnitus Retraining Therapy (TRT)—habituating myself to the tinnitus sound while also using the masking program in my hearing aids. Over the course of my DIY TRT for about three years, I noticed that I finally began to enjoy one noise-free day, sometimes even two, per week. This has been an enduring benefit in the many years since. Jastreboff’s brain model of tinnitus is still the most widely accepted one today. Four controlled studies for tinnitus management were examined during a 2015 seminar series at the National Center for Rehabilitative Auditory Research, with the result that TRT was advocated
as the standard of care, as a component of the Department of Veterans Affairs (VA)’s sequential approach called Progressive Tinnitus Management (PTM). Today, I rarely use my masking devices, and only employ an implanted, removable hearing aid in one ear (the Lyric by Phonak). Although I retired from the U.S. Army in 2001, I continue to work with young military officers who are in their early 30s and 40s, educating them about how I have coped with tinnitus. I have heard similar positive reactions from them about how they have managed their own tinnitus, often a result of an improvised explosive device or a rocket-propelled grenade. They, too, say they are soothed by the masking sounds in their hearing devices, so I am glad the VA is issuing hearing aids to veterans who have a hearing loss. The VA is also piloting working together with active-duty military treatment facilities, and this may result in more consistent and available service member coverage for hearing loss and tinnitus management. Tinnitus as a condition is not considered psychological, even though the phantom sound you hear is a result of something occurring in the brain, and not from an external sound source. However, in psychology, attitudes can exert a powerful influence over behaviors. Making a conscious decision about what is going to be important in your life can be critical to one’s overall well-being. The reality is most of us really do get to choose how we react to life’s challenges. Why not be grateful and positive? In my work with service members, I try my best to encourage people to find their inner strength as they struggle with whatever their condition is. And I try to steer people away from any kind of “snake oil cures,” of which there is an unfortunate abundance. We must continue to fund research in the areas of hearing loss and tinnitus, overseen by highly rated nonprofit organizations like hearinghealthmag.com
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military roundup
hearing health foundation
Hearing Health Foundation and the American Tinnitus Association, on whose board I’ve served as member for several years. So I also urge people to contribute to these nonprofits to advance the research. Based on the science I have reviewed, I think the first step is to find a way to interrupt a very resilient and redundant network of neuronal activity in the brain. This will be paramount for targeting a solution, which may be delivered by way of chemicals or electrical or magnetic stimulation. Ultimately the goal is to quiet things down, so those with tinnitus can hear more of what they want, and less of what they don't.
Share your story: Are you a service member or veteran coping with a hearing condition? Tell us at editor@hearinghealthmag.com.
Colonel John T. Dillard, U.S. Army (Retired), resides in Carmel, California, with his wife of 30 years. A senior lecturer at the Naval Postgraduate School in Monterey, Dillard spent his army career serving in mechanized and parachute infantry assignments and managing programs to bring new technological capabilities to warfighters. He serves on a consumer review panel of tinnitus treatments for the Department of Defense (DoD)’s Congressionally Directed Medical Research Programs and also conducts acquisition policy research for the DoD. Contact him at pa618Faculty@yahoo.com. Read more about the military’s tinnitus treatments in Summer 2012’s “A Landmark Trial” and Summer 2013’s “A Progressive Approach,” both at hhf. org. For references, see hhf.org/fall2017-references.
PROTECTING THE PEOPLE WHO PROTECT US Largely due to noise exposure, hearing loss and tinnitus are the top two health concerns for military service members and veterans. Here’s how the military is working for safer hearing in the service. A Smart Earplug The essential challenge for hearing protection in the military has been how to maintain situational awareness while safeguarding ears. Protection that blocks out the loud sounds of combat—gunshots, explosions, air transport, heavy machinery—reduces the ability to hear voices, footsteps, and other soft sounds critical for readiness. Loud sounds often come without warning, and the protection itself may be uncomfortable. To address this problem, the U.S. Army has developed the Tactical Communication and Protective System (TCAPS), which are earbuds that dampen 10
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military roundup
dangerous noises to safe levels using microphones and noisecanceling technology, while also providing amplification of softer sounds and two-way communication systems. Manufactured by Swedish company Invisio, about 20,000 TCAPS devices costing $2,000 each have been field-tested by U.S. Army soldiers since 2014. “It’s not very often that soldiers are excited about hearing protection, but that’s the feedback consistently received after fitting TCAPS,” says First Lt. Maggie Schad, an audiology expert at Walter Reed National Military Medical Center in Maryland, in a U.S. Department of Defense (DoD) blog post. —Yishane Lee, editor
Hearing Protection in a Pill? A micronutrient found in cheese and yogurt may hold the key to hearing protection. Kathleen Campbell, Ph.D., of Southern Illinois University School of Medicine, is overseeing a phase 3 clinical trial of D-methionine (D-met) administered in a pill to reduce noise-induced hearing loss (NIHL). An amino acid, D-met acts as an antioxidant to mop up free radicals produced by exposure to noise. Campbell first studied D-met nearly two decades ago, finding that it protected against hearing loss resulting from the chemotherapy drug cisplatin. D-met may even be able to reverse NIHL if administered within seven hours. The phase 3 trial is being conducted with the DoD with an estimated December 2017 completion. —Y.L.
Hearing as Part of Overall Health Most hearing loss and tinnitus experienced by service members is the result of excessive exposure to loud noise. Since noise is the most preventable cause of hearing loss, an initiative by the U.S. Air Force called Total Exposure Health (TEH), which focuses on overall health both on and off the job, will measure cumulative noise exposure over the course of 24 hours. While hearing may be within the safe limit at work, spending off-hours at a loud bar or restaurant, mowing the lawn, participating in a loud leisure activity such as motorcycle riding, or even living in a noisy traffic area may push exposure into the danger zone. “Our plan is to create a risk index,” says Richard Hartman, Ph.D., the chief health strategist in the Office of the Air Force Surgeon General. The index will be based on genetics, 24/7 exposure, and clinical disposition (presence of hearing loss, threshold shift, etc.), with recommended protocols such as training, hearing tests, and customized protection. Hartman says in fall 2017, the first TEH noise gathering project will be launched at Moody Air Force Base, Georgia. —Y.L.
loss—for all services, regardless of differences that may exist in individual hearing program practices. According to an October 2016 DoD Hearing Center of Excellence (HCE) report, the rate of hearing loss disability awards is consistently lower for those service members who were, at any point, enrolled in a hearing testing program. The study by the HCE’s Jeremy T. Nelson, Ph.D., and coauthors recommends increasing coverage of DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards. —Peter Osunde
The Navy Investigating NIHL The Office of Naval Research (ONR) has been involved with a range of research on NIHL that is being conducted in institutions across the country. With over two dozen ongoing projects, the research covers three broad categories: medical; assessment and evaluation; and noise reduction. In the medical category is a project by Mark Warchol, Ph.D., of Washington University in St. Louis, who is also a Hearing Restoration Project (HRP) consortium member. In his ONR project, Warchol is investigating whether the reactivation of a specific signaling pathway that triggers hair cell regeneration in birds may induce the same response in mammals. Unlike birds, mammals cannot regrow hair cells after they are damaged or die, leading to permanent hearing loss. Warchol is using a transgenic mouse model that is related to the “DTR mouse” developed by fellow HRP scientist Edwin Rubel, Ph.D., to demonstrate molecular changes in the inner ear. —Y.L.
Past HHF intern Peter Osunde is at The New School for Social Research in New York City, studying politics. For references, see hhf.org/fall2017-references.
Share your story: Are you a service member or veteran coping with a hearing condition? Tell us at editor@hearinghealthmag.com. Support our research: hhf.org/donate
An Ounce of Prevention Service member enrollment in hearing conservation and hearing readiness programs is shown to be consistently associated with a significant reduction in U.S. Department of Veterans Affairs (VA) disability awards for hearing hearinghealthmag.com
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OUR HALFCENTURY JOURNEY THROUGH HEARING LOSS Facing challenges together leads to a deep commitment to help others. By Merv Daniels, with Bev Daniels
in 1963 , at the age of 23 , i failed my first hearing test, given by the local draft board of the U.S. Army. But the tester thought I was faking it—and they needed to fill their quota with a war on the horizon— so I was passed and worked as a medic in Germany and Fort Benning, Georgia. Two years later, I returned to civilian life. A few months into my old job, my boss pulled me aside and said coworkers had commented I was acting distant or unresponsive. Because my boss had grown up with parents with hearing loss, he thought maybe my hearing was the problem. I had it tested and a hearing loss was confirmed. Shortly after I was fitted with my first hearing aid. What a life-changing difference—all because my boss recognized the signs of hearing loss! Being able to hear helped me be more sociable and outgoing, and one night at a roller rink, I was able to chat with the beautiful woman who would become my wife. I married Bev in 1966. Our son was born on Father’s Day 1968, and our daughter in 1971. My hearing began to decline in the 1970s, and I had a stapedotomy operation on my left ear to treat my hearing loss. A few months later I 12
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had the same operation done for my right ear—but it led to a complete loss of hearing in that ear. There is a less than 1 percent risk of this happening, so this was quite a blow. Still, life moved fast, so with my left hearing ear I started work at Disney, a dream job. In Orlando, we began to attend Hearing Loss Association of America (HLAA) meetings, but my hearing continued to decline, and I needed to use increasingly powerful hearing aids and assistive listening devices. I started attending classes for speech-reading as the hearing loss was affecting my ability to communicate at Disney and elsewhere. In 1997, the unthinkable occurred: a total loss of hearing in my “good ear,” my left, at age 58. Nothing could have prepared me for the trauma of going from the hearing world one day to a world of total silence the next. I tried a type of stapedotomy surgery, but it was unsuccessful. The overwhelming realization that I could not hear to fully interact or communicate with family, friends, and coworkers was devastating. I felt out of touch with the everyday things that had previously been a part of my life. I knew I would no longer be able to function at work and had to take early retirement, to face the psychological and emotional effects of a totally different lifestyle.
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Bev was committed to making me hear again. Through her research she learned about the cochlear implant (CI), and in 1998, I was implanted. After four weeks of recovery, it was time to be programmed and turned on. A miracle! I could hear again. Bev and I sat down in the middle of a noisy hospital cafeteria looking at each other across the table, talking to each other, one on one, spontaneously for the first time in two years. I said, “Couldn’t we have picked a more quiet, out-of-the-way place for this?” But now as I think back, what better place than the center of the wonderful noisy environment of a lunchtime cafeteria to celebrate?
When Bev and I recall all the difficulties we faced, we could see that it wasn’t just my problem, or hers, but a shared journey. We decided to commit ourselves to helping others who also are coping with hearing loss. When Bev and I recall all the difficulties we faced, we could see that it wasn’t just my problem, or hers, but a shared journey. We decided to commit ourselves to helping others who also are coping with hearing loss. Besides becoming members of the HLAA, we established HEARS, a support group for people with hearing issues, at Disney. Eventually it became part of HLAA Orlando. At our first HLAA convention in 1999, Bev received the Family Involvement Award for her dedication to supporting and encouraging me and others who experience hearing loss. While at the convention, we also learned about a program developed by the California School of Professional Psychology in San Diego that helps community groups develop better communication strategies for those with hearing loss and their families and friends. To Bev and I this confirmed what we had experienced: the need to learn skills and strategies to prevent and reduce the communication challenges associated with having a hearing loss. We traveled to San Diego for a training session and came home with all the ingredients to put together a six-week workshop that we conducted for two years in Winter Park, Florida, at the Center for Independent Living, a nonprofit that helps people with disabilities.
Dark clouds rolled into our lives once more when Bev was diagnosed with breast cancer in 2003. Now it was my turn to support her as she faced surgery and chemotherapy. She is now a 14-year cancer survivor. In August 2012, I experienced total hearing loss again. Because of the otosclerosis (an overgrowth of bone in the inner ear) and other potential complications, implanting my right ear risked leading to permanent loss of balance. It was too high of a risk. We checked back with our original CI surgeon in Miami who did my implant in 1998, and he agreed to do a re-implantation of my left ear—also risky but our only option for remaining in the world of hearing. When I woke up from the surgery with my head bandaged and my eye taped up, I realized my face was paralyzed. The risk of damaging my facial nerve plus the otosclerosis meant that of the 24 typical electrodes that are inserted with a CI, my doctor could only do 14, and just eight are active. My eye and paralysis, fortunately, recovered after eight long months, and what is so amazing about the CI is that even with only eight active electrodes I can hear and function in the hearing world. My biggest challenge is the understanding of speech, which has been severely affected. As a result, I rely on all of the assistive listening technology available to help fill in the gaps, including a voice recognition smartphone app and TV and telephone captioning. I also advocate for others to take advantage of FM and IR systems as well as hearing loop systems. We continue to be a part of HLAA Support Group Orlando. I have a hard time coming up with words to express the feelings we have for the friends we gained through this organization. They are family. When you are going through a life-changing transition, it is vital that there are people in your life ready and willing to support you. We are committed to sharing our experiences, to advise and help others who are on their own journey with hearing loss and those closely associated with them. We want to help people understand and work through issues related to hearing loss, and to let them know they are not alone.
Merv and Bev Daniels live in Florida.
Share your story: Has a support group helped you cope with a hearing loss? Tell us at editor@hearinghealthmag.com. Support our research: hhf.org/donate
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managing hearing loss
hearing health foundat i o n
Styles of hearing aids include invisible-in-thecanal (the Phonak Lyric, left); receiver-in-thecanal and behind-the-ear (examples from the ReSound LiNX 3D family, near right); and in-the-ear (the Phonak Virto, far right).
How to Buy Hearing Aids 5 practical suggestions to help you to hear your very best. By Barbara Jenkins, Au.D., BCABA
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a friend’s mother needs hearing aids. She has a daughter in the hearing industry, she has insurance to cover hearing aids, she holds a Ph.D. in molecular biology, and she is still overwhelmed and confused by where to go, what the options are, and what is best for her. Sound familiar? There is so much information—and disinformation— available about hearing aids that even some physicians are confused. As with any big purchase, selecting a hearing aid can be difficult and confusing if you don’t have the right information or know the correct questions to ask. Bring a copy of this checklist with you on your next appointment, and feel confident in your decision to improve your life through better hearing.
Hearing Healthcare Checklist 1. Where do I go for a hearing test? Most hearing loss (up to 90 percent) is a result of non-medically treatable issues. But that means as many as one in 10 people will have a medical issue associated with their hearing loss. If this is your first hearing evaluation it would be prudent to see your primary care doctor first, then be referred to a specialist for a diagnostic audiogram (hearing evaluation). Audiologists have a minimum of seven years of university training (master’s or doctorate level). Hearing instrument specialists can perform hearing tests but do not have the medical training to rule out medical issues—causes for hearing loss such as syndromes, Ménière’s disease, Usher Syndrome, sudden-onset, genetics, ototoxic drugs, etc.
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If you know that there is no medically treatable issue associated with your hearing loss, either type of provider should be fine. If you’re in doubt, ask your physician which professional they recommend. They might refer you to an ENT (ear, nose, and throat specialist, or otolaryngologist). please check off the statement below that best applies to you: ____ I have no concern about an underlying medical issue associated with my hearing loss, or I have previously been medically cleared for hearing aids. ____ This is my first hearing exam, and I have concerns about a potential medical issue associated with my hearing loss. I will see my primary care physician, an audiologist, or an ENT to rule out a medical issue first. 2. Where do I buy my hearing aids? Typically, once an audiologist or hearing instrument specialist has evaluated your hearing, you should be able to purchase your hearing aids from them. Requirements differ by state, but generally speaking the professional is trained in hearing aid selection, fitting, and care. Make sure you are comfortable with the quality of care and the options offered by the provider. If only one brand of hearing aid is available, that’s a red flag. Be sure your provider offers a range of choices, in all styles and at all price points. You can also opt to get a second opinion. This will give you additional provider choices, so you can go with
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the person with whom you feel most comfortable. After all, you will be starting a relationship that may last for years. please check off the statement below that best applies to you: ___ I feel confident in the specialist I am seeing. ___ I will schedule a second opinion to be sure I am doing the right thing. I will ask for recommendations from my physician and friends, or do my own research to find a provider with whom I feel most comfortable.
3. What style of hearing aid is best for me? A hearing aid’s style (shape and configuration) is determined by the severity of hearing loss, manual dexterity and vision ability, comfort, and/or cosmetic appeal. Whether you get a larger, behind-the-ear hearing aid, or one that is nearly invisible in the ear canal, the cost is roughly the same. Discuss options with your provider and ask about the benefits and drawbacks to each type of device. Here is a brief overview of hearing aid styles, categorized from a larger size to smaller: Behind-the-ear (BTE); receiver-incanal (RIC) (also known as receiverin-the-ear, RITE): These are currently the most popular due to durability, comfort, and cosmetic appeal. They may be a bit more difficult to put in the ears at first, but since less of the circuitry is inside the ear, they usually offer more natural sound. Also, RICs can be discreet, with only the speaker wire visible at the top of the outer ear.
In-the-ear (ITE); in-the-canal (ITC): This category is among the best for ease of use. Just one piece goes into the ear, with a portion of the device visible outside the ear. Many people like ITEs because they are easy to insert into the ear, and the battery life is better than that of their smaller, ITC cousins. Completely-in-the-canal (CIC); invisible-in-the-canal (IIC): These typically fit deeper into the ear and are a very good choice for people who wear helmets or use stethoscopes. Since they are deep in the canal (making them less visible), the most common complaint is that they may not feel as comfortable as the BTE styles, and depending on usage you must change the batteries once or twice a week. (BTEs and RICs often use larger batteries for more power, and last longer.) please check off this statement when ready: ____ I have reviewed the different styles of hearing aids with my provider and together we have chosen the style that is right for me. 4. Which fidelity level is best for me? Once you have chosen your preferred style of device, you must choose the fidelity (technology) level of the computer chip in the hearing aid. This is where the cost differences in hearing aids become apparent. Most manufacturers have three levels of fidelity in their newest hearing aids as well as in their economy-priced models. The higher the level of technology, the better and faster the hearing aid can separate noise from speech. This means the speech and sound information passed to your brain is more accurate. Every level will help one-on-one conversations in quiet environments; the more advanced chips will boost
clarity and noise reduction even more effectively. In most cases, get the best hearing aid you can afford, but don’t feel pressured into a decision. Take advantage of the 30- to 60-day trial period that is required in most states (in some cases paying a small fee to return the devices).
please check off the statement below that best applies to you: ____ I understand the differences in quality and fidelity of the hearing aids, and I feel confident I am getting the best hearing aid I can afford. ____ I need more information, and possibly a demonstration and/or a trial period to determine which device is best for me. 5. What other special functions do I need for better hearing? In the past few years, new features have emerged that have dramatically changed how we can interact with hearing aids. Rechargeable batteries: Rechargeable hearing aids are now available, requiring changing the battery only once every one to three years. These devices are recharged by placing the entire hearing aid unit on its charging dock. Not having to frequently manipulate the battery door is very helpful if you have vision or dexterity issues or if you tend to forget your batteries. Almost waterproof hearing aids: There are now hearing aids that are so waterproof they actually dry themselves when they get wet. They are also dust- and shock-resistant. These are great for people who frequently spend time outdoors or hearinghealthmag.com
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who just perspire a lot. While it is not recommended swimming with them, these devices should survive taking a shower if you forget to take them out. Bluetooth-enabled hearing aids: Many manufacturers now give you the ability to adjust your hearing aids with your smartphone, using Bluetooth wireless connectivity. You may even be able to stream sound directly to your hearing aids without the use of an additional device like a neck loop. If you’re tech-savvy, this may be for you. Once you’ve gone through the items in this checklist, I hope you feel more confident about making decisions and improving your hearing. The article immediately following has additional hearing aid purchasing tips from the perspective of a consumer who has gone through the process more than once.
Staff writer Barbara Jenkins, Au.D., BCABA, serves as Colorado’s professional state commissioner for people with hearing loss and was awarded the 2010 Leo Doerfler Award for Clinical Excellence by the Academy of Doctors of Audiology. Her office, Advanced Audiology, won the Most Humanitarian Hearing Care Office Award at the 2015 Signia Aspire Conference. For more, see advancedaudiology.com.
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...And Now From a Wearer’s Perspective By Kathi Mestayer
the last time i bought new hearing aids, in 2014, it seemed to take forever—in a good way. My audiologist and I tried different brands, earmolds, receivers-in-canal, settings, programs, features, and controls. It took at least half a dozen office visits, discussing my perceptions, expectations, options, and tradeoffs, plus the tweaking and customizing. My audiologist, Sandy, didn’t seem the least bit annoyed or impatient. At one point, she told me there’s just no way to predict, with 100 percent certainty, what brand or settings will work best for a given patient. So she expects the trial-and-error process. Sandy was a great find. I was referred to her by the wife of one of my husband’s coworkers, also an audiologist, who sang her praises. So I got lucky—I found a terrific audiologist—and that kind of find is now more important than ever. As the hearing aid market and technology get more complicated, we need the best possible experts to help us to navigate, evaluate, make, and revise our choices. From reading the previous article, “How to Buy Hearing Aids,” by Barbara Jenkins, Au.D., BCABA, you know that the first step, if you’ve never had your hearing tested, is to see your primary care physician, and then get a referral. You also know the differences between a hearing instrument specialist (also known as a hearing aid provider or dispenser) and an audiologist. For one thing, the latter has the background to rule out medical causes of hearing loss. Regardless of who tests your hearing, get a paper copy of the test results to take with you. If you decide to go somewhere else for hearing aids, or research how to read your audiogram online, you’ll have the data in your pocket. Beyond this, and because the relationship between you and your provider is so important, here’s what to consider when choosing one: Ask around. Besides asking your primary care physician, get input on specialists from friends and relatives who have had their hearing checked, to see which ones they recommend, and why (or why not). Broaden your network—contact Hearing Health Foundation (hhf.org); find a local chapter of the Hearing Loss Association of America (hearingloss.org); and/or join the Association of Late-Deafened Adults (alda.org). Buying online? Buyer beware. In its “Consumer Information: Buying a Hearing Aid” guide, the Federal Trade Commission (FTC) says: “Buying a hearing aid online or through the mail can be risky, especially if the
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Resist sales pressure about buying a hearing aid. It’s an expensive purchase, so make use of the trial period, mandated in many states, and buy only when you’re satisfied with the answers to your questions. seller does not offer options for adjustments.” Some states do not allow hearing aids to be sold through the mail at all. Poll potential providers. Before making an appointment for a hearing test, call or email providers to help narrow down your choices. As the previous story mentions, a range of hearing aid brands should be available. Consumer Reports says an independent provider should have at least two if not four (or more) different hearing aid brands. Talk about telecoils. T-coils are tiny, copper wire coils inside most hearing aids that significantly improve how well you hear in public spaces where hearing loop systems are installed. When you turn on the T-coil in your hearing aid, it wirelessly picks up the sound transmitted by the loop system and sends it to your hearing aid, which is already optimized to your hearing. T-coils work so well in so many public spaces that forgoing them in your hearing aid is a mistake. Most hearing aids have T-coils, although size may prevent the smallest styles from including them. Ask your provider about how, when, and where to use T-coils and about manual vs. automatic controls. “The automatic-type T-coils will not work in looped spaces,” says Juliëtte Sterkens, Au.D., the HLAA’s hearing loop advocate. This is because the T-coil needs to be very close to the sound source, such as with a cell phone, to be detected and automatically switched on. Ask about real-ear testing. Real-ear testing, or “probe-mic verification,” is a way to directly measure the sound that hearing aids produce in the ear canal. It’s done by placing a narrow silicone tube into the ear canal, past the hearing aid earmold or receiver. The silicone tube makes it possible to detect, in situ, the sound that the hearing aid produces. Because the size and shape of our ear canals vary, it can affect the sound in ways that hearing aid fitting software can’t adjust for perfectly. So the probe-mic test is a valuable reality check as part of a hearing aid fitting. In the June 2017 issue of Hearing Review, Ron Leavitt, Au.D., the founder of the Oregon Association for Better Hearing, and coauthors published results of a study of nearly 100 hearing aid wearers in Oregon. They conducted
probe-mic tests and found that 72 percent were using hearing aids with fitting errors in which the sound level was too low by at least 10 decibels. According to Leavitt, that error level is roughly the equivalent of a 24 percent drop in “speech sound audibility”—a noticeable loss when it comes to understanding speech. The good news is that many providers already have access to probe-mic equipment. A May 2010 study in the Hearing Journal found that, among the 640 audiologists and dispensers surveyed, about half owned or had access to it. But ask before you book an appointment. And most of all, take your time. “Resist sales pressure,” says the FTC guide. “It’s an expensive purchase, so buy only when you’re satisfied with the answers to your questions. Make sure the person you are dealing with puts any promises or guarantees about service and maintenance into the purchase agreement.” Take advantage of the trial period, mandated in many states. It’s tough to keep track of new technology, regulations, and changes in the market. That’s why it’s so crucial to have a qualified, experienced, ethical professional by your side—and why it’s worth the time and effort to find one.
Staff writer Kathi Mestayer serves on advisory boards for the Virginia Department for the Deaf and Hard of Hearing and the Greater Richmond, Virginia, chapter of the Hearing Loss Association of America. For references, see hhf.org/fall2017-references.
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Hearing Health Foundation sincerely thanks Frank and Chris De Francesco (center, shown with their children in a photo from 1998) for designating HHF as a beneficiary in their will.
We Believe
Why in Hearing Health Foundation By Frank and Chris De Francesco
To us, Hearing Health Foundation offers the most practical approach to dealing with hearing-related issues and the best research opportunities to eventually find a cure for hearing loss and tinnitus.
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we were living in brooklyn, new york, when we noticed something amiss about our daughter Karyn. She was about 2 years old. Her older brother, Joe, was jumping around and doing everything. By contrast, Karyn just didn’t seem to be listening to us. We asked our pediatrician about it, and he suggested sending her to a psychologist, thinking she may be mentally handicapped. But the psychologist suspected a hearing loss, which we confirmed through a hearing test in a hospital. We were told about the League for the Hard of Hearing in New York City, now known as the Center for Hearing and Communication. This group helped us figure out how to get hearing health services. Suddenly Karyn’s hearing dropped substantially, and we were told that such a dramatic progression is highly unusual in children under the age of 5. There was a doctor in Manhattan who thought maybe the cause was syphilis, and we thought well, if that is the reason, at least it can be cured. But the tests were inconclusive: Karyn was coming up positive for syphilis, her mother, Chris, was coming up negative, and we thought, this will end in divorce! A second test showed Karyn was negative, too, and we were told to put her on prednisone, a commonly used corticosteroid that alleviates inflammation. But it didn’t help. Karyn is profoundly deaf in both ears. We started speech therapy and—remarkably for back in the 1970s—she was mainstreamed into a Lutheran school on the other side of Brooklyn because one of the teachers there worked at the League for the Hard of Hearing. Karyn was taught spoken English.
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Planned Giving for Hearing Research
“Through the generosity of planned gifts, Hearing Health Foundation (HHF) has been able to fund much of our research and education programs,” says Nadine Dehgan, HHF’s CEO. “We are grateful for those who give generously to create a meaningful, long-term impact on the future.”
If you wish to leave a planned gift, this can be done by designating “Hearing Health Foundation” as the beneficiary of your life insurance policy, or you can simply remember HHF in your will.
To learn more, visit hhf.org/how-to-help. Thank you for your ongoing generosity.
Those who may have already remembered us in their
Please let us know if you have remembered HHF in your estate plan so we may thank you and designate the gift in a manner you wish. Email us at development@hhf.org or call 212.257.6140.
estate plans through our former name, Deafness Research Foundation, should know that their gift will still go to Hearing Health Foundation.
When we moved to California in 1980, our pediatric audiologist in New York City, Jane Madell, Ph.D., put us in touch with an audiologist in San Diego. Judy Horning, Au.D., was a godsend, new as we were to California. She told us the only San Diego school district that would teach Karyn language as well as signing at that time was Poway Unified. And living where we do, with a child with hearing loss, is how we eventually met Ralph Strahm, who was the development director for Hearing Health Foundation (HHF), and Elizabeth (Betsy) Keithley, Ph.D., now a professor emeritus at University of California San Diego and the chair of HHF’s board of directors. By the time Karyn reached high school, her speech was completely typical, and people would say to us they were aware there was a problem but never could have imagined that she is deaf. She speaks well, and clearly. Cochlear implants weren’t advanced enough yet to be an option for Karyn—in the ’80s they were still fairly experimental—and Karyn has what is called “across-theboard hearing.” She does wear one hearing aid, which she started wearing to help with tinnitus she developed in her late teens. She’s 43 now. We set up our family living trust in 1988, and originally designated our children as beneficiaries. Then our son Joe passed away in December 2013, at age 41, from complications from viral pneumonia, and that terribly
sad event plus the experience of Karyn growing up with a hearing loss made us realize we wanted to make a difference in other people’s lives. Our decades-long friendship with Betsy—who became involved with HHF more than 20 years ago, reviewing Emerging Research Grants proposals—also helped cement the deal for us to designate HHF as one of the main beneficiaries of our trust. We support HHF because we believe it offers the most practical approach to dealing with hearing-related issues and the best research opportunities to eventually find a cure for hearing loss and tinnitus.
The De Francescos live in California.
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The author (near left) playing guitar in his bunker at Khe Sanh, Vietnam, in December 1967; and (below right) with a recent Guitars for Vets graduate holding his new acoustic guitar.
Guitars for Vets Watching vets play their guitars, I can see that it brings many of them a peace and calm that I know from personal experience helps to alleviate the impact of chronic tinnitus. 20
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By Bernie Kampf
i spent four years in the u.s. air force, from 1966 to 1970 , starting at age 20. During an eight-month tour in Vietnam I provided mobile power production for communications and navigational efforts. I was also stationed at George Air Force Base in Victorville, California, working on base emergency power and other civil engineering projects. My tinnitus, which I first noticed in my late 20s, was likely caused by that military service. Being stationed in a combat zone with consistent artillery, the close proximity and exposure to aircraft, and the exposure to generators and diesel engines all contributed to my increased risk of hearing damage. Rarely did we use hearing protection—at the time using it meant diminishing your situational awareness. After my service, I worked for over 35 years for AT&T. As a technician I was subject to ongoing audible assaults from working in equipment offices and offices managing high-frequency call switching. In the ’70s and ’80s, I do not recall sound mitigation being provided, but if it was, it was never used as we needed to hear alarms and incoming calls. On top of all that, I played bass and guitar in a band starting at age 14. All these combined experiences—the service, a noisy job, being in a band—likely caused my chronic tinnitus. But it also led to my finding meaningful work with Guitars for Vets (G4V), a group that provides the healing power of music to veterans. In 2009, I met the group’s cofounder, Patrick Nettesheim, at a guitar show near where I live in Illinois. An accomplished musician, Patrick told
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veterans
All these combined experiences—the service, a noisy job, being in a band— likely caused my chronic tinnitus. But it also led to my finding meaningful work with Guitars for Vets (G4V), a group that provides the healing power of music to veterans.
me about G4V, a nonprofit that helps veterans cope with service-related conditions, including tinnitus, by learning to play acoustic guitar. Since I was retired, a veteran, and a musician, I thought this would be a great fit and signed up to help. Soon after meeting Patrick, I opened a chapter at the Edward J. Hines Veterans Administration Hospital, near Chicago. Thanks to its generous sponsors and volunteers, G4V typically offers 10 free, private guitar lessons per veteran, and upon “graduation” we award the vet a new acoustic guitar with all the accessories. We also offer free, open-ended group lessons for up to 10 veterans per group. At Hines, where I am a volunteer chapter coordinator as well as a guitar instructor, we welcomed our first music student in July 2010. Now we have four instructors and will be graduating our 200th vet later this year. Additionally, as G4V’s director of chapter operations, I help oversee the more than 65 chapters around the country, with an additional 20-plus pending. Watching vets play their guitars, I can see that it brings many of them a peace and calm that I know from personal experience helps to alleviate the impact of chronic tinnitus. As for me, I have a mild hearing loss, exacerbated by my tinnitus, which happens to be in a frequency range that tends to make understanding conversations difficult. It is a challenge to discern subtle sounds, especially in noisy situations. Concentrating on the joy of the music helps me ignore the noise, and for a while I no longer notice it. At age 71, I love being able to continue to enjoy and share my love for music. I began playing guitar at age 12, performing with small groups at 14, and playing in bar bands at 16—I grew up in New York City in the 1960s! So I am extremely fortunate to be able to play music with my fellow veterans, teaching them to love music as much as I do and in the process help us all cope with whatever hearing issues we may have.
Bernie Kampf lives in Illinois. One sponsor for Guitars for Vets is the Les Paul Foundation, which also supports HHF’s Emerging Research Grants. Learn more about the recipient of the 2017 Les Paul Foundation Award for Tinnitus Research on pages 32 and 50.
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4+2=6
Decades of Coping With Ménière’s Disease
Cochlear Implants
Insights Into Hearing
By Robert W. Pope when they were young, my daughters dubbed me “Vestibular Man.” They made up lyrics to the tune of a They Might Be Giants song called “Particle Man”: “Vestibular man, vestibular man, gets around the best he can.” I had been diagnosed with Ménière’s disease at age 28—I am now 68—and humor helped in adapting to the dizziness and hearing loss. I have had surgeries to stop the vertigo attacks, starting in 1981 when I was 33. My left ear, before the first surgery, had little hearing left anyway, so the surgeon placed a pin into the cochlea that allowed fluid to drain. It fixed the dizziness, but left me deaf in that ear. I wore a hearing aid, but mainly functioned with just my right ear for 10 years. In 1990 the vertigo returned. My doctor did not want to destroy all my hearing in both ears, so he tried a thenexperimental treatment of injecting gentamicin (an ototoxic drug) into the inner ear. It took three doses, but finally the vertigo episodes stopped. I had hearing in my right ear, fluctuating for the next 10 or so years. Hearing aids in both ears helped. But eventually over the years, the powerful behind-theear hearing aids were not enough for adequate speech recognition. I started feeling my career as a veterinarian was over, as I could no longer communicate well enough to function in the exam room. I knew a cochlear implant (CI) was an option, but I had been turned down as a candidate because my hearing fluctuated, which is common with Ménière’s. At age 64, after failing a hearing test, I was finally a CI candidate. I had to wait a long month before the CI processor could be hooked up. When the day arrived, I was excited but also nervous because although implantation has a very high success rate, failures do occur. My audiologist made the connections and tested my hearing. I don’t know if disappointment or confusion showed on my face, but whatever I expected to happen did not. “What do you hear?” she asked. 22
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“Nothing at all from the CI ear,” I said. “But I seem to hear better from my right ear.” She smiled. This was my first step toward understanding my CIs, and my hearing, better.
Insight #1: Hearing does not occur only in the ear.
The ear and related structures are designed to bring sound to the brain, but it is the brain that interprets the sounds as hearing. My brain had not heard sound from my left ear for over 30 years. Hearing from my CI would take training, adjustments, and time. My hearing improved with each new adjustment, and thus began my wonderful journey back to the world of sound. On one of the first days with my new CI, I was walking with my wife near a field. I heard a very strange noise. It sounded much like a high-pitched chirp. I thought it might be a cricket, and my wife confirmed my guess. I believe I can recognize some sounds if my brain has a memory of them: besides crickets, things like airplanes, birds, cars. But other sounds are troublesome, sometimes even frightening. I cannot recognize buzzers on appliances or electronic instruments. When they go off, I sometimes still jump and ask what it was, in hopes my brain can eventually recognize it.
Insight #2: Communication is essential to meaningful human relationships.
My wife and family never gave up on communicating with me, no matter how frustrating it became. But over the decades my personality and what I was able to do changed drastically because of my hearing loss. I had adapted to the loss by withdrawing from people, places, and activities that became difficult or impossible. When my hearing came back with the CI, I blossomed again. People talk, and I listen—and I am still continually amazed. Work is easier and more enjoyable. I had loved listening
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to people tell stories about their pets. Now, not only am I listening, but I am also laughing, understanding, and asking for another.
Insight #3: Batteries are critical.
Batteries are sound, and dead batteries are silence. Batteries need charging (or changing) and they run out of power at the most inopportune times, such as midconversation. I now always carry a survival bag—batteries, spare parts, and tools— for when my electronics fail. My frequently uttered phrase is “hold that thought—while I change my batteries.” At night when my CI processor comes out, I go into a silent cycle. I cannot hear thunderstorms or the dog barking. I rely on my wife for information. But each morning when I rise and plug in my electronic ear, I get to experience the most amazing miracle of sound again and again and again, every single day.
Insight #4: Hearing is better with two ears.
In January 2017 my second CI processor was activated, and with it a new journey of hearing began. Some people asked me, “Why bother if you are doing well with the one CI?” To be honest, I was comfortable with one. I felt somewhat selfish wanting improvement on an already great and wonderful thing. But if I needed the first CI for me, to be able to hear, the second was for my wife, family, friends, and everyone I interact with, to help me communicate even better. With the second CI, I can hear with two ears, which helps me localize, or pinpoint, the source of a sound. When I walk the dog I can hear a car behind me. I now look in the right direction when someone calls my name. But more than that, sound from the second ear improves overall hearing. It helps the brain decipher the meaning of sounds. During a recent appointment with my audiologist I scored over 90 percent in the speech recognition test!
Insight #5: Two hearing ears mean no more do-si-do.
I used to tell people that I was left-handed and right-eared. Hearing out of my right ear for most of my life has caused me to position myself for best hearing comprehension. At first I had to train my wife and family to walk on my right side. Then after the first CI, I had to retrain them to walk on my left! People who know me were always trying to get on my good side. Walking and talking became a do-si-do. With two CIs we no longer need to square dance.
Insight #6: Wear a hat.
I am not a hat person. But wearing one has become a necessity. It protects the sensitive electronic microphones of my CI from water damage when it rains and wind noise on a breezy day. Several times my CIs have gotten wet from rain, instantly turning my hearing to static. It takes
several hours to dry them out. A hat also protects me from every CI wearer’s kryptonite, metal objects. If my head is close to metal, and I’m not wearing a hat, the magnet from the processor can get sucked off my head and attach to the metal. This results in instant hearing loss of major proportions. The first time this happened I was baffled and spent several minutes looking for my CI on the floor only to discover that it was hanging from a metal pipe above my head.
The ear and related structures are designed to bring sound to the brain, but it is the brain that interprets the sounds as hearing. My brain had not heard sound from one ear for over 30 years. Hearing from my CI would take training, adjustments, and time. Then there is what I call the hat brim effect. Wearing a hat with a brim not only protects the CI but also improves the sound reception. The sound seems to be directed under the brim and into the tiny microphone on the top of the CI processor, amplifying the sound. The improved volume is nothing short of amazing. It makes me want to wear my hat indoors. My two CIs give me the best hearing I have experienced in 40 years. If my hearing loss partly shaped the person I had become, with two CIs, who knows who I will be in the coming years! CI life is truly wonderful.
Robert W. Pope, D.V.M., lives in Wisconsin. His previous four articles for Hearing Health magazine were published between 1997 and 2003 and shared his earlier experiences of coping with Ménière’s disease.
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A GRANDFATHER’S LESSON When an infant is born with a hearing loss, his grandfather takes steps to finally treat his own. By Janice Beetle
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to finally deal with his own hearing loss. He was fitted with hearing aids in both ears and began attending an audiology program hosted by a nearby college. “I can be a stubborn mule sometimes,” Stoller says. “I pushed the whole topic off for a long time. When I realized Pierce might need a device to aid his hearing, I thought to myself, ‘Well if he’s going to wear a device, why can’t I wear one?’” Pierce’s mother, Gabby Stoller Wasserman (Stoller’s oldest daughter), says she and her husband were thrown off by the revelation of Pierce’s hearing loss as no one in either of their families had experienced childhood hearing loss. She says Pierce loves his Cochlear Baha, which is worn using a headband that Pierce can slip on himself. Their audiologist also told the Wassermans about the New York City campus of Clarke Schools for Hearing and Speech. At its five East Coast campuses and through its virtual tVISIT teleservices, the school provides more than 1,200 children who are deaf or hard of hearing with the listening, learning, and spoken language skills they need to succeed. “Clarke calmed me,” Gabby says. Now 2, Pierce began at Clarke with service coordination, a guide through the early intervention process. He also received speech therapy and audiology services via Clarke and participated in its parent-infant group. This fall he is attending a mainstream preschool on Long Island three days
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a week while staying connected to Clarke through tVISIT. His growing vocabulary includes “Riley,” the name of his 4-year-old sister (above, left). As for Stoller, his daughter’s experience has made him especially empathetic toward children with hearing loss. “They have obstacles to overcome. It breaks my heart,” says Stoller, who as a result became involved with Clarke’s Sponsor a Child program. And it’s had a dramatic effect in the acceptance of his own late-onset hearing loss. “I put the hearing aids on and don’t even think about them. Just having general conversation is easier,” he says. “I’m absolutely glad I finally did it.”
Massachusetts– based writer Janice Beetle is a communications consultant for Clarke Schools for Hearing and Speech. For more, please see clarkeschools.org.
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photo credit: david fried (bottom)
fifteen years ago, stu stoller, a chief financial officer, began to have trouble hearing his colleagues in business meetings. Often, he could only make out the dialogue from those sitting beside or in front of him. Stoller visited his doctor for a hearing exam and was referred to an audiologist. He bought a hearing aid but wore it only sporadically before abandoning it. “I wasn’t even close to 60 yet,” says Stoller, now 62, of Long Island, New York. “I didn’t want people to see the device and think of me as someone who was getting older, and I didn’t want an inconvenience in my life. I wasn’t totally convinced yet that I was suffering from hearing loss.” As the years passed, Stoller’s wife, Leslie, and his two daughters encouraged him to seek help as they could tell he wasn’t able to fully engage in family conversations. Stoller put off taking action until his grandson Pierce (shown above, right) was born in September 2015, and the family learned the child would likely have a hearing loss. Pierce was born with microtia and atresia; his right ear and ear canal were not fully formed. This condition affects less than 5 percent of children who are deaf or hard of hearing and is often diagnosed at birth. An audiologist fit Pierce at age 2 months with a bone-anchored hearing device, which uses bone conduction to send sound directly to the inner ear, bypassing the outer and middle ear. Pierce’s procedure encouraged Stoller
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*compared to binaural beamforming in hearing aids for severe to-profound hearing loss (Effect of directional strategy on audibility of sounds in the environment for varying hearing loss severity. Jespersen C, Kirkwood B, Groth J., 2017).
**with Binaural Directionality III compared to omni directionality
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(Binaural Directionality III compared to omni directional. Data on file)
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Being Able to Hear Is Priceless Jim Ryun, who experienced hearing loss as a young child, set the record as the first high school student to break the four-minute mile (3:59:0), a record that lasted 36 years. Ryun has worn Lyric for nine years. Living life without limitations with 100 percent invisible, 24/7, hassle-free hearing provided by Lyric means that he hears all the sounds around him while running, as well as the voices of his 13 grandchildren. By Jim Ryun when i was about five, i had the measles. A very high fever caused me to lose roughly half of my hearing ability, although I didn’t realize it at the time. In grade school, I kept answering questions with the wrong answers and, embarrassed, stopped even trying to answer. A hearing test at school confirmed my hearing loss—but I was told the “moderate” degree meant I would not benefit from hearing aids.
Life is for the living, and you only live once. Why not invest in quality of life and hearing as much as possible? Finally in the early ’90s when I was 44, I had a friend urge me to see an audiologist. The evaluation from the audiologist was encouraging, and I began wearing hearing aids that dramatically changed my hearing, thus enhancing life. As time passed, the Lyric by Phonak became available. The advanced hearing technology in this extended-wear, in-the-ear-canal hearing device improved my hearing even more. Designed for mild to moderately severe hearing loss, Lyric provides a new, crisp, quality sound—for me, this means sounds are now deeper and richer, more complete. And because they are deep in the ear canal, they are invisible, comfortable, and not affected by sweat or the sound of wind. When I am out for a run, I can hear the traffic and footsteps behind me. 26
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Jim Ryun with his wife Anne.
For many folks, hearing devices are expensive; however, being able to hear is priceless. When I was in the House of Representatives, I introduced in 2003 the Hearing Aid Tax Credit to help address the issue of affordability. Versions of the bill remain in the House and Senate today. Quality of life is difficult to measure in dollars and cents. And quality of life is not just for those who have lost hearing but for those around them. Until I was able to treat my hearing loss, my wife Anne, whom I married in 1969, was my hearing aid. She would patiently repeat what our young children were saying. Since we had four children in five years, this was a lot of information to relay. Now, with Lyric, I can hear our grandchildren, all 13 of them. If you think you are losing your hearing, you probably are. Find a good audiologist and get tested. Life is for the living, and you only live once. Why not invest in quality of life and hearing as much as possible?
A track and field silver medalist in the 1968 Olympics, Jim Ryun served as a member of the U.S. House of Representatives for the 2nd Congressional District of Kansas from 1996 to 2007.
Lyric is designed to be worn 24/7 for months at a time. Duration of battery life varies by patient and is subject to individual ear conditions, hearing loss, and listening environments. Depending on battery life, Lyric may be worn up to 120 days. Individual patient needs may vary, and Lyric is not appropriate for all patients. Visit a Lyric provider to determine if Lyric is right for you. Call 844.606.7892 or visit phonak-us.com/lyric.
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1 Individual replacement needs may vary. Duration of device battery life varies by patient and is subject to individual ear conditions. 2 Professional fees may apply. Annual subscription begins the first day of trial. 3 Lyric is water resistant, not waterproof, and should not be completely submerged under water. Lyric is not appropriate for all patients. See a Lyric Provider to determine if Lyric is right for you. Individual patient results may vary. Lyric, Distributed by Phonak, © 2017 Phonak, LLC. All rights reserved.
MS-051727 603
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hearing health foundation
Take care of your health to take care of your hearing, because hearing loss often occurs with other serious health conditions.
your Health, your Hearing 30 to 40 percent
Accelerated rate of cognitive decline among adults with hearing loss compared with those without, according to JAMA Internal Medicine
⁄3
more than
1
Adults who wore hearing aids who experienced mental health improvements, from National Council on Aging research
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Cardiovascular Disease
Heart health and hearing health frequently correspond, according to a 2010 research review in the American Journal of Audiology that found six decades of evidence of both positive and negative correlation. “The most significant positive relationship between improved cardiovascular health and improvements in those auditory systems has been found among older adults,” the Wichita State University researchers conclude. Studies have shown that a healthy cardiovascular system has a positive effect on hearing. One report using data from the Nurses Health Study II and reported in the American Journal of Medicine in 2013 found that a higher level of physical activity is associated with the lower risk of hearing loss in women. Conversely, a higher body mass index and larger waist circumference are associated with higher risk of hearing loss. Also, low-frequency hearing loss may be a marker that predicts the presence or potential development of cardiovascular disease. In 2009, researchers at the Medical College of Wisconsin developed and tested a mathematical formula using audiometric pattern and medical history to predict the probability of cardiovascular diseases and events. Why the link: Poor cardiovascular health causes inadequate blood flow and blood vessel trauma to the inner ear. The inner ear is so sensitive to blood flow that hearing loss, particularly at the lower frequencies, may be an early warning sign of cardiovascular disease.
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Dementia
Multiple studies indicate hearing loss can be linked to the onset of dementia and Alzheimer’s disease. According to research by otologist and epidemiologist Frank R. Lin, M.D., Ph.D., of Johns Hopkins University, individuals with a hearing loss showed a 30 to 40 percent accelerated rate of cognitive decline over the study’s six-year period, compared with individuals with typical hearing. In addition, the more severe the hearing loss, the greater the likelihood of developing a cognitive disorder, and the steeper the decline in mental function. Even subjects with mild hearing loss were found more likely to experience cognitive failures, according to Lin’s 2013 report in JAMA Internal Medicine. An additional 2014 study by Lin in the journal Neuroimage found that people with at least a 25 decibel (dB) hearing loss (categorized as “mild” and higher) had accelerated rates of gray matter shrinkage in the brain compared with those with typical hearing. Similarly, Jonathan Peelle, Ph.D., of the University of Pennsylvania, found a link between hearing loss and gray matter atrophy in auditory areas of the brain. “As hearing ability declines with age, interventions such as hearing aids should be considered not only to improve hearing but to preserve the brain,” says Peelle in Science Daily. A major study in the July 2017 issue of The Lancet by 24 international researchers identified nine lifestyle risk factors for dementia. “Midlife hearing loss” is the highest, contributing to 9 percent of a 35 percent total changeable risk factors, more
hearing health
than smoking or physical inactivity; 65 percent of dementia risk factors are potentially unchangeable. Why the link: Termed “cognitive load,’ the effort it takes those with hearing loss to hear and comprehend creates a regular strain that interferes with normal cognition. In addition, people with hearing loss tend to withdraw socially and the lack of regular interaction leads to mental stagnation.
Depression
+9
percent
The risk midlife hearing loss adds to developing dementia, the highest among nine modifiable, lifestyle risk factors identified in The Lancet
Falls among middle-age and older adults with even a mild hearing loss of 25 decibels are nearly three times more likely, according to the Archives of Internal Medicine
Several studies have confirmed the link between depression and hearing loss, particularly among women. In a 2014 JAMA Otolaryngology–Head & Neck Surgery report using data from the National Health and Nutrition Examination Survey 2005–2010, hearing loss was significantly associated with an increased risk of depression in adults of all ages, but was most pronounced in those ages 18 to 69 years, and depression was higher among women vs. men with hearing loss. A 2010 meta-analysis of 31 studies in Aging Research Reviews reported that hearing loss is among the most common chronic conditions associated with depression in people ages 60 years or older. Working-age adults 35 to 54 years old with untreated hearing loss were found more likely to develop depression and other psychological issues than their hearing peers, according to a 2008 report in the journal Acta Otorhinolaryngologica Italica. However, research by the National Council on Aging in 2000 found that wearing hearing aids may help prevent depression. In its survey of 4,000 people, 36 percent of people who wore hearing aids experienced improved overall mental health, while 34 percent increased their social engagement. A small 2011 study in the Archives of Gerontology and Geriatrics showed that after just three months of use, hearing aids significantly improved emotional and cognitive conditions in patients over age 65. Why the link: Communication, which is vital to social interactions, becomes a source of stress when someone must strain to hear others speak, and frequent misunderstandings may result in embarrassment. Some people with untreated hearing loss begin to avoid
social situations, particularly if they involve loud surroundings like parties or crowded restaurants where understanding speech is even more difficult. Withdrawal tends to progress until the person chooses to remain in silent isolation rather than struggle to hear and communicate publicly. In addition, older patients may feel their hearing loss is a signal that they are simply too old to participate in social activities.
Diabetes
Hearing loss is about twice as common in adults with type 2 diabetes, which accounts for 95 percent of all diabetes cases in the U.S., compared with those who do not have the disease, says the National Institutes of Health. Broken down by age, people under age 60 had 2.61 times the risk of acquiring a hearing loss, while people over 60 had 1.58 times the risk, according to a meta-analysis of 13 studies published in the Journal of Clinical Endocrinology & Metabolism in 2013. Why the link: Diabetes may lead to sensorineural hearing loss by damaging the nerves and blood vessels of the inner ear. This includes sclerosis (hardening) of the auditory artery; thickened capillaries of the stria vascularis, a tissue in the inner ear; atrophy of the spiral ganglion, nerve cells in the inner ear; and damage to the myelin sheath protecting the vestibulocochlear nerve, important for conveying sound and balance information.
Falls
Falls are responsible for numerous injuries and deaths among older adults. According to the Centers for Disease Control and Prevention, more than one in four seniors falls each year, and they may experience brain injuries and hip and other bone fractures after a fall. Research has linked the risk of falls with hearing loss. Middle-age and older adults with a mild hearing loss (25 dB) were nearly three times more likely of having fallen over the previous year than those with no hearing loss, according to Lin of Johns Hopkins, in a 2012 research letter published in the Archives of Internal Medicine. For every 10 dB increase in hearing loss, there was an increased 1.4-fold odds of falling. hearinghealthmag.com
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hearing health foundation
Why the link: Hearing loss decreases awareness of the surrounding environment and increases cognitive load. The brain is overwhelmed with demands on its limited resources to maintain balance and gait, while straining to hear and process auditory input. It is also possible that cochlear disorders affect vestibular function, leading to poor balance.
hearing loss is
2x
more common Among adults with type 2 diabetes, according to the Journal of Clinical Endocrinology & Metabolism And also among adults with iron deficiency anemia, says a JAMA Otolaryngology– Head & Neck Surgery study
additional links between Health and Hearing Loss Anemia
In a 2016 JAMA Otolaryngology–Head & Neck Surgery study examining data from over 300,000 people, Pennsylvania State University researchers found that those with iron deficiency anemia were twice as likely to have a hearing loss when compared with those without anemia. The hearing loss was sensorineural (related to the inner ear) as well as combined (related to the inner ear and the middle ear). A December 2016 article in U.S. News & World Report about the study quoted David Haynes, M.D., Hearing Health Foundation’s (HHF) medical director and an HHF board member, as saying, “These and other studies should put iron deficiency on the radar as one of the factors that may be contributing to hearing loss. Other studies will be needed to confirm the findings, as in all medical research.” The story also quoted Peter Steyger, Ph.D., a member of HHF’s Council of Scientific Trustees, who theorized why there is a link. “Iron is clearly required for normal functioning of the auditory system, as for many other organs, and too little can result in anemia, the loss of hemoglobin in red blood cells to carry oxygen to the tissues in the body,” he says.
Share your story: Do you have a hearing loss and another health condition? Tell us at editor@hearinghealthmag.com. 30
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This article was adapted with permission from materials supplied by Sivantos Inc., one of the leading hearing aid manufacturers worldwide and the manufacturer of Signia (formerly Siemens) brand hearing aids. For more, see signiausa.com. For references, see hhf.org/fall2017-references.
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Ototoxic Medications
While they save lives, chemotherapy drugs may also be ototoxic (harmful to hearing), especially platinum-based chemotherapy medications such as cisplatin and carboplatin. Other potentially ototoxic chemotherapy drugs include bleomycin, vincristine, vinblastine, bromocriptine, methotrexate, and nitrogen mustard. Aminoglycoside antibiotics used to fight serious infections are also known to be ototoxic, along with pain-relieving salicylates and nonsteroidal antiinflammatory drugs (NSAIDs). In addition, phosphodiesterase type 5 (PDE5) inhibitors that treat erectile dysfunction have been linked to sudden sensorineural hearing loss. This drug class includes Viagra, Levitra, and Cialis. Dosage, duration, and method of administration all affect a drug’s ototoxicity risk. —Yishane Lee, editor
For references, see hhf.org/fall2017references. Read about Emerging Research Grants researchers investigating cisplatin ototoxicity on pages 34 and 35.
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research
EMERGING RESEARCH GRANTS
Presenting the 2017 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 14 Emerging Research Grants (ERG) scientists who are working in the areas of Ménière’s disease, tinnitus, central auditory processing disorder (CAPD), hearing health, Usher syndrome, hyperacusis, hearing loss in children, and stria vascularis.
MÉNIÈRE’S DISEASE
One grant was awarded for innovative research that will increase our understanding of the inner ear and balance disorder Ménière’s disease. This grant was supported by The Estate of Howard F. Schum.
The Les Paul Foundation Award for Tinnitus Research was awarded for research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus.
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Massachusetts Eye and Ear, Harvard Medical School Project: Characterization of endolymphatic sac anatomy in early and late onset Ménière’s disease: a clinical radiologic study Goal: To confirm whether the shape and course of a bony canal that traverses the inner ear endolymphatic sac can act as a reliable biomarker to identify distinct types of Ménière’s in patients. This project will potentially lead to an earlier diagnosis of Ménière’s, which currently is identified based on clinical symptoms that can vary greatly, and the development of a novel classification for Ménière’s, in order to inform treatments for the range of Ménière’s patients.
Timothy Balmer, Ph.D.
TINNITUS
32
Ngoc-Nhi Luu, M.D.
Oregon Health & Science University Project: Chronic transmitter exposure in excitatory neurons of the cochlear nucleus generates persistent excitation and could underlie tinnitus Goal: To investigate whether problems with the chronic transmitter exposure of unipolar brush cells (specialized nerve cells that amplify auditory signals and send them to the cochlear nucleus in the brainstem) may be a cause of tinnitus. Understanding if hyperactivity of the cochlear nucleus is a factor leading to tinnitus will eventually help the development of clinical tinnitus treatments. (To read more about Balmer, see page 50.)
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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 conditions affecting the way the brain processes auditory information. All four CAPD grantees are General Grand Chapter Royal Arch Masons International award recipients.
Joseph H. Bochner, Ph.D.
Rochester Institute of Technology Project: Auditory experience, critical periods, and the development of categorical perception in cochlear implant users: a preliminary investigation Goal: To investigate the role of age on the success of cochlear implantation and auditory experience on the development of perceptual (phoneme) categories in prelingually deaf cochlear implant users. This will help to better understand the impact hearing loss may have on central auditory processing when the onset occurs at birth or within the first two to three years of life, before language is acquired.
Angela Yarnell Bonino, Ph.D., CCC-A
University of Colorado Boulder Project: Toddlers’ and preschoolers’ ability to hear speech in noise: assessing performance with a two-interval, observer-based procedure Goal: To develop a reliable behavioral method for measuring speech perception in noise for toddlers and preschoolers. Results from this project will provide information on how typical auditory development unfolds during toddler and preschool years, which may advance the understanding of auditory processing disorders and the effects of hearing loss.
Inyong Choi, Ph.D.
University of Iowa Project: Neural correlates of selective listening deficits in a multiple-speaker environment Goal: To investigate key neural processing systems required for successful speech communication in noisy settings, and how neural processing deteriorates in listeners with degraded hearing ability. This project includes basic neuroscientific research of the central auditory system as well as research on hearing rehabilitation techniques, with the aim to develop clinical tests to identify “hidden” hearing loss currently undiagnosed.
Christina Reuterskiöld, Ph.D.
New York University Project: Rhyme awareness in children with cochlear implants: investigating the effect of a degraded auditory system on auditory processing, language, and literacy development Goal: To assess that the benefits of early cochlear implantation support the development of central auditory processing skills and phonological awareness, and to better understand the implications of early auditory deprivation on auditory processing, language, and literacy learning, which will ultimately lead to improvements in targeted intervention and educational approaches.
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HEARING HEALTH HHF awarded three grants for the best overall hearing research proposals. Roberts' grant is generously funded by The Barbara Epstein Foundation Inc.; Banakis Hartl and Tan’s grants were supported by HHF’s Board of Directors as well as supporters who designated their gifts to fund the most promising hearing research.
Renee Banakis Hartl, M.D., Au.D. University of Colorado Denver Project: Effect of deafness duration on the efficacy of cochlear implants for single-sided deafness Goal: To better predict cochlear implantation efficacy, which is now subject to diverse clinical characteristics, by focusing on one variable (duration of deafness) in a specific etiology (singlesided deafness) using an animal model. This will eventually help determine patient parameters to improve outcomes for cochlear implant use.
Michael Roberts, Ph.D. University of Michigan Project: Cellular and synaptic basis of binaural gain control through the commissure of the inferior colliculus Goal: To understand the fundamental mechanisms for binaural processing in the inferior colliculus—the hub of the central auditory system located in the auditory midbrain with left and right lobes connected by the commissure (nerve fibers)—and how these mechanisms are affected by hearing loss, hearing aids, and cochlear implants. This will help guide advances in the design of auditory prostheses and improve the ability of hearing device users to understand speech in noisy environments.
Xiaodong Tan, Ph.D. Northwestern University Project: Oto-protection of honokiol against cisplatininduced ototoxicity Goal: To determine whether honokiol, an antitumor agent derived from the magnolia plant, may reduce or eliminate the ototoxicity (causing hearing damage) of the commonly used chemotherapy drug cisplatin, and ultimately to determine whether honokiol may be effective for other causes of hearing loss, such as through noise or age.
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 HHF’s Board of Directors and donors who designated gifts to fund Usher syndrome research.
Clive Morgan, Ph.D. Oregon Health & Science University Project: Characterization of USH1F macromolecular complexes Goal: To better understand the three main Usher syndrome type I genetic forms by studying the molecular architecture of and protein complexes in the hair bundle, which converts sound to signals in the inner ear sensory hair cell. Information from these studies will help decipher the molecular basis for genetic abnormalities leading to deafness, such as in Usher syndrome, and help to better understand the functioning of the auditory and vestibular system.
Support our research: hhf.org/donate
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HYPERACUSIS
Two grants were awarded focused on research (e.g., animal models, biomarkers, brain imaging, electrophysiology) that will increase our understanding of the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance. These grants were funded by Hyperacusis Research Ltd.
Senthilvelan Manohar, Ph.D. University at Buffalo Project: Behavioral model of loudness intolerance Goal: To develop and establish a reliable animal behavioral model to understand the pain and annoyance aspects of hyperacusis through testing two animal models together, which will assess emotional reactions to sound as well as the neural interactions between auditory perception and pain sensation. These animal models may help identify the neural and molecular mechanisms underlying hyperacusis and tinnitus.
Jennifer Resnik, Ph.D. Massachusetts Eye and Ear, Harvard Medical School Project: Homeostatic modifications in cortical GABA circuits enable states of hyperexcitability and reduced sound level tolerance after auditory nerve degeneration Goal: To better understand the paradoxical role of central auditory system plasticity as both the cause of—and treatment for—the perceptual consequences of hearing loss. A major step to reach this goal is to understand the compensatory mechanisms, following cochlear damage, that allow for basic sound recovery that may potentially introduce hypersensitivity and cause chronic sensory impairments such as hyperacusis.
HEARING LOSS IN CHILDREN
Oscar Diaz-Horta, Ph.D.
STRIA VASCULARIS
Sandeep Sheth, Ph.D.
University of Miami One grant was Project: The role of FAM65B in the regulation of postawarded for innovative translational modifications of auditory hair cell proteins research focused Goal: To better understand the function of the FAM65B protein, on congenital and an important molecule for hearing, in order to delineate the acquired childhood molecular mechanisms that underlie hair cell development, hearing loss and its etiology, function, and maintenance, and ultimately discover novel strategies for the assessment, diagnosis, and protection and restoration of hearing. treatment. This grant was supported by The Children’s Hearing Institute.
One grant was awarded for innovative research that will increase our understanding of strial atrophy and/or development of the stria. This grant was funded by a generous family foundation with an interest in funding strial atrophy research.
Southern Illinois University School of Medicine Project: Cisplatin-induced oxidative stress down-regulates strial Na+/K+-ATPase and endocochlear potential Goal: To better understand at the molecular and cellular levels how cisplatin, a common chemotherapy drug, harms hearing in the stria vascularis, an important tissue in the inner ear, and to investigate the potential of epigallocatechin gallate (EGCG), a green tea extract that is an antioxidant with anti-inflammatory properties, in the treatment of cisplatin-induced hearing loss.
For more details about these research projects, see hhf.org/erg-2017-grantees. hearinghealthmag.com
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Recent Research by Hearing Health Foundation Scientists, Explained
Central Auditory System Injuries After Mild Blast Explosions blast explosions are a major reason why service members experience hearing loss and tinnitus. While it is well documented how loud sound damages the eardrum, middle ear, and inner ear, what is less understood is how blast explosions, even relatively mild ones, affect hearing. In addition to creating a loud noise, blasts produce a shock wave, a pressure wave faster than the speed of sound. In an August 2017 paper in the Journal of Neurophysiology, Edward Bartlett, Ph.D., a 2009 and 2011 Emerging Research Grants (ERG) recipient, and colleagues at Purdue University used a rat model to compare the effects of a loud noise with a blast shock wave to that of a loud noise only, without a blast shock wave. The researchers found that a single blast exposure with a shock wave produced changes in the central auditory system for at least four weeks. Noise exposure alone had shorter-term effects. The changes to the central auditory system may account for the behavioral issues that veterans experience, such as problems with memory, learning, communication, and emotional regulation. As a result, it is possible that people exposed to blast events are predisposed to developing anxiety, depression, or neurodegenerative conditions such as Alzheimer’s or Parkinson’s diseases later in life. In addition, the specific brain deficits they found showed there may be problems with temporal processing, which can contribute to difficulty understanding speech. Notably, Bartlett and the team were able to measure central auditory aberrations through noninvasive hearing tests already in use, such as otoacoustic emissions and auditory brainstem responses. They allow for the diagnosis of central auditory damage when there are few or no outward signs of blast or hearing injury, especially after a mild blast exposure. The hope is that these portable, easy-to-administer diagnostic tests will aid with evaluating service members exposed to blasts during training or out on the field in order to implement earlier treatments if needed. —Yishane Lee, editor
A 2009 and 2011 ERG scientist, Edward Bartlett, Ph.D., is an associate professor in the department of biological sciences and the Weldon School of Biomedical Engineering, Purdue University, Indiana. Read more about Bartlett in Summer 2012’s “Meet the Researcher” at hhf.org.
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A single blast exposure with a shock wave produced changes in the central auditory system for at least four weeks. The changes to the central auditory system may account for the behavioral issues that veterans experience, such as problems with memory, learning, communication, and emotional regulation, which may lead to later anxiety, depression, or neurodegenerative conditions.
research
EMERGING RESEARCH GRANTS
Recent accomplishments by ERG recipients underscore the importance of supporting early-career scientists who bring fresh approaches to hearing and balance studies.
New Study Suggests Serotonin May Worsen Tinnitus millions of people suffer from the constant sensation of ringing or buzzing in the ears known as tinnitus, creating constant irritation for some and severe anxiety for others. New research by scientists at Oregon Health & Science University (OHSU) and funded in part by Hearing Health Foundation shows why a common antidepressant medication may actually worsen the condition. The study, published in August 2017 in the journal Cell Reports, focused on the action of serotonin, an important neuromodulator in the brain. Researchers examined brain tissue in mice, specifically the dorsal cochlear nucleus where sensory integration occurs and tinnitus is believed to develop. Researchers discovered that neurons known as fusiform cells within this portion of the brain become hyperactive and hypersensitive to stimuli when exposed to serotonin. “We saw that the activity of those neurons went through the roof,” says senior author Laurence Trussell, Ph.D., who is a professor of otolaryngology in the OHSU School of Medicine, a scientist in the Vollum Institute, and on the faculty at OHSU’s Oregon Hearing Research Center. The study could have implications for a common class of antidepressants known as selective serotonin reuptake inhibitors. SSRIs can alleviate symptoms of moderate to severe depression and anxiety by increasing the level of serotonin in the brain. Serotonin is a chemical compound that acts as a neurotransmitter thought to be responsible for maintaining mood balance. However, the new research suggests that SSRIs prescribed to treat anxiety or depression may worsen patients’ tinnitus. Tinnitus is defined as the chronic perception of sound when there is no internal or external acoustic source. “Estimates vary, but at least 10 percent of the U.S. population is affected by tinnitus,” says lead author Zhengquan Tang, Ph.D., a 2013 and 2014 Emerging Research Grants (ERG) researcher and a senior postdoctoral fellow in Trussell’s lab. Tang noted that a review of existing scientific literature indicated that many patients reported an increase in tinnitus soon after they began taking SSRIs.
New research suggests that selective serotonin reuptake inhibitors (SSRIs) prescribed to treat anxiety or depression may worsen patients’ tinnitus. “If you’re a physician treating a patient with hearing loss or tinnitus, you may want to be careful about prescribing a drug that compounds their feelings of anxiety,” says Trussell, who suffers from tinnitus himself. “The SSRI may be enhancing the thing you’re trying to fix.” The OHSU scientists are interested in exploring another area of research focused on a type of ion channel in the membrane of neurons that is activated by serotonin. If the scientists can determine a way to deactivate those channels, they may be able to allow the beneficial effects of antidepressants while limiting the severity of tinnitus. —Erik Robinson, OHSU
Reprinted with permission from Oregon Health & Science University’s communications office. A 2013 and 2014 ERG scientist, Zhengquan Tang, Ph.D., is a postdoctoral fellow at OHSU’s Vollum Institute in the Trussell Lab. For references, see hhf.org/ fall2017-references.
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EMERGING RESEARCH GRANTS
An Animal Behavioral Model of Loudness Hyperacusis one of the defining features of hyperacusis is reduced sound level tolerance. Everyday sound volumes are experienced as uncomfortably loud and potentially painful. 2015 Emerging Research Grants (ERG) scientist Kelly Radziwon, Ph.D., Richard Salvi, Ph.D., and colleagues at the State University of New York at Buffalo have developed a rat behavioral model of loudness estimation utilizing a reaction time paradigm. Rats were trained to remove their noses from a hole whenever a sound was heard. The team then measured reaction time, or how fast the animal responds to a variety of sounds of varying intensities. The more intense a sound is, the faster a listener will respond to it, so the researchers theorized that having hyperacusis would influence reaction time due to an enhanced sensitivity to sound. High-dose sodium salicylate, a nonsteroidal anti-inflammatory drug (NSAID), has long been known to induce temporary hearing loss and acute tinnitus in both humans and animals. If the rats’ responses to sound are faster than they typically were following salicylate administration, then the team theorized it will have developed a relevant animal model of loudness hyperacusis. They also wanted to determine how the dosage of salicylate as well as the frequency of the tone bursts affected reaction time. As reported in the July 2017 issue of Hearing Research, Radziwon and colleagues found that salicylate caused a reduction in behavioral reaction time in a dose-dependent manner and across a range of stimulus frequencies, suggesting that both this behavioral paradigm and the salicylate model are useful tools in the broader study of hyperacusis. Although people with hyperacusis rarely attribute their hyperacusis to salicylate, the use of the salicylate model of hyperacusis in animals provides the necessary groundwork for future studies of noise-induced hyperacusis and loudness intolerance. —Kelly Radziwon, Ph.D., and Richard Salvi, Ph.D.
These two charts show the reaction time-intensity functions for broadband noise bursts for seven rats. The rats are significantly faster following high-dose (300 mg/kg) salicylate administration (top panel; red squares) for moderate- and highintensity sounds, indicative of temporary loudness hyperacusis. The rats showed no behavioral effect following low-dose (50 mg/kg) salicylate (bottom panel).
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A 2015 ERG scientist, Kelly Radziwon, Ph.D., is a research assistant professor in the department of communicative disorders and sciences at the State University of New York at Buffalo. Her ERG grant was funded by Hyperacusis Research Ltd. Read more about Radziwon in Spring 2016’s “Meet the Researcher,” at hhf.org.
research
New Clues to Sound Localization Issues in Fragile X Syndrome fragile x syndrome (fxs), the most common genetic form of autism, is characterized by impaired cognition, hyperactivity, seizures, attention deficits, and hypersensitivity to sensory stimuli, specifically auditory stimuli. Individuals with FXS also experience difficulty with determining the source of a sound, known as sound localization. Sound localization is essential for listening in the presence of background noise such as a noisy classroom. The ability to localize sound properly is due to precise excitatory and inhibitory inputs to areas of the brain. 2016 Emerging Research Grants (ERG) recipient Elizabeth McCullagh, Ph.D., and colleagues hypothesize that the auditory symptoms seen in FXS, specifically issues with sound localization, are due to an overall imbalance of excitatory and inhibitory synaptic input in these brain areas. Investigators looked at the number and size of synaptic structures in different areas of the brainstem responsible for sound localization for several inhibitory neurotransmitters (glycine and GABA) and the primary excitatory neurotransmitter (glutamate) using a mouse model of FXS compared with a control group. The areas of the brainstem responsible for sound localization are connected to one another in a frequency-specific manner, with low frequency sounds stimulating similar areas and the same for high frequency. It was found that most areas of the brainstem examined did not have changes in number or size of structures, but one area—the medial nucleus of the trapezoid body (MNTB)—had alterations to inhibitory inputs that were specific to the frequency encoded by that region. Glycinergic inhibition was decreased in the high frequency region of MNTB, while GABAergic inhibition was decreased in the low frequency region. The August 2017 study in The Journal of Comparative Neurology is the first to explore alterations in glycinergic inhibition in the auditory brainstem of FXS mice. Due to the well-characterized functional roles of excitatory and inhibitory neurotransmitters in the auditory brainstem, the sound localization pathway is an ideal circuit to measure the sensory alterations of FXS. The study points the way to a better understanding of the altered sound processing found in those with FXS. —Pranav Parikh, Kathleen Wallace, and Elizabeth McCullagh, Ph.D.
A 2016 ERG scientist and General Grand Chapter Royal Arch Masons International award recipient, Elizabeth McCullagh, Ph.D., is a postdoctoral fellow at the University of Colorado Denver, Anschutz Medical Campus. Read more about McCullagh in Fall 2016’s “Meet the Researcher,” at hhf.org.
Individuals with FXS also experience difficulty with determining the source of a sound, known as sound localization. Sound localization is essential for listening in the presence of background noise such as a noisy classroom. EMERGING RESEARCH GRANTS
Support our research: hhf.org/donate
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EMERGING RESEARCH GRANTS
Shown in this 3-D model, the histidyltRNA synthetase (HARS) enzyme is instrumental in protein synthesis and thought to be involved in the presentation of Usher syndrome type 3B, one type of an inherited disease leading to hearing and vision loss.
A New Clue to Usher Syndrome Type 3B usher syndrome is an inherited condition that affects both hearing and vision. The syndrome is autosomal recessive, meaning children of parents carrying the gene but who do not display symptoms have a likelihood of developing the disease. There are three clinical types, with types 1 and 2 most common. In type 3A, representing 2 percent of Usher cases, a child is born with typical hearing and vision but develops a progressive loss of both senses during the first two decades of life. In type 3B, patients also experience hallucinations and fluid buildups in the lungs triggered by fever-causing illnesses. There are fewer than 20 known cases of type 3B, all in Amish populations. Susan M. Robey-Bond, Ph.D., a 2012 Emerging Research Grants (ERG) scientist, and colleagues were able to isolate a mechanism involved in the development of Usher syndrome type 3B that is related to temperature sensitivity. They studied histidyl-tRNA synthetase (HARS), an enzyme that is instrumental in protein synthesis and involved in the presentation of type 3B in patients. It had been known that the USH3B genetic mutation encodes a Y454S substitute in HARS in which a serine amino acid is substituted for a tyrosine amino acid. Robey-Bond and colleagues studied the biochemical properties of the USH3B gene to compare the Y454S mutation in the HARS enzyme with its wild-type (non-mutated) form. According to the team’s paper published in June 2017 in the journal Biochemistry, key functions (amino acid activation, aminoacylation, and tRNA binding) were all consistent between the mutated and wild-type genes. However, the researchers found that at an elevated temperature the Y454S substitution was less stable than the wild-type. More specifically, cells from patients containing the Y454S mutation displayed lower levels of protein synthesis, which ultimately could help explain the onset of deafness that these patients experience and possibly their sensitivity to fevers. The paper concludes, “The thermal sensitivity associated with the Y454S mutation represents a biochemical basis for understanding USH3B,” and while the type 3B population is small, it provides a window into the genetic underpinnings for Usher syndrome generally. —Pranav Parikh
A 2012 ERG scientist, Susan M. Robey-Bond, Ph.D., was in the department of biochemistry at the University of Vermont Larner College of Medicine and is now at Illumina, a gene-sequencing research firm.
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research
How Genes Interact to Produce Hair Cells
photo credit: jane g photography (edge)
sensory hair cells are located in the snail-shaped inner ear (cochlea) and convey sound waves to the auditory nerve and from there to the brain. In mammals when these hair cells are damaged or die, they cannot renew and the hearing loss is permanent. Recent studies in auditory research have discovered “sleeping” progenitor cells. Progenitor cells are cells that have not fully differentiated (turned into) into a final cell type, and still show some characteristics of a stem cell, meaning they may still be able to turn into various cell types, such as the different cells of the inner ear. This exciting finding suggested that hearing may be able to be restored by stimulating the remaining “sleeping” cells in the inner ear to replace the damaged hair cells. In order to do so, the steps that lead from a progenitor cell to a final hair cell need to be investigated to identify the trigger for differentiation. 2011 and 2012 Emerging Research Grants (ERG) scientist Judith Kempfle, M.D., and Albert Edge, Ph.D., a Hearing Restoration Project (HRP) consortium member, and colleagues aimed to better understand these steps of hair cell development. They found that Sox2, a progenitor gene, is critical for the development of hair cells from progenitor cells. Published in Scientific Reports in March 2016, the research by the Massachusetts Eye and Ear/Harvard Medical School team showed that Sox2 directly activates the key gene factor for hair cell formation, Atoh1. By describing a direct interaction between Sox2 and the specific activation site for Atoh1, the scientists were able to demonstrate that Sox2 has an active role in the formation of hair cells in the cochlea. It suggests that the right amount of Sox2 is critical in the developing progenitor cells in order for them to become hair cells. In fact, using a model system in which Sox2 was removed from inner ear cells during development, they found the development of inner ear hair cells was halted at an early stage—implying that Sox2 is crucial for the entire process. This groundbreaking discovery has led to an advancement in the understanding of how hair cells are formed from progenitor cells and, specifically, that Sox2 is important for triggering the final differentiation into a hair cell. The knowledge gained from this discovery may be applied in the future to restore hearing from other cell types remaining in the ear after damage. —Pranav Parikh and Judith Kempfle, M.D.
This research conducted at Massachusetts Eye and Ear/Harvard Medical School’s Tillotson Cell Biology Unit was funded by Hearing Health Foundation through its two programs, Emerging Research Grants (ERG) and the Hearing Restoration Project (HRP). Judith Kempfle, M.D., is a 2011 and 2012 ERG scientist supported by the General Grand Chapter Royal Arch Masons International, and Albert Edge, Ph.D., is a member of the HRP. Read more about Kempfle in Fall 2011’s “Meet the Researcher,” at hhf.org.
Hearing may be able to be restored by stimulating “sleeping” cells in the inner ear to replace damaged hair cells. This study provides details about how these cells are transformed into final hair cells. EMERGING RESEARCH GRANTS
Support our research: hhf.org/donate
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Therapies for Hearing Loss: What Is Being Tested?
Hearing Restoration Project consortium member Ronna Hertzano, M.D., Ph.D. (far left), is an assistant professor at the University of Maryland School of Medicine. HHF Council of Scientific Trustees member Debara L. Tucci, M.D., is a professor at Duke University Medical Center in North Carolina. 42
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The team identified 22 active clinical drug trials in the U.S., and reviewed six potential therapies for the treatment of sensorineural hearing loss.
Past HHF intern Pranav Parikh, from New Jersey, is a sophomore at Boston College, where he studies biochemistry and philosophy. Staff writer Kathleen Wallace is pursuing a doctor of audiology degree (Au.D.) at the City University of New York's Graduate Center. For references, see hhf.org/fall2017-references.
Support our research: hhf.org/donate
photo credit: jane g photography (hertzano)
untreated hearing loss is linked to a lower quality of life, physical functionality, and communicative ability. The most common type of hearing loss, sensorineural, is often a result of damage to the delicate sensory hair cells in the inner ear. Because hair cell loss is irreversible, and hearing impairment therefore permanent, new treatment strategies are a welcome sign. In the July 2017 issue of Otology & Neurotology, Hearing Restoration Project consortium member Ronna Hertzano, M.D., Ph.D., and Debara L. Tucci, M.D., a member of Hearing Health Foundation’s Council of Scientific Trustees, along with Matthew Gordon Crowson, M.D., examined the field of emerging therapies for sensorineural hearing loss. The team identified 22 active clinical drug trials in the U.S., and reviewed six potential therapies. Four use mechanisms to reduce oxidative stress believed to be involved in the inner ear cell death. Three of the therapeutic molecules being tested—D-methionine, N-acetylcysteine (NAC), and glutathione peroxidase mimicry (ebselen)—act as antioxidants to mop up free radicals caused by noise or other trauma to the inner ear. (For more about D-methionine, see page 11.) The fourth, sodium thiosulfate, is a chemical found to counteract the ototoxic effects of chemotherapy drugs. The fifth approach is to manipulate the “cell death cascade.” This occurs when cells endure significant stress or injury, leading to the release of free radicals and changes in pH and protein that then kill the cell. Since hair cells do not regenerate like other cells, the cell death cascade causes permanent hearing loss. A trial is underway to make the cochlear neuroepithelium (inner ear tissue) more resilient to cell death signaling, using an inhibitor called AM-111 to block the chain of events leading to cell death. Finally, the sixth approach is a novel hair cell replacement therapy using the gene Atoh1, known to be a vital regulator of hair cell regeneration, causing cells to differentiate (change) into hair cells. Using mouse models, it has been shown that if Atoh1 is blocked, hair cell differentiation does not occur, and if it is induced, hair cell formation occurs, at least in the ears of very young mice. Drug delivery methods to the inner ear are also being investigated. In addition to orally, delivery methods include a topical ear gel, intravenous infusion, and, most revolutionarily, direct injection of viruses to deliver genes to the inner ear. And while many of the drugs had to overcome hurdles to reach late-phase clinical trials, questions about safety, efficacy, and side effects remain, in addition to whether animal model results translate to human biology. —Pranav Parikh
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EMERGING RESEARCH GRANTS Can a chicken cure hearing loss and tinnitus? The answer, incredibly, is YES! Learn more at Hearing Health Foundation’s website.
As one of the only funding sources available to early-stage researchers, Hearing Health Foundation’s ERG program is critical. Without our support, these scientists would not have the needed resources for researching hearing and balance disorders.
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EMERGING RESEARCH GRANTS
Meet the Researcher
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.
Timothy Balmer, Ph.D.
Oregon Health & Science University
Balmer received his Ph.D. in neuroscience from Georgia State University and is now a postdoctoral fellow at Oregon Hearing Research Center at Oregon Health & Science University. A 2017 Emerging Research Grants scientist, Balmer received the Les Paul Foundation Award for Tinnitus Research.
In His Words tinnitus may be caused by persistent cellular activity that leads to the false perception of a sound. My work focuses on understanding a type of neuron (nerve cell) in the auditory system that may underlie this activity. Neurons send signals through neurotransmitters that travel across synapses, from a neuron’s axons to another’s dendrites. the cell type I am focusing on has a unique, single paintbrush-like dendrite. This “unipolar brush cell” is found in the cochlear nucleus, the part of the brain linked by the auditory nerve to the inner ear. The dendritic brush of these cells slows the neurotransmitters from leaving the synapse— they linger at these synapses. Using physiological recording techniques, we found that this leads to persistent excitatory activity, and we reasoned this may lead to tinnitus. Unipolar brush cells have only recently been defined as a distinct cell type—most cell types were defined over 100 years ago. while studying psychology as an undergrad, I became interested in the fundamental biological mechanisms that underlie sensation, perception, and thought. My first lab experience was to study odor sensation in bumblebees. I felt at home in a lab, where I could explore how the brain works by looking at its component parts. from an early age I understood that taking things apart and putting them back together is an effective way to learn how they work. In high school I spent a lot of time rebuilding and modifying cars and engines, giving me experience in design and engineering that I use as a researcher. Troubleshooting an engine that won’t start is an implicit lesson in problem solving and the use of the scientific method. It starts with an observation—“the engine won’t 50
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start”—which leads to a hypothesis about the cause—“there is no spark at the spark plugs”—and testable predictions—“if I can make them spark, the engine will start.” i have tinnitus myself. It is impossible to be certain what caused it, but my guess is that frequent use of power tools without hearing protection may have caused damage. i also repair and modify mechanical watch movements, focusing on those worn by U.S. service members, including a Rolex that kept time on the wrist of a close friend during the Korean War, and an Elgin worn by a pilot in World War II. it is an honor to be funded by the Les Paul Foundation. My uncle is a jazz guitarist here in Portland, and I grew up around guitars—including my uncle’s Les Paul guitar.
Timothy Balmer, Ph.D., received the Les Paul Foundation Award for Tinnitus Research. We thank the Les Paul Foundation for its support of innovative research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus.
We need your help in 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.
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