Hearing Health Summer 2019

Summer 2019 A Publication of Hearing Health Foundation hhf.org

The Genetics Issue Seeking a better understanding of shared hearing conditions, from families to populations

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letter from the chair

DEAR READERS & SUPPORTERS, i am thrilled to introduce to you our new CEO, Timothy Higdon, who brings three decades of leadership and fundraising experience to Hearing Health Foundation (HHF).

A veteran of the U.S. Army Corps of Engineers, Higdon took the helm at the end of May (see page 9). With former U.S. Army Col. John Dillard becoming the chair of HHF’s Board of Directors in July, we have two veterans heading our organization, fortuitous not only for HHF but also for underscoring the connection between hearing conditions and those who serve.

Higdon has certainly hit the ground running. On his second day at HHF, he joined marketing and communications director Lauren McGrath on a trip to Washington, D.C., to meet with the Friends of the Congressional Hearing Health Caucus to discuss hearing healthcare advocacy, and then a few weeks later he and McGrath traveled to Rochester, New York, for the 40th Hearing Loss Association of America (HLAA) convention in June.

The HLAA convention featured a research symposium on the topic of genetics, which is also the theme of this Summer 2019 issue of Hearing Health. Several of our Emerging Research Grants (ERG) alumni spoke at the symposium, including 1994–95

ERG scientist Thomas Friedman, Ph.D., the chief of the Laboratory of Molecular Genetics at the National Institute on Deafness and Other Communication Disorders, who introduced the panel discussion; Zheng-Yi Chen, Ph.D., a 1994 ERG scientist with Massachusetts Eye and Ear; and Patricia White, Ph.D., a 1999 and 2001 ERG grantee who is at the University of Rochester School of Medicine.

As always we are so gratified to see scientists whom we have funded continuing to make significant discoveries in hearing and balance science. Some of these notable findings are detailed in “Decoding Genes” (page 20), and in our recent research section summarizing newly published papers by HHF scientists (page 42). We are also pleased to highlight this coming year’s Hearing Restoration Project plans (page 40).

Finally, we are always happy to share with you personal stories from our community. This issue includes families coming to terms with generations of hearing loss, starting with “Family Ties” on page 10 and, on page 14, a trio of stories from individuals meeting the challenge of Usher syndrome, the leading genetic cause of combined vision and hearing loss. On page 36 one of our interns, who grew up with a hearing loss in a

HHF’s new CEO Timothy Higdon (left), and the incoming chair of HHF’s board, John Dillard. country with limited resources, shares how that experience combined with working at HHF inspired her thesis project on pediatric hearing loss.

We hope you enjoy this issue, and your summer! Thank you for your support.

Elizabeth (Betsy) Keithley, Ph.D. Outgoing Chair, HHF Board of Directors

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HEARING HEALTH The Genetics Issue

Publisher Timothy

Summer 2019, Volume 35, Number 3

As science uncovers genes related to hearing, understanding how they function—on their own and together—will inform future therapies for preventing and treating hearing loss.

Editor Yishane

Lee

Art Director Robin Senior Editors

Higdon, CEO Kidder

Amy Gross, Lauren McGrath

Medical Director David

S. Haynes, M.D.

Staff Writers

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

Features 10 Family Voices Family Ties.

Ava Finnerty 14 Living With Hearing Loss Flying My Way. Ryan Vlazny.

It Takes Courage to Be Vulnerable. Trent Westrick, Au.D. Bodybuilding Against All Odds.

Mersal Faizi 20 Roundup Decoding Genes.

24 Living With Hearing Loss My Ears Had Whiskers.

Ruth D. Bernstein 25 Research A Symposium to Sound Out Hearing Restoration.

Marie Samanovic-Golden, Ph.D. 28 Family Voices Seeing the Signs.

Loraine Alderman, Psy.D. 30 Managing Hearing Loss My First 48 Hours With Hearing Aids. Adam Felman

Departments 03 Letter From the Chair

33 Managing Hearing Loss How to Communicate Better, and More Compassionately, With People With Hearing Loss.

Mary Florentine, Ph.D., Julia B. Florentine, and Michael J. Epstein, Ph.D. 36 Hearing Health Visualizing My Childhood Hearing Loss.

Marjorie Saavedra 38 F amily Voices With Cautious Gratitude. Lauren McGrath 40 Research Hearing Restoration Project Plans Announced for 2019–20.

Peter G. Barr-Gillespie, Ph.D. 42 Research Recent Research

by Hearing Health Foundation Scientists, Explained.

50 Meet the Researcher Babak Vazifehkhahghaffari, Ph.D.

06 @editor

Sponsored

09 HHF News

48 Advertisement Tech Solutions.

26 Meet the Fundraisers The Brueggemans

49 Marketplace

Advertising

advertising@hhf.org, 212.257.6140 Editorial Committee

Peter G. Barr-Gillespie, Ph.D. Robert A. Dobie, M.D. Judy R. Dubno, Ph.D. Dennis Giza Anil K. Lalwani, M.D. Rebecca M. Lewis, Au.D., Ph.D., CCC-A Joscelyn R.K. Martin, Au.D. Board of Directors

Chair: Col. John T. Dillard, U.S. Army (Ret.) Sophia Boccard Robert Boucai Judy R. Dubno, Ph.D. Ruth Anne Eatock, Ph.D. Jason Frank Roger M. Harris David S. Haynes, M.D. Elizabeth Keithley, Ph.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 2019, Hearing Health Foundation. All rights reserved. Articles may not be reproduced without written permission from Hearing Health Foundation. USPS/Automatable Poly To learn more or to subscribe or unsubscribe, call 212.257.6140 (TTY: 888.435.6104) or email info@hhf.org.

Cover Ava Finnerty as an infant with her brother Charles and their grandmother Bessie, on her first trip to the U.S. from Wales in 1949.

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

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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 accuracy of information therein. See hhf.org/ad-policy.

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letters to the editor

hearing health foundatio n

@editor

dear editor: Kudos to Lisa Giles, who wrote “What People With Hearing Loss Want You to Know” in the Spring 2019 issue of Hearing Health. I could have been the writer of that article. Lisa said it all for me.

I miss so much in my Bible study group because, for some reason, people think they should pray to or speak about God in hushed tones with head bowed! In addition, I have a distant family member (who is blessed with excellent hearing) who will repeat something only once and then roll her eyes and say, “Oh, never mind!” leaving me in the dark and feeling reprimanded! Sound familiar?

M. Long

dear editor: I wanted to let you know that I enjoyed the article by Lisa Giles in the Spring 2019 issue. Living with a severe hearing loss is not fun. People just don’t understand.

I have received Hearing Health magazine now for some time. It’s great that people can subscribe for free!

I used to enjoy going to meetings in a nearby town for people with hearing loss, but they stopped having them there and the next nearest town that has them is 50 miles away. I wish someone would start having the meetings again nearer to where I live.

Dawn Woodward, Michigan from the editor: We were happy to see that Lisa Giles’s candid article about living with hearing loss received a lot of positive feedback, including requests for reprints for use by N-CHATT (Network of Consumer Hearing Assistive Technology Trainers) in Illinois and for the administration and nursing staff at a Florida continuing care retirement community. We share additional communication tips on page 33.

dear editor: I am a 60-year-old man who was diagnosed three years ago with moderate

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hearing loss in the midrange of the auditory spectrum. I always like reading your magazine whenever I visit my audiologist and I especially liked the Fall 2018 “Military and Seniors” issue.

However I must point out an error regarding the photo on page 7, which shows the author—Bruce Douglas, D.D.S.—firing from the prone position behind what the article claims is an M1 (i.e., a World War II Garrand) rifle. The rifle in the photo is in reality a version of the BAR (Browning Automatic Rifle), which fires the same round as the Garrand, but unlike the M1, is capable of being fired in fully automatic mode.

Other than that minor detail, I very much enjoyed the issue and especially the articles regarding medical information and breakthroughs in hearing assistance technology. Keep up the good work.

Greg Mactye

dear editor: I’m not a service member but I am a recipient of two cochlear implants. I love getting Hearing Health magazine since the information and articles are so very vital, important, and many times so inspiring.

The “Tackling Tinnitus” and “Total Noise Exposure” articles from Fall 2018 were especially interesting to me since I have had tinnitus for about 60 years.

One thing I would like to mention is the use of acronyms and some words that needed special clarification throughout these articles.

Their constant and numerous use made it difficult to just read the articles and just get the information about what was being said instead of constantly trying to figure out or hunt back to find out again who said or did what, or developed something, or is in charge of this or that, or what a certain definition actually means.

I suggest you make an index of the acronyms, specially used, and uncommon words for the layman. This index could be at the end of the article or there could be one large index at the end of the magazine (for that issue).

This way people could look up quickly any

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letters to the editor

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words or acronyms instead of being thrown off, getting lost in the verbiage, giving up reading, or having to hunt for meanings throughout the article or in dictionaries.

With great appreciation for the work that you, your staff, and all your contributing writers of Hearing Health and Hearing Health Foundation do.

Gerry Chromoy, Florida from the editor: Thank you for the feedback as we completely understand how the acronyms can be overwhelming.

If we have another acronym-heavy issue we will definitely consider including a cheat sheet to keep them all straight!

dear editor: I read your blog post by staff writer Kathi Mestayer about asking for accommodations at a bookstore reading, “Making Friends and Influencing People,” and it resonated with me, as my daughter is profoundly deaf (onset in middle school).

When she graduated from Harvard in auditory neuroscience, the university provided interpreters at the ceremony, but sat my daughter and all of us next to an aisle where people were constantly walking in front of the interpreters, making it

impossible for my daughter to see their signing.

My daughter had told me how difficult it was advocating for accessibility, so I am keenly aware of the challenges that people with hearing loss experience on a daily basis.

Thank you to your writer for sharing her experience. Educating the general population is so very important, and having corporations and employers be educated and sensitized to the needs of people with hearing loss as well as other disabilities is critically important. Sometimes, I don’t think people are intentionally acting out of malice, but are just ignorant or unaware.

I think it is incumbent on people in a position to educate and advocate for people with hearing loss and other disabilities to do so in order to empower those who may just need a helping hand or some minor adjustments in the workplace to be useful, productive individuals who can reach their full potential to make a meaningful contribution to our society and our world. Thank you for all that you do.

Sheryl Gluck, LMSW

We always appreciate feedback from our community of readers. Letters are edited for length and clarity. Please email comments to editor@hhf.org.

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hhf news

NEWS

HHF

Timothy Higdon, HHF’s new CEO, with Lauren McGrath, HHF’s director of marketing and communications.

TIMOTHY HIGDON LEADS HEARING HEALTH FOUNDATION AS NEW CEO hearing health foundation (hhf), the largest nonprofit funder of hearing loss research in the U.S., announces the selection of Timothy Higdon as Chief Executive Officer.

Higdon, who assumed his new position on May 28, 2019, succeeds Interim CEO Margo Amgott.

Higdon’s appointment to CEO is the result of a thoughtful search process led by a search committee of clinicians, scientists, and other members of the HHF Board of Directors supported by Amgott.

“Timothy’s record of accomplishments and commitment to HHF’s mission position him perfectly to lead this robust organization—which I’m grateful to have come to know over the past six months,” Amgott says.

Higdon comes to HHF with 30 years of organizational leadership and fund development experience. He has led teams ranging in size from two people to 200 at prestigious nonprofit institutions, with impressive results.

Most recently, he served as the senior director of strategy and development at New York University (NYU) Langone Medical Center.

He has held executive positions with Amnesty International, Girl Scouts of the USA, and CSS Fundraising.

Previously, he served in the U.S. Army Corps

of Engineers for 18 years, where he attained the rank of major. Like so many other veterans, Higdon lives with noise-induced hearing loss.

“I am honored to join an organization dedicated to the prevention and cure of hearing loss through scientific research,” Higdon says.

“It is thrilling to join the HHF team and be a part of the solution for one of the nation’s most pressing public health concerns.”

An estimated 50 million Americans are affected by this hidden disability. HHF funded the discoveries that birds and fish, when deafened, can restore their own hearing, and now supports a consortium of scientists studying this process for replication in humans.

HHF also funds projects that investigate hearing loss variants, like tinnitus, Ménière's disease, hyperacusis, and auditory processing disorder.

Higdon comes to HHF with 30 years of organizational leadership and fund development experience. He has led teams ranging in size from two people to 200 at prestigious nonprofit institutions, with impressive results.

Higdon earned his master’s degree in public administration from NYU Wagner School of Public Service, and holds a bachelor’s degree in business finance from Indiana University.

“I have tremendous confidence in Timothy to shape the future of HHF,” says Elizabeth Keithley, Ph.D., the chair of HHF’s Board of Directors. “As government funding for hearing research remains inadequate, I am enthusiastic Timothy and the staff will generate the resources and oversee the processes so urgently needed to support our talented scientific grantees. I look forward to working with the HHF team to advance our vision for a world with better hearing loss treatments and cures.”

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Family Ties In a four-decade saga, a baby boomer with three generations of hearing loss in her family discovers the value of her two ears, and one lasting solution for her health.

By Ava Finnerty

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my mother sonia, born and raised in Wales, was the first person I knew with a hearing loss. She concealed it for many years. In my adolescence and young adulthood, I came to learn of her hearing loss, my grandmother’s, and, eventually, my own.

During World War II, my mother served in the Women’s Air Corps in Britain, where it was her duty to (wo)man the barrage balloons on the White Cliffs of Dover. It was there she met and married my father, John Jessen, a U.S. Army Sergeant preparing for D-Day. During the war she gave birth to my oldest brother, and then they both emigrated to the U.S. in 1946 to reunite with my father.

My parents moved to a veterans housing project in Bayonne, New Jersey, to raise our family. My mother was a very private person who largely refrained from sharing her medical issues with my two brothers, my sister, and me. I have a vague memory of her having some kind of ear surgery in the early 1950s, when I was 5 or 6 years old,

family voices

I could tell that my hearing loss was affecting my work. I was well into my career as a high school English teacher. At first, I attributed my inability to understand my students to their mumbling or mouth-covering. But, as the problem worsened, I knew it was me, not them. Only later did I learn my colleagues thought I was aloof because I would not acknowledge their greetings!

Opposite: Shown with extended family, Ava Finnerty (second from right) traces her roots to Wales through her mother Sonia.

Sonia, who married an American, had a hearing loss (top).

Finnerty calls this her “teacher’s blouse,” in this photo taken around 1980, after her stapedectomy (bottom).

but I did not receive an explanation.

Every time we went swimming, my mother plugged her left ear with a large wad of cotton and covered her head with a bathing cap. She told us she had a “hole” in her ear that needed to be protected from water. Incidentally, my mother helped tend bar at my father’s parents’ bar, The Viking, before becoming pregnant with me, but I later learned for certain her hearing loss was not caused by noise.

heavily on her vision, she was more cognizant of others’ facial expressions and body language than most with typical hearing.

At my wedding Grannie impressed me with her grace as a dancer, using the feelings of the bass and drums to move rhythmically. She was a strong and confident woman who’d grown resilient living as a mother and grandmother with a hearing loss during World War II.

A Family Inheritance

A Gradual Process

A strict parent, my mother believed “children should be seen and not heard,” so I thought she often remained silent in response to my questions on purpose, and not because she literally could not hear me.

It was only when I was a teenager that my mother told me the truth about her hearing. She had a severe hearing loss, but she did not treat it. Her small group of friends likely provided some support for her untreated condition.

My mother inherited her hearing loss from her mother, Bessie, who was profoundly deaf. Grannie still lived in the small Welsh village of Pontypool, where I visited her occasionally, first when I was 20, before my own hearing loss had been identified.

Grannie was a voracious writer—I suppose by necessity, because she did not wear hearing aids. She was keenly in touch with her surroundings, able to sense vibrations and read lips adeptly. Relying

My own difficulty hearing came on so gradually it was hard to notice. But I do remember vividly the day I realized the difference between my left and right ears. I was then a parent of three young children, living in Bayonne in a two-family house with my mother. I was cooking while cradling the phone between my right shoulder and right ear.

At one point in the conversation I switched the phone to my left ear and realized I could not hear what was being said. Despite this realization, I compensated for some time, relying on my “good” ear for conversation. It is truly amazing what a person can get accustomed to not having!

Around this time I could tell that my hearing loss was affecting my work. I was well into my career as a high school English teacher. At first, I attributed my inability to understand my students to their mumbling or mouth-covering. But, as the problem worsened, I knew it was me, not them. Only

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family voices

h earing health foundation

later did I learn my colleagues thought I was aloof because I would not acknowledge their greetings!

I developed a meaningful relationship with my mother, incidentally, during the onset of my own hearing loss. She and I cared for my father, helping him with home kidney dialysis every other day, and formed a very close bond. After his death, we spent many hours talking together, and I told her about the difficulty I had hearing my students.

Even though I knew of my mother and my grandmother’s hearing loss, I had concluded I had a buildup of earwax in my left ear. My husband Joseph, who was the chief echocardiography technologist for New York Hospital, was able to refer me to an audiologist at New York Weill Cornell Hospital.

There I learned I had almost no earwax buildup—but I did have a significant hearing loss. I was diagnosed with a 78 percent loss in my left ear and a loss of close to 30 percent in my right.

Successful Surgery

My hearing aids have for certain added to my quality of life. I am a music lover, play-goer, and movie fan. And had I not begun wearing them, I surely would have retired from my teaching career earlier than I wanted to.

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My left ear’s hearing loss was due to otosclerosis, an abnormal growth of bone in the middle ear. Otosclerosis is commonly thought to be inherited but its causes remain unclear. Scientists cite measles infections, stress fractures to tissue surrounding the inner ear, and immune disorders as possible causes. My doctor noted my otosclerosis was accelerated by my pregnancies, and research has since suggested this is possible.

I had a successful stapedectomy on my left ear, a surgical procedure that replaces the stapes bone with a prosthetic device so the bones in the middle ear can again vibrate in response to sound and hearing is restored. The procedure was minimally uncomfortable but did cause severe vertigo, which I was able to control with medication.

In the late 1980s, my mother finally chose to pursue hearing aids but wore them rarely because they emitted a very high-pitched sound. Later in her life, she stopped wearing them completely. Since we shared the two-family home, my family and I always knew what Grandma was playing on her television or radio upstairs at maximum volume. And we lost count of the number of times she shouted “whadjasay?!” to my father.

Mom became increasingly withdrawn. She never wanted to go out on dinner dates or socialize with friends. Only in recent years, after her passing, have I come to understand this preference for isolation.

Over the decades that followed, the hearing in my right ear slowly diminished and I found it increasingly difficult to manage at social events. I wanted to undergo a second stapedectomy, but the audiologist told me this wasn’t recommended.

I was fitted for hearing aids instead. The audiogram showed a moderate hearing loss in my left ear and a severe loss in my right with difficulty hearing low frequencies in both. No wonder I could not hear

family voices

the deep-voiced young men speaking in class!

The audiologist asked if I wanted access to sounds at 180 or 360 degrees. I said 360 because I wanted to hear what my students were saying behind my back. I always told my students that although I wore hearing aids, they needed to speak clearly and be aware that I sometimes surprised myself by what I was able to hear. I specifically told my students to never say “never mind” if I asked them to repeat themselves or speak up, but to repeat and rephrase what they said.

Vigilant About Hearing Well

After retiring as a teacher, Finnerty was elected to her town’s Board of Education as a trustee (opposite and top).

Finnerty with her daughters, who are also teachers.

This was in 2011, when I was 62 years old, and I’ve vigilantly worn my hearing aids since. The devices have, for certain, added to my quality of life. They are not perfect, but I consider them an absolute necessity if I want to hear my grandchildren and other family members. I am a music lover, play-goer, and movie fan. And had I not begun wearing them, I surely would have retired from my teaching career earlier than I wanted to.

I supplement my hearing aids with simple requests and tools. I have no problem telling someone, “I don’t hear as well as I would like to.

Could you say that again?” I retired in 2014, after 42 years of teaching high school English, and then was elected to be a Bayonne Board of Education trustee in 2015. During our meetings I prefer to sit at or near the head of the table to read the lips of the person speaking.

I use closed captions at home watching television. When I babysit, I often go to my grandchildren’s bedroom doors to check on them because I am not sure if they are crying. I love baby monitors that not only light up but also have video for me to easily check.

Both my daughter and daughter-in-law are aware of the genetic predisposition for otosclerosis. In fact, my daughter thinks that her 16-year-old daughter may have some hearing loss. My advice to her was to pay attention—but also that there is a great distinction between “hearing” and “listening,” especially when it comes to adolescents!

Ava Finnerty lives with her husband Joseph in New Jersey. A retired English teacher, she serves on the Bayonne Board of Education as a trustee. Their adult children are Kristen, also an English teacher; Jill, a music teacher; and Sean, a U.S. Navy veteran who served in Iraq.

For references, see hhf.org/summer2019-references.

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

Support our research: hhf.org/donate a publication of hearing health foundation

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Flying My Way living with

USHER

A young man with Usher syndrome chases thrills on coasters and planes.

By Ryan Vlazny airplanes, and learning about their mechanisms, have always made me feel alive. My longtime fascination with all things aerospace inspired my desire to work with computers for a living. But, at times, my hearing and vision loss made for bumpy rides.

I was born profoundly deaf and later diagnosed with Usher syndrome­—which combines deafness, retinitis pigmentosa (progressive vision loss), and problems with balance­—at 8 years old. Usher has many subtypes; I have type 1F. My brother, younger than me by 15 months, has typical hearing and vision. Todd learned sign language before he could speak.

The first word for both of us was “more.”

My parents have typical hearing but became heavily involved in the Deaf community after my diagnosis of hearing loss at age 10 months.

After fitting me with hearing aids, learning sign language, and investigating all their options, they decided I’d learn Signing Exact English (SEE)­—a manual communication system that, unlike ASL, matches English language and 14

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vocabulary­—in place of spoken language.

By the time I was in the 8th grade, I was fully immersed in mainstream classes, thanks to my parents’ commitment to my language development, and had undergone cochlear implantation. While I cannot understand spoken language with my cochlear implant (CI), it allows me to hear laughter, birds, music, and the roar of a roller coaster.

Lucky for me, Usher lets me enjoy roller coaster rides with a perspective different than people with typical hearing and vision. I can more acutely feel the car’s ascent up the hill, the hang time at the top, the speed on the drops, the toggling back and forth on the track, and all the loops and twists in between. These sensations are most fun when I ride an inverted coaster­— like my first “serious” ride in Oslo, Norway­—with the track above me and my feet hanging in the air. I feel like I am flying.

A few years after my CI surgery, actual flying replaced my passion for roller coasters. For my 17th birthday, I had the thrill of riding in a Pitts aerobatic airplane at the airport in Pompano Beach, Florida. The 20-minute charter ride felt like being on a roller coaster ride with 4,000-foot drops above the Everglades. The pilot did tricks that felt similar to vertical loops on a coaster.

Since 2010, I started planning trips to fly

Ryan Vlazny enjoying aerobatic airplanes (above and opposite, top left).

Vlazny, shown with his brother Todd, loves riding roller coasters (opposite, top right).

Vlazny’s first exposure to carnival rides was a carousel at the county fair (opposite, center).

Vlazny wrote this essay in 5th grade (opposite, bottom).

living with hearing loss

on specific types of airplanes. The city doesn’t matter. The plane is the reason for the trip.

My mom and I took an (ordinary) airplane ride to Tallahassee when it was time for me to take the Florida Comprehensive Assessment Test (FCAT), a requirement to graduate high school in the state. There we spoke with government officials about making the test optional for students with hearing loss, and we were successful. Still, even though the requirement had been eliminated, after three tries I passed the FCAT. For the remainder of high school I continued on track, taking advantage of computer-related courses like web design and engineering. I was accepted to the pre-baccalaureate engineering program at the National Technical Institute for the Deaf at Rochester Institute of Technology (RIT), where I enrolled with a major in mechanical engineering concentrating in aerospace.

Some math classes, especially differential equations, were too difficult, and with the support of my adviser, I changed my major to information technology (IT). Unlike with engineering, I felt I was able to fully understand and apply the concepts of IT. As an IT student, I created a greeting card in Adobe Flash, a multimedia software program, about greeting a new student on my make-believe RIT World Airlines. The greeting card was even commended by then-university president William Destler, Ph.D., in a one-on-one meeting.

But few college experiences compare with my opportunity to build my own airplane game in an application development class. The game simulated landing a plane, which other students found fun to play.

Even though I wasn’t an aerospace student, I still got to enjoy some exciting plane rides at RIT. Today I work as a Java developer for a financial technology firm, where I couldn’t be happier, piloting a career I love.

Ryan W. Vlazny lives in Pennsylvania.

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

Support our research: hhf.org/donate a publication of hearing health foundation

summer 2019

15

It Takes Courage to Be Vulnerable

living with

For this audiologist, a hearing loss created an interest in the field, and then provided essential tools for understanding patients—and himself.

USHER

By Trent Westrick, Au.D. millions of people have read brené Brown, Ph.D.’s bestselling books and have viewed her inspiring motivational talks.

For those unfamiliar with Brown, she has spent her career researching courage, vulnerability, and empathy, among other complex topics. While the tenets she researches and discusses are applicable to many facets of everyday life, they are particularly relevant to me, an audiologist with a hearing loss.

When most people think of audiology, terms like courage, vulnerability, and empathy do not immediately come to mind.

Even some experienced audiologists do not regularly use these descriptors. But when more closely considering the relationship between a patient, their family, and the audiologist, courage, vulnerability, and empathy play a significant role.

My journey to becoming an audiologist began after I was diagnosed with sensorineural hearing loss at age 8. Since I was born before the advent of technology

Trent Westrick with his wife Tina in Zion National Park, Utah.

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used in newborn hearing screenings, my hearing loss was not identified until I was older. I believe my mother suspected I had a hearing loss based on my behavior and reliance on visual cues, and then my performance in school began to decline when we started having spelling tests. I also had chronic middle ear infections, so whenever my mom mentioned her concerns about my hearing to doctors, they would just discover another ear infection and assume that was the cause of my behavior. But the diagnosis of sensorineural hearing loss at age 8 ended up being completely unrelated to the middle ear infections.

The day I was fitted with my first hearing aids was memorable and overwhelming as I became aware of a world filled with new sounds. My mom still tells the story of how I walked out of the audiologist’s office and turned around to see whose footsteps I was hearing, not realizing they were my own. As exciting as wearing the hearing aids was, they made me feel vulnerable. I was not

living with hearing loss

While my hearing loss might not be the same degree and configuration as that of my patient, I have more than likely experienced some of their same emotions, including frustration, embarrassment, and vulnerability. My understanding of what patients are experiencing creates space for empathy. Empathy builds trust. ready to let others know I had a hearing loss. Wearing hearing aids at school took courage, with support from my parents and audiologist.

As I experienced the technological advancements in hearing aids firsthand, I became more interested in the possibility of a career in audiology. I was, however, acutely aware of a major obstacle. Talking about my own hearing loss was incredibly difficult. Even with my family, teachers, and especially my friends, even mentioning my hearing loss made me feel vulnerable.

I continued exploring a career in hearing healthcare in college, and quickly came to understand that if I felt uncomfortable talking about my hearing loss and its associated challenges, so too would my patients. As I started graduate school working toward my doctorate in audiology, I slowly overcame this reluctance to discuss my hearing loss.

While gaining clinical experience, I also earned a better understanding of the roles that empathy and vulnerability play in the patient-audiologist relationship.

Although I have become more comfortable talking about my hearing loss, there is still a sense of vulnerability when discussing my progressive vision loss, a result of having Usher syndrome.

Usher is the most common genetic cause of combined hearing and vision loss.

I started experiencing vision changes around age 14, such as difficulty seeing in low light in movie theaters or while walking in the woods at night. I began losing peripheral vision a few years later, which caused me to bump into things. I complained to my optometrist for several

years before I learned about Usher in an undergrad class and essentially self-diagnosed my condition. A few years later I had my first genetic workup and the diagnosis of Usher (type 2A) was confirmed.

That was in 2005. At about the same time, my younger sister was also diagnosed with a sensorineural hearing loss and with Usher. Our older brother is unaffected.

I initially had trouble processing the diagnosis, but after a few years I was fine with knowing I had Usher. That said, I kept it to myself because it was easier than talking about it. And now that my peripheral vision loss has continued to progress and has started interfering with daily life more noticeably, the time has come for me to tell people about it and stop hiding it. I still struggle with sharing the diagnosis, and accepting my worsening vision is also a work in progress. I feel some of the same vulnerabilities familiar from my hearing loss; likewise, I recognize that some of my patients are likely experiencing similar feelings of vulnerability.

I do not feel the need to discuss the details of my own hearing loss with every patient I see, but I am able to reflect on my own experiences in an effort to better understand how each patient is dealing with their own unique challenges related to hearing loss. While my hearing loss might not be the same degree and configuration as that of my patient, I have more than likely experienced some of their same emotions, including frustration, embarrassment, and vulnerability. My understanding of what patients are experiencing creates space for empathy.

Empathy builds trust. a publication of hearing health foundation

summer 2019

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living with hearing loss

hearing health foundati o n

Since I too struggle with disclosing my own hearing and vision issues, I understand the hesitancy patients experience when pursuing assistance for their hearing loss. I recognize the emotions patients feel when I encourage them to disclose their hearing loss to others and to advocate for their communication needs.

When a patient seeks the services of an audiologist, they are putting themselves in a vulnerable position. Not every audiologist has hearing loss, and having a hearing loss is not what makes an audiologist competent.

Audiologists, regardless of their own hearing status, are trained to give their patients the courage to succeed, to help patients advocate for themselves, and to empathize with patients as they describe the emotional and psychosocial challenges of having a hearing loss.

I share my story in this context to shed light on the roles courage, vulnerability, and empathy play in the patient-audiologist relationship. I advise that individuals with hearing loss seek and work with an audiologist who not only understands these complex emotions, but also creates space for their patients to discuss them. As Brené Brown says, it takes courage to be vulnerable.

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

Trent Westrick, Au.D., is an assistant professor and the externship coordinator at Pacific University’s School of Audiology, College of Health Professions, in Oregon.

Support our research: hhf.org/donate

living with

USHER

Bodybuilding Against All Odds

By Mersal Faizi meet elizabeth “elizzy” galvan, a 40-year-old professional bodybuilder from Fargo, North Dakota, who doesn’t fit the stereotype of someone in her chosen sport. She is deaf, lives with Usher syndrome, and has one arm—all so-called limitations that make her success exceptionally inspirational.

Galvan lost her right arm at age 3 in an accident with an old-fashioned washing machine. The same year, she developed a bilateral hearing loss as a result of an illness and was fitted with hearing aids.

Galvan’s parents chose to enroll her in North Dakota School for the Deaf at age 3, where she learned to communicate using American Sign Language (ASL). Decades later, this remains her preferred mode of communication. She also uses notes, speech-to-text, and body language when her conversational partner does not know ASL. 18

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The diagnosis was unexpected. “This explained why I would always bump into things and lose my balance,” Galvan says.

living with hearing loss

After just one bodybuilding session at the gym, Galvan felt energized and ready to improve herself mentally and physically.

“My conditions don’t limit me,” she says. “They motivate me to become stronger.”

During a routine vision screening test in school at age 16, Galvan was diagnosed with Usher syndrome, the most common genetic cause for progressive hearing and vision losses. Usher has three main types and many subtypes; Galvan has Usher type 3. The diagnosis was unexpected because Galvan does not have a known family connection to Usher syndrome. “This explained why I would always bump into things and lose my balance,” Galvan says.

Galvan’s father required she wear her hearing aids while she was in school. Though the devices improved her hearing, Galvan says she disliked them greatly because she found pride in her deafness and didn’t want to change who she was. At age 18, free to make her own decisions, Galvan stopped wearing her hearing aids.

As an adult, Galvan learned how to function comfortably with the help of a friend and Usher advocate. Her friend recommended she ask someone to tap her shoulder when there is a step in front of her, and to use hand gestures close to her face to signal to her that others are present. These methods became necessary when her vision began to worsen, and she was mistaken for being rude when accidentally bumping into people.

A major back surgery in 2015 left Galvan weakened. Through her own research, she discovered bodybuilding would help her regain strength. After just one bodybuilding session at the gym, Galvan felt energized and ready to improve herself mentally and physically. “My conditions don’t limit me,” she says. “They motivate me to become stronger.”

Galvan exercises independently in facilities that fit her needs. Before joining or using a new gym she’ll carefully evaluate it, assessing the equipment, lighting, and spaciousness to ensure it’s safe to work out to avoid further injuring herself.

Still fairly new to bodybuilding, Galvan has already won five awards in the sport. She has earned second and third place medals in competitions and received a trophy for inspiring others from the National Physique Committee, the largest amateur bodybuilding organization in the U.S. Galvan will participate in her next bodybuilding competition in October 2019.

Galvan considers her bodybuilding journey her way of showing people she is capable of defeating the odds.

“Disabilities don’t prevent us from doing anything,” she says. “They just make us do things differently.”

Former HHF marketing and communications intern Mersal Faizi studies corporate communications at Baruch College.

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

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2019

19

roundup

h ear i n g h ealth foundation

Decoding Genes

Research is bringing fresh insights to genetic causes of hearing loss, though there is still much to be puzzled out.

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hearing health

“The majority of hereditary hearing loss is through recessive inheritance where there is no family history of hearing loss, so consideration of a genetic cause may be overlooked…. Our work has shown that genetic mutations can have differential effects on the cochlea and the vestibular organs. Functional changes in hearing do not always predict functional changes in the vestibular system. Indeed, the inner ear vestibular system can be quite resilient to genetic mutations. And, our work has suggested that genetics plays a role in vestibular aging that is different from that in agerelated hearing loss.”

—Sherri Jones, Ph.D., the chair of the department of special education and communication disorders at the University of Nebraska-Lincoln, and a 1997 Emerging Research Grants (ERG) scientist, speaking on the potential for genetic testing for hearing loss to empower audiology practices in the February 2019 issue of The Hearing Journal.

“As far as we know, this is the first time a human mutant protein associated with hearing loss has been shown to be ‘escorted’ by the unconventional cellular secretion pathway. This mechanism may shed light on hhf.org

the process underlying hearing loss associated with other mutant membrane proteins….

Our report highlights the potential of artemisinin to mitigate both hearing and vision loss caused by clarin1 mutations. This could be a repurposable drug, with a safe profile, to treat Usher syndrome patients.” —2002 ERG scientist Kumar N. Alagramam, Ph.D., the lead author of a study showing the malaria drug artemisinin may help prevent a common form of genetic hearing loss, Usher syndrome type 3A, that was published in the Proceedings of the National Academy of Sciences on May 28, 2019. Alagramam is an associate professor at the Case Western Reserve University School of Medicine in Cleveland.

“A genetic diagnosis can help predict response to cochlear implantation. For example, patients with the GJB2 mutation show an excellent response to cochlear implants, while those with genes affecting the cochlear nerve itself showed worse postimplant performance. Knowing this before implantation can help set expectations of post-implant auditory function. [In addition,] certain mutations predispose a patient to acquired hearing loss when exposed to various environmental factors. For example, prescribing the

antibiotic Streptomycin to a patient with the m.A1555G mutation puts the patient at high risk for hearing loss.

We can prevent this type of hearing loss by screening for this mutation prior to giving these types of medications.

[Then] a practitioner prescribing these medications might be alerted by the patient’s electronic health records to warn of the patient’s susceptibility to drug-induced hearing loss.” —Xue Zhong Liu, M.D., Ph.D., FACS, and Jason Rudman, M.D., writing in the April 2019 Hearing Journal.

In August 2018, the National Institutes of Health (NIH) awarded $3 million to Liu, a 2000–03 ERG scientist, and his team to continue a research program applying genomics to hearing loss screening and treatment. A University of Miami School of Medicine professor, Liu has overseen the development of a genomic variant detection platform and phenotypic database of 3,000 DNA samples from people with nonsyndromic hearing loss.

Liu’s institution, noting his earlier $3 million NIH hearing loss research grant, says he is the most successfully funded genetic hearing loss researcher in the U.S.

“No one had shown that noise-induced hearing loss involved the unfolded protein response. It really opens up

roundup

“Identifying deafness genes provides a parts list, but doesn’t identify their function.

When you develop the parts list and start noticing the parts interact with one another in the cell and fit together into a machine, then you begin to understand what the function of that gene might be.”

—Thomas Friedman, Ph.D., the chief of the Laboratory of Molecular Genetics at the National Institute on Deafness and Other Communication Disorders (NIDCD) and a 1994–95 ERG scientist, speaking at a May 28, 2019, NIDCD presentation, “Human Hereditary Deafness Is Complex Yet Easy to Grasp.”

genes

By the Numbers

466 million

people are estimated to have hearing loss, according to the WHO.*

142

“deafness genes”

have been identified as of May 2019, according to the NIDCD.** Of these, 10 are disputed 3 have been refuted, resulting in:

129

gene variations

a lot of potential for identifying different therapeutic options and exploring how targeting the UPR could be effective for lots of kinds of hearing loss that we really don’t have any treatments for.” —Dylan Chan, M.D., Ph.D., an assistant professor of otolaryngology at the University of California San Francisco and co-senior author of a Sept. 6, 2018, paper in The Journal of Clinical Investigation about a compound that was shown to inhibit hair cell death and subsequent hearing loss resulting from noise. The paper, whose contributors include Lawrence Lustig, M.D., a 2002 ERG scientist at New York’s Columbia University, details the effect the gene TMTC4 has on hearing; deleting the gene results in an imbalance in calcium in the hair cell and triggers an “unfolded protein response” by which the cell self-destructs, causing hearing loss.

Chan, Lustig, and colleagues delivered

a known compound, ISRIB (Integrated Stress Response Inhibitor), to mice before noise exposure to block the unfolded protein response, preserving hearing.

“In humans, inner ear development is completed in utero, with hearing onset at about 20 weeks of gestation.

However, genetic forms of congenital deafness are typically diagnosed during the neonatal period. Gene therapy approaches in animal models should therefore be tested after the period of hearing onset, to determine whether they can reverse an existing deafness phenotype. Here, we used a mouse model of DFNB9, a human deafness form accounting for 2–8 percent of all cases of congenital genetic deafness. We show that local gene therapy in the mutant mice not only prevents deafness

(mutations) likely to cause deafness, although: 24 “deafness genes” have limited data.

2 to 3

infants estimated out of 1,000 are

born with a hearing loss.

Up to

60 percent

of childhood deafness has

a genetic etiology. *World Health Organization **National Institute on Deafness and Other Communication Disorders

a publication of hearing health foundation

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roundup

h ear i n g h ealth foundation

Almost

1 in 5

human genes still has an unresolved coding status.

22,210

number of genes, roughly, in the human genome.

15,000

genes in the fruit fly.

30,000

when administered to immature hearing organs, but also durably restores hearing when administered at a mature stage, raising hopes for future gene therapy trials in DFNB9 patients.” —From the abstract of a paper by 2002 ERG scientist Lawrence Lustig, M.D., and colleagues, in the Feb. 19, 2019, Proceedings of the National Academy of Sciences. The research appears to show gene therapy delivered in two parts—to overcome capacity limitations of the gene therapy–delivering adeno-associated virus (AAV)—was successful, as well as demonstrating there may be a bigger window than expected to deliver gene therapy to patients with DFNB9 congenital hearing loss.

genes in corn.

1 percent

portion of human genome occupied by protein-coding genes.

99 percent

portion of DNA that is identical across individuals.

36 percent

of inherited deafness estimated to be syndromic (accompanied by additional disorders).

64 percent

of inherited deafness that is nonsyndromic.

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“Let’s say you are 40 years old. You get your genome sequenced and learn that you carry genetic risk factors for age-related hearing loss. You could prepare yourself and minimize your other risk factors, and, at the first signs of hearing loss, you can get hearing aids fitted. We know that hearing loss contributes to dementia, for example. The earlier you act, the better you can counteract hearing loss and some of the devastating side effects.” —2006 ERG grantee Jung-Bum Shin, Ph.D., a neuroscientist at the University of Virginia, speaking about his team’s finding that the cuticular plate on which the cochlear hair cell develops is important for the hair cells’ ability to detect sound vibrations. As published in their March 8, 2019, paper in Nature Communications, Shin and colleagues, including 1996 and 1997 ERG scientist John Oghalai, M.D., of the University of Southern California, showed the gene LMO7 is vital for the plate’s stability in mice and, when blocked, that the mice gradually developed age-related hearing loss.

“Middle ear infections are very common in kids. By the time they are 1 year old, around half have fever, ear pain, or pus/fluid in the middle ear due to infection. Some of these infections may recur or become chronic, thus requiring surgery.

A number of things predispose people to getting these infections including a lack of vaccinations, lack of breastfeeding, and being around smoking caregivers. But even in the best-case scenario, recurrent or chronic middle ear infections still happen in some kids, which may be due to genetic predisposition.”

—2011–12 ERG scientist Regie SantosCortez, M.D., Ph.D., an associate professor at the University of Colorado School of Medicine, who found common variants of the gene FUT2 associated with recurrent middle ear infections in Filipinos and South Asians and a rarer variant in European-American children. Those with the genetic variants had a much higher chance of getting ear infections, and the team believes the gene modifies the microbiome of the middle ear and makes it more susceptible to infection by specific bacteria.

For references, see hhf.org/summer2019-references.

Support our research: hhf.org/donate

A seamless connection to life - all they have to do is listen.

Give your child access to the latest in hearing technology with the Nucleus® 7 Sound Processor – the industry’s first and only Made for iPhone cochlear implant sound processor.1 Designed specifically for the Nucleus 7 Sound Processor, the Nucleus Smart App allows you to control, monitor and troubleshoot your child’s hearing from the palm of your hand.

You can check device use information and even find a lost sound processor.

®

Find a Hearing Implant Specialist near you: Call: 800 354 1731 Visit: www.IWantYouToHear.com 1. Apple Inc. Use Made for iPhone hearing aids [Internet]. Apple support.

2017 [cited 24 February 2017]. Available from: https://support.apple. com/en-au/HT201466.

2. Cochlear Limited. D1190805. CP1000 Processor Size Comparison.

2017, Mar; Data on file. ©Cochlear Limited 2017. All rights reserved. Trademarks and registered trademarks are the property of Cochlear Limited. The names of actual companies and products mentioned herein may be the trademarks of their respective owners.

©2017. Apple, the Apple logo, iPhone, iPad and iPod touch are trademarks of Apple Inc., registered in the U.S. and other countries.

The Nucleus Smart App is compatible with iPhone 5 (or later) and iPod 6th generation devices (or later) running iOS 10.0 or later.

The Nucleus 7 Sound Processor is compatible with iPhone 8 Plus, iPhone 8, iPhone 7 Plus, iPhone 7, iPhone 6s Plus, iPhone 6s, iPhone 6 Plus, iPhone 6, iPhone SE, iPhone 5s, iPhone 5c, iPhone 5, iPad Pro (12.9-inch), iPad Pro (9.7-inch), iPad Air 2, iPad Air, iPad mini 4, iPad mini 3, iPad mini 2, iPad mini, iPad (4th generation) and iPod touch (6th generation) using iOS 10.0 or later. Apple, the Apple logo, FaceTime, Made for iPad logo, Made for iPhone logo, Made for iPod logo, iPhone, iPad Pro, iPad Air, iPad mini, iPad and iPod touch are trademarks of Apple Inc., registered in the U.S. and other countries. App Store is a service mark of Apple Inc., registered in the U.S. and other countries.

Information accurate as of November, 2017

CAM-MK-PR-335 ISS1 NOV17

living with hearing loss

hearing health foundati o n

My Ears Had Whiskers

By Ruth D. Bernstein

Cindy, a beautiful cafe au lait Siamese kitten with friendly blue eyes, became part of my life when I got my first hearing aid. The aid signaled the first step in learning to cope with the progressive sensorineural hearing loss that is part of the paternal genetic legacy handed down to me and my children.

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the idea because I traveled a lot for business.

That was when I began searching for devices that could do what Cindy did and found I needed an assistive alerting system—flashers for the phone and doorbell, a strobe-light smoke alarm, and a vibrating alarm clock.

Although the technology works well, it doesn’t replace Cindy’s ability to keep me tuned in to the sounds around me. Or her soft fur, loud purr, and unconditional love. I really miss my ears with whiskers.

Consumer advocate Ruth D. Bernstein is on the board of the Hearing Loss Association of America’s New York City Chapter, at hearinglossnyc.org. This story originally appeared on the Center for Hearing and Communication website, at chchearing.org.

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

Support our research: hhf.org/donate

photo credit: istockphoto.com/xesai

cindy and i became friends instantly. During the day, she was a hunter, roaming through our suburban backyard.

Evenings she curled up on my lap as I read in the family room.

At night she slept in my brown velvet chair, guarding it against anyone who might want to claim it from me.

As I grew older I needed a second hearing aid because my hearing loss worsened. Although I was having more trouble understanding speech, I was aware of the sounds around me—my four children coming and going, doors slamming, and the phone and doorbell ringing. Also, there was always someone around to tell me if I missed something.

Then, suddenly, I was divorced and found myself in a totally different world. Cindy and I moved from the house to an apartment in New York City. Even though the cat was my constant companion, I was lonely and frightened. I could no longer hear the phone or doorbell ring, and there was no one to alert me to those sounds.

That was when I discovered my ears had whiskers.

Surprisingly, Cindy seemed to understand my problem.

When the phone rang or someone came to my door she would sit bolt upright, turn her head toward the sound, and twitch her whiskers. If I didn’t react immediately, she would purr so loudly I could feel her body vibrate.

Cindy always made sure she was nearby, sitting on my lap while I read and sleeping with me at night. She was my alarm clock, waking me at 6:30 a.m. by walking across my pillow until I gave her a good morning hug.

Weekends, I got out of bed at 6:30 a.m. to feed her because I was never able to convince her it wasn’t time to get up. Then we would go back to bed together and get some more sleep.

I didn’t realize how dependent I had become on this special alerting system until, at the old age of 17, Cindy developed acute kidney failure and died. I was devastated. I lost my loving companion and extra set of ears. I considered getting another cat but rejected

research

A SYMPOSIUM TO SOUND OUT HEARING RESTORATION

By Marie Samanovic-Golden, Ph.D.

Hearing Health Foundation’s Hearing Restoration Project scientific director Peter G. Barr-Gillespie, Ph.D., and HRP consortium member Tatjana Piotrowski, Ph.D., will speak at a New York Academy of Sciences conference on hearing and hair cell regeneration in October.

hearing loss is the most common sensory disorder, estimated to affect 466 million people worldwide, according to the World Health Organization.

It is especially prevalent among the aging population, resulting in social isolation and decreased quality of life.

Hearing loss may also be a risk factor for dementia and Alzheimer’s disease.

Most cases of acquired hearing loss are due to degeneration and the subsequent permanent loss of cochlear hair cells, which affects mammals but not other species, such as birds and fish. Scientists are working to identify molecular mechanisms that will cause human hair cells to be regenerated in a functional manner.

With this in mind, we at the New York Academy of Sciences are presenting the symposium “Hearing Restoration and Hair Cell Regeneration” in New York City on Oct. 8, 2019. Scientific Organizing Committee member Michael Franti, Ph.D., the director of regenerative medicine at Germany’s Boehringer Ingelheim, proposed the idea; Boehringer Ingelheim is one of several companies and institutions actively working on the regeneration of mammalian hair cells from inner ear stem cells.

Once the theme was set we invited Peter BarrGillespie, Ph.D., Oregon Health & Science University’s chief research officer and the scientific director of Hearing Health Foundation’s Hearing Restoration Project, to deliver the keynote speech. HRP consortium member Tatjana Piotrowski, Ph.D., of the Stowers Institute for Medical Research in Missouri, will also be presenting, along with other leaders in the field.

Open to the public, the conference is aimed at basic, translational, and clinical researchers who work in academia, industry, government, or with nonprofit organizations. There will also be a simulcast webinar for

those not able to attend in person. The Academy, founded in 1817 and open to all who are interested in science, counts over 20,000 members in 100 countries, and part of our mission is to bring communities from multiple locations and sectors to review cutting-edge, cross-disciplinary research, and to address the most pressing global challenges.

In addition to the focus on hearing restoration, the symposium’s goals dovetail with that of HHF’s Emerging Research Grants program that funds innovative, and often early-career, researchers. For this October event, we are reaching out to scientists at various career levels— including a call for poster abstracts and recognizing the top three—and will offer multiple opportunities to network with experts over the course of the day.

We also want to foster interaction and encourage partnerships among academics, pharmaceutical companies, and startups with the overarching aim of further developing innovative treatments for hearing loss. Indeed, basic science has been key in understanding the molecular signaling that initiates regenerative proliferation in the inner ear to regain lost hearing. While further research is essential to expand what we know about the basic biology of hearing, it is an exciting time as some ideas are being taken from the lab to the marketplace.

Marie Samanovic-Golden, Ph.D., is a program manager at the New York Academy of Sciences.

For more about the symposium, see nyas.org/hearingrestoration.

a publication of hearing health foundation

summer 2019

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meet the fundraisers

hearing health foundatio n

5 Years on, a 5K Expands

By Leslie Brueggeman when our son jace was born in 2013, he failed his hearing test more than once before we were released from the hospital. We were referred to a pediatric audiologist in Fargo, North Dakota, where we were living at the time. We were told it was probably just fluid in the ears so we didn’t really think that there were any issues.

Each summer, the Brueggemans’ 5K walk/run race celebrates their son Jace and fundraises for hearing research.

I knew after having a child with a hearing loss that I wanted to spread awareness and find a cure.

Jace was just such a good baby, and nothing startled him. When we saw the audiologist, he was just short of a month old. They hooked him up to the wires and took us into the hearing booth. Our audiologist told us right away that Jace did in fact have a bilateral hearing loss.

We were not sure just how bad it was until a few weeks later, when we got the diagnosis of moderate to severe hearing loss. He was fitted for hearing aids at 3 months old. At first we were devastated, but we are lucky to have great resources and supportive family and friends.

As Jace got older his hearing loss became profound, and at age 2 he received a cochlear implant in each ear, with surgeries eight months apart. To Jace the hearing loss is his normal. He doesn’t know any differently. Our community is small and supportive. Everyone knows Jace and loves him. We did actually visit a university to do genetic testing to find out why he has a hearing loss, but the results were inconclusive.

I knew after having a child with a hearing

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loss that I wanted to spread awareness and find a cure. I went online and found Hearing Health Foundation (HHF), whose mission matches with our own goals. To show our support for HHF, we started doing a 5K fundraiser because it seemed like the most common kind of fundraiser to do. It has been great, reaching hundreds of participants—and this year, for our fifth anniversary, we have expanded to a second location in South Dakota, Sioux Falls, for two 5Ks in July and August.

Jace just finished preschool and skill builders (special education), and this fall he will do pre-kindergarten as well as another year of skill builders and speech therapy. During the school year Jace uses an FM system (we also use it in crowded areas) and has speech therapy three days a week. He has some amazing teachers at the school. We also work with him daily, making sure we enunciate our speech and asking him to repeat words he has problems with. We want to be sure he is saying things correctly, so there is lots of repeating. We also make trips to the Mayo Clinic twice a year for checkups.

I hope that Jace does not ever let his hearing loss hold him back. He is such a bright kid and has a great personality that we believe will take him far.

HHF is very thankful for the ongoing support from the Brueggemans and their dedicated community of family and friends. We appreciate all that they are doing. Learn how to host a fundraiser at hhf.org/how-to-help.

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

Support our research: hhf.org/donate

EMERGING RESEARCH GRANTS

APPLY FOR AN EMERGING RESEARCH GRANT Hearing Health Foundation’s Emerging Research Grants (ERG) program provides seed money to researchers with innovative approaches to hearing and balance science.

Grantees advance knowledge in the following under-researched areas, among others: »» »» »» »» »»

Hearing loss in children Auditory processing disorder Hyperacusis Tinnitus Ménière’s disease

»» Usher syndrome »» Reducing cancer drug ototoxicity »» Links between hearing loss and

diabetes, heart disease, and kidney disease

Within the scientific community, the ERG program is known as a competitive process that awards grants to only the most promising investigators. Recipients are exceptionally well positioned to win funding from the National Institutes of Health (NIH), leading to dramatic innovations in the field. In fact, ERG alumni have gone on to be awarded an average of $91 for every dollar of their ERG grant.

While early career researchers are especially encouraged to apply, ERG awards are open to both early career researchers and senior investigators. The application period for the next ERG program opens in the fall.

For more information, please see hhf.org/erg.

2016 ERG researcher Elizabeth McCullagh, Ph.D., and colleague at the University of Colorado Denver (top); and a microscopic view of a mouse cochlea showing sensory hair cells in green.

SEEING the SIGNS A recognition of symptoms in her son as well as herself provides a shared diagnosis for three generations and, at last, understanding.

Loraine Alderman with her father and son at Disney’s Epcot in Florida.

By Loraine Alderman, Psy.D.

i am a licensed clinical psychologist, a certified school psychologist, and an award-winning author. From this basic description, one might assume I was a good student, but the truth is I had to work five times harder than any of my peers to keep my head above water.

Academics were always difficult. I always had tutors, and there were many tearful nights filled with frustration.

In New York, a high school student has to take state exams known as Regents; you had to pass five subjects to earn a Regents diploma. I passed one, algebra, the first time I took it, and I passed biology the second time. I failed the others by one to five points. In the end, I was able to receive a less-respected “local diploma” from high school.

I never could figure out why school was so difficult for me and why I frequently misinterpreted my assignments.

In my early 20s I thought for sure there must be something 28

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wrong with my hearing, so I had a hearing test. When the results came back showing typical hearing, I thought to myself I was simply not a smart person.

When I went to college, I would try to get the class syllabus ahead of time so I could start my assignments early. My first major was in early childhood education, because I had always wanted to be a teacher. I had a class that involved learning how to teach phonics, but I couldn’t understand the phonetic sounds. I didn’t know why.

My study group tried their best to help me, but I was beyond discouraged. I switched my major the following semester to psychology. I did well, earning As in psychology classes; my only B was in statistics. I still had tearful, frustrated nights in college and graduate school, although I managed to get through with passing grades.

When my son was 2 years old, I felt that something

family voices

When my son was starting 3rd grade, we moved to another school district and I found myself really having to advocate for his needs. In order to do this, I tried to learn as much as possible about APD, a topic not covered in my doctoral program. And it was at this time that I started to recognize a lot of the symptoms of APD in myself. was wrong with him, but everyone told me he was fine and that I was just overprotective. When he was in kindergarten his teacher finally agreed with my worries after he was unable to name the letters of the alphabet or the days of the week.

His teacher advised me to have him evaluated for speech therapy. By the end of kindergarten he was diagnosed with auditory processing disorder (APD, also known as central auditory processing disorder).

At the start of 3rd grade, we moved to another school district and I found myself really having to advocate for his needs. In order to do this, I tried to learn as much as possible about APD, a topic not covered in my doctoral program. This was in the late 1990s and the internet was fairly new, so there was not a lot of available information.

It was at this time that I started to recognize a lot of the symptoms of APD in myself. When I mentioned it to my son’s speech therapist, she suggested that I get myself tested.

I was very nervous during the evaluation, and as I was going to the meeting to get the results, I called my father. I told him that I was afraid to hear the results because if I didn’t have APD then it must mean that I really am not that bright, even though I earned my doctorate in psychology from Pace University in New York.

When the evaluator explained that I had APD and that I did not hear phonetic sounds, I felt so relieved,

decades later (I was in my mid-40s), that there had been a reason for my academic difficulties.

I called my father with my diagnosis. He told me that he had also experienced all of the same symptoms I did when he was in school. He decided to get tested, too, and in his 60s he was also diagnosed with APD. Looking over his immediate family’s history, we are pretty sure that his father and one of his brothers also had APD. It was my father’s idea to write a book about our struggles. My first book, titled “Don’t You Get It? Living With Auditory Learning Disabilities,” was written because we wanted to help other people. My second book, “I Get It! I Get It! How John Figures It Out,” was my idea after hearing my coauthor talk about her children’s book on self-esteem for young girls.

I wanted to write a book to let kids know that they are not alone and that they can be successful despite having APD. I am proud to say that my second book has earned four

awards, and both of my books are sold around the world. I am currently under contract with my publisher for my third book, which is geared toward teens diagnosed with APD. It is due out between late 2019 and early 2020.

I work full-time as a school psychologist and I have a small private practice. As for my son, he is currently a certified physical education and health education teacher and just completed his master’s degree in health education.

Loraine Alderman, Psy.D., is a licensed clinical psychologist and a certified school psychologist. She works in the New York City school system as a school psychologist and has a private practice in Long Island, New York, and her focus is advocacy for children and adolescents with learning disabilities. For more, see psychdocinfo.com.

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

Support our research: hhf.org/donate

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hearing health foundati o n

MY FIRST

48 HOURS

WITH HEARING AIDS

I’ve only worn my hearing aids for two days, and already the impact on my life has been astounding.

By Adam Felman as an editor and writer for medical news today (mnt), based in the U.K., I am constantly exploring the causes and effects of a range of different diseases and conditions. From time to time, I find that a particular article will pop up and alert me to my own health issues. And that is exactly what happened when I looked into deafness and hearing loss around a year ago.

I was going through the questions a doctor might ask to diagnose a hearing loss, and I was staggered to discover how many applied to me and my own ears.

Sure enough, I took these issues to a doctor, and entered the referral process for treatment by an ear, nose, and throat specialist.

After eight months of waiting, I now have two hearing aids. At the time of writing, I have only been wearing them for two days, yet their impact is already significantly greater than I could ever have imagined.

Gradual, Creeping Impact

I’m lucky enough to have retained at least half of my hearing in each ear. At present, I can lead a mostly active, healthy life, I don’t need to communicate with sign language, and my work is unaffected. However, it’s all too easy to dismiss the impact of a gradual, creeping condition such as hearing loss.

It can develop suddenly, or, as in my case, take 20 years to reach a diagnosable level. I will be 30 years old this year, and those 20 years mark a hugely important period in anyone’s life.

Whether you are trying to make an impact as a young professional starting out, rounding off your formal education, building a family, or all of the above, you will undoubtedly be taking account of parts of your life that are becoming increasingly important and complex.

Communication is a huge part of navigating this formative stage. If any

Adam Felman says his first lesson with wearing hearing aids was that no one close to him sees it as a negative life event.

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element of communication is lacking, it can have a significant impact on the way your personality develops, and the methods you use to connect with the outside world.

The big kicker with gradual-onset hearing loss is that you are unaware of how it’s changing you until the symptoms have become moderate to severe.

Socializing Becomes Risky

Every pang of guilt or embarrassment after saying “what?” or “huh?” might lead to another night when you don’t risk going out to socialize. You end up distancing from soft-spoken colleagues, friends, and even family members, simply because the effort it takes to process their speech is so draining.

I’ve forgotten what it’s like to chat with a friend at a concert or even a bar.

Very often, I will have great difficulty separating conversational frequencies from noises in the environment, making it almost impossible to fully focus on what people are saying.

Something as trivial as needing subtitles when watching television programs and movies with other people can feel like a stigma, and also isolating.

Even though your friends are probably understanding, and although subtitles exist to significantly improve the viewing experience for people who cannot hear as well as others, it can still be hard to ignore the underlying feeling of being “different.”

As a result of these fleeting moments and hang-ups, I developed subtle, invisible coping mechanisms to anchor my social interactions. For example, I wheeled out a set of 10 to 15 stock phrases that I cycled through based on tone of voice and general context.

“Absolutely!”

“100 percent!”

“I can fully understand that.”

“Tell me about it!”

None of these seem out of place in a conversation. However, once they become a substitute for genuine responses and coherent conversational flow, they develop into a cornerstone of shame and awkwardness in daily encounters.

Until you start looking at hearing loss as a condition, it simply feels like part of your worldview. Even if it hasn’t yet reached the stage of impairing daily function, it can still strip at least 30 to 50 percent of the human experience from your day.

After writing the MNT article on hearing loss, I followed this journey to hearing aids on my doctor’s recommendation.

Even though I’m missing only one layer of frequencies, the difference is remarkable.

Before the hearing aids, I had developed subtle, invisible coping mechanisms to anchor my social interactions.

For example, I wheeled out a set of 10 to 15 stock phrases that I cycled through based on tone of voice and general context.

Even Food Comes Alive

My new hearing aids are discreet yet powerful—sometimes, to my underused ears, excessively so. A packet of chips opening 20 feet away sounds like it’s crinkling next to my head; I can hear the wheels of a stroller from a balcony five floors up; even the cacophony during bathroom breaks sounds like a National Geographic documentary.

There are unexpected changes, too. My experience of food has completely altered—the additional frequencies adding a lightness of bite and extra crunch that I was previously unaware of. a publication of hearing health foundation

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Using a hearing loop system for the first time at a concert was emotionally overwhelming. My balance and spatial awareness have also greatly improved in these first few days of wearing my hearing aids.

My hearing no longer feels impaired—that is, until I remove the hearing aids. Those few moments in the day without them, such as going to the gym or grabbing a shower, are now pretty draining by comparison.

However, I have heard about 20 birdsongs for the first time in the last 48 hours, and I’ve listened to the phasing hiss of the sea as I’ve never listened before. I was also hit by a hailstorm that might genuinely be the single most impressive thing I’ve ever heard, although admittedly, until two days ago, the bar was not all that high.

I have a lot to learn about life with hearing aids, but my first lesson was that no one close to me sees it as a negative life event. Everyone has been congratulating me as if I’ve just become a parent for the first time.

I’ve realized that however self-conscious you might feel about wearing hearing aids, people only see it as a connection with the world, and this is a huge deal. I see my hearing aids as an opportunity, rather than as debilitating or cumbersome devices.

I’ve realized that however self-conscious you might feel about wearing hearing aids, people only see it as a connection with the world, and this is a huge deal.

I see my hearing aids as an opportunity, rather than as debilitating or cumbersome devices.

A Game Changer

My hearing aids occasionally produce squeals of feedback, and keeping them wedged in my ears can be a challenge, especially while moving around.

However, I’m in the early stages of treatment and already connecting with the world more closely. While my hearing aids are not perfect yet, they remain a genuine game changer.

If conversations have started to become a struggle for you, or if you’ve passed on getting a hearing aid because of the visual aspect, then I urge you to look into your options. Visit your doctor, speak to your insurer about coverage, and weigh up hearing assistance as a real option.

Sound is 20 percent of your experience as a human. Conversation, music, and background noise are all part of keeping a steady headspace and progressing with your day. Protecting and enhancing that is a life-changing step to take for people who can’t process sound as well as others do.

I cannot wait to stick these bad boys in upon waking up tomorrow and seeing what else I can discover for the first time.

This story originally appeared on Medical News Today, medicalnewstoday.com.

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managing hearing loss

HOW TO COMMUNICATE BETTER, AND MORE COMPASSIONATELY, WITH PEOPLE WITH HEARING LOSS

By Mary Florentine, Ph.D., Julia B. Florentine, and Michael J. Epstein, Ph.D.

“Look at me, and take your hands away from your mouth. Please don’t exaggerate your pronunciation.”

A trio of experts with both professional and personal connections to hearing loss share advice for better communication.

They are a distinguished professor emeritus and expert in psychoacoustics (how humans perceive sounds); her daughter who coaches communication skills and researches the link between language, language, and mental health; and an auditory scientist who has investigated hearing better in background noise.

Here they tell us what individuals with hearing loss say works and why these tips are effective.

Many people have learned to use visual information from the mouth and facial expressions along with the sounds they receive to understand speech. The words “bother” and “father” sound very similar to people with hearing loss, but they look different when they are being said. The lips come together for “bother” to make a puff of air to start the word; the air coming through the mouth is continuous at the start of “father.”

Using these cues to understand speech is called “speechreading.”

It used to be called “lip reading,” but we now know that we use more than information from the lips. Facial expressions also help.

Although there are excellent speechreading courses, some people learn to speechread on their own without formal training. Whether a person has had formal training speechreading or not, be sure that they can see your face.

Although many words can be speechread, others cannot. If you go to the mirror and say “mom, bomb,” in a natural manner and speed, they look the same on your lips. Speechreading can be helpful, but do not expect that someone can understand all speech 100 percent of the time using speechreading alone.

Many people exaggerate their pronunciation because they think it will make them easier to understand, but it can actually make it worse. Exaggerated pronunciation changes speechreading cues and may bring unwanted attention from others who can see you.

Speak naturally.

If you are eating while talking, make sure that you swallow the food in your mouth before you start to talk. Holding food in your mouth while talking will also change the speechreading cues.

Don’t talk in the dark; good lighting is important. Don’t talk from the other room unless the listener can see through walls! And get their attention first, such as by asking “Can you hear me?” before speaking. a publication of hearing health foundation

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“If I do not hear you the first time, please repeat with different words.”

Even if you are having difficulty getting them to understand what you are saying, never give up and say, “Never mind.”

You may think that what you have to say may not be that important, and you may be right. But the person with hearing loss wants to know what you said and is likely to feel left out.

Some words are more difficult to hear than others. When a person with hearing loss misses a word, they often ask the speaker to repeat what they said. Most people will repeat the word that the person did not hear the first time.

If the listener does not hear the word again, some people continue to say the same word. Each time the word gets a little louder. This situation is extremely annoying to both the speaker and the listener.

If a person does not hear a word, it may be because the sound of that word might be especially difficult for them to process, and they do not have enough context to help them piece together the meaning. Saying the same thing with different words is a better strategy; it gives them another way to understand the message. If the word is an object and you both can see it, point or gesture. Writing or texting the word can be useful.

Even if you are having difficulty getting them to understand what you are saying, never give up and say, “Never mind.” You may think that what you have to say may not be that important, and you may be right. But the person with hearing loss wants to know what you said and is likely to feel left out. If you are having difficulty getting someone to understand, or you need to finish the conversation, it is better to say, “I’ll tell you later.” But if you say this, remember to tell them later or they will not believe you the next time.

The “I’ll tell you later” response can work well when the conditions for communication are bad or when you need time to think of a way to rephrase (and not just repeat) what you said.

“Let’s try to limit or avoid background noise. I do not hear well in noisy environments.”

It is difficult for people without hearing loss to understand the impact of background noise on a person with hearing loss. This is because they hear differently. People without hearing loss efficiently filter out unwanted noise, except when in the most extreme noise environments. People with hearing loss experience varying amounts of difficulty doing the same thing.

Every time we listen, unless we are in a sound-isolating chamber, we hear the sounds we want to hear mixed with sounds we do not want to hear.

We usually get used to it and can tune out low-level background noises, such as the hum of a refrigerator or ventilation system fans. We can also tune out most other background noises, except when it gets very loud and/or really bothers us, like when a buzzing insect flies by.

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People with hearing loss have difficulty separating out unwanted sounds. Most very low-level sounds are usually not a problem because they are not heard. Moderate and loud sounds that mix with speech can be a big problem.

Noisy restaurants are always a problem, although there are ways to minimize the problem. You could go at a less crowded time, or ask for a quiet table away from the kitchen and the bar. If that doesn’t work, vote with your feet and go to another restaurant with better acoustics. You can look for reviews of quiet restaurants and even rate them yourself.

At home, you have more control over background noise. You can simply turn off the television or radio. If the person with hearing loss is watching television and you need to speak to them, ask them to put the television on pause or mute. If noise is coming from the hall, you can shut the door.

Many other background sounds can occur at home. Don’t talk while washing dishes in the kitchen sink, using a food processor, or any other appliance that makes noise. Outside the home, you have less control over noise. It is usually best not to try to talk while walking outside on a noisy street and when you cannot face your conversational partner. Seek ways to limit background noise or wait until you are in a better environment to communicate.

We hope these strategies help facilitate better communication and engaging conversations for you and your loved ones.

The “I’ll tell you later” response can work well when the conditions for communication are bad or when you need time to think of a way to rephrase (and not just repeat) what you said.

This is excerpted from “How to Talk to People With Hearing Loss,” available at glistentraining. co.uk/book.

Mary Florentine, Ph.D. (far left) is a Matthews Distinguished Professor Emeritus at Northeastern University in Boston. Julia B. Florentine is the director, coach, and trainer at Glisten Training, which she founded, based in the U.K. Michael J. Epstein, Ph.D., is an auditory scientist, writer, filmmaker, and musician.

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hearing health

hearing health foundation

Visualizing My Childhood Hearing Loss

By Marjorie Saavedra

i began experiencing hearing problems when I was 5 years old.

From what my parents told me, I complained to them about ear pain repeatedly and said I couldn’t hear their voices well. At the time we were in Peru. Living in a country with limited resources meant I was never fully diagnosed, but my doctors put in ear tubes to help with my ear pain and discharge.

When we arrived in New York City I was 12, and the new doctor told me I had holes in my eardrums. They did surgery on my left ear, which had become especially bad because the eardrum holes had become bigger. I was on the verge of losing my hearing.

After surgery, I remember feeling slightly ashamed of the condition of my ears. As I grew older I came to realize that I was very lucky to have had my hearing treated; some children are not able to receive treatment and lose their hearing permanently. Today, I’m no longer ashamed to tell someone about my hearing problems, and I’m not embarrassed about my scars.

I think the experience taught me to grow into a more mature, humble person and to never judge another person for any health issues. If anything, I see my medical history as something important to talk about so that others can know the signs of hearing trouble and get treatment.

I still face issues with hearing well. Sometimes in class I can’t hear the professors or speakers with soft voices. Also, loud noises can hurt my ears and give me a headache. But I 36

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have learned to manage these issues by sitting in the front of class or using earplugs.

All of this inspired my thesis project in my major of electronic design and multimedia. Working as an intern at Hearing Health Foundation also definitely influenced my project because it made me realize how much hearing loss can affect people of all ages and backgrounds. It made me want to raise awareness about the importance of hearing health.

I based my project on hearing loss in children because not many people may encounter it in everyday life, and people often believe that hearing loss only affects seniors, or infants who were born deaf. However, there are so many children who have hearing problems at a very young age, and the different types of hearing loss are not often accurately represented.

(Because of my middle ear problems I believe I have conductive hearing loss, which can be inherited.) I wanted to design my project to be educational and approachable, presenting information and statistics in an appealing, easily understandable way in order to reach a wide audience.

The result, titled “Can You Hear Me?,” is a branding project for a hypothetical nonprofit organization that helps families with children from the ages 0 to 9 by providing resources about hearing loss. I used a simple, modern style and rich, vibrant colors to create a family-friendly look. Project elements include a website with integrated animation,

How Can You Spot The Symptoms Of Hearing Loss In Your Child?

Everything sounds like it’s underwater.

A child with hearing loss can show symptoms of poor speech, difficulty in learning, and non–responsiveness during conversations. They might complain of muffled hearing or a feeling of pressure in their ears. For a more comprehensive description of symptoms, visit canyouhearme.us.

How Can You Help Your Child Who Is Suffering From Hearing Loss?

I got my child’s hearing tested because he couldn’t hear his teacher in school.

If your child is hard of hearing, visit canyouhearme.us to find a doctor in your area who can assist with your child's symptoms or connect with a community of parents facing a similar situation.

ABC

2+2

The posters that Marjorie Saavedra created for her thesis project use a simple, modern style and rich, vibrant colors to call attention to pediatric hearing loss.

hearing health

I wanted to design my project to be educational and approachable, presenting information and statistics in an appealing, easily understandable way in order to reach a wide audience. an infographic poster, and two callto-action posters. Collateral elements include a pamphlet, an accordion book, a branding style guide, T-shirt designs, pencils, and stickers.

Our school’s thesis projects are part of a gallery showcase for family, friends, and other students. For my presentation, I arranged my area to resemble a doctor’s office, with toys, seating, and a table with coloring

pages I designed. I was happy to see adults and children alike using crayons to color in the pictures, which also reinforced brand messaging using stats along with the illustrations.

Essentially I wanted to create a warm, friendly waiting room.

Many visitors asked me how I came up with the idea for this project, and I said it was because I myself had a hearing loss as a child. I wanted to bring and spread awareness to families on this important topic, and to show parents that they are not alone. My own family faced the challenges of finding information and support, which is just one reason why I was so motivated to raise awareness about pediatric hearing loss and to provide the resources families need.

HHF web design and development intern Marjorie Saavedra received a B.F.A. in electronic design and multimedia at City College of New York. For more on her thesis subject, the hypothetical nonprofit, see canyouhearme.us.

As part of the gallery showcase for thesis projects, Saavedra mocked up a welcoming doctor’s waiting room that included handouts conveying information in a fun, friendly way.

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

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Heather Mills with her husband Billy and their kids.

WITH CAUTIOUS

Gratitude A mother manages Ménière’s disease with the help of family and friends—and her hearing aids.

By Lauren McGrath

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heather mills never imagined her early adulthood would be interrupted

by Ménière’s disease, a chronic hearing and balance disorder without a cure. She was diagnosed at 21—just within the typical 20-to-50-year-old range of onset—after a slew of tests and follow-up visits with a specialist at the University of Minnesota.

Mills’s symptoms initially included a mild low-frequency hearing loss in one ear, tinnitus, and some ear pressure and pain. Within a few years, her hearing loss became bilateral and worsened from moderate to severe. She was regularly distressed by intense ear pressure, struggled with her balance, and experienced occasional bouts of vertigo.

As Mills learned, Ménière’s affects each patient differently, and she considered herself fortunate not to face drop attacks. These sudden instances of falling to the ground without losing consciousness can be one of the most terrifying symptoms associated with Ménière’s disease.

Mills’s family members also have hearing loss. Her father has lived with a unilateral hearing loss since childhood, while her mother and maternal grandmother both developed high-frequency sensorineural hearing loss in their late 40s. “It never occurred to me that it may one day affect me, too,” she says, recalling her ability to hear whispers across a high school classroom.

Though she followed her doctor’s directions to take diuretics and maintain a low-salt diet for her vestibular symptoms, Mills chose not to purchase hearing aids.

Lacking amplification, she faced difficulty in her job as a legal project specialist, which required frequent verbal interaction with clients, lawyers, and vendors both on the phone and in person. She found herself increasingly dependent on a close friend and colleague who repeated information for her during and after meetings.

Mills’s untreated hearing loss, combined with her constant fear of a sudden vertigo attack, made her feel isolated. In addition to workplace meetings, social outings were a challenge, and the lack of interaction affected her mental state. “I became depressed and lonely, and developed anxiety because of two unknowns—not knowing when my vertigo would strike again, and wondering how I’d continue to work to support my family,” Mills says.

family voices

She says she noticed a sharp decline in her job performance when her “helpful coworker”—her ears— left the law firm. Part of her role required instructing staff about new software, and she was humiliated to find out that her trainees’ questions went unanswered because she did not hear them. “This is when I began to lose confidence in myself,” she says.

Mills realized she had to address her hearing loss.

She says she had delayed taking action for so many years—11 to be exact—because her insurance provided no hearing healthcare coverage.

“All I can say now is I wish I had gotten hearing aids sooner!” she says. Prior to pursuing treatment, Mills says conversations soured quickly when she constantly had to ask other parties to speak up, repeat themselves, or remind them of her hearing loss. Most painfully, Mills was often dismissed by a “never mind” when she asked people to repeat themselves. Now with newfound confidence, Mills says her daily professional communications and social life have dramatically improved.

With her symptoms in remission, Mills considers her life happy and her health stable. Hearing aids have somewhat alleviated her tinnitus, her ear pressure has subsided, and the vertigo spells are very rare. She has also sought treatment for her anxiety and depression.

Mills credits her husband Billy, with whom she has two young children, for his support during her more difficult years. Engaging in online Ménière’s support groups also has been a beneficial coping tool.

Mills is cautiously grateful for her current health, knowing that the unpredictability of Ménière’s could alter her circumstances at any time. She hopes for scientific advancements in Ménière’s research that will one day uncover the causes, more reliable diagnostic procedures, and a cure.

Heather Mills lives in Minnesota with her husband and children. She is a participant in HHF’s Faces of Hearing Loss campaign, at hhf.org/faces. Lauren McGrath is HHF’s director of marketing and communications. For references, see hhf.org/summer2019-references.

Mills’s family members also have hearing loss. Her father has lived with a unilateral hearing loss since childhood, while her mother and maternal grandmother both developed high-frequency sensorineural hearing loss in their late 40s.

“It never occurred to me that it may one day affect me, too,” she says, recalling her ability to hear whispers across a high school classroom.

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research

Hearing Restoration Project Plans Announced for 2019–20 By Peter G. Barr-Gillespie, Ph.D. The goal of the Hearing Restoration Project (HRP) is to determine how to regenerate inner ear sensory cells in humans to eventually restore hearing. These sensory hair cells detect and turn sound waves into electrical impulses that are sent to the brain for decoding. Once hair cells are damaged or die, hearing is impaired, but in most species, such as birds and fish, hair cells spontaneously regrow and hearing is restored. The HRP is tasked with uncovering how to replicate this regeneration process in humans.

Here are the funded HRP projects for the coming year. Thank you for your ongoing support.

Integrative Systems Biology of Hearing Restoration

Seth Ament, Ph.D. University of Maryland School of Medicine

Comparison of Three Reprogramming Cocktails in the Organ of Corti: Cells, Transcriptomes, and Epigenomes

Andy Groves, Ph.D. year 2 | This Baylor College of Medicine project will focus on integrating multiple year 2 | In the past datasets from the HRP year we have been to gain insight into investigating whether hair cell development we are able to use and regeneration and prioritize genetic reprogramming specific “driver” genes that can be techniques to generate targeted to induce regeneration. The new hair cells in the mouse cochlea. main premise is that we will be able The results of this work are extremely to regenerate hair cells if we activate promising: We were able to turn the correct set of hair cell–promoting nonsensory cells of the mouse genes in supporting cells. This cochlea into hair cells. However, we process is called transdifferentiation, consistently find that supporting and it occurs naturally in species cells in our mouse models do not such as birds and fish, but not in respond to reprogramming. This the inner ear of adult mammals. is curious, as supporting cells are HRP researchers have generated the cells responsible for producing numerous genomic datasets that new sensory hair cells in birds and describe cochlear development and fish. In the coming year, we will transdifferentiation in multiple examine supporting cells after our species. By analyzing all of these reprogramming attempts to see data together using sophisticated whether they have been able to network analysis tools, we aim to activate any aspects of a hair cell identify which genes are involved program. We will also test whether in these processes as well as key hair cell death alters the adjacent differences that may explain the supporting cells so that they become inability of human and mouse cells more responsive to reprogramming. to transdifferentiate. Finally, this will help identify genes that can be targeted to enable transdifferentiation.

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Detection of Transcriptome Changes in Single Cells After AminoglycosideInduced Hair Cell Loss in the Chicken Basilar Papilla Stefan Heller, Ph.D. Stanford University

year 3 | Birds regenerate cochlear hair cells by activating dormant supporting cells. This project builds on innovative methods and findings to study how supporting cells are activated when ototoxic drugs cause hair cell death. The project uses single cell analysis (during which we study the complement of genes that are active in many individual cells) to identify triggers that initiate, execute, sustain, and ultimately terminate the regenerative process. Using bioinformatics methods to process the resulting data, we will focus this year on the analysis of the chicken cochlea cell populations isolated at various time points during hair cell regeneration, which will reveal the molecular steps that occur during hair cell regeneration. We have already identified a first candidate gene signaling pathway that may regulate the regenerative process in the chicken cochlea, and we will be confirming that this pathway

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plays a role in hair cell regeneration.

Interestingly, this pathway is not active during inner ear development, which sets it apart from other pathways linked to chicken (and zebrafish) hair cell regeneration; the other pathways are involved in cell development and may not represent a unique regenerative trigger. Finding triggers that specifically control regeneration may lead us toward curing hair cell loss in mammals.

Epigenetics Analysis of Maturation and Regenerative Responses in the Mouse Organ of Corti and Utricle

photo credits jane g photography

Neil Segil, Ph.D. University of Southern California

year 3 | Although hair cell regeneration does not occur in mammals, newborn mice harbor a latent capacity for some regenerative responses.

However, this capability disappears within the first few weeks of life. This observation provides an experimental window that this proposal exploits to address fundamental questions about the failure of hair cell regeneration in mammals. Specifically, we propose experiments to identify those changes in the genetic material, the chromatin, that are responsible for orchestrating the differentiation of new hair cells within the newborn organ of Corti in the inner ear, and to investigate the changes in the chromatin that lead to the failure of regeneration in the adult mammalian inner ear.

Support our research: hhf.org/donate

Implementing the gEAR for Data Sharing Within the HRP

Mouse Model Systems to Interrogate Candidate Genes for Sensory Hair Cell Regeneration

Ronna Hertzano, M.D., Ph.D. University of Maryland School of Medicine

John Brigande, Ph.D. Oregon Health & Science University

year 3 | When a group of geographically dispersed scientists collaborate on hair cell regeneration in three different animal models—chicken, zebrafish, and mouse—and use multiple methods to track how genes “instruct” cells (multi-omics), an enormous amount of data results. The work of visualizing, conceptualizing, and analyzing these data presents a considerable challenge, and as technology has advanced, much of the multi-omic data is generated at the single cell level, resulting in datasets and files that are too big to process with traditional tools, such as Excel worksheets. The gEAR portal (gene Expression Analysis Resource, umgear.org) responds to this need by enabling meaningful visualization and analysis of these complex datasets in the public or private domain—no advanced programming skills required. It has also evolved to become a primary data sharing, visualization, and analysis tool for auditory researchers outside of the HRP to become a platform that supports the hearing research community at large.

year 5 | Given what the HRP has discovered thus far, this project proceeds from the assumption that manipulation of multiple genes that regulate diverse signaling pathways may be required to reprogram supporting cells to become functional hair cells. We must first verify the expression pattern of candidate genes in the inner ear, then develop methods that allow us to modulate multiple candidate genes in each supporting cell in order to transmit the genetic instructions that trigger regeneration.

This proposal’s first goal is to establish improved genome editing via oviductal nucleic acids delivery (iGONAD) technology, allowing us to identify as well as eliminate candidate genes. Our second goal is to turn on multiple genes simultaneously in the same cell using a mouse model. The overall objective is to establish a rapid method to describe candidate gene expression and function in the inner ear at any stage, and to define an approach to determine which genes are critical for modulating signaling pathways for hair cell regeneration.

HRP scientific director Peter G. Barr-Gillespie, Ph.D., is Oregon Health & Science University’s chief research officer and executive vice president. For more, see hhf.org/hrp.

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Recent Research by Hearing Health Foundation Scientists, Explained

Moving Beyond Wnt and Notch Pathways for Hair Cell Regeneration

For more, see hhf.org/hrp and hhf.org/erg.

For references, see hhf.org/ summer2019references.

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there are several active human clinical trials evaluating the safety of inner ear hair cell regeneration therapies, but these therapies’ target mechanisms may be insufficient to stimulate hair cell growth in the adult mammalian cochlea. These approaches rely on the canonical Wnt and Notch signaling pathways and the Atoh1 molecule, which is necessary for hair cell regeneration and is regulated by these pathways.

However, a report published in Molecular Therapy in May 2019 by Anshula Samarajeewa, Bonnie E. Jacques, Ph.D., and Alain Dabdoub, Ph.D., a member of Hearing Health Foundation (HHF)’s Hearing Restoration Project (HRP) consortium, notes that there has been very limited success thus far in regenerating hair cells in adult mammalian cochlea using these signaling pathways. This likely means, the authors write, that researchers will need combined approaches that also use epigenome-editing techniques to address changes to the genetic material and activity that occurs with age.

Both the Wnt and Notch pathways play a role in determining how inner ear cells develop into specific types of cells and multiply, and they are also important in the development of the cochlea as a whole. Activating Wnt pathways and inhibiting Notch pathways can turn supporting cells into hair cells in fetuses and newborn mammals, making these key targets for hair cell regeneration. But both become much less effective as the body ages. Manipulating these pathways in adult animals has led to some success in regenerating hair cells, but these new hair cells tend not to develop fully, do not form necessary connections with auditory neurons, or even survive.

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This lack of success is not because these pathways no longer exist in adults; researchers have found that they are still functional. This suggests that there are epigenetic changes that occur as a result of aging to make the adult cochlea less receptive to regeneration. Targeting epigenetic enzymes in addition to the Wnt and Notch signaling pathways may therefore prove more successful, but researchers still need to determine which part of the chromosome to target. This process would involve gene-editing techniques like CRISPR. This type of epigenome editing has slowed hearing loss in newborn mice, but it has yet to be tried in adult mice. If successful, this technique has the potential to treat hereditary and acquired forms of hearing loss. —Christopher Geissler, Ph.D.

HRP consortium member Alain Dabdoub, Ph.D. (left), is a senior scientist, biological sciences at Sunnybrook Research Institute, University of Toronto. Christopher Geissler, Ph.D., is HHF’s director of program and research support.

For more, see hhf.org/hrp.

Support our research: hhf.org/donate

research

CT Imaging as a Diagnostic Tool for Ménière’s Disease ménière’s disease is an inner ear condition with symptoms including vertigo, hearing loss, and tinnitus, and may be associated with an accumulation of fluid in the inner ear, termed endolymphatic hydrops.

Diagnosis of Ménière’s is entirely based on clinical characteristics, and to date, no classification has been established that can predict the onset or course of the disease. Patients with Ménière’s can have varying degrees of symptoms, so defining subtypes within the Ménière’s population may help establish a classification to improve diagnoses and treatments.

Our previous analysis of cadaveric ears of patients with Ménière’s revealed striking differences within the endolymphatic sac in the inner ear, which regulates endolymph fluid. We had found two different aberrations of the endolymphatic sac—its underdevelopment or its degeneration—among Ménière’s patients, suggesting that the loss of endolymphatic sac cell function and the possible impairment of endolymphatic fluid regulation may lead to Ménière’s. In addition, these two pathologies may be associated with differing clinical traits of the disease.

In our prior work, we examined sections of human cadaveric inner ears with Ménière’s and found differences in the angular trajectory of the vestibular aqueduct (ATVA), the bony canal in which the endolymphatic sac is located.

These differences resembled either the trajectory of typical adults, or the trajectory of early developmental, fetal vestibular aqueducts. ATVA similar to other adults without Ménière’s were associated with late onset of the condition, whereas Ménière’s patients with “fetal” ATVA experienced early onset.

For our paper published in the journal Otology & Neurotology in April 2019, we hypothesized that this difference can be detected with a CT scan (computerized tomography) in patients with early or late onset Ménière’s.

We used a custom-made, open-source web application for angle measurements and applied this technique on high resolution CT imaging of patients with Ménière’s. Comparing the angle measurements of the ATVA, we confirmed the results of the cadaveric study. There was a strong correlation between late onset Ménière’s with a typical “adult” course of the vestibular aqueduct, while early onset Ménière’s was associated with a more straight, “fetal” course of the vestibular aqueduct.

As such our study aims to develop a radiographic screening tool, such as a CT scan of the inner ear, to classify different Ménière’s subtypes. It appears that early onset Ménière’s patients have a different anatomy of the vestibular aqueduct compared with late onset Ménière’s patients.

We want to better understand if these findings also correlate with additional clinical factors, such as specific symptoms or a positive family history for Ménière’s. Ultimately, this may help to further characterize different Ménière’s subtypes in order to better diagnose, predict the course of, and treat the condition. —Ngoc-Nhi Luu, M.D., Dr. med.

EMERGING RESEARCH GRANTS

A 3D reconstruction of the endolymphatic space of a typical human adult inner ear. In Ménière’s disease patients, the anatomy of the endolymphatic sac differs, suggesting that the impairment of the sac’s function to regulate fluid may lead to Ménière’s.

(LSC, lateral semicircular canal; PSC, posterior semicircular canal; SCC, superior semicircular canal.)

Ngoc-Nhi Luu, M.D., Dr. med., is a postdoctoral fellow at EatonPeabody Laboratories, Massachusetts Eye and Ear, Harvard Medical School, and an ENT resident at University Hospital Zurich. Luu’s 2017 ERG grant was generously funded by The Estate of Howard F. Schum. Coauthors on the paper include Judith Kempfle, M.D. (a 2010 ERG scientist), Steven Rauch, M.D. (1990 ERG), and Joseph Nadol, M.D. (1976–77 ERG). a publication of hearing health foundation

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EMERGING RESEARCH GRANTS

Pathways that carry vestibular information to the cerebellum for controlling balance and posture were traced. Direct projections from the vestibular inner ear (green) and indirect projections from the brainstem (magenta) were shown to target different populations of neurons in the cerebellum.

Stability in an Unstable World mice are helping scientists to understand how the world around us remains looking stable even as we move.

While out jogging, you have no trouble keeping your eyes fixed on objects in the distance even though your head and eyes are moving with every step. Humans owe this stability of the visual world partly to a region of the brain called the vestibular cerebellum. From its position underneath the rest of the brain, the vestibular cerebellum detects head motion and then triggers compensatory movements to stabilize the head, body, and eyes.

The vestibular cerebellum receives sensory input from the body via direct and indirect routes. The direct input comes from five structures within the inner ear, each of which detects movement of the head in one particular direction. The indirect input travels to the cerebellum via the brainstem, which connects the brain with the spinal cord. The indirect input contains information on head movements in multiple directions combined with input from other 44

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senses such as vision.

By studying the mouse brain, we have now mapped the direct and indirect circuits that carry sensory information to the vestibular cerebellum. Both types of input activate cells within the vestibular cerebellum called unipolar brush cells (UBCs). There are two types of UBCs: ON and OFF. Direct sensory input from the inner ear activates only ON UBCs. These cells respond to the arrival of sensory input by increasing their activity. Indirect input from the brainstem activates both ON UBCs and OFF UBCs. The latter respond to the input by decreasing their activity.

The vestibular cerebellum thus processes direct and indirect inputs via segregated pathways containing different types of UBCs. The next step in understanding how the cerebellum maintains a stable visual world is to identify the circuitry beyond the UBCs. Understanding these circuits will ultimately provide insights into balance disorders, such as vertigo. —Timothy Balmer, Ph.D., and Laurence Trussell, Ph.D.

This summary originally appeared in the journal eLife. A 2017 ERG scientist who received the Les Paul Foundation Award for Tinnitus Research, Timothy Balmer, Ph.D. (left), is a postdoctoral fellow at the Oregon Hearing Research Center at Oregon Health & Science University (OHSU).

Laurence Trussell, Ph.D., a 1991 ERG alumnus, is a professor of otolaryngology–head and neck surgery at OHSU.

Support our research: hhf.org/donate

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Extended High-Frequency Hearing Loss and Tinnitus: Is There a Link? tinnitus is theorized to possibly arise from decreased central inhibition related to cochlear damage, or hearing loss. A reduction in inhibition function would allow signals that are normally suppressed to be perceived, resulting in tinnitus. However, many individuals with clinically typical hearing also present with tinnitus.

In our earlier study, results indicated that despite an apparently intact peripheral auditory system, inhibitory function was atypical and significantly related to tinnitus severity among a population reporting mild tinnitus. With central inhibition lowered, signals that are typically dampened are able to be perceived, potentially resulting in tinnitus. Our paper also showed the utility of measuring central inhibition through cortical auditory evoked potentials (CAEPs), which are electrical responses in the brain that reveal levels of central inhibition.

Given the prior study’s results, we thought it possible that hearing loss within extended highfrequency thresholds (10, 12.5, and 16 kilohertz), which are not typically assessed in the clinic, may negatively impact inhibitory function and subsequent gating measures.

For our follow-up research, published in the American Journal of Audiology on April 22, 2019, we examined the role of both extended high-frequency thresholds and sensory gating dysfunction, a measure of central inhibition abnormality, in typical-hearing adults with and without tinnitus. Results suggest that extended high-frequency thresholds do not correlate with CAEP amplitude gating indices—in other words, high-frequency hearing loss was not associated with decreased central inhibition.

However, we found an unexpected relationship in the tinnitus group: Those with better (lower) thresholds also presented with worse tinnitus. We believe this finding may be due to typical-hearing adults with better high-frequency hearing to be more aware of internal auditory signals, and thus perceive tinnitus. However, further research is needed to investigate this hypothesis.

In addition, atypical gating performance was

A

B

observed in adults with a Tinnitus Handicap Inventory score over 6, which may demonstrate that tinnitus severity must reach a certain point in order for central gating deficits to be observed, or vice versa. A hierarchical multiple regression showed both extended high-frequency thresholds and atypical gating function to account for a significant 49 percent of tinnitus severity.

Therefore, auditory gating appears to be a useful objective measure for tinnitus severity, at least in adults with clinically typical hearing.

It also appears that the testing of extended high-frequency thresholds is warranted in this population, to be used in combination with CAEP amplitude gating indices. Our laboratory is now conducting studies investigating the utility of auditory gating as a clinical tool for the objective assessment of tinnitus severity in adults with hearing loss. —Julia Campbell, Au.D., Ph.D.

CAEP gating waveforms in A) a typical-hearing subject without tinnitus and B) a typical-hearing subject with tinnitus.

The solid line represents the CAEP response to the first stimulus (S1) in a tonal pair, and the dashed the CAEP response to the second stimulus (S2) in a tonal pair. Typical gating is observed when CAEP S2 amplitude is lower compared with CAEP S1 amplitude (A). Atypical gating occurs when CAEP S2 amplitude is equal to or larger than CAEP S1 amplitude (B).

2016 ERG scientist Julia Campbell, Au.D., Ph.D., CCC-A, FAAA, received the Les Paul Foundation Award for Tinnitus Research. She is an assistant professor in communication sciences and disorders in the Central Sensory Processes Laboratory at the University of Texas at Austin. If you are interested in participating in this research, email julia.campbell@austin.utexas.edu. a publication of hearing health foundation

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EMERGING RESEARCH GRANTS Lasting Effects From Head and Brain Injury traumatic brain injury ( tbi ) is a major public health issue and contributes to injury-related morbidity and mortality worldwide. The estimated economic cost of TBI is estimated to be in excess of $76 billion per year in the United States. Unfortunately, the health effects of TBI are profound. TBI can lead to chronic and debilitating physical and psychosocial symptoms, such as loss of cognitive, sensory, and psychological function. Auditory and vestibular dysfunction has long been recognized as a consequence of head injury, including TBI. In our research “Patient-Reported Auditory Handicap Measures Following Mild Traumatic Brain Injury,” published in The Laryngoscope in May 2019, we examined auditory complaints following traumatic brain injury, as well as changes that occur to the peripheral vestibular system in the postmortem setting. In patients with mild traumatic brain injury (mTBI), we used patient-reported outcome measures to assess auditory complaints. The team found that auditory symptoms and associated handicap were common in patients with non-blast mTBI. For another May 2019 paper in The Laryngoscope, “Peripheral Vestibular Organ Degeneration After Temporal Bone Fracture: A Human Otopathology Study,” we evaluated postmortem specimens from the National Temporal Bone Pathology Registry with head injury. In a cohort of patients with temporal bone fractures, there were distinct peripheral vestibular changes. Collectively, these findings have implications for the pathophysiology and management of symptoms in this patient population.

—Elliott Kozin, M.D.

Elliott Kozin, M.D., is a neurotology fellow at Eaton Peabody Laboratories, Massachusetts Eye and Ear, Harvard Medical School. His 2018 ERG grant was generously funded by the General Grand Chapter Royal Arch Masons International.

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Better Tests for Discovering “Hidden” Hearing Loss conventionally, hearing loss is thought to be a consequence of damage to delicate sensory hair cells in the inner ear (cochlea). However, over the past decade animal studies have shown that nerve endings in the cochlea are considerably more vulnerable to damage than the sensory hair cells, and that such nerve damage is likely to happen before conventionally recognized forms of hearing loss occur.

Unfortunately, damage to cochlear nerve endings cannot be detected by current clinical hearing tests.

Yet, this “hidden” damage can hypothetically still affect hearing in everyday noisy environments such as crowded restaurants and busy streets. Therefore, it is important to develop tests to detect such damage in humans, and there is considerable interest among hearing scientists toward this enterprise.

In our paper published in Neuroscience on March 8, 2019, we considered noninvasive tests that can potentially reveal such nerve damage, and systematically investigated other extraneous sources of variability that might reduce the sensitivity and specificity of these tests. This helped us come up with recommendations for how we can best apply these tests. Funding from Hearing Health Foundation’s Emerging Research Grants contributed to experiments that helped understand and articulate the role of two key variables: how variations in the anatomy of individuals (e.g., brain shape and size) affected our noninvasive tests; and how certain cognitive factors like attention may affect hearing independently of how well the inner ear is capturing the information in sounds.

Armed with the knowledge about these variables and other factors described in the paper, we anticipate that hearing scientists will be able to design more powerful experiments to understand the effects of damage to cochlear nerve endings, and build more powerful tests to detect such damage in the clinic. This work is crucial in enabling clinical translation of the basic science that has been uncovered over the past decade. —Hari Bharadwaj, Ph.D., with Inyong Choi, Ph.D.

A 2015 ERG scientist, Hari Bharadwaj, Ph.D. (far left), is an assistant professor at Purdue University in Indiana with a joint appointment in speech, language,

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and hearing sciences, and biomedical engineering. Fellow ERG scientist Inyong Choi, Ph.D., is an assistant professor in the department of communication sciences and disorders at the University of Iowa. Choi’s 2017 ERG grant was generously funded by the General Grand Chapter Royal Arch Masons International.

A Newly Identified Neuron in a Brain Region Tied to Hearing most of our auditory experience requires extensive and precise computations in the brain. While the neural circuitry underlying these computations has become increasingly clear over the past several decades, there has remained a big gap in our understanding of the neural circuitry in an important brain region called the inferior colliculus (IC).

Located in the midbrain, the IC is the hub of the brain’s auditory pathway. Like an airport hub that processes travelers moving among farflung airports, the IC receives and processes most of the output of lower auditory centers and provides the major source of auditory input to higher brain centers.

Although the IC plays important roles in most auditory functions, including speech processing and sound localization, it has proven difficult to identify the types of neurons (nerve cells) that make up the IC. This has hampered progress because the ability to identify neuron types is a prerequisite for determining how specific neurons interconnect and function within the broader auditory circuitry.

Recently, my lab at the University of Michigan tackled this long-standing problem and successfully identified a novel neuron type called VIP neurons. VIP neurons make a small protein called vasoactive intestinal peptide. Despite its name, previous studies have shown that VIP is made by specific types of neurons in several other brain regions.

Our team, led by postdoctoral fellow David Goyer, Ph.D., hypothesized that VIP is a marker for a class of neurons in the IC. To test this hypothesis, we used a genetically engineered mouse to label VIP neurons with a red fluorescent protein. This made it possible to use fluorescence microscopy to target experiments to VIP neurons in the IC. These experiments revealed that VIP neurons in the IC have internally consistent anatomical and physiological features, supporting the conclusion that IC VIP neurons constitute a distinct neuron type. Examination of the neuronal processes of VIP neurons further revealed that individual VIP neurons likely receive input from a range of sound frequencies. Work by collaborators in the

Schofield Lab at Northeast Ohio Medical University showed that VIP neurons also send output to several brain regions, including to higher and lower auditory centers and to a brain region involved in visual processing.

In another set of experiments, we combined electrical recordings from VIP neurons with a technique called optogenetics, which allows scientists to stimulate specific populations of neurons using brief flashes of light. These experiments revealed that VIP neurons receive input from the dorsal cochlear nucleus, one of the first brain regions in the auditory pathway. The path from the cochlea to VIP neurons is therefore quite short, passing through only three synapses.

Sections of the inferior colliculus, the hub of the brain’s auditory pathway. A newly identified neuron type called VIP neurons, which make a small protein called vasoactive intestinal peptide, have been dyed magenta.

This study, which combined both sets of experiments and was published in eLife on April 18, 2019, showed that VIP neurons are a distinct and readily identifiable class of IC neurons. Based on their features, we hypothesize that VIP neurons play a broadly influential role in sound processing. We and the Schofield lab are currently testing this hypothesis, with a particular emphasis on determining how VIP neurons contribute to speech processing in the IC. —Michael T. Roberts, Ph.D.

A 2017 ERG scientist, Michael T. Roberts, Ph.D., heads the Roberts Laboratory and is an assistant professor at the Kresge Hearing Research Institute, University of Michigan.

Support our research: hhf.org/donate

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Picture Your Company Logo Here Learn about Hearing Health Foundation’s HRP research consortium investigating hair cell regeneration to treat hearing loss and tinnitus.

Hearing Health Foundation’s Emerging Research Grants (ERG) fund innovative approaches toward understanding, preventing, and treating hearing and balance conditions.

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meet the researcher

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EMERGING RESEARCH GRANTS

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

Meet the Researcher Babak Vazifehkhahghaffari, Ph.D. Washington University in St. Louis

Vazifehkhahghaffari received a master’s degree in electrical engineering from the Sharif Institute of Technology, Iran, and a doctorate in computational neuroscience from Universiti Teknologi Malaysia. He is a postdoctoral research associate in the department of otolaryngology at Washington University in St. Louis.

Vazifehkhahghaffari’s 2018 Emerging Research Grant is generously funded by The Children’s Hearing Institute.

In His Words while the cochlear implant (CI) allows access to sound for those with severe hearing loss, perceiving pitch and music and understanding speech in the presence of reverberation, multiple speakers, or background noise remains very limited. To improve the CI, it is important to understand how the implant affects neuronal (nerve cell) behavior in the inner ear by uncovering the properties of neuronal excitability. Neuronal excitability mainly depends on the movement of different ions through the cell membrane and is affected by components such as ionic currents and ion channels. a more precise model of the auditory nerve combined with models of the CI electric field potential will help improve CI stimulation methods by understanding stimulus-response phenomena and their underlying biophysical mechanisms.

An improved model will also help us better understand hearing loss mechanisms by detailing the anatomy and electrophysiology of inner ear sensory hair cells. although i started auditory research just a short time ago, I have extensive experience modeling neurodegenerative diseases such as Alzheimer’s and epilepsy. A “wow” moment for me was when I learned that another lab confirmed, via experimentation, my theoretical model of epilepsy, which had been derived from basic research. It convinced me of the importance of multidisciplinary research—models as well as experiments—to understand the mechanisms of neurological diseases.

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as a kid i remember sitting on a park bench and thinking about how to create a model to predict the trembling of leaves in the breeze. That experience may be the cornerstone of my interest in modeling the behavior of nature. i was born in iran and worked in several countries including Russia and Malaysia. My long family name is actually two parts, but for some reason they were put together in my passport. I often abbreviate it as V-Ghaffari.

Babak Vazifehkhahghaffari, Ph.D.’s grant is funded by HHF partner The Children’s Hearing Institute (CHI). We thank CHI for its generous support of innovative research focused on congenital and acquired childhood hearing loss and its causes, assessment, diagnosis, and treatment.

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

Visit hhf.org/how-to-help.

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