Summer 2020 A Publication of Hearing Health Foundation hhf.org
The Noise-Induced Hearing Loss Issue Scientists research hearing conditions caused by loud sounds
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FEDERAL LAW PROHIBITS ANYONE BUT REGISTERED USERS WITH HEARING LOSS FROM USING INTERNET PROTOCOL (IP) CAPTIONED TELEPHONES WITH THE CAPTIONS TURNED ON. IP Captioned Telephone Service may use a live operator. The operator generates captions of what the other party to the call says. These captions are then sent to your phone. There is a cost for each minute of captions generated, paid from a federally administered fund. No cost is passed on to the CapTel user for using the service. CapTel captioning service is intended exclusively for individuals with hearing loss. CapTel® is a registered trademark of Ultratec, Inc. The Bluetooth® word mark and logos are registered trademarks owned by Bluetooth SIG, Inc. and any use of such marks by Ultratec, Inc. is under license. (v2.6 10-19)
The mission of Hearing Health Foundation (HHF) is to prevent and cure hearing loss and tinnitus through groundbreaking research and to promote hearing health. HHF is outraged and deeply saddened by the tragic deaths of Rayshard Brooks, George Floyd, Breonna Taylor, Ahmaud Arbery, and far too many other Black Americans who should still be here today. We grieve for their families, loved ones, and communities. These horrific acts against Black Americans are not isolated, but systemic. HHF stands in solidarity with the Black community. Taking a stand on this issue is not enough. We can, must, and will do better. One of HHF’s core values is inclusivity. We uphold a commitment to all individuals, regardless of race, gender, sexual orientation, religion, age, or ability. We are assessing our organization’s role in systemic racism so that we can work toward dismantling racial bias. With our hearts and minds wide open, we are listening, learning, and ready to proactively help enact much-needed change.
Summer 2020: The Noise-Induced Hearing Loss Issue Whether at work or play, loud sounds affect all ages and can cause permanent damage, such as hearing loss, tinnitus, and/or hyperacusis.
Timothy Higdon, HHF CEO
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HEARING HEALTH The Noise-Induced Hearing Loss Issue
Summer 2020, Volume 36, Number 3
Publisher Timothy Editor Yishane
Lee
Art Director Robin Senior Editors
Features 06 Hearing Health Expanding the Definition of Noise. Daniel Fink, M.D.
16 Living With Hearing Loss Unlocking the Key. Lina A.J. Reiss, Ph.D.
09 Managing Hearing Loss Accessing 911 in a Crisis. Lauren McGrath
18 Research Science Doesn’t Stop.
10 Managing Hearing Loss Communication in the Time of COVID-19. 12 Living With Hearing Loss Why Suffer? Timothy Higdon 14 Living With Hearing Loss Classic Rock to Classical. Jan Schroeder 15 Living With Hearing Loss Action on Captions. James L. Schulz
20 Meet the Funder Problem-Solving a Problem With Volume. Bryan Pollard 22 Research Social Creatures. Bert Gambini. Les Paul in His Own Words. Sue Baker 26 Research Where Science and Art Meet: Jennifer Stone, Ph.D. 28 Research Recent Research by Hearing Health Foundation Scientists, Explained.
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34 Meet the Researcher Kristy J. Lawton, Ph.D.
31 Advertisement Tech Solutions. 33 Marketplace
Higdon, CEO Kidder
Amy Gross, Lauren McGrath
Medical Director David Staff Writer Kathi
S. Haynes, M.D.
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hello@glmcommunications.com, 212.929.1300 Editorial Committee
Peter G. Barr-Gillespie, Ph.D. Judy R. Dubno, Ph.D. 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 Jay Grushkin Roger M. Harris David S. Haynes, M.D. Elizabeth Keithley, Ph.D. Cary Kopczynski Anil K. Lalwani, M.D. Michael C. Nolan Paul E. Orlin Robert V. Shannon, Ph.D. Hearing Health Foundation 575 Eighth Avenue, #1201, New York, NY 10018 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
Emerging Research Grant was generously funded by the Les Paul Foundation. For more, see page 22.
Hearing Health magazine (ISSN 2691-9044, print; ISSN 2691-9052, online) is published four times annually by Hearing Health Foundation. Copyright 2020, Hearing Health Foundation. All rights reserved. Articles may not be reproduced without written permission from Hearing Health Foundation. USPS/Automatable Poly
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Cover Micheal Dent, Ph.D., with her daughter Avery. Dent’s 2019
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EXPANDING the Definition of Noise In the interest of our hearing and overall health, let’s reexamine what “noise” really means. By Daniel Fink, M.D.
Evidence-Based Noise Levels Affecting Health
30–35 dBA
Sleep disruption, especially if noise is not continuous.
45 dBA
Disturbance of concentration and interference with learning.
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“Sound” and “noise” are used interchangeably in acoustics, electronics, and physics, but have different connotations when applied to listeners. Sound is audible acoustic energy defined as vibrations that travel through the air or another medium that can be heard when it reaches the ears of a person or an animal. Noise is usually defined as unwanted sound. In engineering, noise has the additional connotation of signals that have no meaning and vary over time, whereas sound connotes meaningful signals. A more precise definition of noise is undesired sound—a definition developed by the Acoustical Society of America for the American National Standards Institute and most commonly used in scientific and popular writings. However, this definition needs to be expanded. Definitions give words meanings in both technical and everyday usage. The world appears to be getting noisier. As long as noise is merely defined as unwanted sound, it will be difficult, if not impossible, to reverse this trend. The definition of noise as unwanted sound implies that the perception of noise is purely subjective and that noise is merely a nuisance, ignoring its harmful health impacts. It also implies a value judgment, minimizing the concerns of the person complaining about noise. But desired sounds can also cause auditory damage, and unwanted sound, even if it’s just annoying, can be stressful. Stress causes vascular inflammation, increased cardiovascular disease, and death. That is why I propose an updated definition: Noise is unwanted and/or harmful sound.
The New Secondhand Smoke
Let’s compare noise and secondhand tobacco smoke. Both are simultaneously nuisances and health hazards, but the current definition of noise acknowledges only the nuisance aspect. For most of the 20th century, smoking was viewed as a harmless social habit, and secondhand smoke a mere nuisance. As with noise complaints, those who complained about secondhand smoking had their concerns dismissed. After the first Surgeon General’s Report on Smoking and Health in 1964 established that smoking caused lung cancer, smoking was no longer viewed as a harmless habit. A subsequent Surgeon General’s Report in 1986 documented harm from secondhand smoke exposure among nonsmokers, including cancer in adults and respiratory disease in children. When the Environmental Protection Agency designated secondhand smoke as a Class A carcinogen in 1993, with no known safe lower level of exposure, those complaining about secondhand smoke in restaurants, airplanes, trains, buses, and workplaces were no longer just complaining about a nuisance. They were complaining about a health hazard. Smoking was quickly banned from almost all indoor spaces, and in many
hearing health
cities from outdoor spaces as well, leading to the largely smoke-free world we now enjoy. A new definition of noise that emphasizes the harmful health effects of noise exposure can help us make the world quieter, just as it was made largely smoke-free. According to February 2019 research in the journal Cities & Health, ambient noise levels in cities are high enough to cause hearing loss that is not part of normal aging but largely represents noise-induced hearing loss. Noise damages cochlear synapses, most likely causing the common speech-innoise problem now termed “hidden hearing loss” where people have typical audiograms but can’t follow a conversation in high ambient noise. Even low-level noise exposure may have adverse effects on the brain, according to a 2014 paper in Nature Reviews Neuroscience. As reported in The Lancet and other journals, noise has well-documented, non-auditory health effects, including hypertension, diabetes, obesity, psychological disorders, stress, and cardiovascular disease.
sources: proceedings of meetings on acoustics, acoustical society of america, hearing journal
Nonoccupational Noise Risks
The general perception is that the world is getting noisier, but there is scarce documentation of this in scientific literature. Some specific evidence is available for restaurants. In 1993, the average noise level in restaurants in Dayton, Ohio, was 68.5 A-weighted decibels (dBA). (A-weighting adjusts sound measurements to reflect the frequencies of human speech. A-weighted measurements are almost always lower than unweighted measurements.) In 2018, according to data in the Open Journal of Social Sciences, the average restaurant noise level in New York City was 77 dBA, with bars averaging 81 dBA; 31 percent of restaurants and 60 percent of bars had average noise levels above 81 dBA. While the federal government recommends capping occupational noise exposure at 85 dBA for eight hours, it has issued no guidelines, recommendations, or standards for nonoccupational noise exposure. The only advice for the public regarding nonoccupational noise comes from the National Institute on Deafness and Other Communication Disorders, which states that
60 dBA
Interference with speech comprehension for those with hearing loss.
55 dB
Non-auditory negative health effects.
Remember that the decibel scale is logarithmic. Each 10-point increase (e.g., from 70 to 80 dB) represents a tenfold increase in sound energy but is perceived as twice as loud by humans. So 85 dB of sound has 31.6 times more energy than 70 dB sound. This is equivalent to more than 3,000 percent more energy—not 20 percent more energy, as might commonly be thought.
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70–75 dBA
Interference with speech comprehension for those with typical hearing.
These sound levels are approximate and will vary across individuals. For references, see hhf.org/ summer2020-references.
“long or repeated exposure to noise at or above 85 dB can cause hearing loss.” While this statement is not untrue, it is misleading. As a result, 85 dB became the de facto federal noise exposure standard for the public. It is widely cited by audiologists as the level at which auditory damage begins. It is also used in educational and hearing protection programs and as a volume limit for headphones advertised as safe for children without an exposure time limit. The problem is that without a time limit, 85 dB is not a safe noise level. It is derived from the federal guideline for the workplace, which assumes some exposed workers at the limit will still experience hearing loss. As I reported in the American Journal of Public Health in 2017, the only evidence-based noise level to prevent hearing loss is a timeweighted average of 70 dB over 24 hours. More recently, pediatric audiologist Brian Fligor, Ph.D., and environmental health scientist Richard Neitzel, Ph.D., cowrote a November 2019 Journal of the Acoustical Society of America paper about the hearing loss risk from recreational noise. In a May 2020 New York Times article about increased headphone use among children and teens in the wake of the COVID-19 pandemic and lockdown, Fligor and Neitzel acknowledge the difficulty of assigning numbers to a complex concept, but say a safe limit for most headphone users for an unlimited amount of listening is 70 dB. In addition, in early 2019 the World Health Organization and International Telegraph Union jointly recommended maximum exposures of 80 dB for adults and 75 dB for children, over the course of 40 hours weekly. Remember that the decibel scale is logarithmic. Each 10-point increase (e.g., from 70 to 80 dB) represents a tenfold increase in sound energy but is perceived as twice as loud by humans. So 85 dB of sound has 31.6 times more energy than 70 dB sound. This is equivalent to more than 3,000 percent more energy—not 20 percent more energy, as might commonly be thought. A new definition of noise that includes health hazards can help hearing healthcare professionals in educating patients and the public about protecting themselves from noise. Legislation should be passed at the local, state, and national levels specifying safe noise levels and exposure times for the public, including in restaurants, with signs advising hearing protection and warning labels noting health risks. Noise is unwanted and/or harmful sound.
Daniel Fink, M.D., is the founding chair of The Quiet Coalition; the interim chair of Quiet Communities’ Health Advisory Council; and a former board of the American Tinnitus Association. Fink and Bryan Pollard of Hyperacusis Research Ltd. cowrote the Summer 2015 cover story about nonoccupational noise, and he also shared his own experience with tinnitus and hyperacusis in the Summer 2016 issue, both at hhf.org/magazine. This is adapted from papers in The Journal of the Acoustical Society of America, the Proceedings of Meetings on Acoustics, and the Hearing Journal. For references, see hhf.org/summer2020-references. Share your story: Have you advocated for quieter spaces? Tell us at editor@hhf.org.
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managing hearing loss
Accessing 911 in a Crisis A group of New Yorkers have created a new emergency services app to help people with hearing loss or other communication challenges. By Lauren McGrath Phone calls can be difficult for people with hearing loss, even for those who use hearing aids or cochlear implants, when visual aids like text are unavailable. Emergency phone calls to 911 are perhaps the most dangerous manifestation of this limitation. The ability to communicate with the authorities by text in an emergency is limited to less than 10 percent of the U.S., according to Scott Carlton, the creative director at Saatchi & Saatchi Wellness in New York City. Carlton is one of the creators of Deaf 911, an emergency mobile app that gives people who are deaf or hard of hearing a voice when they need it most. Carlton’s grandmother, with whom he was close, was profoundly deaf. I learned about Deaf 911 during a New York City Hearing Loss Association of America (HLAA) meeting in February 2020, when representatives from NYC Emergency Management addressed individual emergency preparedness and how the city responds during a crisis. We learned the city’s text-to-911 technology, set to start rolling out this summer, would not be fully completed until 2024. Of course, like everyone else in the packed room, we could not have anticipated that just a few weeks later New York City would become the epicenter of the COVID-19 pandemic. It was an audience member, Evelyn Schafer, who informed the room about Deaf 911, which was being tested with a planned launch this fall. Compatible with both iOS and Android technology, the app will facilitate both textto-speech and speech-to-text technology so that a two-way conversation with emergency services is possible. Schafer, a minister at St. Ann’s Church for the Deaf in New York City, is Deaf, uses American Sign Language (ASL), and wears hearing aids. Deaf 911 was jointly developed by Schafer, Carlton, and Delores Hart, Ph.D., an advocate and researcher in the Prelingual/Culturally Deaf field who works with individuals using ASL. (A prelingual Deaf individual is one whose deafness occurred before the full acquisition of language, typically before ages 3 to 5 years old. Members of the prelingually Deaf community utilize American Sign Language as their most proficient and preferred means of communication.) “Members of the Prelingual/Culturally Deaf community have expressed that they have had difficulties in accessing and communicating with EMS, police, and fire. My biggest fear is what this has cost in terms of loss of life and/or health and physical harm,” Hart says.
“At the core of Deaf 911 is the insight that everyone should be given access to emergency services—no matter who they are, no matter what their condition,” Carlton says. After the three came up with the idea, Carlton enlisted a few members of the digital and technical staff at Saatchi & Saatchi, a global communications and advertising agency network, to develop the prototype, including interviewing people with communications challenges about their experiences using emergency services. The app’s creators say Deaf 911 would be the fastest way for a person with hearing loss to contact the authorities. Relay-operator calls can take three minutes or longer and teletypewriting takes more than eight minutes, while Deaf 911 takes just 30 seconds. Part of this is because Deaf 911 has users save their name, address, and telephone number into the app so it can be quickly dispatched to an operator in an emergency. It also includes predictive text to facilitate replies to the most common questions, such as “are you hurt?” Schafer pointed out the app can help people with typical hearing, too, such as those who need emergency assistance but cannot speak out loud, in the case of domestic violence or an active shooter. “At the core of Deaf 911 is the insight that everyone should be given access to emergency services—no matter who they are, no matter what their condition,” Carlton says. As the country navigates the pandemic, efficient, accurate emergency communications are more critical than ever.
Lauren McGrath is HHF’s director of marketing and communications. For more, see deaf911.org. For references, see hhf.org/summer2020-references.
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Communication in the Time of COVID-19 Though social distancing six feet apart and face masks are helping to stop the spread of COVID-19, both public health measures have presented communication challenges for people with hearing loss— and without. In May, Hearing Health Foundation sent out a twoquestion survey to see how our community was coping. With 625 respondents, nearly 90 percent said they are experiencing difficulty communicating. Here are selected responses and advice.
Face Masks
» “Instead of elastics behind the ears, I use a mask with
»
» »
»
ties. Some people wear a baseball cap and sew buttons near their ears. Instead of putting the elastic behind the ears, it is anchored to the buttons.” “I attached the mask elastics to a wide elastic band I had that goes around the head. Also I put the ends of my eyeglass temples under the elastic band, so the only things on the ears are the hearing aids.” “I pull the mask out a little from my face to speak more clearly and hope the person I’m speaking to will do the same.” “I’ve found a face shield that I ordered is better than a mask, because it also covers your eyes and keeps you from touching your face, and protects others from any droplets. Plus it’s more comfortable because it doesn’t scratch your face and keeps your face cooler.” “Face masks with a clear portion or a face shield would help with reading lips, but I would have to ask a stranger to wear them.”
“I pull the mask out a little from my face to speak more clearly and hope the person I’m speaking to will do the same.”
Captioning and Other Technology
» Respondents recommend speech-to-text smartphone
» »
» »
»
» Share your story: Tell us your communication tips at editor@hhf.org.
» »
and/or tablet apps like InnoCaption, Otter.ai, Live Transcribe, and TextHear. Some noted the phone mic may have difficulty picking up the speaker’s voice from a distance, and that the apps may need Wi-Fi for instant captioning and may not work as well outdoors with ambient noise. At home, people are using captioned telephones with captioning services such as CapTel and Hamilton CapTel. Respondents say video calls via Zoom, Skype, Apple FaceTime, and Google Meet are useful for seeing faces and lips. Google Meet has live AI-generated transcriptions, as does Skype, and Zoom is integrating with Otter.ai. FaceTime now also has a group option. Some respondents say they carry a business card or wear a lapel button that says, in large type, that they have a hearing loss. “I carry a notepad in my bag. if someone wants to tell me something and I can’t understand, I ask them to write it down. I carry extra pens, so they can keep the pen if they want after they use it. I also suggest texting, even if we are within earshot.” “Second best to writing things down on paper is using BuzzCards by Sorenson.” BuzzCards are phrases you can set up on your phone to communicate, like flash cards, but it may require being closer than six feet to read. “When my partner and I are out together while wearing our masks, he wears a Mini Mic that streams via Bluetooth into my hearing aids. It’s a big help!” “To make sure I understand, I repeat back what I think I’ve heard—sometimes leading to big laughs!” “SMILE! It can be seen in your eyes!”
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A NEW SOLUTION FOR
SINGLE-SIDED DEAFNESS DO YOU STRUGGLE TO FOLLOW CONVERSATIONS AROUND A NOISY DINNER TABLE? DO YOU HAVE TROUBLE TELLING WHERE SOUNDS ARE COMING FROM? IS YOUR COMMUNICATION WITH YOUR LOVED ONES SUFFERING?
LIVING WITH SINGLE-SIDED DEAFNESS IS NOT EASY. If you answered “yes” to those questions, you may benefit from a new solution that could change your life. The SYNCHRONY Cochlear Implant from MED-EL is the world’s first and only cochlear implant FDAapproved for single-sided deafness. Studies show1 that SYNCHRONY can help listeners hear speech in noisy environments and tell where sound is coming from.
“
Even though I had one good ear, all of the music that I loved and listened to every day was like it was in another room. And I was locked out of that room. Now I can enjoy the feeling of being immersed in music once again. SIMON M.
Recipient and music lover
About three months after getting my implant, my wife said, You’re back to your normal self. I felt that freedom again. It was as much a life-changing, positive experience as losing my hearing was a devasting one. IT IS A REMARKABLE GIFT. RICHARD S. Recipient and avid cyclist
FIND OUT IF SYNCHRONY IS THE RIGHT SOLUTION FOR YOU.
”
Contact a MED-EL expert today. Visit go.medel.com/ssd-us
1 Dillon MT, et al. Effect of Cochlear Implantation on Quality of Life in Adults with Unilateral Hearing Loss. Audiol Neurotol 2017;22:259-71. For information on potential risks and contraindications relating to implantation, please visit www.medel.com/us/isi
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Why Suffer?
Noise-Induced Hearing Loss
The use of hearing aids should be no more of a stigma than self improvement through braces or glasses—and equally as commonplace. By Timothy Higdon
HHF CEO and jazz pianist Timothy Higdon performed last winter at Columbia University in New York City.
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I started learning piano in the 3rd grade, at church with the organist. I kept it up through high school, taking lessons at Butler University in Indianapolis, and while at college at Indiana University, I took music courses at the university’s school of music. During college, I ended up changing course and joining the military, where I served as a combat engineer officer with the U.S. Army Corps of Engineers for 17 years, including in Germany and South Korea. I did a lot of demolition work, military exercises using an armored vehicle, which was pretty much just a diesel engine with four wheels attached. When I left the service, I started my career in sales and marketing at Biomet and IBM, and then switched to the nonprofit world. It was about five years ago, living in New York City, that I came back to piano, taking lessons in jazz piano at Columbia University. Up until then it had all been classical, but I loved listening to jazz and wanted to learn how to improvise on the piano. Classical music is all about reproducing as closely as possible the score as it’s written by the composer, while in jazz you take what is called the lead sheet as a mere suggestion and are encouraged to riff and improvise freely. Jazz piano is like completely learning a new language—how to play all the chords, and studying music theory and jazz music theory to understand how tunes are structured. My goal was to not only learn how to improvise but also to be able to play in an ensemble. I try to practice 12 to 14 hours a week. Last winter I was thrilled to perform at Columbia, and we would have again this spring but then the COVID-19 pandemic reached the U.S. A few years ago I was also fortunate to be able to participate in “jazz camp” for two different years—a real thing for adults, a magical week in Rhode Island that was another amazing experience. When I was discharged from the military, part of the standard physical is a hearing test, and it showed I had a mild, mid-range hearing loss. Over the past two to three years, I have noticed an intermittent tinnitus, and that living in New York City and socializing with friends and colleagues in restaurants or going to the theater or movies has become increasingly difficult. I noticed I was more sensitive to loud places, and the spaces themselves were becoming louder, too. My career to a large extent has been as a professional talker, and I love the conversations and interactions I have with a diverse range of interesting people. But I could tell it was increasingly difficult for me to hear in social settings. I’d cup my hand by my ear and ask again
living with hearing loss
My career to a large extent has been as a professional talker, and I love the conversations and interactions I have with a diverse range of interesting people. But I could tell it was increasingly difficult for me to hear in social settings. what was said, but then eventually I stopped asking people to repeat, instead just nodding my head and missing important parts of the conversation. At times, I would stop trying. It was just getting too hard. Then one day practicing piano I thought that the upper register was not functioning, or that something else was wrong with my upright. I even had the piano tuner come look at the piano to ask him about the high C not being audible. He didn’t know what I was talking about. Finally, and especially as the CEO of Hearing Health Foundation (HHF), I knew I had to get my hearing tested. And frankly when the results came in, even with all these telltale signs, I was very surprised at how much my hearing loss had worsened. I was immediately fitted with a pair of ReSound hearing aids, and my audiologist Shelley Borgia, Au.D., CCC-A, at NYC Hearing, was careful to only give me so much sound at a time. She had me visit three times over two and a half months in order to reacclimate me to all the sounds of everyday life without giving me sensory overload. It was like Dorothy in “The Wizard of Oz” going from black and white to full color. With the piano I can hear all kinds of new levels, and the first time at a restaurant when I was able to hear the person I was dining with—in a loud setting—was wonderful. I’ve been very pleasantly surprised that I can so easily adjust the programming on my hearing aids using a smartphone. Hearing aids these days are so comfortable and invisible that I sometimes forget to remove them before a shower or bath, and they occasionally end up down my shirt if I fall asleep with them on (which puts me in a real panic!). I am also still trying to figure out the best way to wear a face mask with them on. If hearing aids are not working for you, it’s possible you are working with outdated technology. But more than that it’s so important to find an audiologist you work well with and who understands your needs and goals—those of your wallet as well as your hearing environments and lifestyle. If you have a hearing loss, why suffer? The price you pay for not fully hearing well is far, far more than the perceived vanity lost from wearing hearing devices. This type of health benefit should be no more of a stigma than self improvement through wearing braces or glasses, and equally as commonplace. It’s made such a difference to be able to fully function professionally and socially that—as HHF has long promoted—I am proud to be a hearing ambassador, evangelizing to both my personal and professional contacts: Get your hearing tested regularly, and if needed, treated. I promise you’ll have no regrets.
Timothy Higdon is the CEO of Hearing Health Foundation.
Share your story: Tell us your hearing loss journey at editor@hhf.org.
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Classic Rock to Classical
Noise-Induced Hearing Loss
Jan Schroeder with her son Bryan, who plays violin with the Albuquerque Philharmonic Orchestra.
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By Jan Schroeder
When I was in college, I saw the British rock band Jethro Tull in concert in Illinois, and was enthralled and starstruck as I listened to their music blasting through massive speakers only a few feet away from me. Although the sound was a freight train roaring through my ears, the distortion and pain were not enough to budge me from my spot in front of Ian Anderson and his electric flute. My ears rang for three days after that concert. Then at age 42 the tinnitus came back as a constant cacophony of clanking cowbells and rushing water. The audiologist diagnosed me with a severe sensorineural hearing loss in both ears. I cried for days. Sounds were muffled, but worse than that, lacked clarity, as I became accustomed to using hearing aids. I smiled and nodded to missed parts of conversations, pretending to understand when I did not. The struggle to hear and fully function in the hearing world threw me into exhaustion and anger. Surprisingly to me, the audiologist and the ENT both could not say for sure the long-ago concert was the cause of my hearing loss. Now at age 65, I’m on my fourth pair of hearing devices, featuring the latest technology. My audiologist and I are still experimenting to find the most accurate settings for warmth and clarity and also discovering just how intricately these devices can be adjusted to assist my hearing. These rechargeable Oticon hearing aids include the ability to stream using wireless Bluetooth technology that can be synced to my iPhone, and it’s an incredible way to listen to my favorite music. The sound is rich and full. Yes, I still love music, although these days it’s more classical than classic rock. My son Bryan plays violin with the Albuquerque Philharmonic Orchestra, and recently I attended one of his concerts with his older brother Eric. The performance featured a virtuoso solo violinist, and the music was beautiful, the solo outstanding. I noticed, however, that when the soloist and the orchestra played together at roughly equal volume, I could not hear the violinist’s instrument; the sound melted away. When the orchestra played softly in the background or paused, then the sound from his 19th century violin stood out clearly. The tone was so rich that I felt it resonate through my body. After the concert, I said to Eric, “There should have been a microphone for the soloist’s violin. Or maybe he could have used an electric violin, so we could have heard his performance better.” Eric’s expression led me to ask, “Could you hear the soloist’s violin over the orchestra?” He nodded. I was astonished at this new insight into my hearing loss. Apparently, instead of sound being flat like an audiogram printout, it has depth—a thirddimensional quality, like a sculpture, that allows more complex sounds. Significant hearing loss seems to flatten that depth back to a single dimension, which meant that I could not distinguish the sound of one violin separately from the orchestra. I took heart, though, as I’ve grown to trust that hearing devices are potentially only steps away from replicating that third-dimensional capability. After a journey of 23 years of hearing loss, I am grateful to hear as well as I can and for the technology for me to do so.
Jan Schroeder and her family live in New Mexico.
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living with hearing loss
Action on Captions By James L. Schulz
I am a longtime radio broadcaster, the bright, good-morning voice at a number of Wisconsin area stations. After 25 years on the air, I owned an advertising agency where the work was creating radio and TV ads and video scripts all day, every day. So I’ve spent nearly my entire adult life in a studio, wearing headphones. Now, in the latest iteration of life careers, I’m back to playing music (guitar) and singing live in a variety of venues—and paying an awful price for my poor hearing choices early on. I know the broadcasting and the studio work contributed mightily to the hearing loss I now experience. In the Fall 2019 issue of Hearing Health, I read an article on captioning and decided to file a complaint with the Federal Communications Commission (FCC) about a national cable network, Charter/Spectrum, that never captioned any of its programming. Actually, I tried calling my local cable company first. Unable to get through, I followed the advice in the article and went to the FCC website. The procedure with the FCC was easy and intuitive. I handled the entire thing online and was kept abreast of progress via email. I received a call from the cable company a few weeks later. They wanted to be sure that the problem wasn’t my TV (or me!). After a few questions and answers, the cable technician was able to figure out that this network’s programming was in fact captioned, but only in standard definition. Since these days TVs are normally set to high definition by default, it just didn’t show up on this channel. Though it was a simple, three- or four-step process to switch to standard definition for this channel in order to see its captions, and then to reverse the steps to return to high definition for every other channel and their captions, it was rather cumbersome. Hardly worth the effort. The cable tech told me he’d pass on the info “back at the office,” and to help him get some traction I suggested that he mention that the query was in response to an FCC investigation. I thought that would be the last of the matter but, a few days later, I happened to turn on that old movie channel and, lo and behold, it is captioned full-time now! A small victory perhaps, but I’m feeling good that I made a difference. The lack of captioning on that network may have been the result of a technical misunderstanding or simple oversight. (And it’s true that after it was fixed, it inexplicably disappeared at one point, so I had to call again to get it restored—it pays to be viligant!) In any case, I’m enjoying some of those old movies again. The first captioned movie I watched on that channel was “Topaz,” part of an Alfred Hitchcock marathon. I was in heaven! It has been empowering to actually make a difference for people with hearing loss.
James Schulz, whose noise-induced hearing loss likely stemmed from the workplace, encouraged a local TV network to caption its content.
James L. Schulz lives in Waupaca, Wisconsin. To learn more about his music, which he performs under the professional name Jim King, see facebook.com/Jim-King-Show-1282642051745937.
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living with hearing loss
hearing health foundati o n
Unlocking the Key After experiencing balance problems in her 40s, an Emerging Research Grants scientist discovers the cause of her childhood hearing loss. By Lina A.J. Reiss, Ph.D. I became interested in the fields of hearing research, biomedical engineering, and neuroscience in part from my own hearing loss. I have had a severe to profound hearing loss since early childhood. Diagnosed at age 2 ½ and fitted with powerful hearing aids, I learned to speak and listen only with intensive speech therapy. I am the only person with a hearing loss in my family, so we always thought the cause was due to illness or possibly ototoxic medications. In my early 40s, I started having vestibular (balance) problems. It started in November 2018, when we took a big family fishing trip on the Amazon River in Brazil. It involved four flights each way, with the last leg a few hours in a small, unpressurized aircraft. On landing I felt a sensation of fullness and muffled hearing in my left ear that took several minutes to resolve, and a few days later, I noticed some mild imbalance walking around. It was on the return trip when I again experienced problems with my left ear on landing. I was unable to walk straight in the airport, and head movements, especially nodding, caused disorientation. Based on these symptoms, my otolaryngologist and colleague Yael Raz, M.D., and I thought I had benign paroxysmal positional vertigo (BPPV). A common vertigo triggered by movement, BPPV occurs more often with age and is attributed to calcium crystals that get dislodged and move more slowly than fluid through the inner ear’s semicircular canals. I did the recommended treatment for BPPV, the Epley maneuver, and symptoms gradually disappeared after a month.
More Flights, and Imbalance
In the months afterward, I had two other (unavoidable) cross-country flights that put the BPPV diagnosis in question. Now my vertigo was triggered by head nods as well as head turns—and then also head tilts. Walking around stairwells or turning my head to see someone’s face while walking was challenging. I started touching the wall at intervals to keep my balance. Then a new vestibular symptom appeared. In response to loud sounds—especially low-frequency sounds like my husband’s voice—I felt the room start to spin slowly. While disconcerting, this indicated to me that my vestibular cells could now respond to sounds, which would normally only be detected by auditory hair cells. We thought I had superior canal dehiscence (SCD), 16
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discovered by Lloyd Minor, M.D., at Johns Hopkins University in the 1990s, coincidentally while I was a graduate student there. SCD is essentially a hole in the superior semicircular canal, leading to a “third window” (alternate pathway) for sound transmission through the vestibular system that would explain balance responses to sound. This was worrying because SCD often gets worse over time, and outcomes with cranial surgery are not good. But, unlike me, individuals with SCD usually have typical hearing.
Uncovering the Source
When a CT scan, confusingly, came back negative for SCD, Dr. Raz said, “Lina, I think we may have learned the cause of your deafness. You don’t have SCD, but you do have an enlarged vestibular aqueduct, or EVA.” Dr. Raz was also at Johns Hopkins, a resident working with Dr. Minor. She said they had talked about looking at the third window effect in patients with EVA and that it was likely, though there was no published data, that EVA also acts like a third window syndrome, similar to SCD. The EVA itself is not necessarily the cause of the hearing loss but is associated with a mutation of the gene SLC26A4. A 2017 eLife paper discussed how this may
living with hearing loss Opposite page: Before vestibular rehabilitation therapy, Lina Reiss found mountain biking extremely challenging. This page: The four flights to Brazil for a family fishing trip on the Amazon triggered Reiss’s balance issues.
cause EVA by changing how the electrolyte sodium chloride, and thus fluid, is reabsorbed. This affects the development of the inner ear, and possibly plays a role in regulating endolymphatic pressure, which can affect hearing and balance. The diagnosis fit with my progressive hearing loss as a toddler and my adult vestibular symptoms. I realized my imbalance was more like a milliseconds-long “lag” in balance orientation. In contrast, the vertigo in BPPV is described as a slower, room-spinning sensation for 30 to 60 seconds. Also, people with EVA sometimes experience sudden hearing loss after flying, so sudden balance problems after flying are plausible. Genetic testing confirmed I have two different mutations in the SLC26A4 gene. This is consistent with the severity of my hearing loss and my South Korean ancestry, as heterozygous mutations in SLC26A4 are more common in East Asian populations.
After Diagnosis, Treatment
My imbalance issues, while not causing nausea, had a profound, disabling effect on my ability to concentrate, focus, and be productive. Finally after a few months, it had become tolerable, but I still had movement errors. I could ride my bike as long as my head wasn’t moving relative to my body, but if I stopped, turned my head to look for cars, and tried to turn left, I would end up going right instead. I would bend to pick up overripe apples in our yard, twist to throw them in the compost bin, and fall down. I started vestibular rehabilitation therapy using classic exercises, such as focusing on a single letter while moving my head, and proceeding to walking with head turns. It took six months for me to gain small improvements to do this without losing my balance. I moved on to a dynamic posturography machine, which involved standing in a phone booth-like box and trying to maintain my center of gravity while the walls and floor moved, sometimes in different directions. And I began using virtual reality goggles: Watching my husband steer a plane, I kept falling backward every time upward movement was simulated. But after just a few weeks, these simulations ended up being dramatically effective and efficient, compared with the classic exercises. My vestibular function came back to what it was. I also recently got a cochlear implant. In Facebook groups I had joined to learn more about balance problems, I saw that many go undiagnosed and untreated, and even with EVA—one of the most common genetic causes of childhood hearing loss—very little is known, including triggers and susceptibility. My hope is that more research will help us understand all the various types of balance conditions.
In response to loud sounds—especially low-frequency sounds like my husband’s voice—I felt the room start to spin slowly. While disconcerting, this indicated to me that my vestibular cells could now respond to sounds, which would normally only be detected by auditory hair cells.
Lina A.J. Reiss, Ph.D., is an associate professor of otolaryngology–head and neck surgery, and of biomedical engineering, in the Oregon Hearing Research Center at the Oregon Health & Science University’s School of Medicine. A 2012–13 Emerging Research Grants scientist, she wrote about hybrid cochlear implants in our Winter 2014 issue, at hhf.org/magazine. For references, see hhf.org/summer2020-references.
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Science Doesn’t Stop
>> >> >> For Hearing Health Foundation researchers, the nationwide COVID-19 lockdown presented challenges as well as opportunities for creativity.
“In order to keep our work going at home during the pandemic lockdown, I set up my music room into a microscope room. The music stand makes a handy slide display for my Emerging Research Grants (ERG) project investigating hyperacusis. Also taking place in my musical microscope room are recordings for our #QuarantineBand performances that we record and upload to YouTube.” —2019 ERG scientist Gail Seigel, Ph.D. (above), University at Buffalo, State University of New York
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“Although the pandemic effectively put a halt to all experimental work in my lab, it struck at a time where we had just finished the final round of our two-photon imaging experiments, generously funded by my Emerging Research Grant. Given the large datasets we collect in these experiments—hundreds of gigabytes— data analyses often take longer than data collection! So my team and I have been performing our data analyses remotely, from the comfort of our own homes, with more than enough data to keep us busy for a while.” —2019 ERG scientist Pierre Apostolides, Ph.D., Kresge Hearing Institute, University of Michigan
“I am tremendously grateful that I am able to tell my postdocs who receive funding through our HRP/HHFfunded project that they do not have to worry. Most of our lab members do not have local family ties—their families are far away, out of state, and in other countries. The personal level of stress that some individuals have to deal with at this time is truly unprecedented. All of our funding agencies have been exemplary in supporting us, which provides important financial stability, security of a place to live, and an environment where working from home is actually possible. Thank you and HHF’s supporters for this!” —Hearing Restoration Project member Stefan Heller, Ph.D., Stanford University
photo credit: amanda janesick, ph.d., in the lab of hrp member stefan heller, ph.d.
Hearing Health Foundation (HHF) improves the lives of people with hearing loss and related conditions by funding research, connecting researchers, publicizing new findings, raising awareness about hearing loss, and promoting hearing health. Complex and interconnected, hearing loss and its variants impact speech processing, brain function, cognition, interpersonal relationships, psychological well-being, and quality of life. HHF’s advances in one aspect or disease-specific area of hearing often benefit many others, creating pathways to better prevention methods, treatments, and cures. » Fundamental studies of inner ear hair cell function
may shed light on these mechanisms: how hearing loss occurs, how balance is disturbed, how tinnitus is generated, how the brain processes sound and speech, and how cognitive decline accelerates. As a consequence, advancing knowledge in one area of research benefits many related areas and promotes novel therapies.
» Breakthroughs in understanding sensorineural
hearing loss also enlighten studies on tinnitus, hyperacusis, and auditory processing disorders because the biological systems that are involved— the inner ear, the brainstem, and parts of the brain such as the auditory cortex—are shared.
» Developing new means to deliver therapeutic drugs
to the inner ear across the blood-labyrinth barrier may lead to discoveries about that barrier that help scientists prevent damage to hearing as a side effect of other drugs (ototoxicity) or of infection.
» Work on the role of neural circuits in the auditory
processing of speech may improve our understanding of hyperacusis, as these same neural circuits play a role in the brain’s sensitivity to sound.
» Discovering how to regenerate inner ear hair cells
to restore hearing may also help medical researchers and clinicians treat tinnitus, which is often caused by these hair cells “mis-signaling” the brain.
meet the funder
hearing health foundation
Problem-Solving a Problem With Volume By Bryan Pollard
Bryan Pollard (above right) with University at Buffalo professor Richard Salvi, Ph.D., a six-time Emerging Research Grants recipient who is a mentor and scientific adviser to Hyperacusis Research Ltd.
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My own background with experiencing hyperacusis started a little over a decade ago, ironically from a situation where I was trying to make things safer at my home. I had a tree growing partially over the house, and I was concerned that it might come down on the house during a storm. It was quite large, and on the day it was to be cut down I was at home from work to help make sure it wouldn’t hit the house. After the tree trimmers used chainsaws to cut it down, I was quite relieved that it was down safely. I thought the most dangerous part of the work was done. I assumed they would start cutting up the large chunks of the tree to put on a truck, but they instead used this huge, industrial-sized wood chipper to begin chipping up the entire tree, including the large chunks. I remember thinking, “I don’t think I’ve heard anything that loud in my life!” I stood watching it for a few minutes and walked right by the wood chipper to go have my lunch. Little did I know there was significant damage that had already occurred. After a few days I began experiencing various symptoms, including ear pain, which I learned later is a typical trajectory for hyperacusis patients. Using the usual auditory exams, the ENT could find no detectable damage, and I began visiting other audiologists to find help. None of them even mentioned hyperacusis. I discovered the condition myself after searching online using my symptoms. “Hyperacusis”—or hypersensitivity to sounds— captured what I was experiencing. Eventually I found someone 45 miles away who offered the standard Tinnitus Retraining Therapy (TRT) for hyperacusis. I kept with it for over five years, with some modest improvement. My hyperacusis symptoms were fairly typical. When I was exposed to what used to be previously tolerable sounds, my ear would start throbbing but, confusingly, it wouldn’t always happen right away. In fact, sometimes I would think, “Oh, that situation didn’t really bother me,” but after lying down at night my ear would start throbbing. I bought a decibel meter (smartphone apps weren’t available as much at the time) to start noting the decibel levels of my environment to see if they correlated with my symptoms, which included not only throbbing but also ear fullness and tinnitus. I saw that my symptoms would spike in relationship to my surrounding noise levels. My career as an engineer at a high-tech–oriented company was one of solving technical problems. I had studied a number of innovative problemsolving methodologies, including a pattern database. As I started approaching my hyperacusis as I would at work, what became evident to me was that there was no systematic assessment in the research community of the nature of what patients were experiencing. Basic publications about hyperacusis were pretty thin, especially from an in-depth technical perspective, so I started emailing researchers directly. Rather than describing myself as a patient, I reached out saying that I had been trying to understand a particular aspect of their research. I was able to connect with David Mountain, Ph.D., at Boston University, a renowned
meet the funder
researcher who has since passed away. Since he had an electrical engineering background, as I do, and lived close by, I was fortunate to meet him in person. Although he had never studied hyperacusis directly or even heard about it, he had in-depth knowledge of basic auditory functions. Fascinated by this condition and what I described to him, he became a strong partner and advocate and gave me the basis to begin problem-solving. It became clear that if we wanted to significantly influence the research, we needed to create a more formal method to create a coalition of forces that could start to affect what was being researched. A nonprofit became the obvious choice, so we founded Hyperacusis Research Ltd. There was no debate on the name. We knew that unlike other organizations that cover every single dimension of a health condition—the patient side as well as the clinical and research side, a broad basis—we’d have just a small band of volunteers. In those early days we intentionally focused solely on the research dimension in order to make a direct impact.
My career as an engineer at a high-tech–oriented company was one of solving technical problems. I had studied a number of innovative problemsolving methodologies, including a pattern database. As I started approaching my hyperacusis as I would at work, what became evident to me was that there was no systematic assessment in the research community of the nature of what patients were experiencing. What really became our platform for greater influence was our partnership with Hearing Health Foundation (HHF). We were connected through a New York City physician who had hyperacusis. We learned that HHF is the largest nonprofit U.S. funder of hearing and balance disorders, and our collaboration has enabled the research
dollars we obtain to reach a much larger audience through its Emerging Research Grants (ERG) program, through which scientists can gather preliminary data to support an application for a larger grant. In fact one of the leading scientists we have worked with is 2012 ERG recipient Richard Tyler, Ph.D., whose comprehensive 2014 literature review of hyperacusis studies provided the basis for identifying hyperacusis subtypes and noting that they are often variable and co-occurring. The partnership with HHF has given us name recognition in the scientific community while ensuring that we are funding the most promising hyperacusis research. Having collaborated with HHF since 2011, we have also followed the progress of HHF’s Hearing Restoration Project and the many different dimensions that sensory hair cell regeneration covers, from a better understanding of notch-signaling pathways to genetic reprogramming techniques. All are part of what it’s going to take to develop successful hearing restoration. We are excited to learn more about relationships between loudness and pain. In fact I coined the term “noise-induced pain” from the term “noise-induced hearing loss” to help the entire auditory research field understand this is what we’re attempting to uncover. Understanding the pain mechanism, whether it’s certain nerve fibers and/or an inflammatory response, or something else, will help us devise better treatments and find relief.
Bryan Pollard is the founder of Hyperacusis Research Ltd., at hyperacusisresearch.org. Hearing Health Foundation is very grateful for Hyperacusis Research’s longtime support since 2011 of our Emerging Research Grants program, funding seven researchers investigating the mechanisms, causes, diagnosis, and treatments of hyperacusis and other forms of loudness intolerance. For more, see hhf.org/erg. This is adapted from the “Tinnitus Talk Podcast,” produced by the U.K.-based patient organization Tinnitus Hub. For more, see tinnitushub.com and tinnitustalk.com.
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research
EMERGING RESEARCH GRANTS
Social Creatures 2019 Emerging Research Grants recipient Micheal Dent, Ph.D., discovers a surprising factor that can skew research results. By Bert Gambini
Socialization is critical, says Micheal Dent, Ph.D., for humans and animals alike.
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Animal models can serve as gateways for understanding many human communication disorders. Insights into the genetic paths possibly responsible for conditions such as autism and schizophrenia often begin by studying acoustic behavior in mice. But a September 2019 eNeuro study from the University at Buffalo, the State University of New York, suggests that the established practice of socially isolating mice for such purposes might actually make them poor research models for humans, and a simple shift to a more realistic social environment could greatly improve the utility of the future studies. That’s because how mice are raised affects how they hear in the real world, according to Micheal Dent, Ph.D, a professor in University at Buffalo’s department of psychology and coauthor of the paper with Laurel Screven, a postdoctoral research fellow at Johns Hopkins, who was a University at Buffalo graduate student when the research was conducted. Socialization is critical, according to Dent. “We need to raise our animals in more naturalistic situations since it turns out that it has an effect on their acoustic communication, including how they hear and how long it takes to train them on a behavioral task,” says Dent, an expert in the perception of complex auditory stimuli in birds and mammals. “Normally when we do these studies, we isolate the animals for their entire lives,” she adds. “This is not a good model for humans because we’re creating these odd worlds for the mice. It’s not natural. Having the mice live together changes their perception of vocalizations, so clearly it is important.” The findings of the eNeuro paper began as a curiosity Screven expressed to Dent, her dissertation adviser at UB. Screven was interested in the effects of social experience on acoustic communication in mice. Previous research demonstrated that when female mice have babies, their neural responses to calls, or ultrasonic vocalizations, change. Their response depended on whether or not the mice had pups. Screven wondered if the social experience of vocalizations somehow changes the composition of the brain, and changes the composition of the auditory areas of the brain. It was a possibility that had not been previously studied. “We can’t tell what kind of neural effects are taking place from our behavioral research, but what we can say is that we should not be isolating mice,” Dent says. “We should put them together in order to create a more realistic situation, one that’s more applicable to human communication. Knowing how to raise and care for these animals can improve research on human communication.” For their study, Dent and Screven first trained mice to poke their noses through one hole to start a repeating vocalization and then to poke their noses through a different hole when they heard a different vocalization. Mice emit ultrasonic vocalizations (USVs), which vary in frequency, duration, and intensity.
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“Just the finding that the mice train faster when they live together is important for anyone in my line of research wanting to get the data out faster,” Dent says. The collective differences in these characteristics is the call’s “shape.” The shapes perceived by the mice are similar to how humans hear different words. The researchers found that the socially isolated mice required significantly more time to learn to discriminate between the USVs than the socially housed mice, and they used different aspects of the USVs to do it. “The goal of the research in our lab is to first establish the baseline acoustic communication behavior of the mice so in the future we can start understanding communication in mice with genetic manipulations,” Dent says. “If we look at the genes found in humans who stutter, for instance, or have high frequency hearing loss, or accelerated age-related hearing loss, we can see what happens when we knock out those same genes in the mice,” she says. “Eventually, we can attempt to ‘fix’ the disorders in mice, leading to possible treatments for humans.” For Dent, the findings are immediately applicable and she says the next step in her research will be to house mice together in future experiments. In mice, not having regular social contact resulted in abnormal communication skills. “Just the finding that the mice train faster when they live together is important for anyone in my line of research wanting to get the data out faster,” Dent says. “But I also think that creating a more natural living situation for the mice will make the results of these laboratory experiments more relevant for human communication and studying how humans process vocalizations.” And now, during lockdowns caused by COVID-19, Dent adds, “We could easily see similar issues in some or all of the humans socially isolating during the coronavirus pandemic. Like mice, we humans need live interactions on a regular basis.”
Honoring Les Paul’s Legacy Hearing Health Foundation (HHF) is grateful to the Les Paul Foundation and its trustees for their ongoing and generous support of tinnitus research through HHF’s Emerging Research Grants (ERG) program and the Hearing Restoration Project (HRP) since 2013. The 21 researchers the Les Paul Foundation has funded so far have made impressive strides toward a better understanding of tinnitus, as well as developing treatments and therapies that can benefit anyone affected by this condition, including and especially musicians. HHF looks forward to continuing our relationship with the Les Paul Foundation and honoring the legacy of Les Paul, the musician, writer, and inventor. With 90 percent of tinnitus patients also experiencing hearing loss, funding this work is critical to improving quality of life. We are honored to share in Les Paul’s incredible legacy, and the alignment of our missions ensures that millions will be helped by the discoveries made through sustained funding. We appreciate the Les Paul Foundation’s commitment to a tinnitus cure and its trust in HHF’s rigorous grant review process. —Gina Russo
This originally appeared on the website for the University at Buffalo, the State University of New York, at buffalo.edu. Micheal Dent, Ph.D., is a psychology professor at University at Buffalo. Her 2019 Emerging Research Grant was funded by the Les Paul Foundation. For more, see hhf.org/erg and lespaulfoundation.org. For references, see hhf.org/summer2020-references. summer 2020
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Les Paul in His Own Words
By Sue Baker
Guitar legend Les Paul had a hearing loss resulting from being hit accidentally on each ear, years apart.
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Les Paul, who was born on June 9, 1915, and died in 2009, was famous for the solid body electric guitar and many innovations related to recording music. He also had compromised hearing, and wore hearing aids in both ears. Although his hearing loss was not caused by loud noise, almost all of his rock musician friends have had some level of hearing loss from performing and being around loud music. Les and most of his musician friends also have had tinnitus. In his autobiography, “Les Paul in His Own Words,” Les shares how he lost his hearing: “I was very fortunate with my new Gibson deal, but my luck went the other
operating tomorrow.’ … [After the surgery] to my great relief, the constant pounding stopped. Buddy Rich was gone, but so was most of the hearing in that ear, and there was no getting it back. “And then, unbelievably, a very similar thing happened [a few years later] with the other ear when… a guitar player I knew came up behind me to do the ‘guess who?’ thing. Somehow, his hand slapped over my good ear, and I heard the eardrum pop just like before. I didn’t say anything to the guy, never did tell him, but I excused myself and went to my room and just cried because now both ears were blown, and both by accidents where a friend just accidentally hit me in the ear. “I had to have a total of five operations on my inner ear and eardrums. My hearing was permanently impaired, and I’ve been dependent on hearing aids ever since. And one of the things I’m working on now is finding ways to improve the hearing aid.” The book was published in 2006, and Les continued his work on hearing aids until he passed away at age 94. The Les Paul Foundation continues Les’s search to improve hearing through annual funding to Hearing Health Foundation’s Emerging Research Grants program to find a cure for tinnitus.
way when an old friend who came to visit playfully cuffed the side of my head with his open hand.… He didn’t hit me hard, but his open palm just happened to clap over my right ear, and the sudden pressure popped my eardrum. And it was very bad because an infection got started and developed into mastoiditis, which creates a lot of rotten wood and swelling that puts pressure on the brain. “I was left with a constant pounding, like I had Buddy Rich in my ear. This was driving me mad [so] I went to Dr. Moore, an ear specialist at New York Hospital, who took one look and said, ‘We’re
Sue Baker is the Les Paul Foundation’s program director. In addition to supporting tinnitus research through HHF’s Emerging Research Grants program, the Les Paul Foundation also supports grassroots efforts to encourage young people to protect their hearing. Print and video resources are available at lespaulfoundation.org/education. For more, see hhf.org/erg.
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Where Science and Art Meet The Hearing Restoration Project’s Jennifer Stone, Ph.D., shares how she pivoted from studio art to neuroscience, combining two passions. The path from student to researcher is not set in stone. Most researchers take the more traditional route where they choose a field during their undergraduate years and keep with it through master’s and doctoral degrees. Then, there are others who happen upon the field and, unsuspectingly, fall in love with it. Jennifer Stone, Ph.D., a research professor at the University of Washington, falls into the latter category. A self-described “prolific drawer” as a teenager, Stone started her college career as a studio art major at Skidmore College in New York. Stone switched to biology after taking an introductory course she was dreading, after uninspiring experiences in high school. She found herself connecting with the professors in the department and realized biology’s potential as an outlet to continue to express her artistic side. “One of the earliest projects I was involved in was doing scientific illustration with insects,” Stone says. She went out in the winter and collected insect nymphs from the brook near her house, then sketched them at college during January term. Stone says she felt a connection with the faculty and biologists with whom she had studied or worked with and felt a sense of support. “I learned that biology was a nice intersection between arts and science,” Stone says, “and I had really strong interactions with women biologists at Skidmore. Both of these features reinforced my love of biology.” After college, she took two years to explore biomedical research, working in a lab that was studying development of the brain. At that time, her life took another turn— toward neuroscience. After finishing graduate school at Boston University, Stone settled at the University of Washington. Stone conducts research on the sensory hair cells of the inner ear, which she calls the most beautiful cells in the body— 26
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and continues to dabble in art, incorporating illustrations into her papers and teaching. Today, Stone’s research is focused on regenerating hair cells in mammals during adulthood. Hair cells are required for hearing and balance because they are specialized to sense sound waves and head motions. Hair cells die as we age and when we are exposed to ototoxic drugs or high noise levels. Hearing and balance deficits arising from hair cell loss are widespread, particularly in elderly people. Both types of sensory deficit make it hard to socialize and can lead to isolation. Hearing aids amplify sounds in the cochlea and are useful in some types of hearing loss. When hearing loss is severe or profound, cochlear implants can bypass the injured cochlea altogether and directly stimulate the nerve to allow people to hear. Investigators are also developing vestibular implants to provide relief for balance problems such as vertigo.
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Opposite page: This striking microscopic view of the mouse utricle, a balance organ in the inner ear, is an example of how Hearing Restoration Project’s Jennifer Stone, Ph.D., has been able to combine her interest in science with her art (also shown on this page).
photo credit: jennifer stone, ph.d./university of washington
Stone conducts research on the sensory hair cells of the inner ear, which she calls the most beautiful cells in the body— and continues to dabble in art, incorporating illustrations into her papers and teaching.
“But, no treatment cures the problem—the loss of hair cells,” Stone says. “We want to find a way to do that.” The Stone lab focuses on vestibular hair cells, which are required for balance. Although there is no natural regeneration in the auditory sensory organ (the cochlea), vestibular organs have a natural capacity to regenerate some hair cells. This process is limited, but the Stone lab is studying it carefully, with the hope of applying what they learn to promote full hair cell regeneration in both the hearing and balance organs. While her path may have diverged from the typical model, Stone found ways to blend both of her interests, finding a place for biology and art in her career.
Jennifer Stone, Ph.D., a member of HHF’s Hearing Restoration Project, is a research professor in the University of Washington’s department of otolaryngology–head and neck surgery, where she is also the director of research. She is a 1995, 1996, and 2000 Emerging Research Grants alumnus. For more, see hhf.org/research. This is adapted from a story by Ash Shah, the science editor of The Daily, the University of Washington’s student newspaper, at dailyuw.com.
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Recent Research by Hearing Health Foundation Scientists, Explained Charting the Development of the Mouse Cochlea
In the future, scientists may be able to use the data to steer stem cells toward the hair cell lineage, helping to produce the specialized cells they need to test cell replacement approaches for reversing some forms of hearing loss.
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A team of researchers has generated a developmental map of a key soundsensing structure in the mouse inner ear. Scientists at the National Institute on Deafness and Other Communication Disorders (NIDCD), part of the National Institutes of Health, and their collaborators analyzed data from 30,000 cells from mouse cochlea, the snail-shaped structure of the inner ear. The results provide insights into the genetic programs that drive the formation of cells important for detecting sounds. The study datasets are shared on a unique platform open to any researcher, creating an unprecedented resource that could catalyze future research on hearing loss. Led by Matthew W. Kelley, Ph.D., chief of the Section on Developmental Neuroscience at the NIDCD, the study appeared online in Nature Communications on May 13, 2020. The research team includes investigators at the University of Maryland School of Medicine, Baltimore; Decibel Therapeutics, Boston; and King’s College London. “Unlike many other types of cells in the body, the sensory cells that enable us to hear do not have the capacity to regenerate when they become damaged or diseased,” says NIDCD Director Debara L. Tucci, M.D., who is also an otolaryngology–head and neck surgeon. “By clarifying our understanding of how these cells are formed in the developing inner ear, this work is an important asset for scientists working on stem cell-based therapeutics that may treat or reverse some forms of inner ear hearing loss.” In mammals, the primary transducers of sound are hair cells, which are spread across a thin ribbon of tissue (the organ of Corti) that runs the length of the coiled cochlea. There are two kinds of hair cells, inner hair cells and outer hair cells, and they are structurally and functionally sustained by several types of supporting cells. During development, a pool of nearly identical progenitor cells gives rise to these different cell types, but the factors that guide the transformation of progenitors into hair cells are not fully understood. To learn more about how the cochlea forms, Kelley’s team took advantage of a method called single-cell RNA sequencing. This powerful technique enables researchers to analyze the gene activity patterns of single cells. Scientists can learn a lot about a cell from its pattern of active genes because genes encode proteins, which define a cell’s function. Cells’ gene activity patterns change during development or in response to the environment. “There are only a few thousand hair cells in the cochlea, and they are arrayed close together in a complex mosaic, an arrangement that makes the cells hard to isolate and characterize,” Kelley says. “Single-cell RNA sequencing has provided us with a valuable tool to track individual cells’ behaviors as they take their places in the intricate structure of the developing cochlea.” Building on their earlier work on 301 cells, Kelley’s team set out to examine the gene activity profiles of 30,000 cells from mouse cochleae collected at four time points, beginning with the 14th day of embryonic development and ending with the seventh postnatal day. Collectively, the data represents a vast
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photo credit: helen maunsell, nidcd/nih
Single-cell RNA sequencing helps scientists map how sensory hair cells (pink) develop in a newborn mouse cochlea.
catalog of information that researchers can use to explore cochlear development and to study the genes that underlie inherited forms of hearing loss. Kelley’s team focused on one such gene, Tgfbr1, which has been linked to two conditions associated with hearing loss, Ehlers-Danlos syndrome and Loeys-Dietz syndrome. The data showed that Tgfbr1 is active in outer hair cell precursors as early as the 14th day of embryonic development, suggesting that the gene is important for initiating the formation of these cells. To explore Tgfbr1’s role, the researchers blocked the Tgfbr1 protein’s activity in cochleae from 14.5-day-old mouse embryos. When they examined the cochleae five days later, they saw fewer outer hair cells compared to the embryonic mouse cochleae that had not been treated with the Tgfbr1 blocker. This finding suggests that hearing loss in people with Tgfbr1 mutations could stem from impaired outer hair cell formation during development. The study revealed additional insights into the early stages of cochlear development. The developmental pathways of inner and outer hair cells diverge early on; researchers observed distinct gene activity patterns at the earliest time point in the study, the 14th day of embryonic development. This suggests that the precursors from which these cells derive are not as uniform as previously believed. Additional research on cells collected at earlier stages is needed to characterize the initial steps in the formation of hair cells. In the future, scientists may be able to use the data to steer stem cells toward the hair cell lineage, helping to produce the specialized cells they need to test cell replacement approaches for reversing some forms of hearing loss. The study’s results also represent a valuable
resource for research on the hearing mechanism and how it goes awry in congenital forms of hearing loss. The authors have made their data available through the gEAR portal (gene Expression Analysis Resource), a webbased platform for sharing, visualizing, and analyzing large multi-omic datasets. The portal is maintained by Ronna Hertzano, M.D., Ph.D., and her team in the department of otorhinolaryngology and the Institute for Genome Sciences at the University of Maryland School of Medicine. “Single-cell RNA sequencing data is highly complex and typically requires significant skill to access,” Hertzano says. “By disseminating this study data via the gEAR, we are creating an ‘encyclopedia’ of the genes expressed in the developing inner ear, transforming the knowledge base of our field and making this robust information open and understandable to biologists and other researchers.”
This originally appeared on the National Institutes of Health website, at nih.gov. Matthew W. Kelley, Ph.D., is a 1997 Emerging Research Grants (ERG) alumnus and a member of HHF’s Council of Scientific Trustees (CST), the ERG’s governing body; Debara L. Tucci, M.D., is a 1992–93 ERG alumnus and a former CST member; and Ronna Hertzano, M.D., Ph.D., is a 2009–10 ERG alumnus and a member of HHF’s Hearing Restoration Project. For references, see hhf.org/summer2020-references.
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Yap Protein Plays Major Role in Inner Ear Development It turns out that to hear a person yapping, you need a protein called Yap. Working as part of what is known as the Yap/Tead complex, this important protein sends signals to the hearing organ to attain the correct size during embryonic development, according to a June 2020 study published in Proceedings of the National Academy of Sciences (PNAS) from the University of Southern California (USC) Stem Cell Initiative laboratory of Neil Segil, Ph.D. “Our study provides insights into how the hearing organ develops in utero, and offers clues about how to regenerate damaged cells to restore hearing later in life,” says lead author Ksenia Gnedeva, Ph.D. Gnedeva performed the research during her postdoctoral training in the Segil Lab, and is now an assistant professor in the USC Caruso Department of Otolaryngology–Head and Neck Surgery, where Segil is also a professor. As the organ of Corti forms in the embryonic mouse, a population of progenitor cells self-renews and proliferates to achieve the correct size. Then, after approximately two weeks, the progenitor cells begin differentiating into various specialized cell types. The scientists found that the Yap/Tead protein complex plays a major role in this intricate developmental process by regulating the activity of hundreds of genes that control self-renewal and proliferation. The Yap protein serves as a key signal to promote the proliferation of the progenitor cells during the first two weeks of development. If Yap signaling drives proliferation for longer than two weeks, then the inner ear sensory organs grow too large. If Yap signaling breaks down prematurely, then the organ of Corti ends up being too small. By the time mice are born, the progenitor cells in the organ of Corti have finished both proliferation and differentiation, and cannot regenerate if they are damaged—causing permanent hearing loss. Remarkably, when the scientists activated Yap in newborn mice with hearing loss, some of the cells in the organ of Corti began proliferating again, suggesting that regeneration might be possible. “This study provides the first support for the possibility of restoring lost sensory cells for hearing and balance by activating Yap in mice,” says Segil, who is also a professor in USC’s department of stem cell biology and regenerative medicine. “Although we can’t permanently activate Yap in human patients for a variety of practical reasons, we might be able to locally administer a drug targeting related groups of molecules.” —Cristy Lytal 30
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By the time mice are born, the progenitor cells in the organ of Corti have finished both proliferation and differentiation, and cannot regenerate if they are damaged—causing permanent hearing loss. Remarkably, when the scientists activated Yap in newborn mice with hearing loss, some cells in the organ of Corti began proliferating again, suggesting that regeneration may be possible.
This originally appeared on the USC Stem Cell website, part of the Keck School of Medicine of USC, stemcell.keck.usc.edu. Neil Segil, Ph.D. (above far left), is a member of Hearing Health Foundation’s Hearing Restoration Project (HRP), which helped fund this research. HRP members Andy Groves, Ph.D. (center), from Baylor College of Medicine, and Mark Warchol, Ph.D., from Washington University in St. Louis, are among the coauthors on the PNAS paper. For more, see hhf.org/hrp. For references, see hhf.org/summer2020-references.
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EMERGING RESEARCH GRANTS
A Clue to Understand Difficulties With Speech Perception in Noise
Validating an Animal Model of Hyperacusis Hyperacusis is a debilitating hearing condition in which normal everyday sounds are perceived as exceedingly loud, annoying, aversive, or even painful. Epidemiological studies show the prevalence of hyperacusis ranges from 6 to 9 percent of the population, making it an important but understudied medical condition. To learn what is happening in the brain and nervous system when hyperacusis is present, we used soundevoked, functional magnetic resonance imaging (fMRI) to locate regions of abnormal activity in the central nervous system of rats with behavioral evidence of hyperacusis induced with an ototoxic drug (sodium salicylate). We observed enhanced central auditory gain and were able to confirm this electrophysiologically. As published in Hearing Research in April 2020, our results demonstrate for the first time that noninvasive, sound-evoked fMRI can be used to identify regions of neural hyperactivity throughout the brain in an animal model of hyperacusis. In addition, we showed we can experimentally manipulate the degree and type of hearing loss and to objectively quantify biomarkers of hyperacusis that may be used for clinical diagnosis. Often when using fMRI to investigate hyperacusis in humans, the patients cannot tolerate the loud sounds generated by the MRI scanners. An advantage to using mice and rats for these studies is that they have poor low-frequency hearing in regions where there is considerable scanner noise and are not as affected by it. Overall this study allows us to connect human fMRI data with data from the animal model, advancing a greater understanding of hyperacusis. —Kelly Radziwon, Ph.D.
A 2015 and 2018 ERG scientist generously funded by Hyperacusis Research Ltd., Kelly Radziwon, Ph.D., is a research assistant professor in the department of communicative disorders and sciences at the University at Buffalo, State University of New York. 32
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While it is well known that hearing loss degrades speech perception, especially in noisy environments, less is understood as to why some individuals with normal hearing may also struggle with speech perception in noise (SPiN). Several factors appear to contribute to SPiN abilities in adults with typical hearing, including the top-down cognitive functions of attention, working memory, and inhibition. Specifically, inhibition at the cognitive level may be considered as the ability to successfully suppress external auditory information (e.g., background noise) in order to correctly identify and understand the speech signal of interest, requiring the conscious participation of the listener. However, inhibition also exists at a bottom-up, sensory stage as an automatic process even when the listener is not actively involved. Inhibitory function at this level is considered to “gate” sensory information leading to cognitive centers. So it is plausible that deficits in sensory inhibition may allow an excess of auditory noise to reach cognitive centers, overwhelming these resources and resulting in poor SPiN performance. Using high-density EEG (electroencephalogram) testing, our findings suggest that individuals with typical hearing and mild SPiN impairment may present with decreased inhibition at the sensory level, which is reflected in incomplete and atypical activation of their cortical inhibitory networks. This lack of sensory inhibition may allow extraneous noise to reach cognitive centers and interfere with speech perception. With this information, our laboratory is currently conducting studies to investigate the factors that may affect sensory inhibition in the presence of typical hearing (such as noise exposure). —Julia Campbell, Ph.D., Au.D.
Julia Campbell, Ph.D., Au.D., CCC-A, FAAA, is an assistant professor of communication sciences and disorders in the Central Sensory Processes Laboratory at the University of Texas at Austin. A 2016 ERG scientist generously funded by the Les Paul Foundation, she is recruiting participants for this research; email julia.campbell@austin.utexas.edu.
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meet the researcher
hearing health foundation
EMERGING RESEARCH GRANTS
Emerging Research Grants (ERG) As one of the only funding sources available for innovative research, HHF’s ERG program is critical. Without our support, scientists would not have the needed resources for cutting-edge approaches toward understanding, preventing, and treating hearing and balance disorders.
Meet the Researcher Kristy J. Lawton, Ph.D.
Washington State University Vancouver
Lawton received her doctorate in neurobiology and behavior from Cornell University, New York, and then completed postdoctoral research at Reed College, Oregon. She is currently a postdoctoral research associate in the integrative physiology and neuroscience department at Washington State University Vancouver. Her 2019 Emerging Research Grant was generously supported by donors to Hearing Health Foundation.
In Her Words many labs successfully use the zebrafish for drug-induced hearing loss research, but we are one of the very few utilizing them for noise-induced damage. Our device to create noise damage in fish was years in the making, with dozens of prototypes. I got excited about the prospect of using this unique system to study the relatively newly discovered phenomenon of noise-induced synaptic damage seen in mammals, often called “hidden hearing loss” because the deficit isn’t caught by traditional audiograms. Our recent work showed evidence for synaptic loss in zebrafish, so now I am digging into mechanisms that will hopefully be relevant to understanding similar human hearing loss. i’m a first-generation college student in my family, the only scientist, and was an undeclared major for two years. I took a smattering of courses from several disciplines to try to figure out what I wanted to do, and biology was what really pulled me in. I grew up in the countryside, and spent the summers gleefully climbing trees and playing in the fields catching insects, snakes, etc. Animal behavior had always fascinated me, and nowadays I foster kittens. Watching how they develop and interact with the world reminds me of the complexities of our sensory and motor systems. It never ceases to amaze me how well everything performs and integrates.
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in between undergrad and grad school, I decided I wanted to take a break and go do something completely different. So I moved to Japan for a year to teach English in a small remote fishing village. Foreigners were so rare there that they featured me in the newspaper, and many strangers in town would recognize me and greet me. I really miss the food! my father suffers from noise-induced hearing loss from his time in the military and some industrial work. He only recently got hearing aids through the VA and they have helped him tremendously, but they’re still not a perfect fix. Watching him struggle has propelled a shift in my work from a predominantly curiosity-driven passion to a strong desire to perform research that is directly helpful to people.
Kristy J. Lawton, Ph.D., is funded by donors to Hearing Health Foundation who designated their gifts for the most promising research. These projects address the full range of hearing and balance science.
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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