Hearing Health Winter 2022 A Publication of Hearing Health Foundation hhf.org
The Balance Issue Making sense of the connection between hearing and balance
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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 the largest nonprofit funder of hearing and balance research in the U.S. and a leader in driving new innovations and treatments for people with hearing loss, tinnitus, and other hearing and balance conditions. As part of our outreach, we provide this quarterly magazine for free to our vibrant community of readers and supporters, as well as to the dedicated professionals who work with them. Please subscribe at hhf.org/subscribe and make a donation at hhf.org/donate.
Winter 2022: The Balance Issue Both located in the inner ear, the balance and hearing systems share similarities that mean disorders as well as solutions may overlap.
Timothy Higdon President and CEO Hearing Health Foundation
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Hearing Health The Balance Issue
Winter 2022, Volume 38, Number 1 Publisher Timothy Higdon, President and CEO, HHF
Features
Editor Yishane
08 Balance One Ear, Two Systems. Jennifer Stone, Ph.D., and James Phillips, Ph.D. 14 Living With Hearing Loss Just Because Hearing Loss Is Invisible Doesn’t Mean It’s Less Real. Kayleena Pierce-Bohen 16 Balance I’m 24 and Have Hearing and Balance Challenges. Tomoe Miyazaki 18 Technology Exciting Possibilities. Anat V. Lubetzky, Ph.D. 20 Music Spectrum Sounds. Andrew Hugill 22 Balance Check, Check, Check. Mary Horton 24 Hearing Health 6 Ways to Raise Youth Awareness of Noise-Induced Hearing Loss. Rohima Badri, Ph.D.
26 Balance My Seasonal Spins. Shari Eberts 27 Planned Giving A Personal and Professional Connection to the Mission to Research and Cure Hearing Loss. Ellie Daniels 28 Managing Hearing Loss 9 Things to Know About Hearing Loss From the Perspective of the Communication Partner. Pat Dobbs 31 Living With Hearing Loss A Complicated and Special Case. Sarah Kirwan 34 Research Presenting the 2022 Emerging Research Grantees. 40 Research Recent Research by Hearing Health Foundation Scientists, Explained.
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06 @editor 50 Meet the Researcher Megan Beers Wood, Ph.D. Hyperacusis Research
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Hearing Health Foundation (HHF) and Hearing Health magazine do not endorse any product or service shown as paid advertisements. While HHF makes every effort to publish accurate information, it is not responsible for the accuracy of information therein. See hhf.org/ad-policy.
Cover Jennifer Stone, Ph.D., whose research focuses on the balance system, is a 1995, 1996, and 2000 Emerging Research Grants alumna and a member of Hearing Health Foundation’s Hearing Restoration Project.
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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, J.D. Jay Grushkin, J.D. Roger M. Harris 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 Hearing Health magazine (ISSN 2691-9044, print; ISSN 2691-9052, online) is published four times annually by Hearing Health Foundation. Copyright 2022, 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.
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letters to the editor
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dear editor: Recent issues of your quarterly have definitely struck home. I’ve had (thankfully mild) tinnitus for decades, at least going back to an ear infection I had as a university student. It’s usually no more than a background hiss that mostly goes unnoticed unless I’m actually thinking about it. Once in a great while I’ll hear an abrupt spike in it, and then that ear will drop out entirely for a minute or so. Perhaps the worst scare was once, many years ago, when one ear abruptly went totally deaf for several hours, then returned to normal before I’d even had a chance to have it examined. On another occasion I had a bout of severe vertigo, in an airplane van after a flight. The whole world began spinning violently, but fortunately the spin slowed to a stop by the time I reached my hotel. I’ve never understood why anybody would want to expose themselves to extreme noise levels. I don’t think I’ve ever raised the volume on my stereo system beyond 25 to 30 percent. I will say one of the best investments I’ve made is a noise-canceling headset—earbuds with an active noise cancellation circuit—and they also do not put pressure on my outer ears. Then, after constantly seeing the late Les Paul and the Les Paul Foundation mentioned in your quarterly, I was inspired to read his autobiography. I never realized how much he’d accomplished and been through, including getting both ears accidentally boxed, which of course explains the HHF connection. James H.H. Lampert California from the editor: Thank you for writing in. We wanted to note that “Les Paul in His Own Words” is available at les-paul.com.
dear editor: I was inspired to donate to Abe’s bar mitzvah project, after reading about it in your Fall 2021 issue. As a Jewish mother with hearing loss, the family’s story means a lot to me. Mazel Tov!
dear editor: Thank you very much for “10 Tips for Effective Communication” in the Fall 2021 issue and the accompanying article. I intend to look up the book referenced and I know it will be on target! I am very proud to learn of Northeastern’s participation in the world of hearing issues since I am a double graduate of NU (1967, 1997) and had no idea there were great people like Professor Nicole Laffan doing such great work there now. I appreciate your magazine very much. I have subscribed for two or three years now since finding it at the otolaryngologist’s office, and I always learn something I can relate to. Keep up the great work! Nancy Hubbard Boston
dear editor: In the Spring 2021 issue on tinnitus, Tinnitus Retraining Therapy is mentioned as the gold standard for treatment, but does it work for the totally deaf or people with cochlear implants? (Without my CI I am totally deaf.) Googling “Tinnitus Retraining Therapy cochlear implant” brings up mostly articles about how a CI can treat tinnitus, which is useless to the many of us whose tinnitus got worse after their CI was implanted. I am extremely interested in research into problems faced by CI users: tinnitus, hyperacusis, and electronic background noise suppression (not brain retraining). Leah O’Connor Chicago from the editor: Thank you for your feedback. While your specialist will know best, research shows sound therapy may help people with CIs cope with tinnitus. Please see hhf.org/winter2022-references. We always appreciate hearing from our community. Letters may be edited for length and clarity. Please email us at editor@hhf.org.
Ena Brown Via email
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The Entertainment Issue Breaking barriers, raising awareness, and aiming for equity
The Hearing & Overall Health Issue Caring for your hearing is a vital part of physical and mental health
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The Balance Issue Making sense of the connection between hearing and balance
The Tinnitus & Hyperacusis Issue
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We Share Stories and Solutions Hearing Health Foundation’s Hearing Health magazine is an award-winning community resource with inspiring first-person stories and real-world solutions based on the latest research and technology.
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One Ear, Two Systems We are all well aware that we lose our hearing as we age. We also know that congenital mutations and exposure to high doses of ototoxic drugs can damage our hearing. What we may not know is that these same factors can also compromise our sense of balance. Our auditory (hearing) system allows us to communicate and to sense and interpret sounds in the environment. The vestibular (balance) system helps us maintain our equilibrium, orient ourselves in space, and move safely and effectively in our environment. Both sensory systems are crucial for our well-being. A 2009 study from Johns Hopkins University found that just over a third of people older than age 40 experience a balance disorder. Yet, despite how common these deficits are, we still understand very little about the causes, diagnoses, treatments, and prevention of vestibular problems. People who have hearing loss are much more likely to have balance disorders, and vice versa. This is in large part because the primary organs for hearing and balance are located in the inner ear, and they share similar biological and physiological features. As a result, hearing loss and vestibular deficits share many of the same causative factors. The good news is that solutions we develop have the potential to benefit both systems.
How the Systems Work
Our two ears have mirror symmetry, and both are important for collecting and sending information along the eighth cranial nerve to the brain for processing. In the diagram on the opposite page, we can see the different regions of the inner ear 8
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and how they are related to these two senses. A coiled structure, the cochlea, shown on the bottom right, is the sensory organ for hearing. The vestibule, in the top and center region, is where the five sensory epithelia for balance are housed. Three of these epithelia—the cristae— are located in the semicircular canals and sense rotations of the head. Two others, called the maculae, sense static head position and linear motions of the head. The hearing and balance sensory organs work in very different ways, but they also have important commonalities. Along the coiled structure of the cochlea is a sensory epithelium, which looks like a strip that sits on a membrane. When sound waves excite the middle ear, the cochlea’s basilar membrane vibrates, creating a traveling wave. Different regions of the membrane are excited by different frequencies of sound. Sensory cells—called hair cells because of the bundle of hair-like structures on their surface— are distributed across this membrane. When the traveling wave is sufficiently large underneath them, hair bundles are bent, and hair cells are activated, sending a signal to the cochlear branch of the eighth cranial nerve, which relays this information to the brain. Importantly, information from both the left and right cochleae is required for us to optimally localize sounds—to understand which direction sounds are coming from. The two cochleae working together also help us to understand speech in noisy environments. Unlike the cochlea, our vestibular sensory organs detect head position and motion. The macular organs—the utricle and saccule— sense tilts of the head and head motions that
illustration credit: blausen.com staff, medical gallery of blausen medical 2014, via creative commons
Studies of the similarities and differences between the hearing and balance systems are accelerating scientific advances. By Jennifer Stone, Ph.D.
balance
People who have hearing loss are much more likely to have balance disorders, and vice versa. This is mainly because the primary organs for hearing and balance are in the inner ear, and they share similar biological and physiological features. This also means solutions we develop have the potential to benefit both systems. are linear. In these organs, hair bundles are embedded in a membrane that is overlayed by thousands of tiny ear stones, called otoconia. When we move our head in one direction, the otoconia, which are relatively heavy, lag behind. When we tip our heads, the otoconia are pulled down by gravity. This causes the hair bundle to bend, which triggers the vestibular branch of the eighth cranial nerve to send signals to the brain. The saccule and utricle are located at right angles to each other, so they’re more sensitive to different directions of head motion. The utricle is more responsive to head tilts and sideways motions, while the saccule strongly senses motion that is up and down or forward and backward. The three cristae sense angular accelerations (rotations) of the head. The hair cells in the cristae are located inside the organ in a sac, which is called an ampulla, that sits at the end of each curved semicircular canal. The cristae sense head rotations when the hair cells are stimulated by small movements of fluid in the semicircular canals that occur when the head accelerates in a specific plane. When the head rotates in one direction, it will stimulate transient fluid motions in the opposite direction. This fluid movement stimulates the hair bundles to bend, exciting the hair cells and the nerves as well. In this way, each of these five balance organs has different functions, sensing head motions in various directions. The brain receives information from them and compiles it all into a bigger picture. Just as both ears are important for hearing, the brain requires information from both ears to optimally understand how we are oriented in space and how we are moving.
The primary organs for hearing and balance are located in the inner ear, as shown in this detailed view (above) of the inset (top). Also, in this Creative Commons image the semicircular canals are labeled semicircular ducts, which they are also called.
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James Phillips, Ph.D., and Jennifer Stone, Ph.D., study the vestibular system at the University of Washington.
A Shared Origin
The vestibular and auditory organs share a common embryonic origin. Anything that interrupts that development can very well affect both systems, depending on when the change arises. A single gene mutation, for example, may disrupt development of both sensory systems and affect both balance and hearing. Vestibular and auditory senses rely on similar sensorineural elements. These are hair cells, which sense tiny changes in bundle position and transduce them into electrochemical signals, and nerves, which receive the hair cell signals and transmit them to the brain. Another important cell type is present in both systems: the supporting cells. These cells provide structural support to the epithelia and regulate the chemical environment of the sensory organs. Supporting cells also have the potential to form new hair cells after damage, or what we call hair cell regeneration. Environmental factors can injure sensorineural elements in both systems. Ototoxic drugs—including antibiotics like gentamicin and streptomycin—can kill both vestibular and auditory hair cells. Antitumor drugs such as cisplatin may also damage supporting cells and nerve cells. We are all well aware that exposure to prolonged or intense noise causes hearing 10
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damage. There is emerging evidence that these sorts of stimuli may also cause damage to the vestibular organs, particularly to the sensory nerve endings. In the same way that cellular structure and function are similar across both sensory systems, the potential for physical damage is also shared. The vestibular and auditory branches of the eighth nerve carry information from each set of sensory organs to the brain. These nerves travel together through the temporal bone, so any physical disruption, such as a tumor, can affect both hearing and balance. Researchers and clinicians have identified a number of other factors that may lead to measurable losses in balance and hearing function, including microbial infections like CMV (cytomegalovirus), Epstein-Barr virus, and meningitis. We also note a similar decline in function in relation to aging.
What About Regeneration?
No mammals can regenerate auditory hair cells in maturity. They may do so when they’re very young, but once they’ve matured, there’s very little capacity for auditory hair cell regeneration. In a seminal 1993 study, Andrew Forge, Ph.D., and colleagues used gentamicin to damage hair cells in young adult guinea pigs. Forge observed
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We and others in the scientific community believe we will figure out how to fully regenerate vestibular hair cells before determining how to replace auditory cells. The power is having a system that regenerates a little bit—one that can be augmented. It’s easier to rev up a naturally occurring process than to jumpstart something that’s completely stalled. However, information gleaned from investigators of the injured auditory system will no doubt influence studies in the vestibular system. It’s the cross-fertilization of ideas and scientific approaches that lend synergy and momentum to inner ear research. significant damage to the hair cells in the vestibular organ after just a couple of weeks. But about eight to 12 weeks later, he saw that some of these hair cells had been replaced, indicating that there was some capacity for regeneration. Forge and colleagues were understandably excited to find this potential at all in an adult mammal. Subsequent studies from Forge’s lab and others, based on data from biopsies and autopsies, showed that humans may also be able to regenerate some vestibular hair cells. This is broadly significant because even the small natural capacity for regeneration in the vestibular organ provides an opportunity to study how this process is regulated in a system that is incredibly similar to the auditory system. About 10 years ago, my lab switched from studying the hearing organ to the vestibular organ because of the potential that the vestibular system offers. Several other research groups continue to examine how the auditory organ responds to damage to identify the factors that block regeneration there. We and others in the scientific community believe we will figure out how to fully regenerate vestibular hair cells before determining how to replace auditory cells. The power is having a system that regenerates a little bit—one that can be augmented. It’s
easier to rev up a naturally occurring process than to jump-start something that’s completely stalled. However, information gleaned from investigators of the injured auditory system will no doubt influence studies in the vestibular system. It’s the cross-fertilization of ideas and scientific approaches that lend synergy and momentum to inner ear research. It is important to keep in mind that the cochlea has inner and outer hair cells, and they are very distinct from each other and distinct from vestibular hair cells. The vestibular organs have type I and type II hair cells, which likely have unique functions. Understanding how these specific cell types can be regenerated and how they can become incorporated into the respective sensory system poses a unique challenge.
We Need to Know More
There is still so much we don’t know about the vestibular system. We need to learn a lot more before we can develop new ways to treat vestibular disorders. More research is needed on critical topics, including: basic biology (how the vestibular system develops, functions, and degenerates); diagnostics (genetic testing, inner ear imaging, and tests of the vestibular function); preventing ototoxicity and other forms of damage; and
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restoring function to the injured system, perhaps through stem cells, gene therapy, the delivery of pharmaceutical agents directly into the ear, and/or vestibular implants. Understanding these aspects of the vestibular system will provide important insights in how to rebuild the auditory system after injury as well. What’s the best way to promote vestibular research? We need to fund more projects on the vestibular and auditory systems. We need to recruit more people into the field. One way to do that is through Hearing Health Foundation (HHF)’s Emerging Research Grants program, which provides more training to young investigators and may stimulate crossover from other disciplines. We can also take novel approaches to problem solving, such as HHF’s Hearing Restoration Project’s consortium research model, which promotes deep collaboration and open, rapid data sharing. We can leverage insights and technology from vestibular and auditory research to cross-foster ideas and approaches. One of the most exciting aspects of research like that performed by HHF-funded scientists and their colleagues is the leveraging effect built right into the work itself: Each step forward is a move in the right direction in understanding and treating disorders in not just one but two systems that are so critical to quality of life.
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 alumna. To view the captioned webinar recording “One Ear, Two Systems,” which this story is based on, see hhf.org/webinar. For references, see hhf.org/winter2022-references.
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Balance Conditions: A Primer By James Phillips, Ph.D.
Individuals with balance issues are significantly more likely to have hearing loss, and vice versa, suggesting that sensory structures of the inner ear often play an important role. Most balance complaints are transient and do not require any treatment at all, and many are easily treated. For example, benign paroxysmal positional vertigo, which is when the structures of the inner ear deteriorate, can be treated with simple repositioning maneuvers that can be performed in the physician’s office with almost immediate effect. However, a significant number of vestibular disorders become chronic, debilitating issues. If you have a vestibular disorder, the primary thing you’ll experience is a sense that something is horribly wrong. We don’t appreciate the importance of the vestibular sense until it is compromised.
Here are the types of balance conditions:
» Dizziness: an altered sense of your body in space. It’s often accompanied by a feeling of lightheadedness, feeling like you’re unsteady or floating. » Vertigo: a false sense that you or the world are moving. » Imbalance: an inability to maintain your posture, difficulty walking, and falls, which are a leading cause of death in older adults. Older patients with imbalance have an eightfold increase in the probability of experiencing falls. » Oscillopsia: blurred vision when your head moves. There is a “steady-cam” system built into your brain that compensates for head movement by moving your eyes so that your eyes remain fixed on objects of interest. When that system fails, you lose vision, your vision becomes blurred, and the visual world moves dramatically. » Cognitive challenges: trouble concentrating and trouble with memory, also known as brain fog. » Depression and anxiety: Vestibular disorders are debilitating and isolating, so it’s logical that people would experience depression or anxiety, but there actually is an anatomical substrate, or underpinning, for this as well. » Autonomic dysfunction: People with vestibular disorders experience conditions like nausea, vomiting, and difficulty with blood pressure regulation.
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One example of a crossover advance that leverages knowledge of both the auditory and vestibular systems is the cochlear vestibular neuroprosthesis, which is similar to a cochlear implant in that it uses multiple electrode arrays to stimulate specific regions in the inner ear.
Link to Anatomy
There are underlying anatomical connections to these conditions. The vestibular nuclei in the central nervous system receive vestibular information from the inner ear. This data informs movements of the eye, neck, and body; assigns subjective value to self-motion; affects bodily responses such as blood pressure, heart rate, and digestive tract motility; and informs spatial orientation and memory. Vestibular disorders can affect one or both ears. The five vestibular organs in each ear provide a certain degree of redundancy, which is a good thing. The vestibular hair cells are constantly secreting neurotransmitters and the afferent fibers are constantly discharging. They modulate their discharge based on motion inputs. If I turn my head to the right, the afferents from the lateral semicircular canal in the right ear increase their discharge and the afferents from the comparable canal in the left ear decrease their discharge. And vice versa. This means not only does the vestibular system have redundancy but to operate optimally, both ears need to provide information to the brain. When there is no difference between these inputs from each ear, your brain tells you that you are stationary. When you turn your head, there’s a difference between the two ears and that difference tells your brain that you are turning. Acute unilateral loss (loss of vestibular function in one ear) reduces the output from one ear, and that then tells your brain that you’re turning rapidly toward the other ear. It produces whirling vertigo. Bilateral vestibular loss (in both ears) doesn’t produce whirling vertigo, but it does result in profound imbalance, disorientation, and cognitive challenges.
Compensation
How do patients recover from vestibular loss? The central nervous system actually engages in compensatory
mechanisms to restore function. The brain compensates for missing information by adapting and by making substitutions using information from other senses such as vision, touch, and hearing. Currently there are no non-experimental restorative therapies for the loss of vestibular hair cells. Vestibular and auditory scientists are using comparable approaches to treat these problems, and advances in one field can benefit both groups of patients. One example of a crossover advance is the cochlear vestibular neuroprosthesis, which is similar to a cochlear implant in that it uses multiple electrode arrays to stimulate specific regions in the inner ear. Our laboratory has one in development, and there are similar devices at Johns Hopkins and in Europe. Currently being implanted into patients experimentally, this prosthesis is a good example of how the commonality between hearing and balance can help inform treatments.
James Phillips, Ph.D., is a research professor in the University of Washington’s department of otolaryngology– head and neck surgery. He is also the director of the Dizziness and Balance Center at the University of Washington Medical Center (UWMC), the Vestibular Diagnostic Laboratory at UWMC, and the Roger Johnson Clinical Oculomotor Laboratory in the Division of Ophthalmology at Seattle Children’s Hospital.
Share your story: Have you experienced a balance condition? Tell us at editor@hhf.org.
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Just Because Hearing Loss Is Invisible Doesn’t Mean It’s Less Real By Kayleena Pierce-Bohen
When I was in my 20s for a period of about three years, I worked in nightclubs in San Francisco as part of the floor staff. I was subjected to live bands (including heavy metal, rock, punk, and other heavily percussive groups), DJ playlists, and other live performances for four nights a week, for roughly 10 to 12 hours at a time. It was about two years after that period that people around me started suggesting I get my ears checked because I was frequently turning up music and the TV to hear it, speaking louder than normal indoors, and asking friends and family to repeat themselves because I was having difficulty hearing them. When I got an annual physical, my general practitioner suggested I do an auditory exam as well as some imaging. It was at that time I was told I’d experienced some high frequency hearing loss. It was also about that time that I developed a high-pitched whine in my ears (tinnitus), as well as a horrible clicking, which continues even now whenever I try to write at a computer or read a book. I also suffer from vertigo, which is compounded by my hearing loss and makes my life pretty miserable when it appears. I’ll have entire 24-hour periods where I feel like I’m on a ship trying to get my sea legs. Just walking in a straight line will have me clutching a counter or wall for support, which I know looks quite peculiar to other people to whom I seem “fine.” I’ve had to use a cane. I never had anything remotely like vertigo before I had been working in incredibly loud environments.
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Earplugs, But...
I think because I was in my 20s and thought working at a nightclub was fun and interesting, and I was surrounded by talented musicians and entertainers, I didn’t stop to consider that I was going to the equivalent of four concerts a week. Not even concerts with two-hour sets—more like multi-day music festivals with four bands each performing two-hour sets! Earplugs were provided for employees at the nightclubs, and I wore them when I remembered to, but they weren’t always the most effective. I could have invested in better ones. I could have also trusted the older staff members who cautioned against getting too close to the large speakers at the front of the stage. I could have done quieter activities when I wasn’t at work, so as not to compound the hearing issues I was unknowingly subjecting myself to. Back then, it was definitely a case of “hearing loss is something that happens to other people” or “hearing loss is something I’ll experience maybe 20 or 30 years from now.” I had no idea that I would experience it so soon, and have to suffer from it so greatly while in my early 30s. I didn’t realize that hearing loss isn’t something anyone has to suffer from, and that in order to avoid it—whether now or decades from now— there are protective steps I can take.
Living With It
To deal with symptoms related to hearing loss, I now get annual earwax removal (something I didn’t think to do previously) from my general
living with hearing loss
Back then, I thought that “hearing loss is something that happens to other people” or “hearing loss is something I’ll experience maybe 20 or 30 years from now.” I had no idea that I would experience it so soon, and have to suffer from it so greatly while in my early 30s. I didn’t realize that in order to avoid hearing loss—whether now or decades from now—there are steps I can take to protect my hearing. practitioner, and I use white noise machines to drown out the tinnitus wherever possible. I did participate in cognitive behavioral therapy, which is often used successfully to help tinnitus patients, but I didn’t find that it was effective in my case. It’s important to find out what works for each individual because while things like medication may work for some, they won’t work for others. There’s no cure for tinnitus or hearing loss, just ways to make symptoms more reasonable to live with. I now work almost entirely at a computer for my job as a senior writer for an entertainment news site, and I have to take frequent breaks when the tinnitus and clicking get particularly irritating and make it difficult to concentrate. Usually, this involves going into a dimly lit room, turning on a white noise machine, and using a cold compress on my forehead. It’s highly disruptive, but I have developed the system that works best for me, and those around me are understanding when my ears are acting up and I need to do what I have to in order to make the effects more manageable.
I’ve definitely tried to impress upon people I know to protect their hearing when they want to enjoy their nightlife and to take frequent breaks from loud environments. Just making sure people are more aware of something they don’t normally even consider is a step in the right direction. A lot of people think only roadies or mechanics who work on jet engines develop hearing loss when they’re young. I’d like to emphasize that because hearing loss is an invisible condition, it often gets dismissed because those of us who suffer from it “look fine.” This can make dealing with the general public or people who don’t know us very well a challenge. But we do value our close friends and family who support us. Kayleena Pierce-Bohen lives in California. She appears in the video “A Few Words About Hearing,” part of HHF’s Keep Listening prevention campaign. Watch at hhf.org/keeplistening.
Please Be More Aware
Hearing loss is something that sneaks up on you, especially when you’re not thinking about it. Hearing loss affects you in personal ways you don’t expect because it impacts communication, and that can make life difficult and frustrating. If people don’t know you very well, they can often think you’re trying to garner sympathy, and that’s another stressor that was initially frustrating for me to overcome.
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winter 2022
15
living with hearing loss
hearing health foundati o n
I’m 24 and Have Hearing and Balance Challenges By Tomoe Miyazaki
When people think of hearing loss and related conditions and the frustration they can often create, I know well I’m not the first person who comes to mind. I am a 24-year-old, recent college graduate and hiking aficionado, working as a business development associate. My intentions with sharing my story are to show the public that my hearing health circumstances are not restricted to older adults, and to build solidarity with folks in my own age demographic affected by hearing and balance conditions, especially those that remain unresolved. During my third year at University of Michigan, where I was studying biochemistry and French, I became aware of an unfamiliar, jarring sensation—extreme dizziness. If I closed my eyes for just a little bit, I’d feel my surroundings spinning. It was around the same time I recognized my difficulties hearing in large lecture halls. I wondered: How can my classmates hear the professors so much better than I could? Why do I struggle so much to work in groups? This didn’t make sense to me. But when I noticed my grades were suffering considerably 16
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and that I was close to failing a class, I knew it was time to seek medical attention. It is worth noting that I’d had a hearing loss from a very young age that affected my childhood, since I always felt isolated from my peers. But until the dizziness became a problem, I did not seek help for my hearing until college. My first visit was to my primary care provider, who directed me to both a vestibular (balance) specialist and an ENT (ear, nose, and throat) doctor. The ENT suspected a migraine issue and referred me to a neurologist. Fortunately, the neurologist ruled out a neuronal/brain condition, but said my symptoms are strongly indicative of Ménière’s disease, a rare hearing and balance condition that is poorly understood. Troublingly, Ménière’s disease can’t be confirmed for me, which is a typical predicament for many patients with vestibular disorders. My ENT maintains my audiogram doesn’t follow a typical Ménière’s pattern of low frequency hearing loss—mine is high frequency. For now, I am in a trial-and-error stage. Recently, I began a low sodium diet and decreased my alcohol and caffeine intake. I was also directed to get a hearing test twice yearly to
balance Tomoe Miyazaki started experiencing extreme dizziness while in college.
During my third year at University of Michigan, where I was studying biochemistry and French, I became aware of an unfamiliar, jarring sensation—extreme dizziness. If I closed my eyes for just a little bit, I’d feel my surroundings spinning. It was around the same time I recognized my difficulties hearing in large lecture halls. I wondered: How can my classmates hear the professors so much better than I could? Why do I struggle so much to work in groups?
measure significant changes in my audiogram. I do have a measurable sensorineural hearing loss and had been prescribed hearing aids. I admit I do not wear my hearing aids as instructed because of the stigma associated with the devices and my relatively young age. I hope to overcome my hesitation to wear hearing aids. In the meantime, I am learning American Sign Language to facilitate my communication with others. But to this day I still struggle with feelings of shame and anger at myself that I go out of my way to keep this part of me hidden from others. On top of my hearing loss and vertigo, I also live with tinnitus, the sensation of ringing in the ears, and haven’t managed to find any viable solutions. My tinnitus is like a crackling sound and bothers me most when I am exposed to external noise. The condition is so bad that it negatively affects my work and relationships. I hope I can figure out what exactly is causing my hearing and balance symptoms, and in the meantime, I hope my story inspires other young people that there are others out there who may be going through what you’re going through. You are never alone in your journey. I hope
knowing you’re not alone can be a first step to breaking the stigma surrounding hearing loss, as this is part of why I struggle to accept that I have this condition and that I would benefit from hearing aids if I consistently wore them. That said, I know many in the Deaf community don’t necessarily want to use hearing devices, and that choice should be respected. If there is anything I want people to know, it would be that those of us with hearing loss are capable of doing anything except hear. All of us are capable of success in life, no matter what path for communication we choose. Tomoe Miyazaki lives in Michigan. She welcomes comments and questions at tomorin_usa@yahoo.com.
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winter 2022
17
technology
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Exciting Possibilities Studying the influence of combined visual and auditory inputs in different contexts is made simpler through virtual reality. By Anat V. Lubetzky, Ph.D. The general public is increasingly aware of the dangers of falls. Falls can be fatal or lead to an injury that ends in hospitalization and/or the loss of independence. The Centers for Disease Control and Prevention (CDC) reports that more than one in four adults ages 65 or older falls each year, but fewer than half tell their doctor. The CDC also cautions that an individual who has fallen once is twice as likely to fall again. Falls can lead to severe injuries or death. They also impact quality of life if someone enters a vicious cycle due to fear of falling. We know that individuals with vestibular disorders—that is, problems in balance and dizziness due to a disease of the inner ear—who lost their balance in a certain environment are likely to subsequently feel dizzy or anxious in that particular setting. While people with vestibular disorders are at increased risk for falls, they don’t necessarily fall a lot because they may develop avoidance behavior. They think twice about taking the subway because there are stairs, noise, and people. Going to the grocery store may become too triggering. But we know that avoiding everyday activities can affect mental health. We become isolated because we don’t engage with the community and our friends as much as we could. We become more fearful and more anxious. Then we’re not moving as much, and this affects physical health. Without regular physical activity—and exercise is so important for helping us maintain our balance and overall health—our muscles and bones become weaker, and we’re more likely to fall. Being weaker makes it harder to walk regularly, and that also contributes to falling, and the fear of falling makes it less likely we want to do something physical like going for a walk. It becomes a spiral. This is why in balance rehabilitation, the behavioral aspects—the anxiety and fear—are a huge component. Beyond working on muscles and sensory systems, we also need to gain back our confidence.
Going Virtual in Rehab
The use of virtual reality (VR) in physical therapy and rehabilitation started many years ago. Over the past decade, VR has become more affordable and portable, 18
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with minimal lag time. As mentioned before, people with vestibular disorders have problems in visually busy environments, such as a street packed with people, and unstable surfaces, such as a moving train or bus. We created visually complex environments in VR, using a head-mounted display, to provide a graded way for patients to safely experience complex sensory environments in a functional context. Practice should help patients feel less dizzy and more confident in their balance, because VR is a safe environment for learning to combat any “fear avoidance”
People with vestibular issues have problems in visually busy environments with unstable surfaces. Shown here, Anat Lubetzky, Ph.D., is using virtual reality delivered through a head-mounted display to provide a graded way for patients to safely experience complex sensory environments in a functional context.
technology
behavior. Training in a real-life (albeit virtual) context should theoretically be better transferred to daily function because it presents conditions as close as possible to those commonly encountered during daily activities.
Context Matters
My team and I at New York University and the New York Eye and Ear Infirmary of Mount Sinai have been doing VR research for the past six years. There are benefits to this approach that we couldn’t access otherwise, most notably being able to create a gradual, measurable alteration to the sensory situation so we can collect concrete data about balance behavior. We can measure differences in people’s balance in response to different levels of visual, auditory, and cognitive stimuli and potentially break down the user’s sensory dependence in greater detail. In pilot trials we’ve also found that some shifts between two different visual levels can be too subtle to have measurable effects on the user. Another benefit to using VR is the context. Instead of an environment that is very controlled and clinical (in both senses of the word), we can take people (virtually) to the street or onto the subway. We can also customize the environment. In fact, a lot of our scenes have been built for a specific patient story. One patient told us about difficulty navigating airports and flying, so we built a virtual airport where we could gradually increase the sensory load. One major goal is to understand what context does to balance and sensory integration. We know what it does behaviorally, and that it matters a lot in how we feel. But we are still lacking evidence as to whether it actually affects physiological reactions and balance outcomes. Because VR headsets were designed for use in gaming, we can get very accurate head kinematics (movements). We don’t need multiple cameras and markers. By applying
While people with vestibular disorders are at increased risk for falls, they don’t necessarily fall a lot because they may develop avoidance behavior. They think twice about taking the subway because there are stairs, noise, and people. Going to the grocery store may become too triggering.
trackers on any body part, such as the ankles or lower back, we can capture that movement immediately—in one click. This research can be transferred directly to the clinic because the VR equipment is so portable. We can literally go to the patient, as they come to their medical appointments, instead of having the patient come to the research lab, and still obtain accurate information. This portability helps with study recruitment, too!
And What About Sound?
As we advanced through our work in VR, evidence about the relationship between hearing loss and falls emerged. We therefore asked: How does sound play into this? How do noisy settings affect balance? Using VR we can manipulate both the visual and auditory environments— and of course in real life, these are not isolated, and hearing and vision inputs work together. We also asked ourselves whether the opposite—static, steady-state sounds—can help balance. After conducting a review, we saw that several laboratory studies have shown that people can reduce their movement, or what we call postural sway, and adopt a steadier stance when they are presented with broadband white noise. We can therefore imagine a situation where white noise may be helpful for balance. Most studies used speakers for the white noise, but could people use headphones, almost like a sensory “crutch”? We don’t know yet, but it’s something we’re looking at. What type of static sounds might help? Which hardware do you need? Can this treatment work over headphones or not? These questions need answering, but the possibilities are exciting.
This story was originally an interview conducted by the National Institutes of Health. A shorter version appeared on the NIH News in Health website, at newsinhealth.nih.gov. A 2019 Emerging Research Grants scientist, Anat V. Lubetzky, Ph.D., is an associate professor in the department of physical therapy at New York University. She earned a prestigious Early Career Researcher R21 grant from the National Institute on Deafness and Other Communication Disorders to continue her research into the role of hearing in balance control. For references, see hhf.org/winter2022-references.
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winter 2022
19
h e a ri n g h e alt h fo u n dation
Spectrum Sounds A composer shares the music he developed as a result of his experience with Ménière’s disease, thanks to a careful recalibration of his musical tools and the integration of visual and other interpretations of sound. By Andrew Hugill As I write this article, I am in the process of composing seven short pieces under the title “Spectrum Sounds.” These are commissioned by the BBC to be broadcast on their platforms. This is a major milestone for me. Back in 2009, when I was diagnosed with Ménière’s disease, I assumed that my compositional career was over. Having been a successful composer and a professor of music, this was naturally quite devastating, but I had to face the reality that my hearing had disintegrated. To be specific: I have severe unbalanced hearing loss (right ear worse than left) that follows the classic Ménière’s profile of low frequency loss foremost; fluctuating tinnitus in both ears that varies in intensity and character on an hourly basis; and diplacusis, in which the brain registers two different pitches when a single note is played. Even the simplest music can quickly turn into a dissonant and semi-audible mush. The years between 2009 and the present day have therefore been a story of finding ways to compose with Ménière’s. This remains an ongoing project, but it is interesting to explore 20
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how I have managed to get to the present point of effectively renewing my compositional career. There was a major choice to make: Do I revert to writing “dots on paper” in the traditional way, because I at least know what they should sound like even if I cannot hear them myself? Or do I, as a composer who often works directly with digital sound, find a way to continue to do that despite my compromised hearing? In other words, should I deny and fight against my condition, or should I somehow work with it? Broadly speaking, I have chosen the latter course, but it has not been easy.
Everybody Hears Differently
In 2018 I founded the Aural Diversity project, which explicitly sets out to include musicians of all hearing types. Aural Diversity has a tagline which is objectively true: Everybody hears differently! The music business takes little account of hearing differences, so this project tries to raise awareness of conditions such as Ménière’s disease. Aural Diversity was financially supported by GN Hearing, whose wonderful ReSound hearing aids were
a great help to me in opening up musical possibilities, as well as the Arts Council England and the Arts & Humanities Research Council in the U.K. From a personal point of view, it provided the opportunity and motivation to begin composing again. The first outcome was my piece, “Thirty Minutes for Diplacusis Piano.” This began with a systematic attempt to map my own hearing by blocking each ear in turn and recording myself singing perceived pitches, then testing the results with a pitch meter. This produced a list of pitches and the discrepancies between my two ears. Pitch was not the only difference; for example, the perceived amplitude was considerably softer from 138 hertz downward and fell away steadily. I discovered other specific changes in how my hearing worked, with no diplacusis at all in one specific octave, but worsening the higher the range (the piano sounds metallic in that register anyway). The range below middle C is affected by unpredictable amounts of diplacusis note by note, and then in the lower notes it gets even more complex. Using this information, I was able
As a new form of musical notation, the rolling spectrogram became a compositional tool.
to construct a digital instrument that accurately reproduces what I hear when a normal piano is played. Composing for this instrument was extremely challenging. I was hearing my own diplacusis layered onto my own diplacusis! I realized I could only tolerate short pieces, one minute each. I also realized I needed a new form of music notation to help me understand how the sounds I was producing could work together. I used a rolling spectrogram that became a compositional tool where I could line up piano overtones with one another to create meaningful sequences. Videos of these became an integral part of the piece and were screened with the music during the Aural Diversity concerts, giving access to the music even for those who could not hear at all.
Group Setting
This worked well as a means to find some beauty and music in my own hearing, but I also wanted to work with others. “Kelston Birdsong” was written for an ensemble of musicians, ranging from profoundly deaf cochlear implant wearers, through those with a range of conductive and
sensorineural hearing losses, to those with hyperacusis or misophonia (so not exactly a loss, but a heightened sensitivity). The piece works by playing recordings of birdsong that are audible to individual musicians, who then respond with a call, to which the rest of the band respond in their turn. Ménière’s is destructive of musicians’ careers. I spent several years keeping it secret after my initial diagnosis but, at the same time, I was forced to change careers—I am now a professor of creative computing. Some Ménière’s musicians turn away from music altogether and no longer either play or listen. This is something I can well understand, because the very act of listening can become uncomfortable or even painful after a short time. For myself, I have found a way back to composing through a combination of dogged persistence and some extraordinary digital technology. I intend to continue to confront and engage with the reality of my disability in my music. I hope that in doing so, I may encourage others in a similar position to see a more positive future.
This originally appeared in the U.K’s ENT and Audiology News website, at entandaudiologynews. com. Andrew Hugill is a professor in the School of Computing and Mathematical Sciences at the University of Leicester in the U.K. For more, see the Aural Diversity project at auraldiversity.org and andrewhugill. com. You can find his “Thirty Minutes for Diplacusis Piano” at andrewhugill. com/music/thirtyminutes, and “Kelston Birdsong” at andrewhugill. com/music/kelston_birdsong.html.
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winter 2022
21
Check, Check, Check
A constellation of conditions helps explain hearing and balance difficulties. By Mary Horton
22
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Vrroom! “Did you hear that?” I don’t know how many times I asked my family and close friends that question, before I finally quit asking, as they all looked at me like I had lost my marbles. Vrroom! Why won’t it quit? Why do I have a vacuum cleaner running in my head all the time? Am I going crazy? About the time I seriously admitted that I had a hearing problem, I also heard this vacuum cleaner noise constantly. Even after I began wearing hearing aids, it was still there. So, in addition to not really being able to hear conversations because of my hearing disability, anyone speaking to me had to speak clearer and louder than my vacuum cleaner! Okay, it was time for me to learn more. This is what I found. Tinnitus is a phantom sound. Typical-hearing people can hear a pin drop in the quiet, but people with tinnitus hear a constant ringing or roaring in their ears. Mine was neither. It was definitely a vacuum cleaner! Also, tinnitus in some people comes and goes. Mine never went away, day or night. Ever. Ménière’s disease is an inner ear disorder leading to fluctuating hearing loss, tinnitus, and vertigo, or “spinning.” When I first began hearing the constant vacuum cleaner, I did not have vertigo, although I did develop it nearly 20 years later. And when they say spinning, they are not kidding! My otolaryngologist tested my ears in every possible way. Each time, he was surprised that my hearing disorder was different from what was “typical.” Most people with hearing loss, especially in older age (check! ), have difficulty hearing higher voices and tones, but I could barely hear the deeper tones, bass tones, and generally men’s voices—and especially my husband’s. Not a great thing for a happy marriage, even when he is helpful and supportive! Several years later, I actually found a name for my vacuum cleaner. It is hyperacusis. It is your brain being sensitive to, and overamplifying, sounds in your external environment. For some people, hyperacusis is only a minor irritation. Other people experience serious side effects, including loss of balance and seizures. This hearing disorder makes it difficult to handle everyday sounds. Certain sounds may seem unbelievably loud, even though people around you don’t seem to notice them. Stress can further increase this sound sensitivity. The most common cause of hyperacusis is damage to the inner ear from loud noise (like maybe from front row, speaker-adjacent seats at rock concerts for several years when I was a young music reporter?). Another ! Vestibular (balance) hyperacusis is when you experience dizziness or feel unsteady in reaction to everyday sounds. This is what I learned: The hearing and balance systems of the inner ear are both filled with fluid. The movement of this fluid stimulates the sensory cells, making them vibrate. The sounds are sensed and converted into electric signals that the auditory nerve sends to the brain to be interpreted. From what I understand, vestibular hyperacusis is a misinterpretation and confusion of these vibrations and signals, possibly due to nerve damage in the balance organs, throwing the balance system akilter. Instead of feeling pain from everyday sounds, everyday sounds can make you feel dizzy. Let’s add Ramsay Hunt syndrome (herpes zoster oticus) to the whole package, which occurs when a shingles outbreak affects the facial nerve near your ear(s). In addition to the painful shingles rash, this can cause facial paralysis and hearing loss in the affected ear. Yep, another !
balance
My otolaryngologist tested my ears in every possible way. Each time, he was surprised that my hearing disorder was different from what was “typical.” Most people with hearing loss, especially in older age, have difficulty hearing higher voices and tones, but I could barely hear the deeper tones, bass tones, and generally men’s voices—and especially my husband’s. Not a great thing for a happy marriage, even when he is helpful and supportive! I had shingles on my face not once, but twice. What little bit of hearing I had in one ear was almost totally obliterated with my shingles bouts! (And yes, shingles occurs more frequently in older people, as it’s the same virus that causes chickenpox, which most older people have had.) Is it known yet, whether the order of these conditions is interchangeable? No. But, it is interesting to note that in my case, about five years after I finally conceded that I had a hearing problem, I had the first case of shingles, which concentrated solely on the right side of my head, taking most of my remaining hearing in my right ear. I developed the primary symptom (vertigo) of Ménière’s disease 20 years after the vacuum cleaner took up permanent residence in my head. With vertigo treatment, it was found that I have had seizures (another symptom of severe hyperacusis) during much of that time. I have learned to live with my vacuum cleaner, although I tend to avoid gatherings of more than a couple people, or people whom I do not know well. I almost never talk on the telephone, but most friends and many businesses with which I associate routinely know to text or e-mail me, since I cannot hear a phone conversation. I still occasionally have dizzy spells, particularly when getting out of bed, so I am very careful not to get out of bed too quickly, or sit up too quickly. Loss of hearing is isolating in many ways. But there is much to do, many paths to travel, and many people to meet along the way, regardless. Always look at the glass as being half full. You are not alone! Hearing disabled or not, we are all here for a reason. “Listen”… and find it! Mary Horton lives in Alabama. For references, see hhf.org/winter2022-references. Share your story: Have you experienced a balance condition? Tell us at editor@hhf.org.
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winter 2022
23
h e a ri n g h e alt h fo u n dation
6 Ways to Raise Youth Awareness of Noise-Induced Hearing Loss
Creative, engaging ways to share healthy hearing habits through social media can have a positive impact on teens and young adults.
“I wish I had known not to stand so close to the loudspeakers,” says a 17-year-old patient to me during her routine audiometric visit, explaining how her hearing has never been the same—riddled with high-pitched tinnitus and distorted hearing—ever since the fateful concert, one year ago. As many as one in five school-aged children (ages 12-19 years) shows signs of hearing loss as a result of excessive noise exposure, according to the Centers for Disease Control and Prevention (CDC). Noise-induced hearing loss (NIHL) not only affects a child’s hearing ability but their academic performance, social interactions, and overall mental health and well-being. Unfortunately, in my clinical experience, the damage is already done by the time children and young adults like my teen patient become aware of the dangers of noise exposure. There are many resources and tools available for educating and combating NIHL, yet there is a significant disconnect between recommended safe hearing practices and the choices made by the younger generation. The CDC says part of the reason for the gap is a failure to effectively reach and educate the younger population about the dangers of excessive and loud noise exposure. Because NIHL is irreversible but completely preventable, effective and engaging safe hearing practices that resonate with children and young adults are critical. 24
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illustration credit: wikimedia commons
By Rohima Badri, Ph.D.
hearing health
In places where there are loud noises, such as concerts, sports arenas, and school music rooms, attractive visual posters and images with bitesize messages about safe hearing practices can serve as a good reminder. Similarly, “loud noise” warning signs near noise sources such as loudspeakers and construction zones will be extremely beneficial in preventing permanent hearing damage. Here’s how to encourage young people to practice safe hearing habits and prevent hearing damage from excess noise: » Personalize the message. Parents, teachers, and health professionals should discuss the importance of safe hearing practices in a context that is meaningful to young people. Rather than simply stating that noiseinduced hearing loss will cause hearing difficulties in background noise, explain how even mild NIHL can disrupt otherwise enjoyable sports or music concert experiences, or make everyday tasks such as listening to music or following class lectures difficult and tiring. » Show instead of tell. Hands-on fun activities and live, interactive demonstrations of safe hearing practices are more likely to engage children than passive lecturing. Children, for example, can use a sound level meter (free apps available) to measure and compare noise levels in various areas of the school (near loudspeakers, cafeterias, music rooms), listen to sounds with and without hearing protection, and so on. » Youth advocacy. Even better, children can become effective learners by teaching others about NIHL. Children can take an active role in raising awareness and promoting hearing health in their schools, communities, and even online by starting or joining a club or organization. » Reminders and warning signs. In places where there are loud noises, such as concerts, sports arenas, and school music rooms, attractive visual posters and images with bitesize messages about safe hearing practices can serve as a good reminder. Similarly, “loud noise” warning signs near noise sources such as loudspeakers and construction zones will be extremely beneficial in preventing permanent hearing damage. » Easy access to hearing protective devices. Concertgoers are seven times more likely to wear hearing protection during a concert if free earplugs are provided at the venue, according to a 2015 study in the International Journal of Audiology. Making hearing protection readily available (such as in vending
machines) in areas where noise exposure is high and frequent is another extremely useful move to prevent irreversible hearing loss. » Connect, interact, and engage through social media. In the United States, 84 percent of teenagers (13-18 years old) use online resources to obtain healthrelated information, according to a 2015 Media and Communication report—and that number may be even greater now. It’s not enough to simply provide information to this group; instead, engage them through creative storytelling and motivate them to take action through calls to action. For example, start an “earplugs challenge” chain and encourage readers to upload and share photos of themselves at concerts wearing earplugs.
The Value of Healthy Hearing
Many teenagers, like my patient, are suffering or more likely to suffer from irreversible but preventable hearing damage as a result of noise exposure. To reach and engage this younger demographic, we must adapt messages and communicate the value of healthy hearing in a way that engages and resonates with them.
Rohima Badri, Ph.D., lives in New Jersey. To learn about HHF’s Keep Listening hearing loss prevention campaign, see hhf.org/keeplistening. For references, see hhf.org/winter2022-references.
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winter 2022
25
balance
h ear i n g h ealth foundation
My Seasonal Spins
By Shari Eberts
Rising out of my backbend in yoga class, the world shifted, leaving my mind and body reeling from the dizziness. Luckily my knees were planted firmly on the ground so I did not topple over, but it was disorienting and very unpleasant. As I turned around to lie down in shavasana, I knew it was the start of my least favorite time of the year. Each winter as the weather turns colder, my seasonal tinnitus spikes, and the seasonal spins make their unwelcome appearance. It is always a rough period of time as my body acclimates to the changes in temperature and atmospheric pressure that come with winter.
Tinnitus and Vertigo Are Seasonal
It turns out that I am not alone in my seasonal suffering. According to a study in the Journal of Otology, vertigo is in fact seasonal. The researchers found statistically significant seasonal variation in benign paroxysmal positional vertigo (BPPV), which is rotational vertigo induced by head position changes. The correlation of BPPV with temperature and atmospheric pressure changes was also statistically significant. In other words, this type of vertigo comes and goes with the weather. The study also showed meaningful links between BPPV and many other factors, including sex and age. BPPV was found to be most prevalent in middle-aged women, and linked to lower levels of vitamin D, lower bone density, and nasal allergies. None of this is good for me, a 50-plus-yearold woman living in New York City.
My Tips for Managing
Vertigo can be quite debilitating, especially in extreme cases. While my case is mild, here’s how I lessen its impact. Slow down. It’s hard to jump from activity to activity when you feel dizzy and nauseous, so I force myself to slow down, take breaks, and moderate my schedule. Lying down in the dark with my eyes closed can help. Move mindfully. Abrupt head movements can bring on a sudden bout of dizziness, so I move as mindfully and methodically as I can. I take extra time to shift position, to 26
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According to a study in the Journal of Otology, vertigo is in fact seasonal. The researchers found statistically significant seasonal variation in benign paroxysmal positional vertigo (BPPV), which is rotational vertigo induced by head position changes. The correlation of BPPV with temperature and atmospheric pressure changes was also statistically significant. In other words, this type of vertigo comes and goes with the weather. stand up or to lie down, hoping not to shock my system. During my yoga practice, I move in and out of backward and forward bends very slowly, and if I need to, I skip the postures that seem to aggravate the condition. Make diet adjustments. While I personally don’t feel a noticeable impact from changes in diet, the Mayo Clinic suggests avoiding caffeine, alcohol, salt, and tobacco to help curb dizziness. It also recommends avoiding driving or operating heavy machinery in case a bout of dizziness appears without warning. After a few weeks, I am thankful (and know I am lucky) that life usually returns to normal.
Staff writer Shari Eberts serves on the Board of the Hearing Loss Association of America and is a past chair of HHF’s Board of Directors. “Hear & Beyond,” a new book she coauthored with Gael Hannon, is due out in May 2022. This originally appeared on Eberts’s blog, livingwithhearingloss.com. For references, see hhf.org/winter2022-references.
Share your story: Do you get the seasonal spins? Tell us at editor@hhf.org.
planned giving
A Personal and Professional Connection to the Mission to Research and Cure Hearing Loss By Ellie Daniels
Ellie Daniels with her son Frank.
When I was 5, I had an ear infection in my right ear that showed no improvement after taking antibiotics, and it quickly turned into something really serious. The doctors told my parents that there was a growth in my ear, but it was isolated to one of the ear’s tiny bones. I underwent surgery to remove the bone and have it cleaned, then had another surgery to put the bone back in my ear. At age 11, I was at a routine appointment with my ear, nose, and throat specialist and we were told that they needed to operate immediately, because the growth had not been completely removed six years prior. It turns out that a cholesteatoma—an abnormal growth of skin cells—had been developing for years in the back of my ear. It had exposed itself to the lining of my brain, wrapping around the nerve that sends impulses from my brain to the rest of my body. After an eight-hour, open ear surgery, it was removed, but not without removing and/or restructuring a large portion of my inner ear. This all happened so long ago that it’s challenging for me to remember details. The hearing loss in one ear was really considered collateral damage to removing such a scary and aggressive growth, and I think everyone was grateful that this was the only long-term effect I had to deal with. Though, truthfully, I haven’t really “treated” my hearing loss. After the rebuilding of my inner ear and canal, there wasn’t enough tissue for a cochlear implant, and I have never tried to get a hearing aid. I think I made the excuse that I would likely lose this expensive piece of machinery
soon after I got it, but honestly I think the (imagined) stigma of having to use a hearing aid as a young professional is what held me back. So I’ve tried to see my hearing loss as a blessing as much as possible. Over the years it has helped me drown out things like fussing parents, uncomfortably loud music at the gym, and a crying baby during sleep training. I find it especially helpful when I’m trying to sleep and my husband is snoring—I just put my bad ear up! And now coincidentally in my career, as a partner success strategist at FreeWill I have had the unique opportunity to support Hearing Health Foundation with their planned giving program. FreeWill allows you to create your will online, for free, in 20 minutes or less, while also leaving transformational bequests to nonprofits like HHF. I think it’s imperative to support those nonprofits that do the hard work to make a positive impact in our community. The work that HHF does is so important. I encourage everyone to check out freewill.com/hhf and create a legacy dedicated to hearing health and the prevention and cure of hearing loss and tinnitus.
Ellie Daniels lives with her husband and sons, Frank and Wade, in North Carolina.
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9 Things to Know About Hearing Loss From the Perspective of the Communication Partner By Pat Dobbs
If, like me, you have a hearing loss, you know what your hearing challenges are. But what about your friends and family? Do they have challenges communicating with you? I bet the answer is yes. Why wouldn’t they? Hearing loss is a communication disorder that affects all the people in our lives. The challenges our hearing loss presents to our friends and family, also known as our communication partners, depend on factors such as the length and severity of our hearing loss and the depth of our relationships. This article is inspired by what I’ve learned from my teacher and mentor Sam Trychin, Ph.D., a psychologist, educator, author, and creator of his Living With Hearing Loss Program. Here are a few comments I’ve heard informally from friends who live with hearing impaired people (like me) and insights into what both sides may be thinking.
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We don’t acknowledge our hearing loss.
For many of us, it can take years before we acknowledge our hearing loss and get hearing aids. Or we get hearing aids but keep them safely tucked away in a drawer. Both scenarios are frustrating and 28
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annoying for our partners. They have to talk louder, listen to blaring TVs, and hope we understand important instructions and information. It’s a shame that something could be done about these problems, but regrettably, we tend to either deny that there is a problem or hope it will go away on its own.
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We ask our partners to change how they talk to us.
So that we can better understand our partners, we ask them to make direct eye contact when they talk to us, not to talk with their backs turned to us or from another room, talk more clearly, louder but not too loud, slower but not too slow. (Are we demanding or what!) For instance, Debbie says she tries to talk in whichever way it’s best for her husband with a hearing loss to hear her. But sometimes she forgets. After all, it’s different from the way she and other people with typical
hearing have been communicating all their lives. Even when she talks as he has asked her to, her husband may misunderstand what she’s saying and he asks her to repeat. Debbie tries not to get annoyed but admits she does. She understands why her husband doesn’t hear, but it’s still challenging for her. Patience is necessary. Patience, she reminds herself again and again. Laura says it’s hard for her to remember to not call to her husband from another room, as she’s done for years. It’s inconvenient because now she has to go where he is. She misses not being able to whisper a comment at the movies. Now Laura says she waits until she can speak in a normal tone. Then she may forget what she wanted to say. And she says she sorely misses intimate bedtime conversations in the dark because her husband now needs to have light on her face in order to hear her.
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We often bluff.
One of our worst habits is bluffing, pretending to understand what our partner has said when we haven’t. Our partners hate this. They wonder what’s the use of talking if we just mimic back facial expressions or respond out of context. Gary says he can tell when his wife is bluffing and calls her on it immediately. Barbara hates calling her husband on it because he’s sensitive and embarrassed by his hearing loss. She tries to rephrase her questions, hoping he will give her a valid answer, rather than pretend.
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We get insulted when our partners say “never mind.”
There’s nothing we hate more than when our partners say “never mind” when we ask them to repeat a passing punch line. They know that perfect moment is lost and wouldn’t make sense. So how do they feel about repeating that punchline? It’s awkward for them as that critical moment is lost. But if they don’t repeat it, we’ll get angry or hurt or something in between. We all know how we feel if someone says “never mind.” Think about how they feel when they have to repeat something that only works in the moment.
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We can talk to our partners, but often they can’t respond to us.
There are situations when we can talk to our partners, but they can’t respond where we can understand them. Examples are when we’re driving, have a headache, or have auditory fatigue and are too tired to focus on listening. Then there are the times when we ask a question without a simple yesor-no answer, and forget we can’t look at our partners because we’re driving or need to be looking in another direction. We’re forcing them to wait to give us an answer. This is one of Anne’s complaints. It annoys her that her hearing loss husband can talk to her, but when he’s driving or tired, she can’t respond to him because he wouldn’t hear her. Annoying, Anne says, very annoying.
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We make our partners deal with blaring radio and TV.
Unless we’ve learned how to listen to the radio and TV with our hearing aids or assistive listening devices, we turn up the volume so loud that it can hurt our partner’s ears. Ellen told me her husband refuses to get hearing aids. He cranks up the volume of their TV so loud that it’s painful to her ears and she can’t watch in the same room with him anymore. Now he watches TV in another room with a closed door. Even with the door shut, it’s still too loud. Aside from the noise, what she misses most is the intimacy of watching TV together.
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We resist confirming important information.
If our partner is relating important information like when and where to meet them, they may ask us to repeat it to confirm that we’ve gotten it right. Hopefully, we have the grace to do that, but we’re often annoyed at being second-guessed. Henry says that when he asks his hard of hearing wife to repeat critical information, she slams back that she’s got it, only to find out later that she misheard. Of course, this causes problems and total frustration that could easily have been avoided had she confirmed what Henry said or he had written the instructions down.
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We often tune out.
What should our partners do when they know we can’t hear a conversation and tune out? Should they be our ears and repeat everything, or summarize the highlights? Should they let us deal with the situation ourselves? The answer probably depends on how important the information is, the mood, and the setting. John feels awful when he sees his wife left out like that, but he’s never sure what to do. He doesn’t want her to feel ignored but worries that he might be helping her too much. Would it be better for her to advocate for herself? He’s never sure of the right balance. And sometimes, when he does advocate for her, she gets annoyed because she feels he’s overstepping.
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What is clear is that hearing loss is a communication disorder that affects all our relationships. Working through communication problems isn’t easy. Partners that have been together a long time still have their glitches. Good communications mixed with love, humor, and patience helps everyone deal with these difficult and frustrating situations.
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We’re saddened by our loss but so are our partners.
Our partners are saddened by the loss of communication they once had with us because we interact less as our hearing gets worse. We try to avoid social gatherings like family events, or different types of entertainment because it is too difficult for us to understand what is being said. If we do go, people often think we are rude or depressed because we don’t participate. This directly affects our partners. They miss the interaction we used to have. Then there are the private conversations at home. In bed at night, we need to have the light on to see our faces. When we hug, there’s a good chance our hearing aids or cochlear implants will fall out. Our partner’s words of love could provoke romantic responses like “Huh? What’d you say?” Really romantic!
The Five Stages of Loss
Our communication loss is also our partner’s loss. We both go through the five stages of loss: denial, anger, bargaining, depression, and acceptance. Each person experiences them in different ways and at different times. What is clear is that hearing loss is a communication disorder that affects all our relationships. Working through communication problems isn’t easy. Partners that have been together a long time still have their glitches. Good communications mixed with love, humor, and patience helps everyone deal with these difficult and frustrating situations. I have to chuckle to myself— because communicating is the answer to working out our challenges because this is a communication disorder. Thank goodness we can laugh.
Pat Dobbs started to lose her hearing when she was 20 and today is the happy recipient of bilateral cochlear implants. Through most of her life she bought into the stigma of hearing loss until finally she came to terms with it. She then launched the Hearing Loss Association of America Morris County Chapter in New Jersey, serving as president. She also attended Gallaudet University’s two-year peer mentoring program. Inspired by this program, she designed and facilitated a series of workshops on managing hearing loss, and began a coaching practice focusing on the personal, career, and relationship challenges unique to people with hearing loss. Dobbs is the president of SayWhatClub, a global online hearing loss support group and a new resident of Deer Isle, Maine. For more, see Hearing Loss Evolution, at hearinglossevolution.com.
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living with hearing loss
Sarah Kirwan and Carl Deriso married in August 2020, after meeting through a multiple sclerosis self-help group.
A Complicated and Special Case With another condition clouding the symptoms, a diagnosis of an inner ear problem is long in coming but sparks a mission to help others with multiple disabilities. By Sarah Kirwan In June 2010, I stepped off a plane in Los Angeles with a painfully full left ear, muffled hearing, throbbing eyes, vertigo, and a migraine. I also got off that plane with no idea I would straddle the line between a nondisabled and disabled identity for the rest of my life. Over the next eight months, I sat in offices answering questions and completing tests, while medical specialists sat baffled. There was an otolaryngologist, neuroophthalmologist, neuro-psychometrist, rheumatologist, clinical psychologist, audiologist, and neurologist, among others. And the tests were never-ending: from evoked potential tests, MRIs, and batteries of blood tests to vestibular-evoked myogenic potential and neuro-cognitive testing. A decade later, the doctor visits and medical testing continue, but the frenzied pace has slowed and I can finally breathe again. I think back to those times when I was seeing two or three doctors each week, and I can remember one specialist smiling at me and saying, “Yours is a complicated and special case.” At that time, I didn’t realize how right he was.
Searching for a Diagnosis
I am in my early 40s and diagnosed with both multiple sclerosis and superior semicircular canal dehiscence, a very rare inner ear disease affecting 1 to 2 percent of the world’s population. While my MS diagnosis took about eight months, my SSCD went undiagnosed and misdiagnosed for almost nine years. UCLA Health defines SSCD as “a tiny hole (dehiscence) that develops in one of the three canals inside the ear. Healthy individuals have two holes or ‘mobile windows’ in their dense otic capsule bone, but those with SSCD have developed a third hole.” The exact cause of SSCD is unknown. The condition is usually diagnosed in middle age, though it has been seen in small children. The UCLA website adds, “The thinning or absence of bone located in the semicircular canal triggers vertigo, hearing loss, disequilibrium, and other vestibular and auditory symptoms. A common clinical symptom of SSCD, reported by patients, is the abnormal amplification of internal body sounds, such as heartbeats and eye movements.” Hearing internal bodily sounds is called autophony. a publication of hearing health foundation
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Far left: Kirwan before the successful surgical repair of her left ear’s superior semicircular canal dehiscence. Kirwan is the volunteer director of the only women’s wheelchair basketball team in California, having played and coached for years.
MS and SSCD share symptoms, such as fatigue, acquired nystagmus (uncontrollable rapid eye movements), migraines, vertigo, and balance loss—and I was experiencing all of these. I was also experiencing other identifiable MS symptoms, such as numbness and tingling sensations, Lhermitte’s sign (which feels like an electric shock), and muscle spasms. However, the one symptom I was experiencing that could only be connected back to SSCD was autophony. Hearing my organs moving and functioning was so disturbing and debilitating that this symptom combined with every other symptom I was experiencing reduced my life to one of survival and scarcity. It almost sent me into a psychiatric hospital. Or worse yet, over the side of a bridge, as I wrestled with suicidal thoughts and ideations I’d never experienced before.
Autophony-Induced Anxiety
The first time I heard my eyeballs moving, I thought, “There’s no way I could’ve just heard that.” I sat there for a bit, looking from side to side, to see if the noise would stop. It only got louder. I was shocked. The sound was nauseating—a mixture of grinding, whooshing, and fingernails on chalkboards. Then came my heartbeat in my left ear. The more animated and excited I’d become, the louder my heartbeat would get. It finally got to a point where I could barely function or hold conversations with people, as my brain fog worsened. After that, it was the sound of the heels of my feet connecting with the ground or floor beneath me as I walked. Then, my body adjusted its own walking, so the balls of my feet would hit first. I could also hear the mechanics of the muscles in my throat as I swallowed. It was getting more difficult by the 32
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day to hear anything over the noise of my internal organs. I started retreating from life and isolating myself. I was becoming less likely to be the life of the party, as I’d been. For nine years, I lived like this. Each morning, I was greeted by the sound of my heartbeat in my left ear and the sounds of my overwhelming tinnitus that had developed somewhere along the way. During this time, I was diagnosed with major depressive disorder and general anxiety disorder. However, the SSCD continued to go undiagnosed, as providers tied all of these symptoms back to my MS, and insisted I had additional undiagnosed mental health conditions—possibly bipolar depression or schizophrenia. Fearfully, I stopped talking about hearing my internal organs moving. In the summer of 2018, I finally found the answers for myself, when I came across an article about autophony during one of my medical research binges. This led me to SSCD and a self-diagnosis, which I took to my neurologist. I remember pulling out my notebook with printed SSCD articles and explaining to her that this is what I’ve been talking about for all these years. I begged her to conduct a CT scan of my temporal bone, so we would know if my symptoms contributed to SSCD or not and move forward from there. She refused, and once again claimed I had undiagnosed mental health conditions, made sure I was seeing a therapist, and told me there was nothing else going on with my health. When I continued advocating for myself, she said we’d be able to see the inner ear canal in my next MRI, which was wholly untrue, and she became defensive and argumentative. I let it go, fearing retaliation and knowing I would get nowhere. I was keenly aware of how much I needed her—finding a new doctor when you’re barely surviving is not an option.
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Living with multiple invisible disabilities presents numerous challenges that are often misunderstood by the nondisabled community, as well as by some folks within the disability community. My goal is to help individuals, businesses, and communities better understand visible and invisible disabilities, so we can make a more accessible and inclusive world for everyone. Finding Answers
For the next three months, I was too depressed to do much. And then a day came when I felt that familiar feeling of determination rise up within me again. I worked around my neurologist and went directly to UCLA’s department of neurosurgery, where I’d found two world-renowned surgeons researching and providing treatment for SSCD: Quinton S. Gopen, M.D., and Isaac Yang, M.D. I knew they could help me but when I called, in June 2019, I was told I needed an SSCD diagnosis to see them. I burst into tears, unable to hold back nine years of fear, sadness, anxiety, frustration, and loss. Through sobs, I asked, “Where can I get a diagnosis? No one will believe me. Where can I go where they’ll listen to me? No one is listening. Will you please help me? Please.” Hearing the desperation in my voice, the staff member I spoke with took my case to her superiors. One week later, I was sitting in their office. I will never forget when the physician assistant looked into my eyes and said, “We believe you, and we’re going to help you.” Once again, I burst into tears. I did not belong in a psychiatric facility. I did not have bipolar depression or schizophrenia. What I did have was validation and an official diagnosis—bilateral SSCD—meaning both ears are affected. At the time, I was the only patient Dr. Gopen and Dr. Yang had seen who was diagnosed with both MS and SSCD, so they proceeded with caution to treat me. In October 2019, they performed a surgical repair of my left ear SSCD, which includes a skull-base, middle-fossa craniotomy for canal-repair resurfacing, plugging, or capping the bony dehiscence (hole). When I woke up from the surgery, I could no longer hear any of my internal organs. The heartbeat in my left ear was gone, and I could finally hear out of it. For the first time in nine years, the external noises of the world were louder than the internal sounds of my body. It was incredible, but it would take some getting used to. Sounds were extremely loud and I couldn’t handle even the smallest crowds of people, but my body and brain slowly started adjusting. And now, two years post-surgery,
70 percent of the symptoms I initially presented with have been alleviated. Eventually, I will need the same surgery done on my right ear, but I rest easy knowing I won’t have to wait for my symptoms to debilitate me further before I can do so.
Access and Allyship Living with multiple invisible disabilities presents numerous challenges that are often misunderstood by the nondisabled community, as well as by some folks within the disability community. My goal is to help individuals, businesses, and communities better understand visible and invisible disabilities, so we can make a more accessible and inclusive world for everyone. In 2020, I officially launched my woman- and disability-owned small business, Eye Level Communications LLC and, in 2021, the “Incluse This!” podcast. Using an intersectional approach, Eye Level provides strategic consulting and training services for disability access, inclusion, and allyship. The podcast is a safe and coalitional space specifically created for critical conversations that bring disability to the forefront of the greater diversity conversation. While the tools are different, the mission of both is the same—equity for all people living with disabilities. I want to change disability policies and procedures. Most importantly, I want to change mindsets and perceptions, along with how we talk about disability in America and around the world. Sarah Kirwan lives in California. For more, see eyelevel.works.
Share your story: Tell us your hearing loss journey at editor@hhf.org.
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research
Presenting the 2022 Emerging Research Grantees Through the Emerging Research Grants (ERG) program, Hearing Health Foundation (HHF) provides seed money to scientists working across the entire spectrum of hearing and balance research, including many underfunded areas. Since 1958, ERG grants have played a foundational role in the careers of many academic researchers and clinicians in hearing and balance fields. The competitive ERG program awards grants to only the most promising investigators. Recipients are exceptionally well-positioned to win future grants from the National Institutes of Health and other federal research funders, leading to dramatic innovations in the field. Timothy Balmer, Ph.D.
Arizona State University
Project: The role of unipolar brush cells in vestibular circuit processing and in balance Description: The cerebellum receives vestibular sensory signals and is crucial for balance, posture, and gait. Disruption of the vestibular signals that are processed by the vestibular cerebellum, as in the case of Ménière’s disease, leads to profound disability. Our lack of understanding of the circuitry and physiology of this part of the vestibular system makes developing treatments for vestibular disorders extremely difficult. This project focuses on a cell type in the vestibular cerebellum called the unipolar brush cell (UBC). UBCs process vestibular sensory signals and amplify them to downstream targets. However, the identity of these targets and how they process UBC input is not understood. In addition, the role of UBCs in vestibular function must be clarified. Experiments will identify the targets of UBCs, their synaptic responses, and the role of UBCs in balance. A better understanding of vestibular cerebellar circuitry and function will help us identify causes of vestibular disorders and suggest possible treatments for them.
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Long-term goal: To develop a better understanding of the neural circuits that underlie vestibular function. A more complete understanding of the circuitry and physiology of the vestibular cerebellum is necessary to develop therapies for vestibular dysfunction caused by peripheral disorders such as Ménière’s disease. Balmer earned his doctorate in neuroscience from Georgia State University. He is an assistant professor in the School of Life Sciences at Arizona State University. His work investigates how neurons transmit signals to one another through synapses and how networks of neurons process information. His team uses in vitro and in vivo electrophysiology, optogenetics, immunohistochemistry, and computational modeling to understand fundamental mechanisms of sensory processing. Balmer was also a 2017 ERG grantee.
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James W. Dias, Ph.D.
The Medical University of South Carolina Project: Neural determinants of age-related change in auditory-visual speech processing Description: Older adults typically have more difficulty than younger adults identifying the speech they hear, especially in noisy listening environments. However, some older adults demonstrate a preserved ability to identify speech that is both heard and seen. This preserved audiovisual speech perception by older adults is not explained by an improved ability to speechread (lipread), as speechreading also typically declines with age. Instead, older adults can exhibit an improved ability to integrate information available from across auditory and visual sources. This behavioral evidence is consistent with findings suggesting that the neural processing of audiovisual speech can improve with age. Despite the accumulating and intriguing evidence, the underlying changes in brain structure and function that support the preservation of audiovisual speech perception in older adults remain a critical knowledge gap. This project uses an innovative neural systems approach to determine how age-related changes in cortical structure and function, both within and between regions of the brain, can preserve audiovisual speech perception in older adults.
Long-term goal: To identify the unique contributions of sensory processing, attention, and memory to better understand how multisensory integration changes with age. If we can find the cortical structures and connections between structures that facilitate a multisensory mechanism that can compensate for hearing (and vision) loss, then this mechanism can be exploited to improve the communicative abilities of older adults. Dias received his doctorate in psychology from the University of California, Riverside. He completed postdoctoral training in the department of otolaryngology–head and neck surgery at the Medical University of South Carolina, where he is now an assistant professor. His research concerns the multisensory processing of speech and how cross-sensory recruitment may compensate for unisensory deficiencies. Dias’s 2022 ERG grant is generously funded by the Meringoff Family Foundation.
Thank You to Our Grants Reviewers As always, HHF extends its profound thanks to our peer reviewers, a revolving group of research scientists and clinicians who donate their time and expertise to provide comprehensive review of each and every ERG proposal HHF receives. Without these reviewers’ dedication to advancing hearing and balance science, supporting newer investigators’ careers, and sustaining HHF’s mission, HHF could not direct our donors’ support to the innovative and research Support ourpromising research: hhf.org/donate. HHF has had the privilege of funding since 1958. a publication of hearing health foundation
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Subong Kim, Ph.D. Purdue University
Project: Influence of individual pathophysiology and cognitive profiles on noise tolerance and noise reduction outcomes Description: Listening to speech in noisy environments can be significantly challenging for people with hearing loss, even with help from hearing aids. Current digital hearing aids are commonly equipped with noise-reduction algorithms; however, noise-reduction processing introduces inevitable distortions of speech cues while attenuating noise. It is known that hearing-impaired listeners with similar audiograms react very differently to background noise and noise-reduction processing in hearing aids, but the biological mechanisms contributing to that variability are particularly understudied. This project is focused on combining an array of physiological and psychophysical measures to obtain comprehensive hearing and cognitive profiles for listeners. We hope this approach will allow us to explain individual noise tolerance and sensitivity to speechcue distortions induced by noise-reduction
Manoj Kumar, Ph.D. University of Pittsburgh
Project: Signaling mechanisms of auditory cortex plasticity after noise-induced hearing loss Description: Exposure to loud noises is the most common cause of hearing loss, which can also lead to hyperacusis and tinnitus. Despite the high prevalence and adverse consequences of noise-induced hearing loss (NIHL), treatment options are limited to cognitive behavioral therapy and hearing prosthetics. Therefore, to aid in the development of pharmacotherapeutic or rehabilitative treatment options for impaired 36
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processing in hearing aids. With these distinct biological profiles, we will have a deeper understanding of individual differences in listeners and how those profiles affect communication outcomes across patients who are clinically classified with the same hearing status. This study’s results will assist in the development of objective diagnostics for hearing interventions tailored to individual needs. Long-term goal: To advance our understanding of the biological mechanisms of the impaired auditory and cognitive systems with the intent of improving customizable hearing interventions based on listeners’ individual differences. Kim received his doctorate at the University of Iowa, where he studied the neural correlates of variability in speech-in-noise perception using high-density electroencephalography (EEG) under the guidance of Inyong Choi, Ph.D. Kim is currently a postdoctoral research fellow at Purdue University in the laboratory of Hari Bharadwaj, Ph.D. (Both Choi and Bharadwaj are ERG alumni.)
hearing after NIHL, it is imperative to identify the precise signaling mechanisms underlying the auditory cortex plasticity after NIHL. It is well established that reduced GABAergic signaling contributes to the plasticity of the auditory cortex after the onset of NIHL. However, the role and the timing of plasticity of the different subtypes of GABAergic inhibitory neurons remain unknown. Here, we will employ in vivo two-photon Ca2+ imaging and track the different subtypes of GABAergic inhibitory neurons after NIHL at single-cell resolution in awake mice. Determining the inhibitory circuit mechanisms underlying the plasticity of auditory cortex after NIHL will reveal novel therapeutic targets for treating
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Matthew Masapollo, Ph.D.
congenital hearing loss and CIs, with and without sensory feedback.
Project: Contributions of auditory and somatosensory feedback to speech motor control in congenitally deaf 9- to-10-year-olds and adults
Long-term goal: To fully explicate the roles of auditory and somatosensory feedback in the development and maintenance of speech motor control in typical-hearing individuals and individuals with congenital hearing loss who received cochlear implant devices. The findings will allow communication scientists and clinicians to develop novel, mechanistically driven techniques to optimize speech motor instruction to deaf children.
University of Florida
Description: Cochlear implants have led to stunning advances in prospects for children with congenital hearing loss to acquire spoken language in a typical manner, but problems persist. In particular, children with CIs show much larger deficits in acquiring sensitivity to the individual speech sounds of language (phonological structure) than in acquiring vocabulary and syntax. This project will test the hypothesis that the acquisition of detailed phonological representations would be facilitated by a stronger emphasis on the speech motor control associated with producing those representations. This approach is novel because most interventions for children with CIs focus strongly on listening to spoken language, which may be overlooking the importance of practice in producing language, an idea we will examine. To achieve that objective, we will observe speech motor control directly in speakers with
and rehabilitating impaired hearing after NIHL. Also, because auditory cortex plasticity is associated with hyperexcitability-related disorders such as tinnitus and hyperacusis, a detailed mechanistic understanding of auditory cortex plasticity will highlight a pathway toward the development of novel treatments for these disorders. Long-term goal: To identify the molecular and cellular therapeutic targets for treating and rehabilitating the impaired hearing associated with tinnitus and hyperacusis.
Masapollo received his doctorate in communication sciences and disorders from McGill University and completed two postdoctoral fellowships, one in cognitive, linguistic, and psychological sciences at Brown University, and one in speech and hearing science at Boston University. He is the director and principal investigator of the University of Florida’s Laboratory for the Study of Cognition, Action, and Perception of Speech (CAPS), which was established in 2020.
Kumar received his doctorate in neuroscience and pharmacology from West Virginia University and completed his postdoctoral research in the department of otolaryngology at the University of Pittsburgh, where he is currently a research assistant professor. Kumar’s 2022 ERG grant is generously funded by the General Grand Chapter Royal Arch Masons International.
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Robert Raphael, Ph.D. Rice University
Project: Understanding the biophysics and protein biomarkers of Ménière’s disease via optical coherence tomography imaging Description: Our sense of hearing and balance depends on maintaining proper fluid balance in a specialized fluid in the inner ear called the endolymph. Ménière’s disease is an inner ear disorder associated with increased fluid pressure in the endolymph that involves dizziness, hearing loss, and tinnitus. Ménière’s disease is difficult to diagnose and treat clinically, which is a source of frustration for both physicians and patients. Part of the barrier to diagnosing and treating Ménière’s disease is the lack of imaging tools to study the inner ear and a poor understanding of the underlying causes. The goal of this research is to develop an approach to noninvasively image the inner ear and study the internal structures in the vestibular system in typical and disease states. We will utilize optical coherence tomography (OCT), a technique capable of imaging through bone, and observe changes in the fluid compartments in the inner ear. The expected outcome of this research will be the establishment of a powerful noninvasive imaging platform of the inner ear that will enable us to test hypotheses, in living animals, on how ion transport regulates the endolymph, how disorders of ion transport cause disruption of endolymphatic fluid, and how the expression of different biomarkers leads to disorders of ion transport.
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Long-term goal: To establish a new noninvasive method to image the inner ear that can lead to a full biophysical understanding of the molecular mechanisms underlying Ménière’s disease and related inner ear disorders that cause hearing loss, and to inspire new clinical interventions for diagnoses and treatments of inner ear diseases. Raphael received his doctorate in biophysics from the University of Rochester. He did postdoctoral training in the department of biomedical engineering and the Center for Hearing and Balance at Johns Hopkins University. He is currently an associate professor in the department of bioengineering at Rice University where he directs the Membrane and Auditory Bioengineering Laboratory. The lab studies the biophysics of auditory and vestibular hair cells and develops models of ion transport and synaptic transmission in the inner ear. Raphael was also a 2007 ERG grantee.
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Megan Beers Wood, Ph.D. Johns Hopkins University School of Medicine
Project: Type II auditory nerve fibers as instigators of the cochlear immune response after acoustic trauma Description: A subset of patients with hyperacusis experience pain in the presence of typically tolerated sound. Little is known about the origin of this pain. One hypothesis is that the type II auditory nerve fibers (type II neurons) of the inner ear may act as pain receptors after exposure to damaging levels of noise (acoustic damage). Our lab has shown that type II neurons share key characteristics with pain neurons: They respond to tissue damage; they are hyperactive after acoustic damage; and they express genes similar to pain neurons, such as the gene for CGRP-alpha. However, type II neurons are not the only cell type that respond to acoustic damage. The immune system responds quickly after damaging noise exposure. In other systems of the body such as the skin, CGRP-alpha can affect immune cell function. This project looks at the expression of CGRP-alpha in type II neurons after noise exposure. CGRP-alpha will be blocked during noise exposure to see if this affects the immune response to tissue damage.
Long-term goal: To understand the role of CGRP-alpha in the neurons and immune response of the inner ear, which may illustrate a role for type II neurons in pain and inflammation following tissue damage. CGRP-alpha has been a target for therapeutics for painful conditions such as migraine, making it an attractive therapeutic target for pathologies of the inner ear.
Wood received her doctorate in immunology and molecular pathogenesis at Emory University. She is now a postdoctoral research fellow at Johns Hopkins University in the department of otolaryngology. Wood’s 2022 ERG grant is generously funded by Hyperacusis Research.
Please note this 2022 ERG grantee cohort follows directly from the 2020 grantees. The grant year start and end dates changed, as has the way HHF designates grant years. HHF has not skipped a year and there has been no interruption in funding.
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Recent Research by Hearing Health Foundation Scientists, Explained Common Drugs Could Protect Against Noise-Induced Hearing Loss A growing number of people are suffering from hearing loss due to exposure to loud noises from heavy machinery, concerts, or explosions. As a result, scientists have been working to understand the mechanism through which damage to hearing actually occurs. Now, a team led by researchers at the University of Maryland School of Medicine (UMSOM) has published an online interactive atlas representing the changes in the levels of RNA made in the different cell types of ears of mice after damage due to loud noise. These changes in RNA levels are known as changes in “gene expression.” Once they determined the larger trends in gene expression following the damage, the UMSOM scientists then searched a database of FDA-approved drugs to find those that are known to produce opposite patterns of those caused by the noise. From this analysis, the research teams identified a handful of drug candidates that may be able to prevent or treat the damage, and ultimately preserve hearing. The researchers’ analysis was published in Cell Reports in September 2021. “As an otolaryngologist surgeon-scientist, I see patients with hearing loss due to age or noise damage, and I want to be able to help prevent or even reverse the damage to their hearing,” says study leader Ronna Hertzano, M.D., Ph.D., a professor of otorhinolaryngology–head and neck surgery, anatomy, and neurobiology at UMSOM and an affiliate member of UMSOM’s Institute for Genome Sciences. “Our extended analysis gives us very specific avenues to follow up on in future studies, as well as provides an encyclopedia that other researchers can use as a resource to study hearing loss.” The team added their newest data on noise-induced hearing loss to the gEAR—the Gene Expression Analysis Resource—a tool developed by Herzano’s laboratory that allows researchers not trained in informatics to browse gene expression data. Hertzano explains that the inner ear resembles the shell of a snail, with separate fluid compartments and sensory cells along its entire length. The ear functions like a battery with a gradient of ions between the fluid compartments that is generated by the side wall of the shell by adding in potassium. The sensory cells detect sound and then communicate with the neurons that interact with the brain to interpret the signal. The sensory cells are surrounded by support cells. The inner ear also has resident immune cells to protect it from infection. Research supervisor Beatrice Milon, Ph.D., in Hertzano’s laboratory initially did an analysis on the sensory cells and the support cells of the ear in mice. She collected data on the changes in gene expression from before and after noise damage. After making their study known to other researchers in their field, the team heard from scientists at Decibel Therapeutics (led by Joe Burns, Ph.D.) and the Karolinska Institute (led by Barbara Canlon, Ph.D.), who had the gene expression data from the inner ear’s neurons, side wall, and immune cells from before and after noise damage. The teams then combined the datasets and performed their analyses.
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The bioinformatic analyses were led by Eldad Shulman from the lab of Ran Elkon, Ph.D., Tel Aviv University, a bioinformatics expert who has been working collaboratively with Hertzano now for over two decades. Together, they leverage advanced computational techniques and combine them with biological insights to analyze and interpret data, providing impactful insights to the hearing research field. Hertzano says it was so important that they looked at a cell-specific level, rather than looking at the entire ear, because they found that most of the gene expression changes were specific to only one or two cell types. “We expected the subset of neurons typically sensitive to noise and aging to have ‘bad’ changes in genes, so that we could counter them with drugs, but there was no such thing,” Hertzano says. “On the contrary, we found that the subset of neurons that are resistant to noise trauma turn on a program that protects them while the very sensitive neurons had little change in gene expression. We are currently looking into approaches to induce the protective changes in the noise-sensitive neurons to prevent their loss from noise and aging.” In another example, the researchers found that only one out of the four types of immune cells detected showed major differences in gene expression. Additionally, immune-related genes were turned up in all cell types of the inner ear after noise damage, with many of them controlled by two key regulators. The research team took the overall gene expression trends and plugged them into DrugCentral, a database of known molecular responses to FDA-approved drugs, specifically searching for changes that would be opposite of those happening in the noise-damaged cells. They identified the diabetes drug metformin as a potential candidate, as well as some inhaled anesthetic medications used in surgeries and other medications. “Hearing aids and cochlear implants are used to alleviate hearing loss; however, there are no therapies available to prevent or treat hearing loss,” says E. Albert Reece, M.D., Ph.D., MBA, the executive vice president for medical affairs, UM Baltimore, and the John Z. and Akiko K. Bowers Distinguished Professor, and Dean, UMSOM. “The studies that follow up on these findings may eventually lead to medications to prevent occupational noiseinduced hearing loss, for example in factory workers, and to changes in standardizing anesthesia protocols for ear surgery, particularly in hearing preservation procedures.” —University of Maryland School of Medicine This originally appeared on the University of Maryland School of Medicine website, at medschool.umaryland.edu/news. HHF’s Hearing Restoration Project (HRP) member Ronna Hertzano, M.D., Ph.D., is a 2009–10 Emerging Research Grants (ERG) scientist. Hertzano and HRP scientific director Lisa Goodrich, Ph.D., recently hosted a webinar on hair cell regeneration; please see hhf.org/webinar to view the captioned recording.
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Postural and Head Control Given Different Environmental Contexts Balance is known to be specific and context-dependent. Context is defined as the circumstances that form the setting for an event, statement, or idea, and in terms of which it can be fully understood and assessed. The majority of studies reporting that balance is context-dependent refer to the task: standing steady (static balance) or moving (dynamic balance); performing a balance task alone or with an additional cognitive task such as memory or calculation; standing on a stable or an unstable surface, etc. For example, improving one’s singleleg stance time is not expected to transfer to faster or more stable walking. Current virtual reality technology allows context-based testing of multisensory integration and balance using head mounted displays (HMDs). By creating diverse environments that provide different contexts (e.g., a street vs. the clinician’s office) we can be better positioned to assess changes in balance performance potentially induced by cognitive and emotional aspects, such as postural threats, fear of imbalance, or symptoms related to past experiences within specific environments. In our study published in Frontiers in Neurology in June 2021, we investigate how postural sway and head kinematics change in healthy adults in response to different levels of combined auditory and visual perturbations (changes): static visuals without sounds, and then two levels of moving visuals and dynamic sounds of varying intensity. These gradual perturbations were created in two different contexts, an abstract stars scene or a city scene. Our first finding was that the current settings were too subtle to test differences between responses to low and high visuals and sounds. We therefore combined the low and high data and compared the ‘static visual, no sound’ vs. dynamic scenes. That prevented us from being able to isolate the role of sounds in this specific protocol. Future studies should isolate each modality in the presence of the other. Our second finding was that for most measured parameters (side-to-side postural sway and head movement, pitch and roll head movement), an increase in movement between the static and dynamic scenes was
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Researchers used two visual reality contexts to implement gradual changes in visual and auditory input: abstract stars (below, left) and a city scene.
greater in the city than the stars scene. These findings support the importance of context in the study of sensory integration and confirm the feasibility of an HMD setup to evaluate balance in different contexts. We also explored the feasibility of this novel HMD assessment in individuals with unilateral peripheral vestibular hypofunction and monaural hearing (hearing on one side only). The majority of the vestibular group moved more than controls when the scenes were dynamic, particularly in the city scene. The monaural hearing group was more diverse, with slightly more than half performing similarly to controls. Those who performed differently had either prior vestibular rehabilitation or reduced hearing compared with the others. Fall risk in people with hearing loss has been shown in older adults, and our pilot data suggests balance impairments in people with single-sided hearing are more likely to arise in older participants with moderate dizziness. Future studies utilizing HMDs should further assess the impact of aging with and without hearing loss on postural performance in a larger sample with a specific diagnosis of the hearing loss type as well as control for vestibular symptoms in those with hearing loss. —Anat V. Lubetzky, Ph.D. A 2019 ERG scientist, Anat V. Lubetzky, Ph.D., is an assistant professor in the physical therapy department at New York University. For more about Lubetzky’s research, see page 18.
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Cochlear Organoids Reveal HIC1’s Role in Hair Cell Differentiation Sensory hair cells in the cochlea translate sound into electrical signals that they transmit to auditory neurons. These cells are lost over the course of life through cumulative damage from aging, infection, certain medications, and loud sounds. Sensory hair cells do not grow back, and consequently hearing loss is irreversible. In contrast, non-mammals, like birds and fish, can regenerate their damaged hair cells. Recent studies have also found spontaneous regeneration of hair cells after damage in the newborn mouse cochlea and balance organ. This regeneration involves a series of molecular pathways whereby precursor progenitor/stem cells (which are a subset of supporting cells that reside adjacent to the hair cells) begin to express the key hair cell transcription factor ATOH1 and differentiate into hair cells. However, this capacity for regeneration disappears during maturation of the mammalian cochlea, suggesting that regeneration is possible but repressed after development. Understanding this molecular blockade hindering progenitor cell to hair cell conversion promises to reveal key steps needed to reverse hearing loss. Studying these pathways has traditionally been limited by complex transgenic mice and a few sets of relevant tools in a dish. The advent of inner ear organoids, which yield an expanded pool of progenitor cells, revolutionized our ability to study this process robustly in a dish. We derive organoids by first isolating and expanding progenitor cells from newborn mice cochleae and then differentiating them to hair cells. We can genetically modify the organoids to study how specific proteins affect this process. We focused on the HIC1 (hypermethylated in cancer 1) protein, as its role regulating ATOH1 has been established in other systems. It has been previously shown to directly bind to and suppress the regulatory regions around the Atoh1 gene during cerebellar development, and Hic1 deletion appears to permit Atoh1 expression and differentiation of Paneth cells in the intestine (like hair cells, Paneth cells express Atoh1). We hypothesized that HIC1 could contribute to repression of the Atoh1 gene in the cochlea through transcriptional regulation and interaction with Wnt, a key signaling pathway important in cochlear development. As we reported in Stem Cell Reports in April 2021, we found that across various time points (ages), Hic1 is expressed throughout the mouse sensory epithelium. In cochlear organoids, HIC1 knockdown (suppression) induces Atoh1 expression and promotes hair cell differentiation, while HIC1 overexpression hinders differentiation. We go on to study HIC1’s interaction with Wnt signaling which appears to be critical to its mechanism. Our findings reveal the importance of HIC1 repression of Atoh1 in the cochlea. It also demonstrates the power of combining the organoid model with the genetic toolkit to study key regulators of hair cell differentiation, which we hope will be leveraged to advance our understanding of hair cell development and regeneration. —Dunia Abdul-Aziz, M.D. A 2019 ERG scientist, Dunia Abdul-Aziz, M.D., is an otolaryngologist at Mass Eye and Ear and an instructor in otolaryngology–head and neck surgery at Harvard Medical School.
This is a representative brightfield image of inner ear organoids. Within the organoids, a much larger proportion of cells in which Hic1 is knocked down (short-hairpin RNA to Hic1, or shHic1), marked by a red reporter) demonstrate overlapping expression of Atoh1 (green) compared with untreated organoids or organoids that are treated with nontargeting shRNA. This study further shows that these cells express other hair cell markers consistent with their differentiation into hair cells.
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Novel Small Molecule Promotes Synaptic Regeneration In Vitro Hearing loss is associated not only with the loss of sensory hair cells in the inner ear, but also with the loss of nerve cells called spiral ganglion neurons (SGNs), as well as the synapses between surviving hair cells and SGNs. Improving SGN survival, neural outgrowth, and synaptogenesis (synapse formation) could lead to significant gains for patients with hearing loss. Neurotrophic factors (molecules that regulate the growth and survival potential of neurons) might promote the survival of SGNs and the rewiring of sensory hair cells by surviving SGNs. In a paper published in Frontiers in Cellular Neuroscience in July 2021, my team and I detail how we have pioneered a hybrid molecule approach to maximize SGN stimulation through the use of small molecule analogues of neurotrophin-3 (NT-3). NT-3, in addition to brain-derived neurotrophic factor (BDNF), is the primary neurotrophin in the inner ear during development and throughout adulthood. Both have demonstrated potential for SGN survival and neurite outgrowth. We have previously shown that a small molecule BDNF analogue can promote SGN neurite outgrowth and synaptogenesis in vitro. There is evidence that NT-3 will have a greater regenerative capacity in the cochlea than BDNF in a number of contexts. We therefore sought to develop a similar approach for NT-3 using 1Aa, a small molecule analogue of NT-3. To maximize the potential for drug delivery to the bone-encased cochlea, we also studied the activity of a bone-binding derivative of NT-3. This hybrid molecule links 1Aa to risedronate, which is a clinically used bisphosphonate molecule that avidly binds bone for the treatment of osteoporosis, to create Ris-1Aa. Using an in vitro mouse model, we demonstrate that both 1Aa and Ris-1Aa stimulate neurite outgrowth and synaptogenesis in SGN cultures at a significantly higher level compared with controls. This result provides the first evidence that a small molecule analogue of NT-3 can stimulate SGNs and promote regeneration of synapses between SGNs and inner hair cells. This work furthers the development of an effective
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The small molecule 1Aa promotes neurite outgrowth in vitro. Below, neurites were stained with neuronal marker TuJ (red), and nuclei were labeled with DAPI (blue). The scale bar represents 100 μm. Images are representative of four independent experiments.
drug delivery platform for the inner ear that uses the cochlear bone as a depot for prolonged neurotrophic stimulation of SGNs. Our method may bypass the pitfalls of systemic administration—increased risk of side effects and insufficient levels of drug delivery—and the dangers related to opening the cochlea. As we have now described novel small conjugated molecules with neurotrophic activity in vitro, we anticipate that other small molecules with desired activities within the cochlea could potentially be delivered via this platform. —David Jung, M.D., Ph.D. A 2018 ERG scientist, David Jung, M.D., Ph.D., is an otolaryngologist at Mass Eye and Ear and an assistant professor in otolaryngology–head and neck surgery at Harvard Medical School. Coauthor Judith Kempfle, M.D., is a research fellow in otolaryngology–head and neck surgery at Mass Eye and Ear and a 2010–2011 ERG awardee. Another coauthor, Albert Edge, Ph.D., is the Eaton-Peabody Professor of Otolaryngology–Head and Neck Surgery at Mass Eye and Ear and a member of the Hearing Restoration Project consortium.
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Verifying a Novel Method for Assessing Speech Motor Skills in Children With Cochlear Implants Auditory input is essential to the acquisition and maintenance of speech production skills, and yet speech production has been scarcely studied in children and adults with impoverished auditory input since cochlear implants (CIs) became a standard treatment. A critical piece of the puzzle in understanding the development and maintenance of speech motor control in CI users is that we need to better understand how these individuals time, sequence, and coordinate speech movements. During conversational speech, typical hearing individuals usually produce six to nine syllables per second, using approximately 100 different muscles in their laryngeal and supralaryngeal vocal tracts. Recent technological advances have greatly improved researchers’ ability to track the motions of the speech articulators (tongue tip, tongue body, lips, and jaw). Our University of Florida Cognition, Action, and Perception of Speech Lab allows the use of electromagnetic articulography (EMA) to directly record speech movements within the inner reaches of the vocal tract on a millisecond-by-millisecond basis, and localize where in the vocal tract the movements occur. The tracking is performed by using magnetic fields to localize the positions of sensors temporarily attached to the articulators during speaking. However, the use of EMA in children and adults with CIs had been curtailed because it was not clear that direct measures of speech motor actions could be made without negative interactions between CIs and EMA, since both devices make use of magnetic fields. In our current study, published in the Journal of the Acoustical Society of America Express Letters in August 2021, my team and I demonstrated for the first time that there is minimal crossinterference between the devices, suggesting that EMA is a promising method for assessing speech motor skills in children with CIs. Our team is now working to establish optimal methods for collecting articulographic data from CI users. Collectively, the current findings lay the foundation for future research aimed at developing novel, mechanistically driven rehabilitation protocols to optimize speech motor instruction for deaf children. By combining principles and tools from engineering and computer
Matthew Masapollo, Ph.D., affixes articulography sensors onto the lips, tongue, and jaw for speech movement tracking of a cochlear implant (CI) user. Three transmitter coils, located at the vertices of an equilateral triangle above the head of the CI user, generate and radiate out a series of magnetic fields, which track the positions of the sensors in near real-time during a speaking task.
science with cognitive and linguistic science, we envision developing robotic devices to deliver speechlike patterns of somatosensory input to the vocal tracts of children who use CIs as they learn to listen to speech sounds through their CI processor. —Matthew Masapollo, Ph.D. A 2022 ERG recipient, Matthew Masapollo, Ph.D., is the director and principal investigator of the University of Florida’s Laboratory for the Study of Cognition, Action, and Perception of Speech, which was established in 2020.
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Common Loud Noises Cause Fluid Buildup in the Mouse Inner Ear—Which May Be Easily Resolved Exposure to loud noise, such as a firecracker or an ear-splitting concert, is the most common preventable cause of hearing loss. Research suggests that 12 percent or more of the world population is at risk for noise-induced loss of hearing. Loud sounds can cause a loss of auditory nerve cells in the inner ear, which are the cells responsible for sending acoustic information to the brain, resulting in hearing difficulty. However, the mechanism behind this hearing loss is not fully understood. Now, a new Frontiers in Cell and Developmental Biology study from Keck Medicine of University of Southern California (USC) links this type of inner ear nerve damage to a condition known as endolymphatic hydrops, a buildup of fluid in the inner ear, showing that these both occur at noise exposure levels people might encounter in their daily life. Additionally, researchers found that treating the resulting fluid buildup with a readily available saline solution lessened nerve damage in the inner ear. “This research provides clues to better understand how and when noise-induced damage to the ears occurs and suggests new ways to detect and prevent hearing loss,” says John Oghalai, M.D., an otolaryngologist with Keck Medicine, the chair of the USC Caruso Department of Otolaryngology–Head and Neck Surgery, and the lead author of the study. A previous study he conducted on mice exposed to blast pressure waves simulating a bomb explosion linked nerve damage with fluid buildup in the inner ear. For this study, Oghalai and colleagues wanted to explore the effect of common loud sounds ranging from 80 to 100 decibels (dB) on the mouse inner ear. After the exposure, they used an imaging technique known as an optical coherence tomography to measure the level of inner ear fluid in the cochlea, the hollow, spiral-shaped bone found in the inner ear. Up until exposure to 95 dB of sound, the inner ear fluid level remained typical. However, researchers discovered that after exposure to 100 dB—which is equivalent to sounds such as a power lawn mower, chainsaw, or motorcycle—the mice developed inner ear fluid buildup within hours. A week after this exposure, the mice were found to have lost auditory nerve cells. However, when researchers applied hypertonic saline, a salt-based solution used to treat nasal congestion in humans, into the affected mouse ears one hour after the noise exposure, both the immediate fluid buildup and the long-term nerve damage lessened, implying that the hearing loss could be at least partially prevented. These study results have several important implications, according to Oghalai, especially as the loss of nerve cells in the inner ear is known as “hidden hearing loss” because hearing tests are unable to detect the damage. “First, if human ears exposed to loud noise, such as a siren or airbag deployment, can be scanned for a level of fluid buildup—and this technology is already being tested out— medical professionals may have a way of diagnosing impending nerve damage,” he says. “Secondly, if the scan discovered fluid buildup, people could be treated with hypertonic saline and possibly save their hearing.” Oghalai also believes the study opens a new window into understanding Ménière’s
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Up until exposure to 95 decibels (dB) of sound, the inner ear fluid level remained typical. However, researchers discovered that after exposure to 100 dB—which is equivalent to sounds such as a power lawn mower, chainsaw, or motorcycle—the mice developed inner ear fluid buildup within hours. A week after this exposure, the mice were found to have lost auditory nerve cells. However, when researchers applied hypertonic saline, a salt-based solution used to treat nasal congestion in humans, into the affected mouse ears one hour after the noise exposure, both the immediate fluid buildup and the long-term nerve damage lessened, implying that the hearing loss could be at least partially prevented.
disease, a disorder of the inner ear that causes vertigo, tinnitus, and hearing loss. “Previously, inner ear fluid buildup was thought to be primarily linked to Ménière’s disease. This study indicates that people exposed to loud noises experience similar changes,” he says. Oghalai hopes this study will lead to further research on the reasons ear fluid buildup occurs, and encourage the development of better treatments for Ménière’s disease. —Keck Medicine of University of Southern California This originally appeared on the Keck Medicine of USC website, at news.keckmedicine.org. John Oghalai is a 1996–1997 ERG scientist. The study was supported by the National Institute on Deafness and Other Communication Disorders. For links to all the publications in this section, see hhf.org/winter2022-references.
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meet the researcher
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Meet the Researcher
Emerging Research Grants (ERG) As one of the leading 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.
Megan Beers Wood, Ph.D.
John Hopkins University School of Medicine
Wood received her doctorate in immunology and molecular pathogenesis at Emory University in Atlanta. She is now a postdoctoral research fellow at Johns Hopkins University School of Medicine in the department of otolaryngology. Wood’s 2022 Emerging Research Grant is generously funded by Hyperacusis Research. i was intrigued to find that neurons in the cochlea expressed genes similar to pain-sensing neurons. Since my background is in immunology, I’m interested in whether the alpha-calcitonin gene-related peptide (CGRPα) in those neurons interacts with immune cells after noise exposure, like in other organs. I looked for CGRPα protein in type II peripheral endings after exposure and saw promising results. My current project allows me to learn more about this exciting observation, which may help with hyperacusis—an elevated sensitivity to everyday sounds. i was interested in science from a very young age and wanted to be a doctor. My first “research” project was in 5th grade when I looked into how Super Glue could be used instead of stitches for some wounds. I am also lucky to come from a family with several scientists. On one side was my grandfather, who was a forester with a Ph.D. in plant biology, and on the other side I have two cousins who are scientists—one a chemist working for the Smithsonian Institution and the other a scientist working in biotech. So I would say we are all a curious bunch, and that was encouraged at home by nature walks with my parents and science kits to play with. as a toddler i was diagnosed with juvenile idiopathic arthritis. My experiences in teaching hospitals were very inspiring. One of my pediatric rheumatologists even oversaw my independent research study in high school, which was my first real introduction to scientific literature.
my goal as a researcher has been to explain rare phenomena. I recently gave a talk for Hyperacusis Research. It was very rewarding, as I was able to interact with people experiencing the condition. It really helped me understand the mechanisms underlying hyperacusis. i grew up around music, so the experiential side of hearing has always been important to me. My grandmother wore hearing aids. I saw firsthand how uncomfortable they are and how isolated she became when the batteries got low. over the summer i worked in a community garden. We grew 70 pounds of cucumbers and 15 varieties of tomatoes! I like to think I’m following in my grandfather’s footsteps as he grew abundant vegetables. I also enjoy embroidery. I find free-handing shapes lets me slow down. It keeps my hands busy while I think over complex problems. —Heather Chambers
Megan Beers Wood, Ph.D., is generously funded by Hyperacusis Research. We thank them for their support of studies that will increase our understanding of the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance.
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. 50
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