38 minute read
Living With Hearing Loss My Cochlear Implant Adventure. Ruth D. Bernstein. An Unexpected Side Effect. Henry Klein. Moving on From Ménière’s. Alice Sheinman
Older Adults and Cochlear Implants
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Top: During mapping sessions, the cochlear implant computer program gets adjusted to Ruth Bernstein’s hearing ability.
Above: Bernstein’s MED-EL implant processor is attached using magnets.
My Cochlear Implant Adventure
A hearing loss consumer advocate shares how she decided to get an implant at age 86, and what happened next. By Ruth D. Bernstein
I’ve been dealing with my deteriorating hearing for four decades. As the years went by and my hearing continued to get worse, my ever-patient audiologist encouraged me to consider a cochlear implant (CI). I was reluctant because I have a long history of hypersensitivity and allergic reactions to medications and anesthesia. My allergy to the plastic used in hearing aids also did not bode well for CI devices.
Over a 20-year period, I was evaluated five times for CIs. Each time I came away feeling it wasn’t time yet. I was still able to have one-on-one conversations in quiet spaces, use the phone, and hear music, which was surprising given the fact my audiogram said I was technically deaf.
Seven years ago, when I turned 80, I realized conversations were getting more difficult. I needed a Bluetooth streamer or captioning to use the phone. I felt physically exhausted by the huge amount of energy my brain was burning to keep me hearing. One day I suddenly lost my balance on the subway. To find out why, my general practitioner sent me to Lawrence Lustig, M.D., head of the department of otolaryngology–head and neck surgery at Columbia University.
After many tests, Dr. Lustig concluded I had vertigo, for which there was no physical explanation. He suggested, and I agreed, I was a candidate for a CI because my ears were in good shape internally. I controlled the vertigo by using Sea-Band acupressure wristbands. Happily, it disappeared after three months.
I was evaluated at Columbia, and the results were not surprising: I was almost deaf in both ears. I had finally found a surgeon who understood my physical needs and a CI audiologist who understood my auditory needs. It was time to move forward.
Finding the right CI team is a very personal decision. I encourage anyone considering an implant to interview several teams to find the right fit. One size does not fit all.
After talking with Dr. Lustig, my general practitioner agreed to the operation because I would be under anesthesia for less than two hours, something he was concerned about because of my age, then 86. First, we had to decide which ear should be implanted. Although my right ear has been my “worse” ear all my life, hearing with my left ear had recently become erratic. We chose the left ear for the CI in the hope it would become more reliable.
Trying Out Options
Now it was time to choose a device that wouldn’t cause an allergic reaction. That process took 18 months, as I went through a series of
patch tests for the various CIs. Ultimately, we chose the MED-EL Synchrony implant and the Rondo 2 processor, which uses a magnet to attach to the back of my head, avoiding the plastic over-the-ear hook I’m allergic to.
Surgery took place at Columbia in late August 2019. It started at 7:30 a.m. I had no dizziness or nausea afterward and was home eating lunch at 2 p.m. The three-week recovery period was uneventful. I was not totally cut off from the world because the hearing aid in my right ear was working—and I knew what to expect, thanks to my wide circle of friends with CIs, who shared their experiences, and the professionals who imparted their knowledge.
I decided to forgo all extracurricular activities for the month of September, which allowed me time to heal and eliminated any possible stressful hearing situations. I found I had to nap every afternoon for three weeks. Dr. Lustig explained I needed to rest because it took my body time to eliminate the anesthesia in my system.
I told family and friends to contact me by email or text; although I use InnoCaption for operator-transcribed phone calls, I asked for no phone calls, please! Otter.ai, the app that does instant AI transcription on smartphones, became an inseparable part of my life. A week after my implant, I went to a friend’s funeral. Sitting three rows back from the podium at the funeral parlor, I was able to read every word said on the Otter.ai transcription on my iPhone.
More Energy
The day after my CI was activated, I started meeting weekly with my speech-language pathologist at the Center for Hearing and Communication. We practiced sounds, words, and sentences I had difficulty understanding. She assigned online lessons on my iPad and helped me learn to use the assistive listening equipment that comes with my CI. At Columbia I also started monthly mapping sessions, the electronic process that adjusts the CI computer program in my implant.
The range of sounds I can now hear is amazing. I can hear the timer on my iPhone ring from two rooms away, and now I know my slippers make a swishing sound on wooden floors. The elevator in my building makes a clicking sound at every floor as it travels to the 22nd floor. That’s a lot of clicks I haven’t heard since I moved into the building in 1976!
With the CI, my brain responds easily to the sounds it is hearing. I’m not physically exhausted at the end of every day because my brain no longer works in high gear all the time, something this 87-year-old great-grandmother really appreciates.
In a story about preventing dementia in The New York Times in February 2020, Justin Golub, M.D., of Columbia Doctors says, “Better hearing is better for you and better for your mind…. Hearing is good for your brain, the more hearing you have the better.” My brain and I agree 100 percent!
Ruth D. Bernstein is a consumer advocate and a board member of the Hearing Loss Association of America’s New York City Chapter, hearinglossnyc.org. A version of this story originally appeared on the Center for Hearing and Communication website, chchearing.org. Lawrence Lustig, M.D., is a 2002 Emerging Research Grants alumnus. For references, see hhf.org/fall2020-references.
Share your story: Tell us your hearing loss journey at editor@hhf.org.
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Older Adults and Cochlear Implants
Reading lips and using a strong, body-worn hearing aid to help me detect the direction sounds were coming from got me by for years. Then, in my 70s, a cochlear implant in my right ear brought me back to the real world.
An Unexpected Side Effect
By Henry Klein
At age 30 in the spring of 1960, I was diagnosed with otosclerosis and underwent stapedectomy surgery for my left ear. Due to a sneeze a few days after, the pin flew off the mount and ruptured the inner ear. I was left with no hearing in the left ear.
The right ear with a simple hearing aid was fine. I was told a hearing aid will always benefit me due to the very slow progression of otosclerosis.
In the early 1980s at age 53, I underwent heart bypass surgery. The cardiologist said that my cholesterol must be controlled and prescribed neomycin, an antibiotic. I took this medicine for eight years. At first, I noticed difficulty hearing on the phone. Then difficulty hearing during regular conversations.
The cardiologist declared that my hearing loss was not due to the neomycin, but another specialist told me neomycin was the cause and that it had gradually destroyed the nerves around the cochlea. Neomycin is ototoxic (harmful to hearing). The hearing loss in my right ear was profound. As a result of this experience, I strongly urge everyone to be fully aware of the side effects of any drug or supplement used. Check and check again—asking your prescriber but also doing your own research—and then check once more.
Back then as the CEO of an electrical contracting firm, I needed to be able to communicate via telephone. I discovered the VCO (voice carry over) relay service, and got captioned phones for my office and home.
Lipreading (speechreading) courses and a very strong, body-worn hearing aid to help me detect the direction sounds were coming from got me by for about 12 years. Then at age 73, 18 years ago, I had cochlear implant surgery for my right ear. The implant brought me back to the real world.
During my time of profound, not yet treated deafness, my wife developed Alzheimer’s. The communication between us was challenging and tough. It was hard for her to understand that I needed to be facing her and read her lips. But we have found that persistence, determination, and love conquers all.
I am now 91 years old. Wearing a face mask can be awkward. Sometimes I don’t recognize myself in the mirror. I recently heard this joke: A guy tried to rob a bank without wearing a face mask and the bank clerk said, “I’m not allowed to help you because you aren’t using a mask!” Humor, empathy, and resilience will get us through.
Henry Klein lives in Maryland.
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Older Adults and Cochlear Implants
Alice Sheinman and her dog Harry, who has helped her cope with Ménière’s disease.
Moving on From Ménière’s
The hearing and balance condition Ménière’s disease brings changes at home, work, and play. By Alice Sheinman
About eight years ago when I was 46, a few days after having a very stressful cataract surgery, I started to trip over my own feet. My balance has never been very good, but this was out of the ordinary, even for me. I had to ask my husband to come home from work to take care of our 8-year-old son. After the attack passed, I noticed that I wasn’t hearing very well out of my right ear. I went to the doctor, who thought it was a sinus infection and gave me an antibiotic.
More vertigo attacks followed, and eventually I was diagnosed with Ménière’s disease. I found an ENT who specializes in this chronic vestibular disorder that affects balance and hearing.
I was put on a diuretic to help with fluid retention and underwent endolymphatic shunt surgery, where a tube was permanently put behind my right ear to drain excess fluid. The vertigo attacks finally stopped, but I started having tinnitus and vestibular migraines.
To stop the headaches, I tried different medications that made me tired, gave me brain fog, and caused my hair to fall out. I told my doctor, “I pay too much for these highlights to see them on the bottom of the bathtub!” I decided to stop all of them and just take the diuretic, so at least I still have my hair.
Then more recently, a couple years ago, the Ménière’s started affecting both ears. It caused a fluctuating hearing loss, so I was never sure from day to day if I would be able to hear or not. This was devastating. I am already legally blind, with only about 25 percent vision in my right eye. Hearing had always been how I navigated the world.
I didn’t want to live as a person with no hearing and low vision. I became very depressed. I am a musician, and play the alto saxophone in a local community orchestra. I’ve been playing the instrument since I was 12 years old and wondered if I could continue.
I had another endolymphatic shunt surgery, this time in my left ear. Afterward I was home feeling sorry for myself. My sister called and said she was getting a puppy to be her dog’s playmate, and there were other puppies available—did I want one? After years of telling our son we couldn’t get a dog because of the responsibility involved, this time I said yes, and we brought Harry home two months later.
Harry has really helped me to cope. He is always there for a cuddle when I need it. I’ve also seen a psychotherapist, who has helped me cope with the depression and anxiety from having Ménière’s and the challenge of making my family and friends understand what it’s like to have it.
Making Changes
In the beginning of 2018, my ENT suggested a cochlear implant (CI) for my right ear. He explained that I would need a “backup” for when the hearing in my left ear went. After surgery and activation, I was lucky that I was able to understand words immediately.
During this time, I kept teaching cooking class to special needs high school students. The students were very kind to me, perhaps because they had their own disabilities to deal with. Every morning I would tell them if it was a bad or good hearing day, and they were very accommodating.
I still play alto saxophone in a local orchestra. After getting my first I used a Roger Select microphone placed on the kitchen worktable. On a bad hearing day, the cochlear implant, I could see that rehearsal was much harder than students would pick it up to talk into it, so I could hear them. What I never told them was that the mic I thought it would be. I told the allowed me to hear them from across the room. conductor that as much as I had This made for some very entertaining times! But as much as I loved my work and students, enjoyed coming, I felt like I might my inability to hear was becoming more of a problem for me. I taught in a commercial kitchen be a liability. He asked me if where exhaust fans, ovens, and other kitchen playing in the orchestra would equipment were constantly running—not an ideal listening environment. help my rehabilitation with the Although my boss was very understanding, I started to think about another career that fit with my abilities and realized that perhaps the best job implant. I said yes, that I thought it would. He said that I was staying. for me would be to help people just like me. I looked into the field of vocational rehabilitation. I was so happy, I started crying. This I saw I could work with people one on one or in group has shown me such kindness. small groups and use skills that I developed while teaching and at previous jobs in management and administration.
Who better to work with people with disabilities than someone who is disabled and can empathize with them? In fall 2019, I started my master’s in rehabilitation counseling at Rutgers University in New Jersey. The online format fits with my hearing ability.
I still play alto saxophone in a local orchestra. After getting my first CI, I could see that rehearsal was much harder than I thought it would be. I told the conductor that as much as I had enjoyed coming, I felt like I might be a liability. He asked me if playing in the orchestra would help my rehabilitation with the implant. I said yes, that I thought it would. He said that I was staying. I was so happy, I started crying. This group has shown me such kindness.
When the hearing in my left ear diminished, my ENT recommended a second CI. I got the surgery and, in February 2020 before the pandemic lockdown, the implant was activated. My mapping appointments for March and April were canceled, but even without the mapping the left ear came along. I graduated from hearing Charlie Brown’s teacher to hearing Share your story: Tell us Alvin and the Chipmunks. your hearing loss journey at
I continued with school, finishing my second semester and the summer editor@hhf.org. session. I am glad to be in school as it’s given me a purpose during this unsettled time, and I am hopeful that when our orchestra is able to again meet in person, the two implants will help me hear the music better. Support our research:
Life has taken me on a path that I never expected to be on. I’ve realized hhf.org/donatethat we need to live our own life as best we can, trying to focus not on what a disease has taken away from you but on what you can still do.
Hearing Restoration Project Plans Announced for 2020–21 By Peter G. Barr-Gillespie, Ph.D.
Hearing loss occurs when sensory hair cells of the inner ear (cochlea) are damaged or die. The goal of the Hearing Restoration Project (HRP) is to develop therapeutic methods to convert the cells that remain after damage into new, completely functional sensory hair cells, restoring hearing. We know that in most species—but not mammals, like humans and mice—hair cells robustly regenerate on their own after damage to the auditory system. The HRP is tasked with uncovering how to replicate this regeneration process in humans.
Here are the funded HRP projects for the coming year. Thank you for your ongoing support.
Integrative Systems Biology of Hearing Restoration
Seth Ament, Ph.D. University of Maryland School of Medicine
year 3 | The broad premise of the HRP is to identify molecules that could control hair cell regeneration. To do this, we are studying cell types and regenerative processes in multiple contexts and species, then integrating these data together to identify mechanisms that could potentially be turned on in the mouse cochlea to drive transdifferentiation (activating the correct set of hair cell–promoting genes in supporting cells). The role of this systems biology project is to provide the necessary data integration “glue,” binding together the results from the data generation projects. We will combine much of the data that is being generated by the HRP to advance our knowledge of hair cells, supporting cells, conversion of one cell type to another, and the potential for regeneration. Through modeling all of the available HRP data, we will identify regulatory molecules that may contribute to regeneration.
Comparison of Three Reprogramming Cocktails in the Organ of Corti: Cells, Transcriptomes, and Epigenomes
Andy Groves, Ph.D. Baylor College of Medicine
year 3 | Each cell type in the human body is defined by its activation of a unique combination of genes that endow each cell type with unique properties. The activation of these genes is achieved by special proteins known as transcription factors, or switches, responsible for switching on appropriate genes in one cell type and preventing inappropriate genes from being activated. In recent years, investigators have identified a number of these transcription factors that cause the formation of hair cells. The goal this year is to examine why these cocktails of transcription factors are able to reprogram nonsensory cells, but not supporting cells of the inner ear, to become hair cells.
Detection of Transcriptome Changes in Single Cells After AminoglycosideInduced Hair Cell Loss in the Chicken Basilar Papilla
Stefan Heller, Ph.D. Stanford University
year 4 | Birds robustly regenerate their cochlear hair cells through the conversion of dormant supporting cells into new hair cells. Our project uses selective, high-sensitivity methods to reveal the molecular changes in supporting cells after their activation by, for example, ototoxic drugs that cause hair cell death. By examining the responses of many single cells, we have begun to identify triggers that initiate, execute, sustain, and ultimately terminate the regenerative process. Recent experiments have confirmed that the two halves of the chicken cochlea (neural and abneural) predominantly use different regeneration mechanisms. Using bioinformatics methods to process the resulting data, we will analyze chicken cochlea cell populations isolated at various time points during hair cell regeneration and specifically characterizing the two distinct responses. By examining regeneration in an animal that replaces hair cells after damage, we will be able to find triggers that may be activated in mammals to reverse hair cell loss.
Epigenetics Analysis of Maturation and Regenerative Responses in the Mouse Organ of Corti and Utricle
Neil Segil, Ph.D. University of Southern California
Andy Groves, Ph.D. Baylor College of Medicine
year 4 | Although hair cell regeneration does not occur in mammals, newborn mice harbor a latent capacity for some regenerative responses. However, this capability disappears within the first few weeks of life. This observation provides an experimental window that this proposal exploits to address fundamental questions about the failure of hair cell regeneration in mammals. Specifically, we propose experiments to identify those changes in the genetic material, the chromatin, that are responsible for orchestrating the differentiation of new hair cells within the newborn organ of Corti in the inner ear, and to investigate the changes in the chromatin that lead to the failure of regeneration in the adult mammalian inner ear.
Implementing the gEAR for Data Sharing Within the HRP
Ronna Hertzano, M.D., Ph.D. University of Maryland School of Medicine
year 4 | One of the successes of the HRP has been the development of the gEAR portal (gene Expression Analysis Resource, umgear. org). The gEAR has many public and private datasets, and these complex datasets can be compared by scientists without the need for sophisticated programming expertise. The gEAR is also the primary data sharing, visualization, and analysis tool for auditory researchers outside of the HRP, becoming a platform that supports the hearing research community at large. This year we will build on past successes, continuing to support data upload, develop new visualization tools, and further enable the greater research community to exploit this resource.
Mouse Model Systems to Interrogate Candidate Genes for Sensory Hair Cell Regeneration
John Brigande, Ph.D. Oregon Health & Science University
year 6 | As HRP scientists detect and characterize genes that are hypothesized to participate in the activation or inhibition of hair cell regeneration, methods for altering or disrupting those genes are critical to the demonstration of their importance. This project aims to couple together two sophisticated methods for manipulating genes in mice, the so-called CRISPR-READI and i-GONAD methods. CRISPRREADI enables efficient, large gene edits, and i-GONAD simplifies the delivery of reagents for gene editing. Together, the proposed CRISPRREADI-GO method should allow for rapid and efficient gene editing to be put to use by HRP investigators.
Support our research: hhf.org/donate
HRP scientific director Peter G. Barr-Gillespie, Ph.D., is a professor of otolaryngology and the chief research officer and executive vice president at Oregon Health & Science University. For more, see hhf.org/hrp.
EMERGING RESEARCH GRANTS
2020 Emerging Research Grants Announced By Christopher Geissler, Ph.D.
Scientific researchers have, like all of us, faced numerous challenges this year due to the COVID-19 crisis but have continued the science throughout, whether at home or in labs with reduced occupancy, driven by a dedication to advance knowledge and contribute to the treatment of hearing loss and other hearing and balance disorders.
We are deeply grateful that donors to Hearing Health Foundation (HHF) have shown a similar dedication. Following a rigorous review process, our Scientific Review Committee and Council of Scientific Trustees, comprising senior expert scientists and physicians from across the U.S., have chosen seven especially meritorious Emerging Research Grants (ERG) projects to fund for the upcoming year (October 1, 2020 to September 30, 2021).
Starting with this 2020–2021 ERG cycle, HHF is increasing the available annual amount per project from $30,000 to $50,000. The additional funds will have a profound impact on the scope and ambition of the projects being undertaken, and the increase in funding and the decision to make grants renewable for a second year attracted an exceptionally high-quality pool of applicants, with application numbers increasing 67 percent over 2019.
We are pleased to be able to support the work of these promising researchers and look forward to learning about the advances they will undoubtedly make in the coming year and beyond.
Congratulations to this year’s ERG recipients:
patients with Ménière’s disease
James Dewey, Ph.D.
University of Southern California Project: Filtering of otoacoustic emissions: a window onto cochlear frequency tuning
Mishaela DiNino, Ph.D. Calvin Wu, Ph.D.
Carnegie Mellon University Project: Neural mechanisms of speech sound encoding in older adults Generously funded by the Meringoff Family Foundation
Z. Ellen Peng, Ph.D.
University of Wisconsin-Madison Project: Investigating cortical processing during comprehension of reverberant speech in adolescents and young adults with cochlear implants issue of Hearing Health, coming out in January,
Generously funded by the General Grand Chapter Royal Arch Masons International
Pei-Ciao Tang, Ph.D.
Indiana University Project: Elucidating the development of the otic
Bryan Ward, M.D.
Johns Hopkins University Project: The effect of fluid volume on vestibular function and adaptation in
Ross Williamson, Ph.D.
University of Pittsburgh Project: Characterizing tinnitus-induced changes in auditory corticofugal networks
University of Michigan Project: Development and transmission of the tinnitus neural code Generously funded by the Les Paul Foundation
Christopher Geissler, Ph.D., is HHF’s director of program and research support. The Winter 2021 lineage using stem cell–derived organoid systems
will have additional details about these projects. For more, see hhf.org/erg.
Recent Research by Hearing Health Foundation Scientists, Explained
Steps Toward the Better Understanding of Hearing Loss
The cellular diversity of the inner ear has presented a technical challenge in obtaining molecular insight into its development and function. The application of technological advancements in cell type–specific expression enables clinicians and researchers to leap forward from classic genetics to obtaining mechanistic understanding of congenital and acquired hearing loss. This understanding is essential for development of therapeutics to prevent and reverse diseases of the inner ear, including hearing loss.
For our paper in The Laryngoscope in June 2020, we compared and contrasted major approaches for cell type–specific analysis of the ear—fluorescence–activated cell sorting (FACS), ribosomal and RNA pulldown techniques, and single cell RNA–seq (scRNA–seq)—using published and original data. After demonstrating the strengths and weaknesses of these approaches, we conclude that to maximize the utility of these approaches, it is important to match the experimental approach with the tissue of origin, cell type of interest, and the biological question. Often, a combined approach is required.
Finally, there are new tools for the visualization and analysis of complex expression data. Our gEAR platform collates cell type–specific gene expression from the ear field and provides unprecedented access to both clinicians and researchers to gene expression data.
In a second study in the journal Otolaryngologic Clinics of North America in August 2020, my team and I investigate noise and its role as acoustic trauma to the inner ear. While not funded by Hearing Health Foundation (HHF), our review of hearing loss and tinnitus has broader implications for HHF’s Hearing Restoration Project (HRP).
In the report, we detail the many types of hearing loss, the broad categories being conductive hearing loss; sensorineural hearing loss (itself divided into temporary, hidden, and permanent); and hearing loss of central origin. All indications are that tinnitus, when not caused directly by a central nervous system issue (such as stroke), is always associated with one or more forms of hearing loss.
This strong comorbidity indicates that it is unlikely there will be a cure for tinnitus independent of a cure for hearing loss. It also points to tinnitus potentially
being an early symptom of an underlying auditory injury before measurable audiometric changes. That said, the relationship between the characteristics of the hearing loss and the perception of tinnitus does not follow any clear rules.
In a third paper in Development in September 2020, our team determined the role a critical protein, GFI1, plays in the development of hair cells. GFI1 may dictate whether an embryonic hair cell matures into a functional adult hair cell or becomes a cell that functions more like a nerve cell (neuron). The research builds on research from my dissertation, when I discovered that the hearing loss from mutations in the protein POU4F3 appeared to largely result from a loss of GFI1 in hair cells. The new data explains the importance of GFI1 in experimental protocols to regenerate hair cells from stem cells.
In the future these regenerative methods, which are being pioneered by numerous groups in the field, will have the potential to be used for patients who have experienced hearing loss. While not yet ready for prime time, the cumulative work of the field and work funded by HHF through the HRP brings hope for a brighter future. —Ronna Hertzano, M.D., Ph.D.
A 2009–10 ERG scientist and a member of HHF’s Hearing Restoration Project, Ronna Hertzano, M.D., Ph.D., is an associate professor in the department of otolaryngology–head & neck surgery at the University of Maryland School of Medicine. For more, see umgear.org.
EMERGING RESEARCH GRANTS
Investigating the Interaction of Auditory and Pain Pathways
As the intensity of a sound increases, typical-hearing listeners experience an increase in loudness, but for levels above 120 decibels (dB), listeners not only perceive the sound as extremely loud, but also painful—the aural threshold of pain. Some individuals with hearing loss and other neurological disorders perceive even moderateintensity sounds as both painful and loud, a condition known as pain hyperacusis.
At the same time, in many individuals moderateintensity sounds have been shown to reduce sensitivity to pain, a phenomenon termed audio-analgesia. This has led to the use of white noise and music in some medical settings. And it has long been known that very intense sounds around 130 dB can evoke aural pain, termed audio-hyperalgesia, but it is not clear whether pain sensitivity is also exacerbated.
We tested the hypothesis that hearing loss could alter pain sensitivity by exposing rats to an intense noise leading to hearing loss. Three and four weeks after the noise exposure, pain sensitivity significantly increased. To our knowledge, these results show for the first time that noiseinduced hearing loss can lead to increased thermal pain sensitivity.
To investigate the multisensory interactions between auditory and pain pathways, we performed a series of experiments using a rat model, with results published in Hearing Research in August 2020. After confirming that sounds up to 90 dB reduced pain sensitivity (specifically thermal, or pain from heat), a major new finding was that high intensity sounds exacerbated the experience of thermal pain. Our results show for the first time that audioanalgesia in rats is limited to intensities below 100 dB, that thermal pain sensitivity begins to increase above 110 dB, and that audio-hyperalgesia emerges for intensities near the aural thresholds of pain.
We also modified the opioid pain pathway by treating rats with a high dose of the opioid fentanyl, known to induce increased pain sensitivity after its effects diminish. In ambient noise, pain sensitivity remained typical 10 days after the fentanyl dose. But at 90 to 110 dB, pain sensitivity increased, indicating that the pre-treatment with fentanyl had converted audio-analgesia to audio-hyperalgesia.
Finally, we tested the hypothesis that hearing loss could alter pain sensitivity by exposing rats to an intense noise leading to hearing loss. Three and four weeks after the noise exposure, pain sensitivity significantly increased. To our knowledge, these results show for the first time that noise-induced hearing loss can lead to increased thermal pain sensitivity.
Taken together, our results suggest that auditory and pain pathways interact in ways that depend on intensity, hearing loss, and opioid pain signaling, data that may be relevant to better understanding pain hyperacusis. —Senthilvelan Manohar, Ph.D.
A 2017 ERG scientist generously funded by Hyperacusis Research Ltd., Senthilvelan Manohar is a research assistant professor at the University at Buffalo, the State University of New York. Coauthors on this paper include ERG alumni Kelly Radziwon, Ph.D., and Richard Salvi, Ph.D. Radziwon’s 2015 and 2018 ERG awards were also funded by Hyperacusis Research Ltd.
Traumatic Brain Injury Affects Auditory Quality of Life Even With Typical Hearing Thresholds
Traumatic brain injury (TBI) is a major cause of morbidity and mortality in the United States and worldwide. There are an estimated 3.2 to 5.3 million individuals in the United States who are living with a TBI-related disability, and the estimated societal cost is in excess of $76 billion per year. Auditory conditions, including hearing loss, tinnitus, and hyperacusis, have been recognized as a common consequence of TBI, even in the absence of a temporal bone fracture.
As published in the journal Otolaryngology–Head & Neck Surgery in June 2020, we evaluated adult patients with typical hearing thresholds and a history of TBI for subjective hearing loss, tinnitus (ringing in the ears without a sound source), a feeling of ear fullness, hyperacusis (sensitivity to sounds), and autophony (unusually loud hearing of one’s own voice). We also administered questionnaires specific to certain hearing conditions: the Hearing Handicap Inventory for Adults, the Tinnitus Handicap Inventory, and the Hyperacusis Questionnaire.
Our study was among the first to investigate audiometric patterns, auditory conditions, and associated handicap in individuals following TBI with pure tone averages in the typical range. Participants with TBI not only commonly reported a range of auditory conditions, but also almost half the participants sustained head injury six years before participation in this study, suggesting the chronic burden of these symptoms.
We concluded that despite typical hearing thresholds, individuals with TBI experience a decrease in auditory quality-of-life metrics. —David Jung, M.D., Ph.D., and Elliot Kozin, M.D.
A 2018 ERG scientist, David Jung, M.D., Ph.D. (above left), is an assistant professor of otolaryngology–head and neck surgery, Harvard Medical School/Massachusetts Eye and Ear. A 2018 ERG scientist generously funded by the General Grand Chapter Royal Arch Masons International, Elliot Kozin, M.D. (above right), is an otolaryngologist at Massachusetts Eye and Ear.
Support our research: hhf.org/donate
EMERGING RESEARCH GRANTS
A Multidisciplinary Approach to Overcome Pediatric Listening Difficulties
Listening difficulties occur in children diagnosed with auditory processing disorders (also known as central auditory processing disorders) and may co-occur in children who have developmental language disorder or attention/memory deficits. Persistent listening difficulties negatively affect children’s learning and functioning.
For our paper published in the journal Attention, Perception, & Psychophysics in June 2020, we examined children’s susceptibility to auditory distraction and its relation to working memory capacity in 125 school-age children ages 7 to 11 years old with typical hearing and nonverbal IQ scores.
We found that the children’s susceptibility to background auditory distraction did not diminish with age. We also did not find a significant association between this susceptibility and children’s working memory capacity. Since children’s working memory improvements with age do not appear to help them ignore sound distractions, it is crucial to enhance target speech in children’s learning environments.
In another study published in the American Journal of Audiology in August 2020, we compared 26 typicalhearing school-age children reported to have listening difficulties with 26 age-matched children with no listening difficulties or parent concerns, on multiple auditory, language, memory, and attention measures. Novel to our approach was using multiple tasks for working memory, measuring different aspects of language ability, and measuring the accuracy and speed of retrieving information from long-term memory/stored knowledge.
We found that children with listening difficulties needed a greater signal-to-noise ratio than children with no listening difficulties. Grammatical knowledge, nonverbal IQ, and general attention did not significantly differ between the groups.
Overall group scores of children with listening difficulties were not low enough to be classified as clinically poor. This is consistent with evidence that many children assessed for auditory processing problems do not fulfill diagnostic criteria even though they present with comorbid learning problems. Importantly, functional difficulties in children with listening difficulties as reported by parents were more significant relative to children’s formal test results. This indicated that commonly used assessments may not fully capture the children’s listening challenges.
Evidence from our studies sheds light on some sensitive areas for assessment and intervention. Our most recent data (in a paper under review) show that larger word knowledge, stronger vocabulary networks, and accurate and efficient retrieval of stored knowledge from long-term memory help children perform significantly better while listening in complex auditory situations.
Assistive listening devices, contextualized robust language-based intervention, and educational supports are important, and can be best achieved when a child’s difficulties are discussed using a framework of multiple sources of potential deficits instead of a single, disciplinespecific, diagnostic label. This multidisciplinary approach can help avoid confusion that parents face regarding appropriate management. —Beula Magimairaj, Ph.D., CCC-SLP
A 2015 ERG scientist generously funded by the General Grand Chapter Royal Arch Masons International, Beula Magimairaj, Ph.D., CCC-SLP, is a research scientist at Utah State University.
Effects of Premature Birth on the Auditory System
Nearly 380,000 preterm births or deliveries prior to 37 weeks are recorded annually in the United States. It is also estimated that 15 million preterm births occur globally each year. Recent reports indicate the prevalence of preterm births continues to rise, impacting as many as 1 in 10 infants. According to information acquired from 2015 to 2017, premature birth rates are 49 to 50 percent higher in African American women (14 percent) when compared with Caucasian women (9 percent), indicating the presence of a health disparity issue.
The probability of substantial health issues and longterm disabilities increases as gestation at the time of birth decreases. If the effects of prematurity on children’s development of auditory skills are better understood, hearing healthcare professionals will be able to provide more individualized intervention recommendations and improve long-term outcomes for children born prematurely.
In our August 2020 paper in The Hearing Journal, we review causes of prematurity and the typical development of the auditory system. The sensory system, which includes the auditory system, develops in a very specific way inside the womb. This process is interrupted and occurs differently when development continues outside of the womb due to a premature birth. Specifically, exposure to frequencies of sounds, noise, and noise levels is very different within the neonatal intensive care unit. Information on this topic is critical for audiologists to know because this early sensory interruption can affect the brain’s ability to process sound. This can have long lasting consequences for those children born prematurely and can include central auditory processing disorders for this special population.
We examined central auditory processing skills in adolescents ages 12 to 15 years with a premature birth history. Those skills were then compared with their age-matched peers who have a full-term birth history. The central auditory processing skills accessed in that project included binaural integration (combining information coming from both ears); binaural separation (listening to one message, while ignoring a competing message); binaural interaction (detecting where sounds are coming from); rapid speech (speech that is fast and distorted); temporal patterning (distinguishing sound patterns); temporal resolution (separating gaps or sounds Major findings from our study are that adolescents who were born prematurely had significant difficulties with central auditory processing skills in the areas of binaural integration and speech-in-noise.
from each other); speech-in-noise (understanding speech in the presence of background noise); and auditory memory (repeating what is heard).
Major findings from our study are that adolescents who were born prematurely had significant difficulties with central auditory processing skills in the areas of binaural integration and speech-in-noise. Our research is ongoing and being assessed in other pediatric age groups. A major recommendation of this paper is early monitoring of central auditory processing skills by audiologists for this population of prematurely born children. —Alisha Lambeth Jones, Au.D., Ph.D.
A 2018 ERG scientist generously funded by the General Grand Chapter Royal Arch Masons International, Alisha Lambeth Jones, Au.D., Ph.D., is an associate professor in the department of communication disorders at Auburn University in Alabama.
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EMERGING RESEARCH GRANTS
Association Between Blood Pressure and Cochlear Function
High blood pressure (BP) is a common chronic condition in the United States with an estimated prevalence among adults of 31 percent, or 69 million. In addition to an increased risk of stroke and heart disease, elevated BP may also increase risk of hearing loss. In fact, the two commonly co-occur. Numerous studies have evaluated the association between hearing loss and risk factors for cardiovascular disease, including high BP. However, data from population- and laboratory-based studies remains inconclusive, and most prior work has focused on the effects of BP level on behavioral responses to sounds.
Our study published in Ear and Hearing in August 2020 extended previous work by examining the effect of BP on auditory status using extended highfrequency audiometry (which measures behavioral responses to sound) and distortion product otoacoustic emissions (DPOAEs), a noninvasive, objective test of inner ear (cochlear) function. Sixty individuals took part in this study and underwent a health assessment in addition to comprehensive audiological testing.
Participants were placed into one of two groups according to their BP level: “optimal” (systolic/diastolic BP <120/<80mm Hg) or “nonoptimal” (systolic ≥120 or diastolic ≥80mm Hg, or requiring the use of antihypertensives).
Initial findings suggest significant correlations between diastolic BP and behavioral hearing thresholds. We also identified a correlation between diastolic BP and DPOAE levels in the mid-frequency range. However, more in-depth statistical analysis indicated that other factors such as age and gender are more important drivers of impaired auditory function than BP level. Contrary to our hypothesis, we found that subtle BP elevation was not associated with poorer hearing or cochlear dysfunction. A greater elevation in BP (that is, hypertension itself) may be associated with more pronounced effects on inner ear function, warranting further investigation. In addition, our study suggests that DPOAEs may be a viable tool to characterize the relationship between risk factors for heart disease (and in particular, stage 2 hypertension) and hearing health. Follow-up investigations are underway. —Rachael R. Baiduc, Ph.D.
For references, see hhf.org/fall2020-references.
A 2018 ERG scientist, Rachael R. Baiduc, Ph.D., is an assistant professor in the department of speech, language, and hearing sciences at the University of Colorado Boulder, where she is also the director of the Hearing Epidemiology and Research Diagnostics (HEARD) Laboratory.
