Hyperacusis

2019-2020 Emerging Research Grantees Announced

By Christopher Geissler, Ph.D.

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Hearing Health Foundation (HHF) is proud to announce the recipients of Emerging Research Grants (ERG) for the upcoming year (July 1, 2019 — June 30, 2020). Following a rigorous review process, our Scientific Review Committee and Council of Scientific Trustees, comprised of senior expert scientists and physicians from across the US, have chosen fourteen especially meritorious projects to fund, covering a broad range of hearing and balance science. 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.

This year’s ERG recipients are:

Dunia Abdul-Aziz, M.D.
Massachusetts Eye and Ear
Project: Targeting epigenetics to restore hair cells

Pierre Apostolides, Ph.D.
Regents of the University of Michigan
Project: Novel mechanisms of cortical neuromodulation

Michael Dent, Ph.D.
University at Buffalo
Project: Noise-induced tinnitus in mice
Generously funded by The Les Paul Foundation

Vijayalakshmi Easwar, Ph.D.
University of Wisconsin Madison
Project: Neural correlates of amplified speech in children with sensorineural hearing loss
Generously funded by The Children’s Hearing Institute

Kristi Hendrickson, Ph.D.
University of Iowa
Project: Neural correlates of semantic structure in children who are hard of hearing
Generously funded by General Grand Chapter Royal Arch Masons

Hao Luo, Ph.D.
Wayne State University
Cochlear electrical stimulation induced tinnitus suppression and related neural activity change in the rat's inferior colliculus
Generously funded by General Grand Chapter Royal Arch Masons

Kristy Lawton, Ph.D.
Washington State University Vancouver
Project: Characterizing noise-induced synaptic loss in the zebrafish lateral line
Generously funded by General Grand Chapter Royal Arch Masons

Anat Lubetzky, P.T., Ph.D.
New York University
Project: A balancing act in hearing and vestibular loss: assessing auditory contribution to multisensory integration for postural control in an immersive virtual environment

David Martinelli, Ph.D.
University of Connecticut Health Center
Project: Creation and validation of a novel genetically-induced animal model for hyperacusis
Generously funded by Hyperacusis Research

Jameson Mattingly, M.D.
The Ohio State University
Project: Differentiating Ménière's disease and vestibular migraine using audiometry and vestibular threshold measurements

Vijaya Prakash Krishnan Muthaiah, P.T., Ph.D.
University at Buffalo
Project: Potential of inhibition of Poly ADP Ribose Polymerase as a therapeutic approach in blast induced cochlear and brain injury.
Generously funded by General Grand Chapter Royal Arch Masons

William “Jason” Riggs, Au.D.
The Ohio State University
Project: electrophysiological characteristics in children with auditory neuropathy spectrum disorder
Generously funded by General Grand Chapter Royal Arch Masons

Gail Seigel, Ph.D.
The Research Foundation of SUNY on behalf of the University at Buffalo
Project: Targeting microglial activation in hyperacusis

Victor Wong, Ph.D.
Burke Medical Research Institute
Project: Targeting tubulin acetylation in spiral ganglion neurons for the treatment of hearing loss

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Ears On Fire

by Ben Meltzer 

I attended my first concert, Harry Chapin, at age 3. This was on March 24, 1977 at Painters Mill Music Fair in Owings Mills, Maryland. 14 years later, at the same venue, I saw my first of some 35 Bob Dylan concerts. I was a big concertgoer who wore earplugs only sporadically.

I had a great deal of additional noise exposure in an office where my colleagues and I were subjected to routine fire alarm testing during working hours. And for several weeks, I sat near a squealing printer that ran continuously.

In early 2007, I started feeling fullness in my left ear. Over the next nine months, it worsened. I experienced my first bout of fleeting tinnitus or SBUTT: sudden brief unilateral tapering tinnitus.

An ENT told me aural fullness is a common symptom of temporomandibular joint disorder, or TMJ, so I visited my dentist, who made me a mouthguard. It made my symptoms worse.

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Two weeks later, I awoke to find ordinary sounds were painfully loud. The honk of a car horn was excruciating, as was the roar of a lawnmower.

More debilitating than this knifelike pain from sound, however, was a constant burning pain in the ears that existed independent of sound. This symptom confounded the audiologist and ear doctors I saw. 

The loudness discomfort test I took at a prominent hospital indicated severe hyperacusis, but the audiologist there said she had never encountered a patient with constant ear pain. The institution’s inexperience with my symptoms unnerved me. 

Over the next two years–with time, quiet, and ear protection–I slowly improved. Then, two big noise insults in the same week proved catastrophic. At the dentist, with a dental drill whirring to my right, I felt something break in my right ear. A few days later, I was near the fire station when an air-raid siren blasted, loud enough to alert the whole town’s firefighters.

From then on, my symptoms of noise trauma―aural fullness, tinnitus, hyperacusis, and burning pain―intensified. The trauma turned my right ear red and veiny, and it became sensitive to wind and cold. When my hair grows longer, I feel it on my right ear more acutely than on my left. So I now have a “good ear” and a “bad ear,” with similar symptoms that differ in severity. 

Worst of all, the right ear could no longer tolerate an earplug or being covered with a protective earmuff. I get severe pain from merely covering the ear.  This symptom has mitigated slightly. I can now–more than 10 years post-injury–wear earmuffs for around 5 minutes on a good day and still not at all on a bad one.  Sometimes I feel as though there’s a lit match inside my ear. 

A noise injury worsens readily. For hyperacusis sufferers such as myself, quiet makes the condition better; noise makes it worse. Among sufferers this is indisputable, but medical practitioners bizarrely treat quiet as harmful. Too often they think the patient’s chief concern is hearing loss, rather than an intolerable perception of sound. 

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Two nonprofits currently researching noise-induced pain and pushing the field forward are Hyperacusis Research and Hearing Health Foundation. New discoveries, with scientific tools and techniques only recently available, show that noise is far more destructive than previously believed. 

I plan to donate my ears to the Temporal Bone Registry so that researchers will be able to examine them after my death to better understand hyperacusis, relieve it, and put an end to the misery endured by those like me.

Ben Meltzer is from Baltimore and now lives with his wife in New York.

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Quantifying the Effects of a Hyperacusis Treatment

By Xiying Guan, Ph.D

A typical inner ear has two mobile windows: the oval and round window (RW). The flexible, membrane-covered RW allows fluid in the cochlea to move as the oval window vibrates in response to movement from the stapes bone during sound stimulation.

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Superior canal dehiscence (SCD), a pathological opening in the bony wall of the superior semicircular canal, forms a third window of the inner ear. This structural anomaly results in various auditory and vestibular symptoms. One common symptom is increased sensitivity to self-generated sounds or external vibrations, such as hearing one’s own pulse, neck and joint movement, and even eye movement. This hypersensitive hearing associated with SCD has been termed conductive hyperacusis.

Recently, surgically stiffening the RW is emerging as a treatment for hyperacusis in patients with and without SCD. However, the postsurgical results are mixed: Some patients experience improvement while others complain of worsening symptoms and have asked to reverse the RW treatment. Although this “experimental” surgical treatment for hyperacusis is increasingly reported, its efficacy has not been studied scientifically.

In the present study, we experimentally tested how RW reinforcement affects air-conduction sound transmission in the typical ear (that is, without a SCD). We measured the sound pressures in two cochlear fluid-filled cavities—the scala vestibuli (assigned the value “Psv”) and the scala tympani (“Pst”)—together with the stapes velocity in response to sound at the ear canal. We estimated hearing ability based on a formula for the “cochlear input drive” (Pdiff = Psv – Pst) before and after RW reinforcement in a human cadaveric ear.

We found that RW reinforcement can affect the cochlear input drive in unexpected ways. At very low frequencies, below 200 Hz, it resulted in a reduced stapes motion but an increase in the cochlear input drive that would be consistent with improved hearing. At 200 to 1,000 Hz, the stapes motion and input drive both were slightly decreased. Above 1,000 Hz stiffening the RW had no effect.

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The results suggest that RW reinforcement has the potential to worsen low-frequency hyperacusis while causing some hearing loss in the mid-frequencies. Although this preliminary study shows that the RW treatment does not have much effect on air-conduction hearing, the effect on bone-conduction hearing is unknown and is one of our future areas for experimentation.

A 2017 ERG scientist funded by Hyperacusis Research Ltd., Xiying Guan, Ph.D., is a postdoctoral fellow at Massachusetts Eye and Ear, Harvard Medical School, in Boston.

We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

 
 
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Introducing the 2018 Emerging Research Grantees

By Lauren McGrath

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Hearing Health Foundation (HHF) is pleased to present our Emerging Research Grants (ERG) awardees for the 2018 project cycle.

Grantee Tenzin Ngodup, Ph.D., will investigate neuronal activity in the ventral cochlear nucleus to help prevent and treat tinnitus.

Grantee Tenzin Ngodup, Ph.D., will investigate neuronal activity in the ventral cochlear nucleus to help prevent and treat tinnitus.

15 individuals at various institutions nationwide—including Johns Hopkins School of Medicine, University of Minnesota, and the National Cancer Institute—will conduct innovative research in the following topic areas:

  • Central Auditory Processing Disorder (CAPD)

  • General Hearing Health

  • Hearing Loss in Children

  • Hyperacusis

  • Tinnitus

  • Usher Syndrome

Our grantees’ research investigations seek to solve specific auditory and vestibular problems such as declines in complex sound processing in age-related hearing loss (presbycusis), ototoxicity caused by the life-saving chemotherapy drug cisplatin, and noise-induced hearing loss.

HHF looks forward to the advancements that will come about from these promising scientific endeavors. The foundation owes many thanks to the General Grand Chapter Royal Arch Masons International, Cochlear, Hyperacusis Research, the Les Paul Foundation, and several generous, anonymous donors who have collectively empowered this important work.

We are currently planning for our 2019 ERG grant cycle, for which applications will open September 1. Learn more about the application process.

WE NEED YOUR HELP IN FUNDING THE EXCITING WORK OF HEARING AND BALANCE SCIENTISTS. DONATE TODAY TO HEARING HEALTH FOUNDATION AND SUPPORT GROUNDBREAKING RESEARCH: HHF.ORG/DONATE.

Grantee Rachael R. Baiduc, Ph.D., will identify  cardiovascular disease risk factors that may contribute to hearing loss.

Grantee Rachael R. Baiduc, Ph.D., will identify
cardiovascular disease risk factors that may contribute to hearing loss.

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HHF Partner Hyperacusis Research Shares 14-Year-Old’s Heartbreaking Story to Fight Noise Intolerance

Photo Credit: Hyperacusis Research

Photo Credit: Hyperacusis Research

By Lauren McGrath

Hearing Health Foundation (HHF) Emerging Research Grants (ERG) grant funder Hyperacusis Research—a nonprofit dedicated to developing effective treatments for hyperacusis and to funding research that will eliminate the underlying mechanisms that cause hyperacusis—has a new reason to fight to cure the noise intolerance disorder.

Cindy, 14 years old, has suffered from hyperacusis since she was blasted in the face with an airhorn one year ago. The blast almost immediately prompted “a burst of pain in [her] ear” that made it “feel like someone was stabbing [her].” Six months and several doctors’ visits later, an occupational therapist recognized her symptoms and diagnosed her with the disorder, which causes Cindy to experience pain at low levels of sound relative to what a person with typical hearing can withstand.

Once a happy and social eighth-grader, Cindy now rarely leaves her home. Secluded from the painful sounds of the outside world, her house has become “her sanctuary,” her mother explains. Her intolerance of everyday noises like the school cafeteria and teachers’ voices has forced her to leave public school in exchange for an isolating homeschool experience. “The thing I hate most is that I can’t see friends,” Cindy shares.

Cindy suffers from one of four hyperacusis subtypes called pain hyperacusis. The other three types, according to Hyperacusis Research, are loudness hyperacusis (which causes moderately intense sounds to be perceived as very loud), annoyance hyperacusis (which causes negative emotional reactions to sounds), and fear hyperacusis (which prompts an aversive response to sounds that causes anticipatory response and avoidance behavior). Specific medical treatments, at the moment,  do not yet exist for pain hyperacusis.

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Those inspired to help Cindy can donate to Hyperacusis Research to advance the ontological knowledge of hyperacusis through research grants, including those awarded to HHF’s ERG investigators.

Since 2015, Hyperacusis Research has generously funded grants for a total of five ERG investigators focused on hyperacusis at the University at Buffalo, Oregon Health and Science University, and Massachusetts Eye and Ear Infirmary. You can learn more about our ERG researchers’ efforts to better understand the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance here.

We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

 
 
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HHF Launches Faces of Hearing Loss Campaign

Think of someone you know who has hearing loss. Who do you see?

You envision a relative, but you are not thinking of your 4-year-old niece. A neighbor comes to mind, but not the 32-year-old who lives across the street.

This is a trick question. Hearing loss—and related conditions like tinnitus, Ménière's disease, and hyperacusis—can affect anyone, anywhere. Hearing loss is your 4-year-old niece, your 32-year-old neighbor, your colleague in her mid-20s. Hearing loss affects every age, race, ethnicity, and gender.

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No one is immune from developing a hearing and balance disorder—and hearing loss has no single face. To refute common misconceptions that it only affects older adults, HHF has collected images of individuals living with a hearing condition to capture the diversity of its impact across the country. These are HHF’s “Faces of Hearing Loss.”

Participants shared their picture, current age, state of residence, type of hearing condition, and the age at onset or diagnosis. Among the tens of millions of Americans with hearing loss are an 11-year-old boy in Oregon, an 80-year-old woman living in Washington, and a 47-year-old man in North Dakota. These individuals may never meet, but “Faces of Hearing Loss” connects them through their shared experiences.

If you or a loved one has hearing loss, please consider participating in “Faces of Hearing Loss” by completing this brief form, sending in picture, and answering a few basic questions. If you are the parent of a child under 18, you may sign a release form on their behalf.

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An Animal Behavioral Model of Loudness Hyperacusis

By Kelly Radziwon, Ph.D., and Richard Salvi, Ph.D.

One of the defining features of hyperacusis is reduced sound level tolerance; individuals with “loudness hyperacusis” experience everyday sound volumes as uncomfortably loud and potentially painful. Given that loudness perception is a key behavioral correlate of hyperacusis, our lab at the University at Buffalo has developed a rat behavioral model of loudness estimation utilizing a reaction time paradigm. In this model, the rats were trained to remove their noses from a hole whenever a sound was heard. This task is similar to asking a human listener to raise his/her hand when a sound is played (the rats receive food rewards upon correctly detecting the sound).
 

FIGURE: Reaction time-Intensity functions for broadband noise bursts for 7 rats.    The rats are significantly faster following high-dose (300 mg/kg) salicylate administration (left panel; red squares) for moderate and high level sounds, indicative of temporary loudness hyperacusis. The rats showed no behavioral effect following low-dose (50 mg/kg) salicylate.

FIGURE: Reaction time-Intensity functions for broadband noise bursts for 7 rats.

The rats are significantly faster following high-dose (300 mg/kg) salicylate administration (left panel; red squares) for moderate and high level sounds, indicative of temporary loudness hyperacusis. The rats showed no behavioral effect following low-dose (50 mg/kg) salicylate.

By establishing this trained behavioral response, we measured reaction time, or how fast the animal responds to a variety of sounds of varying intensities. Previous studies have established that the more intense a sound is, the faster a listener will respond to it. As a result, we thought having hyperacusis would influence reaction time due to an enhanced sensitivity to sound.

In our recent paper published in Hearing Research, we tested the hypothesis that high-dose sodium salicylate, the active ingredient in aspirin, can induce hyperacusis-like changes in rats trained in our behavioral paradigm. High-dose aspirin has long been known to induce temporary hearing loss and acute tinnitus in both humans and animals, and it has served as an extremely useful model to investigate the neural and biological mechanisms underlying tinnitus and hearing loss. Therefore, if the rats’ responses to sound are faster than they typically were following salicylate administration, then we will have developed a relevant animal model of loudness hyperacusis.

Although prior hyperacusis research utilizing salicylate has demonstrated that high-dose sodium salicylate induced hyperacusis-like behavior, the effect of dosage and the stimulus frequency were not considered. We wanted to determine how the dosage of salicylate as well as the frequency of the tone bursts affected reaction time.

We found that salicylate caused a reduction in behavioral reaction time in a dose-dependent manner and across a range of stimulus frequencies, suggesting that both our behavioral paradigm and the salicylate model are useful tools in the broader study of hyperacusis. In addition, our behavioral results appear highly correlated with the physiological changes in the auditory system shown in earlier studies following both salicylate treatment and noise exposure, which points to a common neural mechanism in the generation of hyperacusis.

Although people with hyperacusis rarely attribute their hyperacusis to aspirin, the use of the salicylate model of hyperacusis in animals provides the necessary groundwork for future studies of noise-induced hyperacusis and loudness intolerance.


Kelly Radziwon, Ph.D., is a 2015 Emerging Research Grants recipient. Her grant was generously funded by Hyperacusis Research Ltd. Learn more about Radziwon and her work in “Meet the Researcher.”

We need your help in funding the exciting work of hearing and balance scientists. Donate today to Hearing Health Foundation and support groundbreaking research: hhf.org/donate.

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Noise-Induced Brain Inflammation May Result in Painful Hearing

By Drs. Senthilvelan Manohar, Kelly Radziwon, and Richard Salvi

What do jet engines, sirens, and rock bands have in common? The sounds they emit are so intense that they are not only loud, but also painful, sometimes evoking a painful sensation around the external ear. The acoustic threshold for pain, 130-140 dB SPL, is intense enough to destroy or damage the delicate sensory hair cells, supporting cells and auditory nerve fibers in the inner ear. The axons from the auditory nerve deliver their messages to neurons located in the cochlear nuclei in the brainstem. 

In a recent paper published in Molecular and Cellular Neuroscience, Drs. Baizer and Manohar at the University at Buffalo were surprised to find that intense noise exposures that destroyed the sensory hair cells in the rat inner ear led to a prolonged period of auditory nerve fiber degeneration in the cochlear nucleus in the brainstem (Bazier et al., Neuroscience 303 (2015) 299–311). Nerve fiber degeneration was still occurring 6-9 months post-exposure, nearly a third of the rat’s lifespan. In brain regions where the fibers were degenerating, there was robust upregulation of brain immune cells (microglia), indicative of long-term neuro-inflammation triggered by the release of inflammatory molecules in the brain. Since sensory nerve fibers (e.g., pain, touch) from the face, head, neck and shoulders (facial, trigeminal and spinal nerves) enter the cochlear nucleus, the long-term neuro-inflammation occurring in this region could lower pain thresholds (hyperalgesia). If this were to occur, much lower, moderate-intensity sounds (60-80 dB) might be sufficient to cause hyperacusis (loudness intolerance) with ear pain.

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With funding from the Hearing Health Foundation obtained by Drs. Radziwon* and Manohar to identify pain-related molecules in the auditory pathway as a result of noise exposure, Drs. Manohar, Adler, and Salvi carried out a second study in which they measured noise-induced changes in the expression (amount) of genes involved in the synthesis of proteins known to be involved in neuropathic pain and neuro- inflammation. Interestingly, the researchers found that intense noise exposure significantly altered the expression of six genes (Ccl12, Tlr2, Oprd1, II1b, Ntrk1 & Kcnq3) in the cochlear nucleus (Manohar et al., Molecular and Cellular Neuroscience 75 (2016) 101–112). These results suggest that noise-induced inflammation in the parts of the central auditory pathway that also processes sensory information related to pain might, in turn, activate the central pain pathway thus producing ear pain. Determining whether neuro-inflammation is directly responsible for ear pain will open the door for novel interventions to treat hearing loss and hyperacusis.

*Kelly Radziwon, Ph.D., is a 2015 Emerging Research Grants recipient. Her grant was generously funded by Hyperacusis Research Ltd. Learn more about Radziwon and her work in “Meet the Researcher.”
 

We need your help in funding the exciting work of hearing and balance scientists. Donate today to Hearing Health Foundation and support groundbreaking research: hhf.org/donate.

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Introducing HHF's 2016 Emerging Research Grant Recipients

By Morgan Leppla

We are excited to announce the 2016 Emerging Research Grant recipients. This year, HHF funded five research areas:

  • Central Auditory Processing Disorder (CAPD): research investigating a range of disorders within the ear and brain that affect the processing of auditory information. HHF thanks the General Grand Chapter Royal Arch Masons International for enabling us to fund four grants in the area of CAPD. 
     
  • Hyperacusis: research that explores the mechanisms, causes, and diagnosis of loudness intolerance. One grant was generously funded by Hyperacusis Research.
     
  • Méniere’s Disease: research that investigates the inner ear and balance disorder. One grant was funded by the Estate of Howard F. Schum.
     
  • Stria: research that furthers our understanding of the stria vascularis, strial atrophy, and/or development of the stria. One grant was funded by an anonymous family foundation interested in this research.
     
  • Tinnitus: research to understand the perception of sound in the ear in the absence of an acoustic stimulus. Two grants were awarded, thanks to the generosity the Les Paul Foundation and the the Barbara Epstein Foundation.

To learn more about our 2016 ERG grantees and their research goals, please visit hhf.org/2016_researchers

HHF is also currently planning for our 2017 ERG grant cycle. If you're interested in naming a research grant in any discipline within the hearing and balance space, please contact development@hhf.org.

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2015 Emerging Research Grants Approved!

By Laura Friedman

Hearing Health Foundation is excited to announce that the 2015 Emerging Research Grants (ERG) have been approved by our Board of Directors, after a rigorous scientific review process. The areas that we are funding for the 2015 cycle are:

  • Central Auditory Processing Disorder (CAPD): Four grants were awarded for innovative research that will increase our understanding of the causes, diagnosis, and treatment of central auditory processing disorder, an umbrella term for a variety of disorders that affect the way the brain processes auditory information. All four of our CAPD grantees are General Grand Chapter Royal Arch Masons International award recipients.

  • Hyperacusis: Two grants were awarded that is focused on innovative research (e.g., animal models, brain imaging, biomarkers, electrophysiology) that will increase our understanding of the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance. Research that explores distinctions between hyperacusis and tinnitus is of special interest. Both of our Hyperacusis grants were funded by Hyperacuis Research.

  • Ménière’s Disease: Two grants were awarded for innovative research that will increase our understanding of the inner ear and balance disorder Ménière’s disease. One of the grants is funded by The Estate of Howard F. Schum and the other is funded by William Randolph Hearst Foundation through their William Randolph Hearst Endowed Otologic Fellowship.

  • Tinnitus: Two grants were awarded for innovative research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus. One of the grants is funded by the Les Paul Foundation and the other grantee is the recipient of The Todd M. Bader Research Grant of The Barbara Epstein Foundation, Inc.

To learn more about our 2015 ERG grantees and their research proposals and goals, please visit: http://hearinghealthfoundation.org/2015_researchers

Hearing Health Foundation is also currently planning for our 2016 ERG grant cycle. If you're interested in naming a research grant in any discipline within the hearing and balance space, please contact development@hhf.org.

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