For the 2012 Emerging Research Grant cycle, Hearing Health Foundation supported research in the following areas:



+ Renjie Chai, Ph.D.


Stanford University
Characterization of Wnt-responsive progenitor cells in the mammalian cochlea

Hearing loss is a common sensory disorder affecting nearly 50 million adults in the United States alone. The majority of hearing loss is caused by the loss of the inner ear sensory hair cells, which, in mammals, lack the ability to regenerate. In this proposal, we will gain insights into the regenerative potential of the mammalian cochlear hair cells, with long term goal to improve the current treatment of hearing loss via hair cell regeneration. The Wnt signaling pathway has been found to play a crucial role in maintaining the stem cell population in several organ systems. Recently our laboratory has found a transient expression of active wnt signals in the mouse cochlea, and found 2 inner ear progenitor cell populations marked by two Wnt downstream target genes. This project has been designed to systematically investigate the role of the Wnt pathway in maintaining these two Wnt responsive progenitor cell populations.

Research area: hair cell regeneration

Long term goal of research: To use hair cell regeneration and cell-base therapy to treat patients with sensorineural hearing loss.

Renjie Chai, Ph.D. received his Ph.D. at Baylor University and is now working as a postdoctoral fellow in the Department of Otolaryngology at Stanford University.

+ Wei-Min Chen, Ph.D. and Ani Manichaikul, Ph.D

University of Virginia
Susceptibility to chronic otitis media: translating gene to function

Each year in the United States, over $5 billion is spent on healthcare for inflammation of the middle ear (ME) known as otitis media (OM) in children. Some children develop chronic middle ear infections known as chronic otitis media with effusion and/or recurrent otitis media (COME/ROM). Our goal is to find genetic factors that increase risk for COME/ROM in children. The discovery of causal variants would increase knowledge of novel genes and pathways involved in COME/ROM pathogenesis.

Research areas: otitis media, genetics

Long term goal of research: Findings from our research are expected to improve the clinical prevention of chronic infections; therefore decreasing pediatric antibiotic use, surgery, and deafness.

Wei-Min Chen, Ph.D. is an assistant professor at the University of Virginia Department of Public Health Sciences and Center for Public Health Genomics. He earned his Ph.D. at Johns Hopkins School of Public Health. His research focuses on the design and statistical analysis of human gene mapping data.

Ani Manichaikul, Ph.D. is an assistant professor at the University of Virginia Department of Public Health Sciences and Center for Public Health Genomics. She received her Ph.D. in biostatistics at the Johns Hopkins School of Public Health. Her research focuses on statistical genetics and genetic epidemiology in human cohorts, as well as translational research bridging mouse and human genetic studies.

+ Yoojin Chung, PH.D.

Massachusetts Eye and Ear Infirmary
Restoring binaural hearing with cochlear implants in early-onset deafness

Many profoundly deaf people wearing cochlear implants still face challenges in everyday situations such as understanding conversations in crowds. This is because, even with cochlear implants in both ears, they have difficulty making full use of subtle differences in the sounds reaching two ears to identify where the sound is coming from. This problem is especially acute in children with congenital deafness. We will study how perceptual training can help the brain to develop the circuitry for processing this precise information in animals with early-onset deafness. Results from the study will eventually lead to new sound processors and rehabilitation strategies specifically adapted for bilateral cochlear implants.

Research areas: neural coding of cochlear implant stimulation, central auditory plasticity

Long term goal of research: are to improve treatments for children with early-onset deafness by studying how neural mechanisms for binaural processing are altered by auditory deprivation during development and whether these effects can be reversed by CI stimulation.

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Chung received a Ph.D. in biomedical engineering from Boston University in Massachusetts. She is a postdoctoral fellow at Massachusetts Eye and Ear Infirmary and Harvard Medical School. Dr. Chung is a Royal Arch Masons award recipient. The Royal Arch Masons support Emerging Research Grants in the area of Central Auditory Processing Disorder (CAPD). See all researchers who have received or are currently receiving funding from the Royal Arch Masons.

+ Elizabeth A. Hurd, Ph.D.

University of Michigan
Investigating the role of Chd7 during noise-induced hearing loss

Mice born with loss of Chd7, the gene mutated in human CHARGE syndrome, exhibity middle ear defects and resistance to acoustic trauma. Preliminary results show that deletion of Chd7 in adult mice (using tamoxifen inducible Cre line) also results in variable resistance to acoustic trauma, even in the absence of middle ear defects. This suggests important functions for Chd7 in regulating hair cells and neuronal integrity in adult cochlea. The objective of this research is to identify how loss of Chd7 influences susceptibility to acoustic trauma in the mature cochlea.

Research area: noise-induced hearing loss (NIHL)

Long term goal of research: to help identify novel genes and molecular pathways involved in protection from NIHL and provide rationale for designing new therapies.

Elizabeth Hurd, Ph.D. received her BSc and Ph.D. in Biochemistry from the University of Bristol, United Kingdom. She completed 10 years of postdoctoral training at the University of Michigan in the Departments of OB/GYN and Pediatrics. She is currently a Research Investigator in the Department of Pediatrics at University of Michigan.


+ Israt Jahan, M.B.B.S, Ph.D.

University of Iowa
Misexpression of Neurog1 combined with delayed deletion of Atoh1 provides a novel model

Contemporary research is focusing on the regeneration of hair cells in hearing loss using Atoh1. Reconstitution of organ of Corti requires proper organization of the two types of hair cells, inner and outer hair cells, as well as supporting cells. Recent data showed that level of Atoh1 determines the degree of survival of different types of hair cells. Jahan’s previous work demonstrated the survival of some organ of Corti-like cells without Atoh1 if replaced by a closely related transcription factor, Neurog1. It is now Jahan’s intent to investigate the effect of Atoh1 substitution with Neurog1 combined with a delayed loss of Atoh1 expression in viable animals. This combined mutant offers for the first time a critical test of presumed causalities of molecular mechanism that regulate the patterning of the organ of Corti, including hair cell and supporting cell differentiation.

Research area: hair cell regeneration

Long term goal of research: Define the correct dose and duration of Atoh1 expression for type-specific hair cell development which will provide novel insights into hair cell regeneration.

Jahan received her Ph.D. in medical sciences from Gifu University in Japan. She began postdoctoral training in inner ear neurosensory development at Creighton University in Nebraska before moving to the University of Iowa to continue her work.

+ Kelvin Y. Kwan, Ph.D.

Rutgers University
Identification of Transcription Factors for Hair Cell Regeneration

In mammals, when hair cells die from ototoxic drugs or loud noises, they are not replaced. Kwan’s efforts are focused on identifying transcription factors that promote repopulation and replacement of lost sensory hair cells. Transcription factors are DNA binding proteins that play crucial roles in global gene regulation. By repurposing transcription factors that are normally expressed during hair cell development, he plans to promote regeneration by controlled cell division to repopulate lost hair cells before differentiating nascent cells into hair cells.

Research area: hair cell regeneration

Long term goal of research: To use a cocktail of small molecules that activates expression of transcription factors or their associated signaling pathways in order to promote functional auditory hair cell regeneration and alleviate hearing loss.

Kwan received his Ph.D. in biochemistry from Harvard University in Massachusetts. He completed his postdoctoral training in auditory neuroscience at Harvard Medical School where he investigated the process of hair-cell mechanotransduction. He is an assistant professor and the Duncan and Nancy MacMillan Faculty Development Chair in the Life Sciences in the Department of Cell Biology and Neuroscience at Rutgers University in New Jersey.

+ Sean Eric Low, Ph.D.

Rockefeller University
Ascertaining the contribution of piezo proteins to mechanotransduction in zebrafish hair cells

The proteins that mediate the transformation of mechanical forces into electrical signals within the sensory cells that convey the senses of hearing and balance have yet to be identified. This lack of knowledge has undoubtedly hindered the identification of therapeutic compounds capable of alleviating the complications that arise from disorders in hearing and balance, such as deafness and vertigo. Recently, a member of the novel piezo protein family has been shown to contribute to cutaneous mechanosensation, raising the possibility that related family members may contribute to hearing and balance. Dr. Low will utilize the simple vertebrate commonly known as zebrafish, to address this possibility.

Research area: fundamental auditory research

Long term goal of research: To identify therapeutic agents that can restore normal hearing and balance in individuals who have either lost these senses, or suffer from conditions caused by abnormal activity in the sensory cells that mediate them.

Sean Low Ph.D. received a B.S. in Cellular and Molecular Biology in 2001, and a Ph.D. in Neuroscience in 2008 from the University of Michigan. Desiring to focus on sensory transduction he sought out a postdoctoral position in the Saint-Amant lab at the University of Montréal from 2009 – 2011, where he examined the role of a piezo protein in cutaneous mechanotransduction. These studies evolved into an interest in mechanotransduction processes in general, and a second postdoctoral position with Dr. Hudspeth at The Rockefeller University beginning in 2011.

Low is also The Todd M. Bader Research Grant of the Barbara Epstein Foundation, Inc., Recipient. This research award is funded in part by The Todd M. Bader Research Grant of The Barbara Epstein Foundation, Inc.

+ Lina Reiss, Ph.D.

Oregon Health & Science University
Changes in Residual Hearing in a Hearing-impaired Guinea Pig Model of Hybrid Cochlear Implants (CIs)

The goal of the current study is to understand mechanisms of hearing loss with “hybrid” or “electro-acoustic” cochlear implants (CIs), a new type of CI designed to preserve low-frequency hearing and allow combined acoustic-electric stimulation in the same ear. Hybrid CI users perform significantly better than standard CI users on musical melody recognition, voice recognition, and speech recognition in the presence of background talkers. However, approximately 10% of hybrid CI patients lose all residual hearing, and another 20% lose 20-30 dB after implantation. We hypothesize that in addition to surgical trauma, electrical stimulation through the hybrid CIs also damages cochlear cells, leading to the residual hearing loss (HL). Aim 1 is to determine the contribution of electrical stimulation to the residual HL in hybrid CI guinea pigs with noise-induced steeply-sloping high frequency hearing loss (NIHFHL). Aim 2 is to examine the effect of electrical stimulation on the cochlear pathology. The findings will guide the development of strategies to prevent hearing loss with electrical stimulation, and allow extension of the hybrid concept to all cochlear implant recipients with usable residual hearing.

Research area: Cochlear implants

Long term goal of research: To improve residual hearing preservation with “hybrid” or “electro-acoustic” cochlear implants (CIs), a new type of CI designed to preserve low-frequency hearing and allow combined acoustic-electric stimulation in the same ear.

Lina Reiss Ph.D. has been an Assistant Professor in the Department of Otolaryngology-Head and Neck Surgery at Oregon Health and Science University (OHSU) since 2010. Previously, Reiss was a postdoctoral scholar at the University of Iowa where she conducted research in the Hybrid cochlear implant clinical trials. Reiss earned her doctorate in Biomedical Engineering from Johns Hopkins University in 2005.

+ Susan M. Robey-Bond, Ph.D.

University of Vermont and State Agricultural College
The Role of a Mutation in Histidyl-tRNA Synthetase in Usher-like Syndrome Deafness

An Usher-like syndrome, comprising deafness, blindness, and fever-induced hallucinations was recently discovered, caused by recessive inheritance of a mutation in histidyl-tRNA synthetase (HARS). The HARS enzyme is required for protein production in cells: it attaches the amino acid histidine to a transfer ribonucleic acid (RNA) molecule which activates and transports the amino acid to the ribosome for protein synthesis. We will measure the effects of this mutation on the molecules required for protein synthesis. Preliminary results suggest HARS may be chemically modified by the cell, and that mutant HARS is modified differently, which is evidence HARS may have roles in the cell separate from its known function in protein synthesis. We additionally propose to determine the interactions of HARS and mutant HARS with other cellular proteins, specifically in cells derived from embryonic mouse inner ears, as a first step in elucidating a different role for HARS in hearing.

Research area: Usher and Usher-like syndrome deafness

Long term goal of research: Our long term goal is to describe the specific role HARS, and the HARS mutation, plays in sensory cell development and maintenance. With a greater understanding of the proteome - the expressed proteins and protein interactions of a cell - during different stages of development of affected cells, we hope to discover more potential avenues for therapy to prevent or alleviate symptoms of Usher and Usher-like syndromes.

Susan Robey-Bond, Ph.D. received a B.A. in chemistry from Macalester College in Minnesota and her Ph.D. in toxicology from the University of Rochester, New York. She conducted postdoctoral research at Cornell University and the University of Vermont on antioxidants and DNA repair mechanisms before her appointment as a Research Associate in Biochemistry, studying histidyl-tRNA synthetase, at the University of Vermont.

+ Isabelle Roux, Ph.D.

Johns Hopkins University
Mechanisms involved in efferent synapse formation and maintenance in cochlear hair cells

This research aims at understanding the molecular mechanisms that underlie the formation and maintenance of the connections between the sensory hair cells and efferent nerve fibers that provide feedback from the brain to the ear. Such fibers are important modulators of inner ear activity. Our investigation includes different approaches (electrophysiology, confocal microscopy, and mouse genetics) in parallel.

Research area: synaptic transmission in the inner ear

Long term goal of research: to understand the developmental machinery in the inner ear, which can lead to the ability to treat deficits in their function.

Isabelle Roux, Ph.D. received her M.S. degrees in genetics and human genetics in 2001 from the Paris VII Denis-Diderot University in Paris, France. She received her Ph.D. in human genetics in 2006 from the same university studying how mutations of the gene OTOF could lead to the human congenital deafness DFNB9. In 2007, she joined The Johns Hopkins School of Medicine in Baltimore as a post-doctoral fellow, where she is studying cochlear physiology.


+ Rebecca Seal, Ph.D.

University of Pittsburgh
Role of outer hair cell glutamate release in cochlear function and dysfunction

Outer hair cells are vital for normal hearing. Although the cells are known to amplify the cochlear response to sound using an electromotile mechanism, they also signal to type II spiral ganglion neurons through the regulated release of glutamate. However, the function of this signaling remains unknown. Similar to inner hair cells, glutamate signaling by outer hair cells may influence sound transmission as well as the maintenance of spiral ganglion afferents. In the adult, cholinergic efferents play a critical role in maintaining outer hair cell viability and the innervation pattern of these fibers may also be influenced by the released glutamate. Thus, there are several potential mechanisms by which loss of glutamate signaling by outer hair cells could cause hearing loss. This proposal aims to address these possibilities.

Research area: fundamental auditory research

Long term goal of research: To provide new information about the role of hair cell signaling in hearing and in disorders of the auditory system including hearing loss. These analyses will inform decisions on therapeutic strategies for the restoration of hearing and for other disorders that may be derived from aberrant cochlear function.

Rebecca Seal Ph.D. received her Ph.D. in Neuroscience from Oregon Health and Sciences University and completed her postdoctoral training in sensory circuits at the University of California, San Francisco. She is currently an Assistant Professor in the Department of Neurobiology at the University of Pittsburgh.

Seal is also recipient of the C.H.E.A.R. endowment, created to support an annual Sensory-Neural Deafness Research Grant. C.H.E.A.R. (Children Hearing Education and Research) was absorbed into Hearing Health Foundation in 1991, and we are very proud to continue their legacy of funding research in sensory-neural deafness.

+ Bradley J. Walters, Ph.D.

St. Jude Children's Research Hospital
Potential regeneration of auditory hair cells in the opossum, Monodelphis domestica

Millions of Americans suffer from sensorineural hearing loss: a disability that is permanent and on the rise as the iPod generation ages. For those who would seek to regain hearing, one potential solution may be offered by the regeneration of sensory hair cells within the inner ear. However, this is, as yet, unachievable in humans. Despite this, many non-mammalian vertebrates, like fish, amphibians, reptiles, and birds, naturally regenerate their sensory cells and regain their ability to hear, suggesting that humans lost this regenerative potential at some point during their evolution. It is hoped that discovering the critical differences between the hearing organs of these non-mammals and humans will allow us to manipulate the human ear to behave more like a bird’s or a reptile’s, and allow for human auditory cells to be regenerated. However, there are many differences between the ear of a human and that of a chicken, and narrowing down the search for the most important differences represents a daunting task. Marsupials, like the gray short-tailed opossum, represent an intermediate group, sharing many similarities with both humans and with non-mammals. Of particular interest, supporting cells in the opossum inner ear retain the ability to proliferate well after birth, an indication that these marsupials may possess some regenerative ability. This project aims to determine whether or not opossums are capable of hair cell regeneration or recovery of hearing and to characterize the extent of supporting cell proliferation that occurs both during development and after hearing loss. From this, comparisons will be able to be made between the various model species (e.g. chickens, opossums, humans) to gain a better understanding of which differences between the various hearing organs are essential for regeneration, and which differences are detrimental or unrelated.

Research area: hair cell regeneration

Long term goal of research: The long term goal of the proposed research is to apply what we learn from proliferative and potentially regenerative processes in opossums and incorporate these discoveries into a comparative approach where we may discover key differences that either allow for regeneration to occur in the mammalian cochlea, or, conversely, prevent it. Once these differences are identified, we can begin to develop drugs and/or gene therapies for pre-clinical testing.

Bradley Walters, Ph.D. received his Ph.D. in integrative biology at Lehigh University in Pennsylvania. He is a postdoctoral research fellow in the Department of Developmental Neurobiology at St. Jude Children’s Research Hospital in Tennessee.

+ Yasheng Yuan, Ph.D.

Massachusetts Eye and Ear Infirmary
Regeneration of auditory neurons using stem cells

Hearing loss is usually permanent, and there are no effective interventions available to reverse symptoms by repair of damage. The overall goal of this research is to develop a cell-based therapy to replace auditory neurons. We have shown that neural progenitor cells derived from mouse embryonic stem (ES) cells transplanted into the auditory nerve send out fibers that grow to hair cells and to the cochlear nucleus. In this proposal, we will specifically address this issue through the use of a new mouse ES cell line for tracing the grafted cells and new procedures for detection of the synapses. We will connect the location of the new synapses with auditory function throughout the frequency range of the cochlea. Our study is composed of two related, specific aims. In the first aim we will assess auditory function after cell transplantation. In the second aim we will connect the synaptic counts to functional improvement in specific frequency regions.

Research areas: sensorineural hearing loss, stem cells and regeneration

Long term goal of research: to find biological treatments for hearing loss. Hearing loss has lifelong consequences for individuals and their family. Hearing is mediated by hair cells, which convert sound vibrations into electric signals that are conveyed to the brain through the auditory nerve. Damage to hair cells and the auditory nerve result in hearing loss. Since mammals lack the regenerative capacity to replace hair cells and auditory nerve, hearing loss is usually permanent and there is no effective intervention to reverse the loss of these cells. Our previous work has demonstrated that neurons derived from stem cells can survive and re-innervate the cochlea. The proposed work will investigate a new approach to cell transplantation that will allow us to directly measure cell replacement and its effect on hearing.

Yasheng Yuan, Ph.D is a postdoctoral fellow in the laboratory of Albert Edge, Ph.D., at Massachusetts Eye and Ear Infirmary with an academic appointment at Harvard Medical School. He is an ENT surgeon and an associate professor at Shanghai ENT hospital, Fudan University, China.


+ Keith Bryan, Ph.D.


University of Iowa
Investigating the Role of CaBP1 in KCNQ4 Channel Modulation

KCNQ4 potassium channels play an important role in controlling the responsiveness of auditory hair cells to sound stimulation. Mutation of the gene encoding this channel cause deafness in humans, which is typically due to improper functioning of these channels in the ear. I have identified a novel interaction between Ca2+ binding protein 1 (CaBP1), which is highly expressed in auditory hair cells, and KCNQ4. The goal of this research is to evaluate the functional consequences of this interaction on the cellular localization and biophysical properties of KCNQ4 channels in auditory hair cells.

Research area: fundamental auditory research

Long term goal of research: To understand at the molecular level how hair cells function normally in sound detection and develop novel therapeutic strategies for treating patients with inherited forms of hearing loss.

Bryan earned a M.S. in biochemistry and a Ph.D. in biochemistry from the University of Iowa. He is a postdoctoral fellow in the Department of Molecular Physiology and Biophysics at the University of Iowa.

+ Elizabeth A. Dinces, M.D., M.S.

Albert Einstein College of Medicine
Effects of aging on selective attention in complex, multisource sound environments

Dinces’ basic science research focuses on understanding how the brain processes sounds into meaningful language and includes auditory scene analysis in the elderly, sound intensity processing in children, and development of auditory processing after cochlear implantation. The value of learning the role of attention and understanding the active and passive processes of stream segregation in aging populations will be to help develop therapeutic strategies to improve listening and understanding in noisy sound environments of aging adults.

Research area: fundamental auditory research

Long term goal of research: to explain mechanisms of auditory scene analysis, which is how the auditory system processes sound into meaningful elements, that break down with aging.

Elizabeth Dinces, M.D., M.S. received her B.A. in chemistry from Amherst College in 1987. She went on to earn an M.D. in 1991 and a M.S. in clinical research in 2004, both from the Albert Einstein College of Medicine in the Bronx, N.Y. Dr. Dinces is a Royal Arch Masons award recipient. The Royal Arch Masons support Emerging Research Grants in the area of Central Auditory Processing Disorder (CAPD). See all researchers who have received or are currently receiving funding from the Royal Arch Masons.

+ Sung-Ho Huh, Ph.D.

Washington University
Role of FGF's in Cochlear Sensory Epithelium

Congenital sensorineural hearing loss is one of the most common hereditary disabilities, affecting 1 in 1000 children. Fgf20 null mice have congenital hearing loss associated with loss of sensory cells, and inactivation of both Fgf9 and Fgf20 result in a shortened cochlea. The goal of our research is to understand the cellular and molecular functions of Fgf9 and Fgf20 in inner ear development in vivo. The ultimate goal of this research is to learn how to direct the regeneration of malformed or damaged sensory tissue to restore or improve hearing.

Research area: hair cell regeneration

Long term goal of research: My long term goal is to understand how Fgf signaling regulates the development, maintenance and repair of sensory hair cells and supporting cells in the cochlea. Due to lack of regenerative ability in humans after loss or dammage of hair cells, it is critical to identify signals that can reactivate developmental pathways and thus permit repair and regeneration of the damaged cochlea. Studying the mechanisms that regulate cochlear development will provide valuable clues about molecules that can be tested for regenerative activity and will thus benefit future translational studies aimed at inducing hair cell regeneration in adult humans.

Sung-Ho Huh, Ph.D. received B.S. and M.S. from the Korea University, South Korea, in 1999 and 2001, and a Ph.D. from Washington University in 2009. Since then he has been working as a postdoctoral fellow at Washington University School of Medicine.


+ Kirill Vadimovich Nourski, M.D., Ph.D.

University of Iowa
Temporal Processing in the Human Auditory Cortex

My research area is the function of the auditory cortex—the hearing center in the brain. Some neurosurgical patients undergo an operation in which arrays of electrodes are temporarily implanted in the brain for clinical diagnostic purposes. This provides a unique opportunity to study how the auditory cortex works, by measuring its activity (“brain waves”) directly from the brain. My personal project involves measuring the brain’s responses to the timing information of sounds and the ability of the brain to accurately follow this timing and use this information to build a coherent percept of the environment. I want to understand where and how, specifically, timing cues are processed in the auditory cortex. Patients with cochlear implants are largely dependent on timing and rhythm cues to understand speech and communicate. On the other hand, people who have auditory processing disorders, may be impaired in their ability to process that kind of information. In order to come up with new ways of assisting people with auditory processing disorders, it’s important to understand how the timing and rhythm of speech is usually handled by the brain.

Research area: fundamental auditory research

Long term goal of research: Beyond this study, my long term goal is to better understand how the different areas that comprise the auditory cortex in humans are organized and what specific roles they play in processing information about sounds, particularly, as it relates to perception of speech. Ultimately, this knowledge will contribute to finding new and/or improved solutions for people with hearing loss and auditory processing disorders.

Kirill V. Nourski, M.D., Ph.D. received an M.D. from St.-Petersburg State University, St.-Petersburg, Russia, in 2001, and a Ph.D. in Neuroscience from The University of Iowa, in 2006. Dr. Nourski is a Royal Arch Masons award recipient. The Royal Arch Masons support Emerging Research Grants in the area of Central Auditory Processing Disorder (CAPD). See all researchers who have received or are currently receiving funding from the Royal Arch Masons.

+ Regie Lyn P. Santos-Cortez, M.D., Ph.D.

Baylor College of Medicine
Identification of genes that predispose to chronic otitis media in an indigenous population

The study aims to identify genes predisposing to otitis media by studying gene variants that are identified from a complex pedigree within an indigenous population that has a high prevalence of chronic otitis media. The study population is ideal for gene mapping due to the limited number of founders and marriages only within the indigenous population. Next-generation sequencing will be performed in order to quickly and cost-effectively detect the causal genetic variants for otitis media that fall within the mapped genomic region. The discovery of gene variants predisposing to otitis media opens great possibilities towards increased knowledge of pathophysiology, prediction of the likelihood of otitis media through genetic diagnosis, and development of innovative treatments for otitis media.

Research areas: otitis media, genetics

Long term goal of research: The discovery of genes predisposing to otitis media will lead to increased knowledge of the disease process behind otitis media and development of new diagnostic and treatment strategies for otitis media. The study findings are expected to benefit not only the indigenous population but also otitis media patients from other populations, as the genes that will be found can be followed up in other populations and as new therapies are developed through knowledge of genes predisposing to otitis media.

Regie-Lyn Santos-Cortez, M.D., Ph.D. graduated from the University of the Philippines Manila College of Medicine – Philippine General Hospital for both her medical education and residency in otorhinolaryngology. She studied genetic epidemiology in Erasmus Medical Centre Rotterdam, the Netherlands and did most of her PhD work on the genetics of non-syndromic hearing impairment at the Leal lab at Baylor College of Medicine, Houston, Texas, USA. She is now Assistant Professor at the Center for Statistical Genetics, Department of Molecular and Human Genetics at Baylor.

Santos-Cortez is also the Collette Ramsey Baker Research Award recipient. This research award is made in memory of Hearing Health Foundation’s founder, Collette Ramsey Baker, and is awarded to an Emerging Research Grantee that upholds Collette’s founding principles of pursuing new, innovative aspects of hearing research.

+ Zlatka P. Stojanova, Ph.D.

House Research Institute
Epigenetic Regulation of the Atoh1 gene during development and regeneration of the mammalian organ of Corti

The Atoh1 gene is both necessary and sufficient for auditory hair cell formation during normal development. It is also one of the first genes to be upregulated during regeneration in non-mammalian vertebrates. The project is investigating novel mechanisms of Atoh1 gene regulation that involve epigenetic modifications (not due to changes in DNA sequence). During the 2nd year renewal we will analyze the mechanistic links between the discovered epigenetic state of the Atoh1 gene and the Atoh1 gene expression.

Research areas: hair cell regeneration, genetics

Long term goal of research: To better understand how is Atoh1 gene regulated in order to reverse the failure of hearing regeneration in the mammalian organ of Corti.

Zlatka Stojanova, Ph.D. received a B.S in Biotechnology and a M.S. in Genetic Engineering in her native Bulgaria, and a Ph.D. in Human Genetics from the University of Utah. Currently she is a postdoctoral scientist at the House Research Institute, Los Angeles.



+ Richard S. Tyler, Ph.D.


University of Iowa
Literature review on hyperacusis, recruitment, misophonia, phonophobia, and mechanisms

The funded research will result in a thorough review of the literature, documenting causes, mechanisms, measurement and treatment. It is the intent that the review will provide a comprehensive document that clinicians and researchers will be able to use to understand hyperacusis and to improve current treatment approaches, and to suggest future treatment directions.

Research areas: cochlear implants, tinnitus

Long term goal of research: The long-term goal is to provide a systematic, comprehensive review of the entire field of hyperacusis. By providing such a widespread and comprehensive review of hyperacusis, we should be able to provide the background necessary to direct research to find cures.

Richard Tyler, Ph.D. is a Professor in the Department of Otolaryngology and in the Department of Communication Sciences and Disorders at the University of Iowa. He was trained both as a clinical audiologist and then as a Psychoacoustician. He has served on The National Academies Institute of Medicine Committee on Noise and Military Service: Implications for Hearing Loss and Tinnitus, the World Health Organization Panel on ‘Burden of disease from Environmental noise and Tinnitus and the Committee for Revision of the Veterans Administration Schedule for Rating Disabilities (hearing loss and tinnitus).


+ Andy K. Groves, Ph.D.


Baylor College of Medicine
Development of Biomarkers to Study Strial Development and Degeneration

The sensory hair cells of the cochlea are able to detect sound vibrations. Hair cells need a source of potassium that helps them to convert sound energy into electrical energy that is sent to the brain. Hair cells in our cochlea are bathed in a potassium-rich fluid called endolymph, and the potassium is constantly pumped into the endolymph by a specialized group of cells in the cochlea called the stria vascularis. As humans get older, the stria vascularis can degenerate, and so the “battery” that supplies potassium to the cochlea runs down, and we lose our hearing. The goal of this project is to understand how the stria vascularis develops, and to devise ways of looking at changes in this structure with age.

Research areas: the development and regeneration of the inner ear, stria vascularis development

Long term goal of research: We hope this knowledge may allow us to repair or slow down damage to the cochlea and lessen the effects of age-dependent hearing loss.

Andy Groves, Ph.D. was born in London and studied Natural Sciences at the University of Cambridge. He did his Ph.D. training at the Ludwig Institute for Cancer Research at University College London, where he studied the early development of the nervous system. He moved to California for postdoctoral training at the California Institute of Technology, and it was there that he changed his research focus to study the development and regeneration of the inner ear. Dr. Groves was recruited to Baylor College of Medicine in the summer of 2008.


+ Kevin K. Ohlemiller, Ph.D.


Washington University
Cellular and Genetic Bases of Age-Associated Strial Degeneration and EP decline in NOD congenic mice

The electric currents that run through cochlear sensory cells are largely driven by a specialized cochlear structure called the stria vascularis. The work of the stria requires a lot of energy, so that it is densely vascularized (hence the name). Loss of strial blood vessels is thought to be a common cause of age-related hearing loss. Not everyone shows signs of this kind of pathology, however, so that there must be forms of certain genes carried by some people that act as ‘risk’ genes. People who carry ‘risk’ genes may be more likely to experience loss of strial blood vessels, and ultimately loss of the stria itself. In 2008 we discovered that a particular breed of mice (NOD mice) start out with a normal stria, but then show loss of strial vessels, followed by loss of the stria beginning from both ends of the cochlea and progressing toward the middle. These changes were accompanied by other distinctive anatomic features that may tell us something about the process, or may be unrelated. By crossing these mice with another strain that does not show pathology, we will be able to determine what pathologic features are inherited together (thus caused by the same genes), how many genes are involved, and their approximate locations. Any gene(s) we find may have human counterparts that exert similar effects.

Research areas: auditory physiology/pathophysiology, cell biology of hearing and deafness

Long term goal of research: Finding ‘risk’ genes may not point directly to cures or allow us to predict who will lose their hearing. Nevertheless, identifying the genes, gene networks, and gene products will help pinpoint key reactions that can be tweaked pharmacologically. We are among the first to seek out mouse strains with pathology of the stria vascularis and to use these to uncover genes that promote strial degeneration in mice, and possibly in humans.

Kevin Ohlemiller, Ph.D. received his Bachelor of Science degree in biology from Indiana University, then his PhD in neuroscience from Northwestern University under the mentorship of Dr. Jonathan Siegel. After performing post-doctoral work with Dr. Nobuo Suga at Washington University in Saint Louis, he joined the faculty at the Central Institute for the Deaf, now merged with the Department of Otolaryngology at Washington University School of Medicine.