Four grants were awarded for research that will increase our understanding of the causes, diagnosis, and treatment of CAPD, 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.
+ Joseph H. Bochner, Ph.D.
Rochester Institute of Technology
Auditory Experience, Critical Periods, and the Development of Categorical Perception in Cochlear Implant Users: A Preliminary Investigation
My project will investigate the role of age on the success of cochlear implantation and auditory experience on the development of perceptual (phoneme) categories in prelingually deaf cochlear implant users. The research will demonstrate the degree to which these cochlear implant users can categorize speech sounds, which will improve our understanding of speech perception and the effects of early auditory deprivation on the overall success of cochlear implantation.
Long-Term Goal: To understand the impact of sensorineural (peripheral) hearing loss may extend to central auditory processing when the onset of hearing loss occurs at birth or within the first two to three years of life. This project will study the nature and extent of this impact on the development of categorical speech perceptions in prelingually deaf cochlear implant users. Findings from our research will inform the delivery and development of auditory training programs within the context of pediatric audiology and auditory rehabilitation, as well as the provision of amplification devices to young children.
Dr. Bochner is a professor and department chair at the National Technical Institute for the Deaf at Rochester Institute of Technology. Bochner studied language and audition at the University of Wisconsin, receiving his Ph.D. in in 1983. He has been involved in the language sciences, deafness, and higher education for four decades, and conducted research on the acquisition of language and literacy skills, speech perception and production, and American Sign Language.
+ Angela Yarnell Bonino, Ph.D., CCC-A
University of Colorado - Boulder
Toddlers’ and preschoolers’ ability to hear speech in noise: Assessing performance with a two-interval, observer-based procedure
Children require access to acoustic information in order to develop speech and language. However, this information is often degraded because of competing sounds in the environment. While it is clear that children’s ability to listen in noise substantially improve between infancy and entering school, we do not know how and when this process unfolds during the intervening years.
The objective of this project is to develop a reliable behavioral method for measuring speech perception in noise for toddlers and preschoolers. This approach will build upon a recently developed testing method, in which a child’s behavior is judged by an experimenter using a two-interval, two-alternative testing paradigm . The children’s response to the stimulus is further shaped by training them to perform a conditioned play-based response to the sound. The proposed research will test the hypotheses that reliable data can be collected from toddlers and preschoolers and that speech-in-noise abilities improve dramatically during this time period. Results from this project will provide us information on how typical auditory development unfolds during the toddler and preschooler years, which may advance our understanding of the potential underpinnings of auditory processing disorders and the effects of hearing loss.
Learn more about our research by watching this video.
Long-Term Goal: To examine toddlers’ and preschoolers’ auditory functioning with stimuli that are believed to be highly related to speech and language abilities. A second long-term goal is then to translate this research to the clinical setting by developing behavioral measures that can be used by audiologists to assess children’s auditory abilities, including early diagnosis not only of hearing loss but also central auditory processing disorder and other auditory conditions. This research will make progress toward this goal by establishing a reliable behavioral method for testing speech-in-noise abilities of toddlers and preschoolers.
Angela Yarnell Bonino, Ph.D., is an assistant professor in the Department of Speech, Language, and Hearing Sciences at the University of Colorado Boulder. She completed her clinical training in audiology at Vanderbilt University and her Ph.D. and postdoctoral training at the University of North Carolina at Chapel Hill.
+ INYONG CHOI, PH.D.
University of Iowa
Neural correlates of selective listening deficits in a multiple-speaker environment
Choi’s lab studies how human brains understand speech in noisy everyday settings, how central brain functions are affected by hearing loss, and how we can improve hearing-related brain functions with training.
This study will investigate several key neural processing systems required for successful speech communication in noisy social settings, and how neural processing deteriorates in listeners with degraded hearing ability. As such the project involves basic neuroscientific research of the central auditory system and translational research on hearing rehabilitation techniques.
Long-term goal: To develop clinical tests to identify specific hearing deficits that are currently undiagnosed and raise awareness of such “hidden” hearing loss.
Inyong Choi is an assistant professor in the Department of communication sciences and disorders at the University of Iowa. He received a Ph.D. in electrical engineering from Seoul National University, South Korea, with focus on acoustics and psychoacoustics. After industry research at the Samsung R&D Center and postdoctoral studies in the field of auditory neuroscience and neuroimaging at Boston University, he started his lab at the University of Iowa in August 2015.
+ CHRISTINA REUTERSKIÖLD, PH.D.
New York University
Rhyme Awareness in Children with Cochlear Implants: Investigating the Effect of a Degraded Auditory System on Auditory Processing, Language, and Literacy Development
Successful literacy is critical for a child’s development. Decoding written words is mostly dependent on the child’s processing of speech sounds, requiring a certain level of awareness of speech sounds and words in order to develop literacy skills. If the benefits of early cochlear implantation support the development of central auditory processing skills and phonological awareness, children with cochlear implants (CIs) would be expected to acquire phonological awareness skills comparable to children with typical hearing.
However, past research has generated conflicting results on this topic, which this project will attempt to remedy through investigating rhyme recognition skills and vocabulary acquisition in children who received CIs early in life.We will also shed light on the importance of central auditory processing during a child’s first years of life for developing strong literacy skills.
Long-Term Goal: To better understand the implications of early auditory deprivation on auditory processing, language, and literacy learning in children, which will ultimately lead to improvements in targeted intervention and educational approaches for these children.
Dr. Christina Reuterskiöld received her Ph.D. in Medical Science with a concentration in communication sciences and disorders from Lund University in Sweden. She holds an M.Sc. in speech-language pathology from Boston University and from Lund University and is certified as a speech-language Pathologist by the American Speech-Language-Hearing Association and the Swedish Board of Social Care. She is currently an associate professor and the chair of the department of communicative sciences and Disorders at New York University.
HHF awarded three grants for the best overall hearing research proposals. These grants were generously supported by the board of HHF as well as supporters who designated their gifts to fund the most promising hearing research.
+ Renee Banakis Hartl, M.D., Au.D.
University of Colorado, Denver
Effect of deafness duration on the efficacy of cochlear implants for single sided deafness
One of the large challenges in attempting to predict outcomes of cochlear implantation is due to the diverse clinical characteristics of implant candidates. Our initial studies have worked to isolate the effect of one variable (duration of deafness) in a specific etiology (single-sided deafness) on implant efficacy. By studying auditory neurophysiological responses to cochlear implant stimulation in an animal model for single-sided deafness, we can investigate objective performance and changes in brainstem physiology.
Long-Term Goal:To determine the parameters in patients that may improve outcomes for cochlear implant use. Using the information obtained from this animal model, we can begin to investigate the factors that most closely mimic normal physiology, helping to predict which patients may expect to see the best outcome from implantation.
Renee Banakis Hartl, M.D., Au.D., is a resident in the department of otolaryngology at the University of Colorado, Denver. She received both her doctorate degree in audiology and her medical degree from Northwestern University.
+ Michael Roberts, Ph.D.
University of Michigan
Cellular and synaptic basis of binaural gain control through the commissure of the inferior colliculus
Deficits in binaural hearing make it difficult for users of cochlear implants and hearing aids to localize sounds and follow speech in everyday situations. One of the most important sites for binaural computations is the inferior colliculus (IC). Located in the auditory midbrain, the IC is the hub of the central auditory system, receiving most of the ascending output of the auditory brainstem and much of the descending output of the auditory cortex. The left and right lobes of the IC communicate with each other through a massive connection called the commissure. Recent data from in vivo recordings show that commissural projections shape how IC neurons encode sound location. This suggests that important binaural interactions arise through the IC commissure, but the cellular and synaptic basis of these interactions are largely unknown. Understanding these interactions will provide foundational knowledge to guide future efforts to restore binaural hearing.
Long-term goal: To understand the fundamental mechanisms for binaural processing in the auditory midbrain and how these mechanisms are affected by hearing loss, hearing aids, and cochlear implants. To use knowledge of these mechanisms to guide advances in the design of auditory prostheses and improve the ability of device users to understand speech in noisy environments.
Michael Roberts, Ph.D., is an assistant professor at the Kresge Hearing Research Institute at the University of Michigan.After receiving his doctorate cell and molecular biology at the University of Texas at Austin, he then completed postdoctoral fellowships at Oregon Health and Science University and the University of Texas at Austin. Roberts’ 2017 ERG award is generously funded by The Barbara Epstein Foundation Inc.
+ Xiaodong Tan, Ph.D.
Oto-Protection of Honokiol Against Cisplatin-Induced Ototoxicity
Cisplatin is a common chemotherapy medication that known to be ototoxic (damaging to hearing), but most proposed drugs to counteract this side effect compromise the antitumor effects of cisplatin. Honokiol is an antitumor agent derived from the magnolia plant that has been shown to have synergistic effects with cisplatin in cancer treatment because it activates an enzyme that protects healthy cells and suppresses tumor cells. As a result, honokiol may have a strong protective effect for cochlear hair cells. This study will investigate the hearing protective properties of honokiol using tissue cultures in the lab as well as through direct drug administration in an animal model.
Long-Term Goal:To develop a new therapeutic approach for chemotherapy that includes hearing protection by reducing or eliminating the ototoxicity of the common chemotherapy drug cisplatin, and ultimately to determine whether honokiol may be effective for noise-induced or age-related hearing loss.
Xiaodong Tan, Ph.D., is a research assistant professor in the department of otolaryngology–head and neck surgery at Northwestern University.Tan received his Ph.D. in biomedical sciences from Creighton University in Nebraska and completed postdoctoral research at the University of Wisconsin-Madison and Northwestern University.
Two grants were awarded focused on 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. These grants were funded by Hyperacusis Research.
+ Jennifer Resnik, Ph.D.
Massachusetts Eye and Ear, Harvard Medical School
Homeostatic modifications in cortical GABA circuits enable states of hyperexcitability and reduced sound level tolerance after auditory nerve degeneration
Sensorineural hearing loss due to noise exposure, aging, ototoxic drugs, or certain diseases reduce the neural activity transmitted from the cochlea to the central auditory system. These types of hearing loss often give rise to hyperacusis, an auditory hypersensitivity disorder in which low- to moderate-intensity sounds are perceived as intolerably loud or even painful. Previously thought as originating in the damaged ear, hyperacusis is emerging as a complex disorder. While it can be triggered by a peripheral injury, it develops from a maladaptation of the central auditory system to the peripheral dysfunction. My research will test the hypothesis that the recovery of sound detection and speech comprehension, may cause an overcompensation that leads to an increase in sound sensitivity and reduced tolerance of moderately loud sounds.
This hypothesis will be tested using a combination of chronic single unit recordings, operant behavioral methods and optogenetic interrogation of specific sub-classes of cortical interneurons. By understanding how brain plasticity is modulated, we will gain deeper insight into the neuronal mechanism underlying aberrant sound processing and its potential reversal.
Long-Term Goal:To better understand the paradoxical role of central auditory system plasticity as both the cause of—and treatment for—the perceptual consequences of hearing loss. A major step to reach this goal is to understand the compensatory mechanisms, following cochlear damage, that allow for basic sound recovery while potentially introducing hypersensitivity and causing chronic sensory impairments such as hyperacusis.
Jennifer Resnik, Ph.D., received a doctorate in neuroscience from the Weizmann Institute of Science in Israel. She is currently a postdoctoral research fellow at Massachusetts Eye and Ear, Harvard Medical School, in the lab of Daniel B. Polley, Ph.D, studying the mechanisms and clinical implications of auditory brain plasticity.
+ Senthilvelan Manohar, Ph.D.,
University at Buffalo
Behavioral Model of Loudness Intolerance
High-level noise causes discomfort for typical-hearing individuals. However, following cochlear damage, even moderate-level noise can become intolerable and painful, a condition known as hyperacusis.
One of the critical requirements for understanding and finding a cure for hyperacusis is the development of animal models. I have developed two new animal behavior models to study the pain and annoyance components of hyperacusis. The Active Sound Avoidance Paradigm (ASAP) uses a mouse’s innate aversion to a light open area and preference for a dark enclosed box. In the presence of intense noise, the animal shifts its preference to the light area. The Auditory Nociception Test (ANT) is based on a traditional pain threshold assessment. Although animals show an elevated pain threshold in the presence of 90 and 100 dB, at 110 and 115 dB they show a reduced pain tolerance. Using these two tests together will allow me to assess emotional reactions to sound as well as the neural interactions between auditory perception and pain sensation.
Long-Term Goal: To develop and establish a reliable behavioral model to understand the pain and annoyance aspects of hyperacusis and touse these behavioral models to identify the neural and molecular mechanisms underlying hyperacusis and tinnitus.
Senthilvelan Manohar, Ph.D., received a doctorate in stress physiology at Madras University, India. As a postdoctoral fellow at the University at Buffalo, he studies hyperacusis, noise-induced pain, and sound aversion.
One grant was awarded for innovative research focused on congenital and acquired childhood hearing loss and its etiology, assessment, diagnosis and treatment. This grant was generously supported by our partner The Children's Hearing Institute.
+ Oscar Diaz-Horta, Ph.D.
University of Miami
The role of FAM65B in the regulation of post-translational modifications of auditory hair cell proteins
Recent genetic studies have identified the FAM65B protein as an important molecule for hearing. In this study we will search for inner ear hair cell proteins interacting with FAM65B in order to further delineate FAM65B’s function. We will focus on FAM65B’s role in the modification of its partner proteins. These studies will help characterize molecular aspects of hearing and how hearing loss occurs when they are disrupted.
Long-Term Goal: To develop novel strategies for protection of hearing and restoration of hearing loss by delineating molecular mechanisms that underlie hair cell morphogenesis, function, and maintenance.
Oscar Diaz-Horta, Ph.D., is an assistant scientist in the department of human genetics at the University of Miami. Diaz-Horta received his Ph.D. in biomedical sciences from the University of Havana, Cuba, and completed postdoctoral research at the University of Miami.
One grant was awarded for innovative research that will increase our understanding of the inner ear and balance disorder Ménière’s disease. This grant was generously supported by The Estate of Howard F. Schum.
+ Ngoc-Nhi Luu, M.D., Dr. med
Massachusetts Eye and Ear, Harvard Medical School
Characterization of endolymphatic sac anatomy in early and late onset Ménière's disease: a clinical radiologic study
Ménière’s disease is an inner ear disease that affects the vestibular organs of the inner ear and presents with various symptoms such as vertigo, hearing loss, and tinnitus. There is no known cause, but it is believed that Ménière’s can be associated with an abnormal accumulation of fluid in the inner ear, called endolymphatic hydrops, which is produced in an appendix to the vestibular system, called the endolymphatic sac. Currently, diagnosis of Ménière’s is entirely based on clinical symptoms, and since these can vary greatly in patients and even mimic other diseases, it has made identifying Ménière’s difficult, delaying treatment of symptoms. Previously, using radiologic imaging, we had found striking differences in the vestibular aqueduct, a bony canal that the sac traverses, among those with either early or late onset Ménière’s. This project is to confirm whether the shape and course of the vestibular aqueduct can act as a reliable biomarker to identify distinct types of Ménière’s in patients.
Long-Term Goal: To diagnose Ménière’s more quickly and to develop a novel classification for early and late onset Ménière’s. This may lead to future research focusing on developmental differences in early and late Ménière’s, ultimately leading to better treatments for the range of Ménière’s patients.
Ngoc-Nhi Luu, M.D., Dr. Med, received her medical degree at the University of Tuebingen, Germany, followed by a doctoral degree from the Hearing Research Centre Tuebingen, working on an in vitro model for drug screening procedures. For her postdoctoral fellowship she is currently working on inner ear pathologies and inner ear regeneration in the lab of Albert Edge, Ph.D., at the Eaton Peabody Laboratory at Massachusetts Eye and Ear Infirmary, Harvard Medical School.
One grant was awarded for innovative research tthat will increase our understanding of strial atrophy and/or development of the stria. This grant was funded by a generous family foundation with an interest in funding Strial Atrophy research.
+ Sandeep Sheth, Ph.D.
Southern Illinois University School of Medicine
Cisplatin-induced oxidative stress down-regulates strial Na+/K+-ATPase and endocochlear potential
Cisplatin is a widely used chemotherapy treatment for various solid tumors. Unfortunately, its use sometimes results in permanent hearing loss,. Understanding the pathophysiology of cisplatin ototoxicity (toxicity to the ear) is crucial for the development of novel treatments to combat this serious side effect.
Preliminary studies from our lab suggest that cisplatin appears to reduce the sodium/potassium activity of the cochlear fluid maintained by the stria vascularis, an important tissue in the inner ear, which leads to hearing damage. However, this suppressive effect by cisplatin may be restored through epigallocatechin gallate (EGCG), a green tea extract that is an antioxidant with anti-inflammatory properties. This project aims to investigate the potential of EGCG in the treatment of cisplatin-induced hearing loss.
Long Term Goal:To better understand cisplatin ototoxicity at the molecular and cellular level in auditory organs including the stria vascularis, and how these changes trigger strial atrophy leading to hearing loss; and to eventually develop alternatives to ototoxic chemotherapy drugs or drugs to counteract the ototoxicity of chemotherapy treatments.
Sandeep Sheth, Ph.D., received his doctorate in pharmacology at Southern Illinois University School of Medicine, where he is now a postdoctoral fellow.
One grant weas awarded for innovative research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus. Dr. Balmer received the Les Paul Foundation Award for Tinnitus Research.
+ Timothy Balmer, Ph.D.
Oregon Health & Science University
Chronic transmitter exposure in excitatory neurons of the cochlear nucleus generates persistent excitation and could underlie tinnitus
The dorsal cochlear nucleus in the brainstem receives not only auditory signals directly from the ear but also multisensory input from other areas of the brain. However, the sources of these inputs are unclear. We do know the inputs are processed through unipolar brush cells (UBC), a type of nerve cell in the cochlear nucleus that amplifies signals. This cell derives its name from its single paintbrush-like dendrite, which shows persistent excitation due to chronic neurotransmitter exposure. My project is to investigate whether problems with the multisensory inputs or with the chronic neurotransmitter at the UBC synapse lead to hyperactivity of the cochlear nucleus, which is associated with tinnitus.
Long term goal: To investigate whether chronic transmitter exposure in nerve cells of the cochlear nucleus may be a cause of tinnitus, which eventually may lead to clinical tinnitus treatments.
Timothy Balmer, Ph.D., is a postdoctoral fellow at Oregon Hearing Research Center at Oregon Health & Science University. Balmer received his Ph.D. in neuroscience from Georgia State University working on the development and plasticity of visual circuits with Dr. Sarah Pallas.
One grant was awarded for research to increase our understanding of the mechanisms, causes, diagnosis, and treatments of Usher syndrome, the most common cause of combined blindness and deafness. This grant was generously supported by funders who designated their gifts to fund Usher's research and the board of HHF.
+ Clive Morgan, Ph.D.
Oregon Health & Science University
Characterization of USH1F Macromolecular Complexes
Usher syndrome (USH) is an autosomal recessive disorder characterized by sensorineural hearing loss, retinitis pigmentosa, and variable vestibular dysfunction. Usher syndrome type I (USH1) is the most severe form of the disease. Most USH1 genes have been identified, including myosin VIIa (USH1B), harmonin (USH1C), cadherin 23 (USH1D), protocadherin 15 (USH1F), SANS (USH1G), and CIB2 (USH1J). In the Barr-Gillespie Lab I have developed a new hair bundle isolation strategy to facilitate the separation of individual protein complexes from the hair cell membrane.
My research will study the three main USH1F isoforms (PCDH15-CD1, -CD2, and -CD3). We will make isoform-specific monoclonal antibodies, and use these to isolate protein complexes from the chick and mouse ear. We aim to decipher the molecular pathways leading to the differing localization and functional differences in the key USH1F isoforms, which will help to understand the differing prognoses for individuals with mutations in the PCDH15 gene.
Long-Term Goal: To better understand the molecular architecture of the hair bundle, and eventually to examine mechanotransduction complexes (that convert sound to signals) during the development of the hair cell bundle and to compare complexes from auditory and vestibular organs. I am also utilizing the protein material generated during this project to examine other protein complexes in hair bundles and elsewhere in the inner ear. Information from these studies will help decipher the molecular basis for genetic abnormalities leading to deafness, and help to better understand the functioning of the auditory and vestibular system.
Clive Morgan, Ph.D., received his doctorate in biochemistry from University College London. After completing postdoctoral research there and at the University of Manchester in the U.K., he is currently at Oregon Health & Science University with Peter Barr-Gillespie, Ph.D.