Announcing 2019 Capita Foundation Auditory Research (CFAR) grant award recipients

Prof. Karen Avraham

Tel Aviv University

Project Title: "Gene therapy in a mouse model of human hearing loss DFNB76"

Adeno-associated virus (AAV) has become very popular in the gene therapy field in recent years, and several proof-of-principal studies in the field of hearing loss have demonstrated its potential for treatment of genetic deafness. AAV has major advantages for gene therapy. Most importantly, AAV appears to elicit no immunological response in humans and recombinant AAV is also almost entirely incapable of integration, which enhances its safety. Several synthetic AAVs have been engineered to transduce cells in the inner ear with high efficiency and have been used to demonstrate rescue of auditory and vestibular function in deafness mouse models.  We hope our efforts will lay the groundwork for future development of gene therapy in humans. In addition, we hope this work will contribute to the understanding of the roles of nesprin proteins in cellular processes in general, and specifically in outer hair cell physiology.

The point of no return? These outer hair cells are in excellent form, arranged in three rows, just a day before they start deteriorating due to an absence of the gene SYNE4. Alterations in the DNA sequence of the SYNE4 gene leads to deafness in children and adults. Models are being used to test methods of gene therapy, in order to rescue hearing due to defects in the SYNE4 gene.


Credit: Shahar Taiber, MD-PhD student

Karen Avraham - Genomics of Deafness laboratory, Tel Aviv University

Valeriy Shafiro, Ph.D.

Rush University

Project Title:  "Validation of the Basic Auditory Skills Evaluation (BASE) 

battery for internet home testing of cochlear implant listeners"

Accessible internet-based auditory testing can have a wide reach beyond the typical clinical settings and empower people with cochlear implants to take more active roles in monitoring their progress and rehabilitation. This project will evaluate a comprehensive online battery of tests in three general areas of auditory function: basic spectro-temporal processing, nonlinguistic meaningful sounds, speech perception in quiet and in noise. The results will provide a basis for further developing and implementing easily accessible and affordable online diagnostic tests and online auditory training programs for people with hearing loss.

Joseph C. Toscano, Ph.D.

Villanova University

Project Title:  "Improving speech recognition for listeners with auditory neuropathy"

Our research examines effects of hearing difficulty on speech perception, a problem that affects many adults even if they have normal hearing thresholds. These cases of "hidden hearing loss" can result in problems coding intensity differences at higher sound levels  and, in turn, can disrupt perception of certain acoustic cues used to distinguish speech sounds. This project will investigate a new technique to improve speech recognition by filtering the sound signal to make these intensity differences more salient, compensating for deficits associated with hidden hearing loss.

Saad Bhamla, Ph.D.

Georgia Tech University

Project Title: “The LoCHAid: A low-cost, open-source hearing aid for Age Related Hearing Loss”

Victor Wong, Ph.D.

Burke Neurological Institute

Project Title: "TARGETING α-TUBULIN AND MIRO ACETYLATION TO 

ENHANCE NEURITE OUTGROWTH IN SPIRAL GANGLION NEURONS"

  My long-standing research interests lie in identifying molecular mechanisms for axonal regeneration after nervous system disease or injury. Axons in the adult nervous systems have little capacity to regenerate after injury. Although hearing regenerative capacity have been documented in avian and amphibian species, the reversal of hearing loss in mammals has been a persistent challenge. Although most therapeutic strategies have focused on the replacement of hair cells (HCs); there also is a need to address the subsequent degeneration of the innervating spiral ganglion neuron (SGN) for functional recovery of hearing. Moreover, very little is known about how to promote SGN neurite growth. There is, therefore, a critical and unmet need to determine how to enhance SGN peripheral neurite growth. The focus of my research is to examine how post-translational modifications can change the fate of SGN neurite growth. Specifically, the main objective is to understand how acetylation of microtubules, which affect microtubule stability, and acetylation of Miro, a mitochondrial transport protein, can impact SGN neurite growth under pathological conditions. Ultimately, I wish to capitalize these biological processes (i.e., microtubule dynamics and axonal transport) into therapeutic strategies to encourage neural regeneration and repair.

Dr. Amineh Koravand

University of Ottawa

Project Title:  “Investigating the Temporal Resolution Capacity in School Aged Children via Neurophysiological Measurement. Pilot Study.”

Prof. Koravand's research deals with the relationship between the peripheral and central auditory systems in children. Her goal is to develop neurophysiological measures (biological markers) to assess the central auditory functions of children during early childhood, to prevent disorders while brain plasticity is still significant.