Agenda

Deafness is the most common form of sensory impairment in humans and frequently of genetic origin. Using mouse models, we have been investigating the mechanisms by which mutations in genes encoding components of the mechanotransduction machinery of hair cells affect hair cell function. More recently, we have begun to study the mechanisms by which sensory input regulates the wiring of auditory sensory neurons into circuits to transmit sound to the brain and how sensory deficits affect circuit wiring. Our studies demonstrate that genetic defects that affect hair cell function have profound impact on peripheral and central auditory function. These studies are significant for the development of therapeutic approaches to treat deafness because they show that rescue of hair cell defects also has to consider effects on sensory circuitry.