NBIO Presents: Ulrich Mueller, PhD (Johns Hopkins University)

The Sound of Silence: Mechanotransduction, auditory perception and the molecular pathogenesis of hearing loss

Deafness is the most common form of sensory impairment in humans and frequently of genetic origin. Using mouse models for human deafness, we have identified components of the mechanotransduction machinery of cochlear hair cells, which transforms mechanical stimuli into electrical signals. Our findings demonstrate that the mechanotransduction machinery is an intricate multi-component machinery. 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 mechanotransduction have a profound impact on peripheral and central auditory circuitry. Our studies implicate activity-dependent mechanisms in auditory circuit wiring and maintenance, and provide a molecular entry point to study not only deafness but also other pathological conditions such as tinnitus.