Abstract: Series 112, Lecture 1

The Harvey Lectures Series 112 (2016—2017)

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Lecture #1: Thursday, October 20, 2016 — Watch Video of Lecture

Understanding Hearing Mechanisms in Light of Genetic Disorders of this Sensory System: From Sound Detection to Auditory Perception

Christine Petit, MD, PhD

Christine Petit, MD, PhD

Professor & Chair of Genetics and Cellular Physiology at College de France
Professor at Institut Pasteur

College de France & Institut Pasteur

Paris, France

Dr Petit's Website

Hearing is unique among the senses in its increasing contribution to communication during evolution, culminating in the development of language and music by humans. The molecular mechanisms underlying the development and functioning of the auditory system remained unknown until the start of the 1990s. We developed a genetic approach based on the identification of deafness genes in humans, and then carried out multidisciplinary studies, mostly in genetically engineered mouse mutants, to obtain insight into the operation of this sensory system and the molecular networks underlying this functioning. This lecture will describe the progress we have made, on both aspects, towards understanding sound processing by the auditory sensory cells, the hair cells, and, in particular, the way in which their sensory antennae, the hair bundles, work to mediate the interplay between their various functions. It will show how this research has, in parallel, revolutionized our knowledge of sensorineural deafness. Based on recent results, a new focus is emerging, highlighting the molecular mechanisms of auditory perception and revealing an overlooked side of inherited deafness that should lead to a reappraisal of therapies for hearing impairment.