From deafness genes to hearing mechanisms: harmony and counterpoint

Abstract

The study of hereditary hearing impairments provides a unique opportunity to deal with two objectives simultaneously: (i) identification of the causative genes and the underlying pathogenic process in each form of deafness; and (ii) elucidation of the molecular and cellular mechanisms of hearing. This review highlights the breakthroughs achieved during the past 12 years, with respect to their medical impacts and advances in basic scientific knowledge. To date, this research relies extensively on mouse models to study human forms of deafness. But, can mouse models sustain genetic approaches to study the physiology and pathophysiology of the auditory system and to develop and test drugs?