Explosive progress is being made in genetic studies of hearing and deafness from the clinical and basic research perspectives. Greater than half of hearing loss is estimated to have a genetic basis. Recent studies of hearing and deafness have identified a dozen genes that cause nonsyndromic hearing disorders. Deafness can be inherited in an autosomal recessive, autosomal dominant, X-linked, or mitochondrial manner. Mutations in one gene, connexin 26 (encoding the gap junction protein beta 2), may be responsible for half of all autosomal recessive nonsyndromic deafness. With new mandates for hearing screening programs for newborns in many states, for the first time, the new information on the genetics of hearing loss can be used to diagnose the cause of hearing loss in some children and to understand better the molecular biology of hearing.