Hereditary hearing loss: From human mutation to mechanism

► Gene discovery for deafness facilitates genetic counseling. ► Cellular mechanisms leading to deafness elucidated by gene discovery. ► Actin-bundling protein TRIOBP is essential for stereocilia function in the inner ear. ► An inverted-duplication of TJP2 leads to DFNA51 dominant hearing loss. ► Mutations in microRNA-96 lead to DFNA50 dominant hearing loss.