Genetics of Hearing Loss

Since 1995, advancements in human genetics have elucidated many of the causes of hearing loss. Currently, over 150 genes are identified as responsible for nonsyndromic sensorineural hearing loss, and more than 200 are linked to syndromic forms (for review, see Hereditary Hearing Loss Homepage, https://hereditaryhearingloss.org). Genetic epidemiology is better understood for congenital hearing loss than for progressive adult-onset forms. It is estimated that three-quarters of early-onset hearing loss has a genetic origin, the remaining causes being acquired (prenatal cytomegalovirus infection, meningitis, sound trauma, temporal bone injuries). This article is an update of the main etiologies in genetic hearing loss.