Genetic Hearing Loss

Excerpt

Hearing loss is the impairment of auditory function, which can have significant long-term consequences on social and language development. It can develop prelingually (before the acquisition of speech/language) or post-lingually (after the acquisition of speech/language). Hearing loss can be classified as conductive hearing loss (CHL), caused by the reduction of sound transmission through the external or middle ear to the inner ear, and sensorineural hearing loss (SNHL), which is caused by dysfunction of the inner ear or auditory nerve. Mixed hearing loss features both a conductive and sensorineural component. Sensorineural hearing losses can be categorized into acquired (e.g., noise-induced) and inherited (e.g., genetic). This article will focus on the genetic mechanisms, diagnosis, and management of genetic SNHL.