Congenital and genetic sensorineural hearing loss.

One in 750 neonates will have a sensorineural hearing loss of which approximately 40% will be due to a genetic defect. The causes of congenital and genetic sensorineural hearing loss are abundant, and their associations with other diseases are quite diverse. The early detection of hearing loss is of the utmost importance because it can affect development even within the first year of life. A familiarity with the numerous causes of sensorineural hearing loss and their associated abnormalities allows for early detection. Rehabilitation of the hearing loss as well as treatment of the associated organic defects and assistance through genetic counseling is often indicated.