Noncongenital hereditary hearing loss in children. Prospective documentation

Younger siblings of children with sensorineural hearing loss of possible hereditary cause underwent interval audiologic examination. Seven siblings (in unrelated families) were found to have progressive sensorineural hearing loss despite early audiograms documenting normal hearing levels for age. Continued testing of these children allowed for early identification and intervention. We advocate regular otolaryngologic and audiologic follow-up even after normal audiologic assessments are made for younger siblings of children with documented sensorineural hearing loss, unless a definite nongenetic origin of the hearing loss in the older child is known. Recessive sensorineural hearing loss with onset in infancy or childhood may present with no antecedent family history and with normal behavioral audiograms early in life.