Otitis media and congenital perilymphatic fistula as a cause of sensorineural hearing loss in children.

In all infants and children who have progressive, fluctuating or sudden sensorineural hearing loss, the possibility of a congenital perilymphatic fistula should be considered. Factors determined to be highly suggestive of the presence of a congenital perilymphatic fistula as the cause of sensorineural hearing loss or vertigo, or both, include the following: mixed conductive and sensorineural hearing loss; antecedent sudden physical exertion or barotrauma; congenital deformities of the external ear and head; and abnormal findings on computed tomograms of the temporal bone, especially Mondini-like ear dysplasias. In a series of 37 children who had a congenital perilymphatic fistula treated at the Children's Hospital of Pittsburgh, 28 (76%) had had documented otitis media in the past or a history of middle ear disease. This finding should alert the clinician to the possibility of the presence of a congenital perilymphatic fistula when sensorineural hearing loss develops or progresses during an episode of otitis media. Perilymphatic fistula is caused by either congenital ossicular deformities or abnormalities of the labyrinthine windows or coexistence of both conditions. The likelihood of there being no further deterioration in hearing after surgical repair of a perilymphatic fistula is high. Every infant and child with unexplained hearing loss or disequilibrium or both deserves an attempt to uncover the cause at the earliest possible age.