This study underscores the importance of considering a perilymph fistula, an abnormal communication between the inner and middle ear, in any child with a progressive sensorineural hearing loss. A concomitant complaint of intermittent dizziness or observed spells of imbalance (56%) is another indication of the disease. Six of 16 patients (37.5%) with proven fistulas were less than 2 years of age at the onset of symptoms. Unless the fistula is identified and repaired by grafting, the ear may become totally deaf. This would be disastrous in the presence of bilateral fistulas, which occur commonly in children (56% of patients had symptoms of bilateral disease). Our experience in recognizing and treating fistulas and thereby saving and restoring hearing is described.

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