Five cases are presented of children with rapid onset of sensorineural hearing loss, disequilibrium, or both, who were found at exploratory tympanotomy to have a perilymph fistula. Four of the children had histories suggesting that antecedent barotrauma or physical exertion contributed to the development of the fistula. One child with congenital unilateral craniosynostosis had a residual temporal bone abnormality on the same side as the perilymph fistula. Two children had identifiable anatomic abnormalities in the middle ear. A classification of perilymph fistula is proposed that describes a congenital, an acquired, and a combined type of fistula. Inner ear fluid dynamics and patency of the cochlear aqueduct appear to be important factors in pathogenesis. Children with unexplained fluctuating or sudden onset of sensorineural hearing loss, and children with unexplained disequilibrium or vertigo should be suspected of having a perilymph fistula. The history can be singularly important in raising the suspicion that a perilymph fistula may be present. Although audiometric, vestibular, and radiographic studies can be helpful, there is no way to prove the presence or absence of a fistula without directly viewing the middle ear. Tympanotomy with repair of the fistula does not assure improvement in hearing.