Two emerging perilymph fistula "syndromes" in children.

This paper proposes two relatively common "syndromes" seen in children with perilymph fistulas using illustrative case reports. One, a 2 1/2-year-old child with bilaterally symmetric progressive sensorineural hearing loss, was found to have bilateral oval and round window fistulas. The other was a child who presented originally with an unexplained unilateral sensorineural loss. It was only when hearing deteriorated in the opposite ear several years later that a perilymph fistula was suspected and confirmed by tympanotomy. The clinical presentations and other details of these cases diverge widely, but may represent two emerging syndromes of perilymph fistulas in children: 1. a congenital or hereditary predisposition, and the other, 2. sudden hearing loss in the "good" ear of children with an unexplained sensorineural hearing loss in the opposite ear.