A 12-year-old female had iritis, episcleritis, papilledema and vestibuloauditory dysfunction. The diagnosis was atypical Cogan's syndrome as described by Cody and Haynes.
The initial symptoms were high fever and injected bulbar conjunctiva.
Attack of vertigo, as in Ménière's disease, appeared 2 months after the onset. The results of equilibrium and glycerol tests, and electrocochleography suggested endolymphatic hydrops in the inner ear.
Within 4 weeks after the initiation of steroid therapy, hearing improved and ocular symptoms disappeared without papilledema. At the time of discharge, she had no ataxia, but bilateral CP was present in the caloric test.
We considered that the disorder of the inner ear in Cogan's syndrome is hydrops due to vascular insufficiency.
