Audiologic features of the X-linked progressive mixed deafness syndrome with perilymphatic gusher during stapes gusher.

Stapes gusher sometimes occurs at the moment the vestibule is opened. A flood of perilymph can be expected as a complication of stapes surgery in all males affected with the X-linked progressive mixed deafness syndrome. Audiologic features, such as a mixed hearing loss and elicitability of stapedial reflexes, are reported for eight affected males in a large Dutch family.