Hearing and otopathology in Apert syndrome

Otologic and audiologic records on patients wit phenotypic characteristics of Apert syndrome were reviewed. Eustachian tube function was evaluated in five patients. The otologic findings indicate that patients with Apert syndrome have serous otitis media and its sequelae develop during infancy, with persistence into adulthood. This situation is analogous to cleft palate and brings to light another form of probable congenital Eustachian tube dysfunction. Stapedial footplate fixation was noted in one patient, and a dehiscent jugular bulb was seen in two others. The middle ear disease contributes to auditory sensory deprivation in the form of a persistent conductive hearing loss.