Otopathology in cranial facial dysostosis

Thirty patients with Crouzon's disease were evaluated longitudinally for type, incidence, and progression of otopathologic changes. The spectrum of otopathologic manifestations included otitis media with effusion, tympanic membrane atelectasis, tympanic membrane perforation, cholesteatoma, and grade I microtia. The frequency of ear disease progressed from 37% during infancy to 62% in older patients. The degree and type of hearing loss were also analyzed. Radiographic cephalometric measurements of the nasopharynx and temporal bone illustrated growth disturbances of the nasopharyngeal dimensions that worsened with increasing age. Our findings suggest that patients with Crouzon's disease are at risk from birth for otitis media with effusion and its sequelae and that the consequent incidence and severity of otopathologic changes increase with age.