The otologic manifestations of mucopolysaccharidoses

Patients with mucopolysaccharidoses are frequently hard of hearing. This disorder is often conductive in nature and considered the result of chronic serous otitis media. Such patients frequently undergo myringotomy. A coexisting progressive neurosensory hearing loss in some children is recognized far less often. Examination of temporal bones in such patients suggests arrested development including incomplete pneumatization. Hair cell dysfunction is implicated in the neurosensory component. Close communication among pediatrician, otolaryngologist, and speech and hearing personnel is essential to maximize the communicative skills of these patients.