The otodystrophies: diagnosis and differential diagnosis.

The imaging evaluation of hearing loss is usually focused on inflammatory entities, especially chronic otitis/ cholesteatoma and cerebellopontine angles lesions, particularly acoustic tumors. This discussion concerns several developmental and acquired entities. The developmental entities referred to as the otodystrophies include otosclerosis, Paget's disease, fibrous dysplasia, and osteogenisis imperfecta. Otosyphilis and postradiation temporal bone changes are also included because of the overlapping imaging appearances.