Rare cases of temporal bone osteodystrophy (literature review and description of clinical cases).

Otodystrophies are a group of diseases affecting the temporal bone in addition to affecting other bones of the body, resulting in conductive, sensorineural (neurosensory) or mixed hearing loss. Conductive hearing loss occurs due to involvement of the external auditory canal, auditory ossicles or oval window. Sensorineural hearing loss occurs as a result of involvement of the auditory capsule or internal auditory canal. The etiology of otodystrophies can be metabolic, genetic or unknown. The most common form of otodystrophies is otosclerosis. However, in clinical practice there are other, much rarer manifestations of otodystrophies: osteopetrosis, osteogenesis imperfecta, fibrous dysplasia, Paget's disease. Knowledge of such manifestations of the disease, their differential diagnostic features and approaches to treatment are of great clinical importance for a practicing physician. The article presents clinical observations of rare manifestations of otodystrophies of the temporal bone. A review of literature data on the diagnosis and treatment of these diseases is presented.