hearing loss

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Cochlear Otosclerosis

History: 30 year old female with a history of hearing loss. The term for this finding is cochlear otosclerosis. Synonyms include cochlear otospongiosis, retrofenestral otosclerosis, and Beethoven Malady. The diagnosis in this case was osteogenesis imperfecta, however, paget disease, fibrous dysplasia, and otosyphillis can cause these findings.