Pathology of otosclerosis: a review

Otosclerosis is a bone disorder of unknown etiology confined to the otic capsule. Failure of remodeling of newly formed vascular, woven bone (otospongiosis) results in sclerotic bone (otosclerosis) with abnormal osteons. Involvement of the oval window causes conductive hearing loss. Electron microscopic, histochemical, and biochemical studies identify normal cellular and matrix components of otosclerotic bone without providing clues to the abnormal bone formation and resorption. Plastic-embedded, nondecalcified histologic sections with in vivo tetracycline labels permit the study of mineralization rates to separate this disorder from other bone dyscrasias that have similar histopathologic appearances. Characterization of the cells, matrix, and their mediators can yield an understanding of abnormalities that disorder bone.