Pathophysiology of otosclerosis

Objective: To review current knowledge of the pathophysiology of otoscleros is and to review hypotheses for the amelioration of this disease. Data Sources: Review of the literature and experimental observations by the authors. Conclusions: Otosclerosis is a localized disease of bone remodeling within the otic capsule of the human temporal bone. Unlike other similar bone dise ases, it does not occur outside of the temporal bone. These lesions seem to begin by resorption of stable otic capsule bone in adults, followed by a r eparative phase with bone deposition. There are clearly genetic factors tha t lead to this disease, but measles virus infection and autoimmunity also m ay play contributing roles. Surgical correction of the conductive hearing l oss is highly effective, but nonsurgical intervention has not yet been show n to prevent or slow the disease. Of the factors that may inhibit this proc ess, fluorides, cytokine inhibitors, and bisphosphonates, third-generation bisphosphonates appear to hold the most promise.