Cholesteatoma of the cerebellopontine angle

Congenital cholesteatoma is the third most common tumor found in the cerebellopontine angle. It must be differentiated from acoustic neuromas, meningiomas, metastatic tumors, arachnoid cysts and lipomas. Symptoms include hemifacial spasm, progressive facial paralysis, hearing loss, tinnitus, vertigo, pain and otorrhea. Radiologic and magnetic resonance imaging frequently can be useful to establish a preoperative diagnosis. The treatment of choice is total removal of the lesion. Complete removal with preservation of normal structures is the most difficult and technically exacting procedure performed by the neurotologic surgeon. The clinical features and results from a series of 19 cases, nine of which extended into the cerebellopontine angle, are discussed.