Thirty cases of congenital cholesteatomas of the cerebellopontine angle (CPA) treated over a period of 20 years are reviewed with regard to their clinical features, the pathophysiology of their symptoms, and their management. The predominant symptoms were related to cranial nerves VII and VIII and headaches. Signs and symptoms were divided into those caused by local involvement of the cholesteatoma, increased intracranial pressure, or both. Diagnostic procedures from invasive procedures such as ventriculography to more sophisticated noninvasive procedures including computed tomography and magnetic resonance imaging are discussed. The surgical management of 27 congential cholesteatomas was accomplished via a posterior cranial fossa approach. Total excision of the cholesteatoma was the aim but was carried out in only five (18%) patients because of concern regarding the preservation of nearby neurovascular structures. Partial excision was possible in 22 (82%) patients. To minimize re-formation, the residual cholesteatoma was carefully cauterized with the aid of the operating microscope and bipolar diathermy without damaging surrounding neurovascular structures. Follow-up has ranged from 2 to 15 years, with four (13%) cases of re-formation.
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