Posterior cranial fossa extension of congenital cholesteatoma in children: Report of two cases

Objectives: To present the results of surgical treatment and long term follow-up of two cases of congenital cholesteatoma in young children extending into the posterior cranial fossa. Method: A retrospective review of 264 children, surgically treated for cholesteatoma of the temporal bone between 1999 and 2008 was performed. The presenting symptoms, tympanic membrane findings, surgical findings, methods, and results were investigated retrospectively. Results: 30 cases satisfied the criteria of congenital cholesteatoma. Two patients presented radiological and surgical evidence of extension of the cholesteatoma in the posterior cranial fossa. Post-operative air-bone gap changes were not significant. No recurrence is observed to date. Conclusion: Congenital cholesteatoma is a different disease entity from acquired cholesteatomas, no previous otorrhea episodes are described and the tympanic membrane appears to be intact; the first symptom is usually hearing loss, but in very young children, one side hearing loss may be misdiagnosed, therefore CC may have severe intracranial complications as onset symptomatology. Pre-operative CT imaging is useful for the evaluation of the extension of the disease and planning the appropriate surgical technique.