Hemangioblastoma of the internal acoustic canal in a patient with von Hippel-Lindau disease: a case report and review of the literature.

We report a case of von Hippel-Lindau disease in a 55-year-old woman who presented with an intracanalicular hemangioblastoma and discuss the otologic manifestations of this disease. A review of the literature revealed no previous reports of this entity originating in the internal acoustic canal.