Hemangioblastoma of the Internal Acoustic Canal in a Patient With von Hippel–Lindau Disease

Abstract

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.

Hemangioblastoma of the Cerebellopontine Angle

“…3 One in 4 HMBs is associated with von Hippel Lindau (VHL) disease. 4 A cerebellar HMB is found in 44% to 72% of patients with VHL disease 5 ; however, involvement of the CPA is uncommon. When present within the CPA, HMBs may mimic VSs because the 2 entities have similar magnetic resonance imaging (MRI) characteristics.…”