Endolymphatic Sac Tumors in von Hippel-Lindau Disease

Endolymphatic sac tumors are benign tumors of the temporal bone, originating from the endolymphatic sac or duct. These tumors are among the least common manifestations of von Hippel-Lindau disease (4–16% incidence). Bilateral endolymphatic sac tumors are pathognomonic for von Hippel-Lindau disease. Audiovestibular symptoms are common, including hearing loss, vertigo, and tinnitus. Diagnosis is largely based on clinical history and imaging findings. No universal screening or surveillance recommendations exist in the current literature. Complete surgical resection is the standard treatment. Radiation and chemotherapy treatment options are alternative treatment options for poor surgical candidates.