Tumors of the Endolymphatic Sac in von Hippel–Lindau Disease

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Abstract
Tumors of the endolymphatic sac are locally invasive neoplasms arising in the temporal bone that can cause hearing loss, tinnitus, vertigo, aural fullness, and facial-nerve dysfunction. They occur sporadically but are most often associated with von Hippel–Lindau disease; in such instances, they are frequently bilateral. The natural history, mechanisms underlying the early symptoms, anatomical origin of endolymphatic-sac tumors, and optimal timing of their treatment are unknown.