Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report

Journal of Neurosurgery
Jay JagannathanH Jeffrey Kim

Abstract

Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively,...Continue Reading

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Citations

Sep 14, 2011·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Marie Louise Mølgaard PoulsenMarie Luise Bisgaard
Apr 5, 2012·Diagnostic Pathology·Yan-Hua SunMei-Quan Xu
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