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Previous Volume 350:2481-2486 June 10, 2004 Number 24 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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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.

We describe three cases of von Hippel–Lindau disease that illustrate the following features of endolymphatic-sac tumors: morbid hearing loss due to a radiologically undetectable microscopic tumor in the endolymphatic sac or duct; initial symptoms . . . [Full Text of this Article]

Case Reports

Patient 1

Patient 2

Patient 3

Methods

Temporal Bones

Tumors

Results

Histologic Features of the Temporal Bones

Analysis of Surgical Specimens

Discussion

Source Information

From the Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke (R.R.L., A.O.V., E.H.O.), the Neuro-Otology Branch, National Institute on Deafness and Other Communication Disorders (H.J.K.), and the Diagnostic Radiology Department, Warren G. Magnuson Clinical Center (J.A.B.), National Institutes of Health, Bethesda, Md.; and the Department of Otolaryngology, Children's Hospital Medical Center, University of Cincinnati, Cincinnati (D.I.C.).

Address reprint requests to Dr. Lonser at the Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bldg. 10, Rm. 5D37, Bethesda, MD 20892, or at lonserr@ninds.nih.gov.

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