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Indian J Otolaryngol Head Neck Surg. 2014 Jan;66(Suppl 1):352-5. doi: 10.1007/s12070-011-0395-3. Epub 2011 Dec 27.

Endolymphatic Sac Tumour: A Neoplastic Cause for Meniere's Syndrome.

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  • 1Madras ENT Research Foundation, No. 1, 1st Cross Street, Off 2nd Main Road, Raja Annamalipuram, Chennai, 600028 India.

Abstract

Patients with intractable vertigo often present a diagnostic dilemma to the treating physician. A wide spectrum of diseases, ranging from those of the labyrinth onto the central nervous system, may present predominantly with vertigo. In some cases, it requires the clinical acumen of an experienced neuro-otologist, to decipher these vertiginous symptoms and arrive at a definitive diagnosis. Meniere's syndrome is one such phenomenon, where the endolymphatic hydrops may be attributable to varied aetiology. We report a case of sporadic (non-syndromic) Endolymphatic Sac Tumor which presented to us, mimicking a classical Meniere's syndrome. We discuss its clinical presentation, diagnostic modalities, operative technique and histo-pathological features. The case is reported along with a review of the world literature on this tumor, highlighting the diagnostic and management protocols advocated for this rare entity. Endolymphatic sac tumor is a rare entity which may masquerade as a classical case of Meniere's syndrome, wherein the triad of symptoms (as in endolymphatic hydrops) may not be alleviated by the usual treatment protocols. Tracing the aetiology of these symptoms, remains the most crucial factor in treating such patients.

KEYWORDS:

Endolymphatic hydrops; Endolymphatic sac tumor (ELST); Vertigo; Von Hippel–Lindau’s disease (VHL)

PMID:
24533415
PMCID:
PMC3918290
DOI:
10.1007/s12070-011-0395-3
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