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September 2007

Endolymphatic Sac Tumors in von Hippel-Lindau Disease

Author Affiliations

Copyright 2007 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2007

Arch Otolaryngol Head Neck Surg. 2007;133(9):857. doi:10.1001/archotol.133.9.857


Mechanisms of Morbid Hearing Loss Associated With Tumors of the Endolymphatic Sac in von Hippel-Lindau Disease 

John A. Butman, MD, PhD; H. Jeffrey Kim, MD; Martin Baggenstos, MD; Joshua M. Ammerman, MD; James Dambrosia, PhD; Athos Patsalides, MD; Nicholas J. Patronas, MD; Edward H. Oldfield, MD; Russell R. Lonser, MD

Context:  Endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and cause irreversible sensorineural hearing loss (SNHL) and vestibulopathy. The underlying mechanisms of audiovestibular morbidity remain unclear, and optimal timing of treatment is not known.

Objective:  To define the underlying audiovestibular pathophysiologic mechanisms associated with ELSTs.

Design, Setting, and Patients:  Prospective and serial evaluation of patients with von Hippel-Lindau disease and ELSTs at the National Institutes of Health between May 1990 and December 2006.

Main Outcome Measures:  Clinical findings and audiologic data were correlated with serial magnetic resonance imaging and computed tomographic imaging studies to determine the mechanisms underlying audiovestibular dysfunction.

Results:  Thirty-five patients with von Hippel-Lindau disease and ELSTs in 38 ears (3 bilateral ELSTs) were identified. Tumor invasion of the otic capsule was associated with larger tumors (P = .01) and occurred in 7 ears (18%) causing SNHL (100%). No evidence of otic capsule invasion was present in the remaining 31 ears (82%). Sensorineural hearing loss developed in 27 of these 31 ears (87%) either suddenly (14 ears [52%]) or gradually (13 ears [48%]), and 4 ears had normal hearing. Intralabyrinthine hemorrhage was found in 11 of 14 ears with sudden SNHL (79%; P < .001) but occurred in none of the 17 ears with gradual SNHL or normal hearing. Tumor size was not related to SNHL (P = .23) or vestibulopathy (P = .83).

Conclusions:  Vestibulopathy and ELST-associated SNHL may occur suddenly due to tumor-associated intralabyrinthine hemorrhage or insidiously, consistent with endolymphatic hydrops. Both of these pathophysiologic mechanisms occur with small tumors that are not associated with otic capsule invasion.