JAMA
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 . . . [Full Text of this Article]