The vestibular aqueduct: site of origin of endolymphatic sac tumors.

Object: Although endolymphatic sac tumors (ELSTs) frequently destroy the posterior petrous bone and cause hearing loss, the anatomical origin of these neoplasms is unknown. To determine the precise topographic origin of ELSTs, the authors analyzed the imaging, operative, and pathological findings in patients with von Hippel-Lindau disease (VHL) and ELSTs.

Methods: Consecutive VHL patients with small (
Results: Ten consecutive VHL patients (6 male and 4 female) with 10 small ELSTs (
Conclusions: ELSTs originate from endolymphatic epithelium within the vestibular aqueduct. High-resolution imaging through the region of the vestibular aqueduct is essential for diagnosis. Surgical exploration of the endolymphatic duct and sac is required for complete resection.