Surgical outcomes in patients with endolymphatic sac tumors

Objective: To determine the surgical outcomes in patients with endolymphatic sac tumors (ELSTs). Study Design: Retrospective review of patients at a referral-based otology-neurotology practice. Methods: A review of the records from the House Ear Clinic revealed 16 patients treated for ELSTs from 1971 to 2002. This article reports the treatment outcomes for the 14 patients for whom clinical data were available. Results: Sensorineural hearing loss, tinnitus, and dizziness were the most common presenting signs and symptoms. Six patients presented with facial weakness, and three patients had symptoms characteristic of Meniere's syndrome. One patient suffered from Von Hippel-Lindau disease. Patients underwent microsurgical removal and were followed for an average of 59.6 months. Patients that presented with normal facial function maintained excellent postoperative function, and hearing was preserved in two patients with small tumors. Two patients suffered persistent, progressive disease despite multiple attempts at microsurgical removal and radiotherapy. Both had incomplete resections of their initial tumors. A third patient developed a small recurrent tumor that was successfully managed by a second attempt at microsurgical removal. Conclusions: Taken together with other reports, these results suggest that ELSTs are best managed by complete surgical resection. This can generally be accomplished with minimal additional morbidity.