Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome

Objective: To determine the incidence of bilateral endolymphatic sac tumors in von Hippel-Lindau syndrome and to describe the technique and results of hearing preservation surgery for small endolymphatic sac tumors in a series of patients with von Hippel-Lindau syndrome. Study Design: Analysis of the literature to determine the incidence of bilateral endolymphatic sac tumors and a retrospective case review of hearing preservation surgery for removal of small endolymphatic sac tumors in four patients with von Hippel-Lindau syndrome. Setting: Tertiary care academic medical centers. Patients: Four patients with von Hippel-Lindau syndrome (three with bilateral endolymphatic sac tumors) and progressive sensorineural hearing loss in which preoperative imaging studies revealed in situ or small endolymphatic sac tumors without ipsilateral labyrinthine destruction. Intervention: All four patients had complete surgical excisions of the endolymphatic sac tumor via one of three surgical approaches with the goal of hearing preservation. One patient had bilateral surgery. Main Outcome Measures: Audiometric and radiographic. Results: Nearly one-third (30.2%) of patients with von Hippel-Lindau syndrome and endolymphatic sac tumors have bilateral disease. All four patients (five cars) maintained serviceable hearing postoperatively after surgical excision of the endolymphatic sac tumor via a variety of approach options. Conclusion: The discovery of a small or in situ endolymphatic sac tumor affords the patient the option of surgical removal with hearing preservation. This is critical in the patient with von Hippel-Lindau syndrome who is at risk for bilateral disease and complete bilateral anacusis if tumor growth progresses.