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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 ears) 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.

I realize I practiced otology and neuro-otology during a golden era, but I have to admit that I didn't appreciate how important it was until I sat down to outline this article. How fortunate I was to have lived and practiced during these developing years of neuro-otology. How fortunate to have worked with the likes of Howard and William House, James Sheehy, James Crabtree, David Austin, and John Shea. How fortunate to have had the opportunity to teach residents and fellows in association with my private practice. So I envy the young otologist-neuro-otologist and the future you have. You stand on the brink of great discoveries. And like my generation, you stand on the shoulders of giants. Good luck.