Bilateral acoustic tumors (Neurofibromatosis 2) have now been shown to arise in patients with gene abnormalities separate from those with von Recklinghausen's disease (Neurofibromatosis 1). The management of patients with this disease has long posed a particularly challenging problem for the neurotologist. This retrospective study reviews our series of 38 patients and updates a previous report. Thirty-four patients underwent surgical excision of tumors on one or both sides. Hearing conservation was attempted in 20 with hearing preservation possible in eight. Recent advances in understanding the hereditary etiology of this disease, changing methods for diagnosis, preferred surgical techniques, and guidelines for appropriate patient management are discussed.