Early proactive management of vestibular schwannomas in neurofibromatosis type 2

OBJECTIVE: The treatment of patients with neurofibromatosis Type 2 has alwa ys been challenging for neurosurgeons and neurotologists. Guidelines for ap propriate management of this devastating disease are controversial. METHODS: A retrospective study of 28 patients with neurofibromatosis Type 2 who underwent 40 middle fossa craniotomies for excision of their acoustic tumors is reported. Eleven patients underwent bilateral procedures. The stu dy focused on hearing preservation and facial nerve results for this group of patients. The 16 male patients and 12 female patients ranged in age (at the time of surgery) from 10 to 70 years, with a mean age of 22.6 years. Th e mean tumor size was 1.1 cm (range, 0.5-3.2 cm), and the majority of tumor s were less than 1.5 cm. RESULTS: Measurable hearing was preserved in 28 ears (70%), with 42.5% bein g within 15 dB pure-tone average and 15% speech discrimination score of pre operative levels. In 55% of cases there was no change in the hearing class, as defined by the American Academy of Otolaryngology-Head and Neck Surgery . Of the 11 patients who underwent bilateral operations, 9 (82%) retained s ome hearing bilaterally. After 1-year follow-up periods (mean, 12.8 mo), 87 .5% of patients exhibited normal facial nerve function (House-Brackmann Gra de I). CONCLUSION: Early surgical intervention to treat acoustic tumors among pati ents with neurofibromatosis Type 2 is a feasible treatment strategy, with h igh rates of hearing and facial nerve function preservation.