Management strategy of vestibular schwannoma in neurofibromatosis type 2.

OBJECTIVES

To discuss our management strategy of vestibular schwannoma in patients with neurofibromatosis type 2 (NF2).

STUDY

DESIGN

Retrospective study.

SETTING

Quaternary referral skull base center.

METHODS

Thirty-eight NF2 patients who had undergone 48 operations at the Gruppo Otologico between January 1988 and December 2008. The mean age at time of surgery was 36.3 years (range, 17-65 yr), and the average tumor size was 3.1 cm (range, 0.6-6 cm). There were 27 female and 21 male ears, and 25 cases were right side ears, whereas 23 were left sided. The average follow-up time was 3.7 years. Surgical approaches, hearing, and facial nerve functions, as well as hearing rehabilitation and facial nerve reconstruction outcomes, are discussed.

RESULTS

Total tumor resection was achieved in 44 cases (92%). Facial nerve function was postoperatively House-Brackmann grades I-III in 36 cases (77%); it was grade I in 17 cases (35%) and grade II in 8 cases (17%). In 7 cases, hearing preservation was attempted, and a measurable hearing has been recorded in 5 cases (71%). Auditory brainstem implant was inserted in 25 cases, and concomitant cochlear implants were inserted in 5 cases.

CONCLUSION

Early diagnosis and treatment of bilateral vestibular schwannoma in patients with NF2 will achieve the best outcomes regarding facial nerve, hearing preservation, and postoperative complications. The watchful waiting policy will decrease the chance of reaching these goals. Cochlear implants and auditory brainstem implant have made hearing rehabilitation possible in NF2 patients who had bilateral sensorineural hearing loss.