Abstract Otology 2000 D13-4Early management of neurofibromatosis type II
Derald E. Brackmann MD, Jose N. Fayad MD, William Hitselberger MD
House Ear Clinic Inc. USA-Los Angeles CA
Introduction: The management of patients with neurofibromatosis type II (NF2) has always posed a challenging problem for neurosurgeons and neurotologists. Guidelines for appropriate management of this devastating disease are controversial. Methods: A retrospective study of 24 patients with neurofibromatosis type II who underwent 34 middle fossa craniotomies for excision of their acoustic tumors is reported. Ten patients underwent bilateral procedures. The study focused on hearing preservation and facial nerve results in this group of patients. Results: There were 13 males and 11 females. Age at the time of surgery ranged between 10 and 70 years. The size of the tumor ranged between 0.5 cm and 2.5 cm with the majority of the tumors being between 1 and 2 cm. The hearing was preserved in 24 ears (77.42%), lost in 7 ears (22.58%). Three recently operated patients did not have a follow-up at this time. In the immediate postoperative period 28 cases (82.35%) had a grade I House-Brackmann. Three cases had a grade II (8.82%). One case had a grade III. One case had a grade V, and one had a grade VI. Long-term facial nerve results will be reported. Discussion and Conclusions: Based upon these data, we conclude that early surgical treatment of acoustic tumors in NF2 patients is the treatment of choice.