Hearing preservation surgery for neurofibromatosis type 2-related vestibular schwannoma in pediatric patients

Object. The authors reviewed the proportion of pediatric patients with neurofibromatosis Type 2 (NF2) in whom hearing was preserved after middle fossa resection of vestibular schwannoma (VS). Methods. In this retrospective chart review the authors examined the cases of 35 children with NF2 who had undergone middle fossa resection (47 surgeries) between 1992 and 2004 in a neurotological tertiary care center. Surgical outcome was assessed using pure-tone average (PTA) thresholds obtained before and immediately after resection. Speech discrimination scores (SDSs) and pre- and postfacial nerve grades were also recorded. In 55% of surgeries, hearing of less than or equal to 70 dB PTA was maintained postoperatively. The American Academy of Otolaryngology-Head and Neck Surgery Class A hearing (PTA <= 30 dB and SDS >= 70%) was preserved in 47.7%. Facial nerve function was good (House-Brackmann Grades I or II) in 81% of the patients. Twelve patients had bilateral middle fossa resections; in nine (75%) of these patients hearing was maintained postoperatively in both ears. Conclusions. More than half of the children with NF2 in the authors' cohort experienced hearing preservation after middle fossa resection was performed for VS. The authors recommend this approach for preserving hearing in children with NF2.