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  • Author or Editor: Rick A. Friedman x
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William H. Slattery III, Laurel M. Fisher, William Hitselberger, Rick A. Friedman and Derald E. Brackmann

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.

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Robert C. Rennert, Danielle M. Levy, Jillian Plonsker, Jeffrey A. Steinberg, Rick A. Friedman, John R. Crawford and Michael L. Levy

Pediatric cerebellopontine angle (CPA) meningiomas are extremely rare and are usually treated with a retrosigmoid surgical approach or radiation. The authors present the use of a middle fossa approach for the treatment of a symptomatic CPA meningioma in a 22-month-old female. The patient initially presented at 17 months with isolated progressive, long-standing right-sided facial weakness. MRI demonstrated a 5.0 × 5.0–mm right CPA lesion just superior to the cisternal segment of cranial nerve (CN) VII, which demonstrated growth on interval imaging. At 22 months of age she underwent a successful middle fossa craniotomy, including wide exposure of the porus acusticus, allowing for a gross-total resection with preservation of CNs VII and VIII. Pathological analysis revealed a WHO grade I meningioma. The patient remained neurologically stable on follow-up. The middle fossa approach can be used to safely access the CPA in properly selected pediatric patients.