Pediatric Sporadic Vestibular Schwannomas

Objective: Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, response to surgical treatment, and recurrence rates in these younger patients.

Methods: A retrospective review of all patients 21 years old and younger with unilateral vestibular schwannomas and without neurofibromatosis type 2, who underwent surgical resection by the senior authors (D.E.A. and J.P.L.) between 1997 and 2016. Presentation, surgical characteristics, clinical outcomes and follow-up were reviewed.

Results: Thirteen patients were identified, ranging from 12 to 21 years old (mean: 16). Common presenting symptoms included hearing loss (92%), ataxia (69%), headache (46%) and tinnitus (38%). At the time of surgery, mean tumor size was 3.2 cm, with four 1 cm tumors, a trend different from current literature. Five underwent a retrosigmoid approach, three underwent a translabyrinthine approach, and five underwent a combined approach. Postoperatively, 10 patients (77%) had House–Brackmann grade I facial nerve function and 3 (23%) had grade II with a mean follow-up of 70 months. Four cases had residual tumor following their first surgeries and three (75%) of those had a significant radiographic regrowth that required a second surgery or radiotherapy treatment.

Conclusion: We report on the clinical findings related to sporadic pediatric acoustic neuromas, with the largest series of small tumors surgically resected reported in the literature. Additionally, residual tumors in pediatric patients have a higher rate of regrowth than in their adult counterparts.