Vestibular schwannomas in children

Objective: This article highlights the clinical presentation and management issues of unilateral vestibular schwannomas in children. We demonstrate ho w the presentation differs from neurofibromatosis type 2 (NF2) and from adu lt unilateral vestibular schwannomas. Study Design: This article is composed of a series of three cases and a lit erature review. Setting: The study was performed at a university hospital (tertiary referra l center). Patients: Three children, aged 9, 11, and 13 years, with histologically con firmed vestibular schwannomas were studied. All children under 16 years of age in the world literature with unilateral vestibular schwannomas were rev iewed. Intervention: Analysis of presentation and surgical management of these thr ee children and those children reported in the literature. Main Outcome Measure: Pattern of presentation relative to children with NF2 and people with adult unilateral vestibular schwannomas. Results: Two patients had multiple cranial nerve weakness and recurrence, a nd one patient had successful removal of the tumor with preservation of all functions of the cranial, nerves, including the facial nerve. Conclusion: Vestibular schwannomas in children are very uncommon. It is lik ely that it is the first manifestation of NF2, but it may also be a variant of sporadic vestibular schwannomas. A presentation of three cases and a re view of 36 other cases in the literature demonstrates how the presentation is different from adult sporadic vestibular schwannomas and NF2 because it lacks primary audiological symptoms. The study also provides evidence of no n-NF2 vestibular schwannomas presenting in children and suggests that it is likely that these are a variant of unilateral sporadic vestibular schwanno mas. The search for the features of NF2 in these cases remains mandatory.