title: [Neurofibromatosis 2 (bilateral acoustic neurofibromatosis)].
abstract: We report a personal series of 28 patients with neurofibromatosis 2 (NF-2), emphasizing the differences from classical NF-1. The hallmark of NF-2 is bilateral acoustic neuromas with initial symptoms usually occurring in the second or third decade. The natural history may lead to bilateral deafness, but hearing loss may also be a complication of surgery. NF-2 is frequently accompanied by additional intracranial tumors (particularly multiple meningiomas). Half of our patients had a spinal space-occupying lesion. NF-2 is inherited as an autosomal dominant trait, and many patients appear to represent new mutations.
authors.affiliation: Neurochirurgische Klinik, Universitätsspital Zürich.
authors.forename: C
authors.initials: C
authors.lastname: Yalcinkaya
authors.forename: A
authors.initials: A
authors.lastname: Sarioglu
authors.forename: E
authors.initials: E
authors.lastname: Boltshauser
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003638:Deafness / Q000209:etiology*; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D009464:Neuroma, Acoustic / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed
vernacular_title: Neurofibromatose 2 (bilaterale akustische Neurofibromatose).