Journal of Clinical Otolaryngology Head and Neck Surgery
The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery
종설

제2형 다발성 신경섬유종증 1례

도홍림1, 황찬승1, 홍영호1, 김훈1
Hong Lim Do1, Chan Seung Hwang1, Young Ho Hong1, Hoon Kim1
1중앙대학교 의과대학 이비인후과학교실
1Department of Otolaryngology, College of Medicine, Chung Ang University, Seoul, Korea

© Copyright 1997 The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery. This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published Online: May 31, 2020

ABSTRACT

Type 2 Neurofibromatosis is one of the neurocutaneous syndromes and it is an autosomal dominant hereditary disease characterized by the development of bilateral vestibular schwannomas.

The diagnostic criteria are met by an individual who has (1) bilateral 8th nerve masses even with appropriate imaging techniques, or (2) a first degree relative with type 2 neurofibromatosis and either a unilateral acoustic neuroma or two of the following : neurofibroma, meningioma, glioma, schwannoma, or juvenile posterior subcapsular lenticular capacity.

Recently we have experienced a case of type 2 neurofibromatosis, manifested with a collateral vestibular schwannoma in a 27-year-old female patient, which was treated with surgical excision. So we report the case with review of literature.

Keywords: Bilateral vestibular schwannomas; Neurofibromatosis