title: [Neurofibromatosis type 2].
abstract: OBJECTIVE
  To recognise the predisposing factors, clinical manifestations, diagnosis and treatment of neurofibromatosis type 2 (NF2).
  
  
  METHODS
  The clinical data of one NF2 case was reported and the literatures were also reviewed.
  
  
  RESULTS
  The patient was diagnosed at a much later stage than onset. Progressive hearing loss and tinnitus were the initial symptoms. MRI scan indicated space-occupying lesions in the bilateral cerebellopontine angles, bilateral cavernous sinuses and bilateral cervical parts of the patient. The patient was diagnosed as NF2 according to the National Institutes of Health (NIH) criteria, and received operation on the left acoustic tumor. The tumor was proved to be schwannomas by pathological test. The hearing loss and the facial nerve paralysis (House-Brackmann II) had appeared after operation.
  
  
  CONCLUSIONS
  NF2 is an autosomal dominant, highly penetrant disease which is characterized by bilateral vestibular schwannomas. Early diagnosis and management for tumor is very important for survival and hearing preservation. The "golden standard" in terms of diagnostic precision is the magnetic resonance imaging (MRI) scan with gadolinium enhancement.
authors.affiliation: Department of Otolaryngology, Union Hospital, Huazhong University of Science and Technology, Wuhan, 430022, China. gongss@mails.tjmu.edu.cn
authors.forename: Shusheng
authors.initials: S
authors.lastname: Gong
authors.forename: Guangli
authors.initials: G
authors.lastname: Chen
authors.forename: Gang
authors.initials: G
authors.lastname: Zhong
authors.forename: Kaisheng
authors.initials: K
authors.lastname: Yan
authors.forename: E
authors.initials: E
authors.lastname: Zhang
authors.forename: Pei
authors.initials: P
authors.lastname: Chen
authors.forename: Ning
authors.initials: N
authors.lastname: Lin
authors.forename: Xiu
authors.initials: X
authors.lastname: Nie
mesh_terms: D000328:Adult; D006801:Humans; D008297:Male; D016518:Neurofibromatosis 2 / Q000175:diagnosis / Q000628:therapy