[Neurofibromatosis type 2].
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.
Gong
Shusheng
S
Department of Otolaryngology, Union Hospital, Huazhong University of Science and Technology, Wuhan, 430022, China. gongss@mails.tjmu.edu.cn
Chen
Guangli
G
Zhong
Gang
G
Yan
Kaisheng
K
Zhang
E
E
Chen
Pei
P
Lin
Ning
N
Nie
Xiu
X
D000328:Adult; D006801:Humans; D008297:Male; D016518:Neurofibromatosis 2 / Q000175:diagnosis / Q000628:therapy