Fichten A, Bourgeois P, Desaulty A, Louis E, Lejeune J-P
Clinique de Neurochirurgie, Hôpital Roger-Salengro, CHRU, 59037 Lille Cedex, France.
Neurochirurgie. 2006 Feb;52(1):37-46. doi: 10.1016/s0028-3770(06)71168-7.
Intracranial facial nerve schwannomas are rare neoplasms. Preoperative diagnosis is difficult because of non-specific clinical presentations (deafness, facial paralysis sudden or progressive) and radiological differential diagnosis (petrous bone tumor, vestibular schwannoma). Treatment depends on localization and has to be discussed for each case.
Seven cases (four men and three women) of intracranial facial nerve schwannomas were retrospectively studied.
Before treatment, we found deafness in six cases (two sudden and four progressive), a facial palsy in five cases (three sudden and two progressive). Five patients had deafness and facial palsy. One patient had only headache. Three schwannomas were supra and intra-petrous, two in the cerebello-pontine angle, and two were plurifocal (petrous bone, internal auditive canal and cerebellopontine angle). Six patients were operated on with an oto-neurosurgical procedure. After treatment, facial palsy always worsened (requesting secondary hypoglosso-facial anastomosis in cinq cases). Only one case of transmission deafness improved after ossiculoplasty. One patient is still under clinical and radiological observation.
Diagnosis is difficult and made operatively in half of patients. A large tumor requires surgery, but surveillance can be a good option for a small one, considering the risk of postoperative facial palsy.
颅内面神经鞘瘤是一种罕见的肿瘤。由于临床表现不具特异性（耳聋、突发性或进行性面瘫）以及放射学鉴别诊断（岩骨肿瘤、前庭神经鞘瘤），术前诊断较为困难。治疗方案取决于肿瘤的位置，需针对每个病例进行讨论。
对7例颅内面神经鞘瘤患者（4例男性，3例女性）进行回顾性研究。
治疗前，6例患者出现耳聋（2例突发性，4例进行性），5例患者出现面瘫（3例突发性，2例进行性）。5例患者同时存在耳聋和面瘫。1例患者仅有头痛症状。3例神经鞘瘤位于岩骨上及岩骨内，2例位于桥小脑角，2例为多灶性（岩骨、内听道及桥小脑角）。6例患者接受了耳神经外科手术。治疗后，面瘫均加重（5例患者需要二期舌下神经-面神经吻合术）。仅1例传导性耳聋患者在听骨成形术后听力有所改善。1例患者仍在接受临床及影像学观察。
诊断困难，半数患者通过手术确诊。对于较大的肿瘤需要进行手术，但考虑到术后面瘫的风险，对于较小的肿瘤，观察也是一个不错的选择。
