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2型神经纤维瘤病患者双侧前庭神经鞘瘤手术切除后听觉和前庭功能的保留

Preservation of auditory and vestibular function after surgical removal of bilateral vestibular schwannomas in a patient with neurofibromatosis type 2.

作者信息

Black F O, Brackmann D E, Hitselberger W E, Purdy J

机构信息

R.S. Dow Neurological Sciences Institute, Good Samaritan Hospital, Portland, Oregon 97210, USA.

出版信息

Am J Otol. 1995 Jul;16(4):431-43.

PMID:8588642
Abstract

The outcome of acoustic neuroma (vestibular schwannoma) surgery continues to improve rapidly. Advances can be attributed to several fields, but the most important contributions have arisen from the identification of the genes responsible for the dominant inheritance of neurofibromatosis types 1 (NF1) and 2 (NF2) and the development of magnetic resonance imaging with gadolinium enhancement for the early anatomic confirmation of the pathognomonic, bilateral vestibular schwannomas in NF2. These advances enable early diagnosis and treatment when the tumors are small in virtually all subjects at risk for NF2. The authors suggest that advising young NF2 patients to wait until complications develop, especially hearing loss, before diagnosing and operating for bilateral eighth nerve schwannomas may not always be in the best interest of the patient. To the authors' knowledge, this is the first reported case of preservation of both auditory and vestibular function in a patient after bilateral vestibular schwannoma excision.

摘要

听神经瘤(前庭神经鞘瘤)手术的效果仍在迅速改善。进展可归因于多个领域,但最重要的贡献来自于对导致1型神经纤维瘤病(NF1)和2型神经纤维瘤病(NF2)显性遗传的基因的识别以及钆增强磁共振成像的发展,用于早期解剖学确认NF2中典型的双侧前庭神经鞘瘤。这些进展使得几乎所有有NF2风险的受试者在肿瘤较小时就能进行早期诊断和治疗。作者认为,建议年轻的NF2患者等到出现并发症,尤其是听力丧失后再诊断和手术治疗双侧第八神经鞘瘤,可能并不总是符合患者的最佳利益。据作者所知,这是双侧前庭神经鞘瘤切除术后患者听觉和前庭功能均得以保留的首例报道病例。

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