听神经瘤之听力保留策略和听力重建技术

作为桥小脑角区最常见的良性肿瘤,听神经瘤(vestibular schwannomas,VS)由于生长部位关键,肿瘤习性特殊,常给患者带来严重的脑神经功能障碍甚至生命威胁。随着诊疗技术的进步,该病的治疗目标已从早期的控制肿瘤,发展为越来越强调重要脑神经功能即面神经、听神经功能的保留,以不断提高患者的生活质量。相较面神经,听神经功能的保留是更具挑战性的难题。VS大多数为单侧、散发,双侧听神经瘤则与2型神经纤维瘤病(NF2)密切相关,其听力保留的预后更差。目前,VS的听力保留策略主要包括保守治疗、显微手术及放疗等,几种策略各有千秋,如何针对每个患者选择适应证、制定个性化的、最优化的听功能保留及重建策略,是耳外科专家、神经外科专家及放射肿瘤学家们高度关注和热议的话题。本文结合解放军总医院耳鼻咽喉科对听神经瘤37年诊疗经验和临床研究探索历程,并且系统阐述了国内外最新进展,对我国听神经瘤诊治会起到一定的推动作用。虽然临床技术有很大进步,但还有许多问题尚待解决。听神经瘤包括NF2缺乏理想动物模型,其确切的发生机制、针对性的药物研发和各种干预手段效果的客观评价等研究在国内外都还是空白。期待未来能有更多的理念和技术手段不断涌现,可以让更多的患者受益。

The Strategies of Hearing Preservation and the Technique of Hearing Function Rehabilitation of Vestibular Schwannoma

Vestibular schwannoma(VS),also known as acoustic neuroma,is the most common benign tumor in the cerebellopontine angle(CPA)region.Because of its unique location and growth patterns,VS can result in serious cranial nerve morbidity and even mortality.Its treatment has progressed from tumor control in the early days to now including preservation of hearing and facial nerve function,with improvement of quality of life as a main objective.Recent advancements in techniques have reduced the risk of facial nerve dysfunction,but hearing preservation/rehabilitation remains a critical challenge.Most VS cases are unilateral and sporadic.Bilateral VSs are associated with neurofibromatosis type 2 which has a worse prognosis for hearing preservation.Currently,conservative management,microsurgery and radiotherapy are treatment options for VS,although it often presents a treatment dilemma for both the surgeon and patient.How to come up with an initial individualized management strategy that can minimize morbidities from tumor progression while maximizing the duration of useful hearing is debated among otolaryngologists,neurosurgeons and radiation oncologists.Based upon 37 years of experience in VS treatment and research at the PLA General Hospital Department of Otorhinolaryngology,this article systematically reviews the latest progress regarding VS at home and abroad,in an attempt to facilitate advances in the diagnosis and treatment of VS in China.Despite great improvement in clinical techniques,there are many problems to be solved,including lack of ideal animal models of NF2-VS and poor understanding of it exact mechanisms.Much remains to be done to develop targeted drugs and objective efficacy evaluation for various interventions.It is expected that more ideas and technical means will emerge in the future,which can benefit more patients.