应用国产化多通道听觉脑干植入设备重建2型神经纤维瘤病患者听觉功能的临床试验分析  

Clinical trial of the domestic multi-channel device of auditory brainstem implant for auditory rehabilitation in patients with neurofibromatosis type 2

作　　者：赵赋[1] 王博[2] 李仕维[2] 王兴朝[2] 张晶[1] 王振民[2] 李朋[2] 杨智君[2] 刘丕楠[1,2] Zhao Fu;Wang Bo;Li Shiwei;Wang Xingchao;Zhang Jing;Wang Zhenmin;Li Peng;Yang Zhijun;Liu Pinan(Beijing Neurosurgical Institute,Capital Medical University,Beijing 100070,China;Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University,Beijing 100070,China)

机构地区：[1]首都医科大学,北京市神经外科研究所,100070 [2]首都医科大学附属北京天坛医院神经外科,100070

出　　处：《中华神经外科杂志》2021年第4期369-374,共6页Chinese Journal of Neurosurgery

基　　金：“十二五”国家科技支撑计划(2012BAI12B03);北京市卫生健康委员会神经损伤基础研究与临床转化平台建设(PXM2020_026280_000002);国家自然科学基金(81372715,61671187)。

摘　　要：目的评价应用国产化多通道听觉脑干植入设备(ABI)对2型神经纤维瘤病(NF2)患者进行听力重建的安全性和效果.方法前瞻性纳入2015年12月至2016年2月首都医科大学附属北京天坛医院神经外科收治的存在单侧或双侧听力损伤、且需要切除前庭神经鞘瘤的NF2患者.手术采用枕下经乙状窦后入路,在切除肿瘤后进行ABI电极植入.术中通过记录电诱发听觉脑干反应(EABR)辅助定位耳蜗核,以确保电极贴附于耳蜗核表面.采用自体脂肪填塞侧隐窝以固定ABI刺激电极.通过长期临床随访观察应用ABI的疗效和不良反应.结果本研究共纳入5例存在听力损伤或丧失的NF2患者.术侧的前庭神经鞘瘤均获得全切除.术中所有患者均顺利植入ABI,并通过电刺激成功记录到EABR波形.术后2~4周进行开机调试和言语训练,5例患者均可在安静环境下感知不同程度的环境音.随访时间为57~60个月,所有患者均未出现与开颅手术或设备植入相关的并发症.术前双侧听力丧失的2例患者开机后连续使用ABI 54、27个月,其术后环境音的平均识别率分别为62.5%和49.6%,封闭环境中单音节和双音节词语的平均识别率分别为42.5%和33.8%,同时结合读唇后具有较好的言语沟通能力.1例患者因对侧耳听力完全丧失而尝试开机调试和进行言语训练2个月,其余2例患者因对侧仍有听力,至末次随访仍未开机.结论初步研究表明,应用国产化多通道ABI是一种安全、有效的听力重建方法,术中良好的耳蜗核定位和术后言语训练是提高疗效的关键.Objective To evaluate the elinical ffect and safety of domestic muli channel auditory brainstem implant(ABI)device for auditory rehabilitation in Chinese neurofibromatosis type 2(NF2)patients.Methods We prospectively enrolled NF2 patients with unilateral or bilateral hearing impairment who needed resection of vestibular schwannoma and were adnitted to Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University from December 2015 to February 2016.The surgery was performed via the suboccipital retrosigmoid approach.The ABI electrode was implanted after tumor removal.During the operation,the elecrically evoked auditory brainstem response(EABR)was recorded to assist in localizing the cochlear nucleus to ensure that the electrodes were attached to the surface of the cochlear nucleus.The lateral recess was packed with autologous fat to fix the ABI stimulation electrodes.Long-term clinical follow-up was conducted to observe the fficacy and adverse events of the application of ABI.Results A total of 5 NF2 patients with hearing impairment or loss were included in this study.All the vestibular schwannomas on the surgical side were completely removed.During the operation,all patients were sucesfully implanted with ABI equipment and EABR waveforms were sucessfully recorded through electrical stimulation.Device progamming and spech training were carried out at 2 to4 weeks after the operation,and 5 patients could perceive diferent degrees of environmental sound in a quiet environment.The fllow-up time was 57 to 60 months,and all patients had no complications related to craniotomy or device implantation.In 2 patients with bilateral hearing loss before operation,the ABI was used continuously for 54 and 27 months after the device was turned on.'The avernage recognition rates of postoperative environmental sounds were 62.5%and 49.6%,respectively.The average recognition rates of monosyllable and two-syllable words in a closed environment were 42.5%and 33.8%respectively,and the patients had good verbal communicati

关 键 词：2型神经纤维瘤病 神经外科手术 听力 前庭神经鞘瘤 听觉脑干植入设备 

分 类 号：R739.4[医药卫生—肿瘤]