内耳共同腔畸形电刺激听觉脑干诱发电位辅助人工耳蜗植入术及其疗效评估

目的:探讨内耳共同腔畸形儿童人工耳蜗植入的诊断、手术后调机及听力言语康复效果.方法:回顾性分析术前影像学证实common cavity内耳畸形的语前聋人工耳蜗植入儿童19例,术中采用电刺激听觉脑干诱发电位(EABR)辅助评估听神经通路;并筛选临床资料相近的19例正常耳蜗结构的语前聋儿童人工耳蜗植入者配对,分析术前听力、...     

Mondini内耳畸形患者的多通道人工耳蜗植入

目的探讨Mondini内耳畸形患者人工耳蜗植入方法及手术前后听力评估和术后言语康复效果。方法回顾性分析经乳突面隐窝进路行人工耳蜗植入的19例Mondini内耳畸形患者的临床资料，并将其中10例患者植入后听力情况与10例耳蜗发育正常的人工耳蜗植入者(对照组)进行比较。结果19例Mondini内耳畸形患者中，32个电极全部植入者12例，28个电极植入者2例，26个电极植入者2例，22个电极植入者2例，20个电极植入者l例；术中7例发生多量脑脊液流出(ozze)，5例发生脑脊液井喷(gusher)，7例无脑脊液外溢。两组患者术后听力接近，均在30～40dB HL左右，无严重并发症发生，17例Mondini内耳畸形患者术后听觉言语康复效果与耳蜗发育正常者接近，2例稍差。长期效果有待进一步观察。结论人工耳蜗植入适用于Mondini内耳畸形患者，但手术前应进行全面的听力学及影像学评估。     

共同腔畸形人工耳蜗手术适应证及手术方法的探讨

目的：探讨共同腔畸形人工耳蜗手术适应证以及人工耳蜗电极植入人路的选择。方法：在对重度或全聋患者进行人工耳蜗植入术前影像掌检查中,发现了6例耳蜗、前庭、外半规管呈共同腔畸形,其中5例有残留听力,1例未查到残留听力。结果：6例影像学检查呈共同腔畸形患者中,对5例有残留听力患者进行了人工耳蜗植入,其中3例选择了常规入路植入电极,2例选择了经乳突侧入路植入电极,术后均建立了人工耳蜗的听觉反应。1例因未查到残留听力,放弃了人工耳蜗手术治疗。结论：有残留听力的共同腔畸形患者,如果能够接受术后听觉言语识别效果差的事实,可以进行人工耳蜗手术。无残留听力或无法了解到有听觉反应的共同腔畸形患者,在现有技术条件下应放弃人工耳蜗植入手术。     

不同内耳畸形人工耳蜗植入效果分析

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHODS: Among 410 patients who received multi-channel cochlear implantations from 1996 to 2004 in Beijing Tongren Hospital, 82 patients were diagnosed with inner ear malformations and implanted. A retrospective analysis was performed about the surgical characteristics and mapping characteristics after implantation. RESULTS: (1) All patients had auditory sensations. (2) Gusher was more common than the normal cochlear implantation. (3) The electrodes were inserted in the "cochleostomy" in full length of 80 Patients, but 2 pairs of electrodes remained outside of "cochleostomy" in 2 patients. (4) No serious complications occurred after implantation. (5) The impedance of the electrodes, the T level and C level were similar with the normal cochlear implantation. The results had no significant difference in compare with normal cochlear group (P > 0. 05). (6) The abilities of speech discrimination and spoken language were improved through rehabilitation. CONCLUSIONS: The cochlear implantation can be performed safely in inner ear malformations. The outcome of hearing rehabilitation for patients with inner ear malformations are similar to those children with normal cochlear structure followed the multi-channel cochlear implantation.     

多通道人工耳蜗植入在Common cavity畸形患者中的应用

目的：探讨Common cavity畸形患者人工耳蜗植入后的语言康复效果。方法：对6例Common cavity畸形患者采用经乳突外半规管进路行人工耳蜗植入,并与6例耳蜗发育正常组进行听力情况比较。结果：32个电极全部植入者3例,28个电极植入者1例,26个电极植入者1例,22个电极植入者1例。与正常组比较,术中术后并发症差异无显著性意义。术中脑脊液多量流出者2例,少量流出3例,无流出1例,两组术后听力接近,均在30-40dBHL左右,无严重并发症发生,大多数患者术后听觉语言康复效果与政治家耳蜗者接近,少数稍差。长期效果有待进一步观察。结论：人工耳蜗适用于Common cavity畸形患者,但手术前应进行全面的听力学及影像学评估。     

面神经畸形人工耳蜗植入围手术期处理

面神经是耳科手术需重点保护的结构之一,而面神经发育畸形会显著增加面神经损伤的风险。人工耳蜗植入(cochlear implantation,CI)是治疗重度感音神经聋患者的重要手段之一,面神经畸形(Facial nerve aberra-tion,FNA)常影响其手术径路的选择,增加手术风险。本文就面神经发育、FNA的分类、以及FNA患者行CI手术的围手术期处理予以综述,为临床提供参考。     

共同腔畸形患者的前庭功能研究进展

共同腔畸形(common cavity deformity, CCD)是由于胚胎发育第4周时耳囊发育停止导致的一种严重内耳畸形(inner ear malformations, IEMs),临床表现为极重度听力损失、平衡功能、大运动功能发育迟缓,如出现头部控制及独立行走的延迟。目前,人工耳蜗植入(cochlear implantation, CI)手术是内耳畸形包括CCD患者恢复听觉和生活质量的重要技术之一,国内关于CCD患者的前庭和平衡功能的研究较少。本文旨在综述CCD患者前庭功能的发展以及CI对CCD前庭功能的影响研究进展,以期为该畸形患者CI术前术后前庭功能评估及保护残余前庭功能提供参考依据。     

先天性内耳畸形的人工耳蜗植入

目的 探讨先天性内耳畸形引起重度感音神经性聋者人工耳蜗植入的有关问题。方法 2001年1月至2003年4月间对内耳畸形引起极重度感音神经性聋18例进行了人工耳蜗植入术。结果 18例中前庭水管11例，Waardenburg综合征3例，Mondini畸形3例，Usher综合征1例。全部病例采用Nucleus24型人工耳蜗，其中对前庭水管5例采用Contour植入体，其余病例采用直电极植入体。术中发现前庭水管11例开骨窗后仅有外淋巴搏动，但无井喷(脑脊液漏)，电极植入顺利。Waardenburg综合征3例和Mondini畸形3例中各有1例伴发圆窗骨性封闭畸形。结论 前庭水管者人工耳蜗植入手术顺利，术后效果与耳蜗发育正常者相同。如Mondini和Common Cavity等内耳畸形者行人工耳蜗植入时术前应准确评估畸形的程度及伴发的畸形，要充分估计手术难度和避免术后可能出现的脑脊液耳鼻漏及其颅内感染。     

Implantation of the common cavity malformation may prevent meningitis

Abstract

Objectives and importance

Children with certain congenital malformations of the inner ear, including those with a common cavity defect, have a higher incidence of spontaneous cerebrospinal fluid (CSF) leak and resulting meningitis. However, they may also benefit from cochlear implantation. We suggest that surgical management may be possible that both prevents meningitis and provides hearing rehabilitation during the same procedure.

Clinical presentation

A 2-year-old girl with bilateral common cavity defects who had previously undergone cochlear implantation developed contralateral CSF leak resulting in meningitis.

Intervention

After resolution of the infection, cochlear implantation was performed at the same time as definitive CSF leak repair. Simultaneous cochlear implantation and repair of the CSF leak successfully decreased the chance of recurrent meningitis in this case. She has been deriving hearing benefit from the bilateral implants.

Conclusion

This case suggests a role for cochlear implantation to be combined with simultaneous CSF leak repair in children with a cochlear malformation. Furthermore, bilateral cochlear implantation at an early age may be warranted in these patients before CSF leaks and meningitis have occurred.     

Endoscopically guided placement of prefabricated cochlear implant electrodes in a common cavity malformation

OBJECTIVE: To devise a safe and effective method of optimal customized electrode placement in the common cavity of children with cochleovestibular malformations. METHODS: Specialized electrodes were manufactured on the basis of three-dimensional data obtained from the high resolution computed tomography (HRCT) scans of the temporal bones of these two children. Electrode positioning was achieved with direct endoscopic view of the cavity utilizing a three-hole common cavity technique. RESULTS: Optimal electrode positioning in apposition to the medial neuroepithelium in the common cavity was verified visually intraoperatively. Postoperatively, minimal stable electrical current levels were found to be required. CONCLUSIONS: Custom-designed electrodes have the potential to offer improved results in children with common cavity malformations. Intraoperative direct positioning may further improve these results.     

先天性内耳畸形并中耳畸形患者多通道人工耳蜗植入术3例报告

目的：探讨先天性内耳畸形并中耳畸形患者行多通道人工耳蜗植入术的方法及效果。方法：1995年5月-2002年5月我院为3例罕见的先天性内耳畸形并中耳畸形患者经乳突进路行人工耳蜗植入术,分别植入27、28、32个电极,植入后3个月行声场测听。结果：3例患者均成功地行人工耳蜗植入,术中、术后无严重并发症发生,术后声场测听听阈达35-40dBHL。结论：罕见的先天性内耳畸形并中耳畸形患者也可行人工耳蜗植入术,术后效果满意。     

人工耳蜗植入术后改良耳蜗位平片的探讨

目的 探讨改良后耳蜗位平片摄影位的临床应用价值。方法 21例人工耳蜗植入术后患儿(年龄30d～4岁)分别摄许氏提倡的耳蜗位平片摄影位和我们改良后的许氏耳蜗位平片摄影位,用Cohen(c)方法测出蜗管内电极数,将术中得数与2种片所得共3组数据行统计处理t检验。结果 2种平片均能清晰地显示蜗管内电极的位置和形态,2组X片结果与手术结果基本吻合,3组数据比较差异无统计学意义。结论 改良后耳蜗位平片摄影位方法简便,易使4岁以下患儿接受,且能满足了解人工耳蜗植入术后精确状态的需要。     

内耳发育畸形患者人工耳蜗植入术后的疗效分析

目的分析内耳发育畸形患者行人工耳蜗植入术后的疗效。方法回顾性分析2011年1月~2017年7月在西南医科大学附属医院行人工耳蜗植入术的30例内耳发育畸形患者的临床资料,其中大前庭水管综合征(large vestibular aqueduct syndrome,LVAS) 8例,Mondini畸形（不完全分隔Ⅱ型）7例,同时伴有LVAS和Mondini畸形10例,内听道狭窄5例。随机选取同时期临床资料相匹配的30例耳蜗结构正常的人工耳蜗植入患者作为对照组。采用听觉行为分级标准和言语可懂度分级标准进行评估。比较两组患者的术前听力、术中情况以及术后听觉言语康复情况。结果3例LVAS患者在术中耳蜗钻孔后出现外淋巴液不同程度的外涌,1例Mondini畸形患者术中出现脑脊液井喷现象。两组患者术后均获得有意义的听觉反应,术后的听觉言语能力相比较,差异无统计学意义（P>0.05）。同组患者,术后3,6,9,12个月的听觉言语能力均较各自术前均有明显提高（P<0.05）。结论内耳发育畸形患者不是人工耳蜗植入术的绝对禁忌证,其术后言语康复效果与内耳结构正常者无明显差异,但手术难度比内耳结构正常者要大,术前应根据不同的畸形类型制定个性化的手术方案,以减少并发症的发生。     

内耳畸形患儿的人工耳蜗植入

目的：探讨几种内耳畸形患儿的人工耳蜗植入效果。方法：对10例内耳结构异常的感音性耳聋患儿进行了人工耳蜗植入。结果：术后随访0．5～4．0年，10例基本达到了较满意的听觉言语恢复效果。结论：内耳畸形息儿行人工耳蜗植入，应严格进行术前听力学与影像学的评估，严格掌握手术的适应证，可以达到较满意效果。