Mondini畸形多道人工耳蜗植入的效果分析

目的　探讨Mondini畸形患者人工耳蜗植入手术的可行性效果及手术特点。方法　我院自 1996年开展多道人工耳蜗植入术以来 ,截止到 2 0 0 2年 10月已经开展手术 30 0例 ,其中典型的Mondini畸形患者 15例 (2 8耳 )。本组对这 15例 (15耳 )植入手术患者进行回顾性分析 ,就Mondini畸形人工耳蜗植入手术特点、开机调试结果、言语康复与耳蜗形态正常的人工耳蜗植入者进行了比较。任意抽出 15例 (30耳 )耳蜗结构正常的人工耳蜗植入者作为对照组与Mondini畸形者进行比较。结果　①Mondini畸形术中 11耳出现井喷现象 ,较对照组多见 ,且井喷比较汹涌 ;严重者术中及术后需要使用甘露醇降颅压 ;② 13例 (13耳 )电极全部植入耳蜗内 ,2例 (2耳 )遗留 2对蜗外电极 ;③术后无面瘫、脑脊液漏、脑膜炎等手术并发症 ;④所有Mondini畸形患者人工耳蜗植入术后都有听觉 ;⑤开机调试时电极反应阈值、最大舒适阈值及阻抗值与对照组数值接近 ,经统计学比较差异无显著性 (P>0 0 5 )。术后平均听阈 (35dBHL)与对照组 (30dBHL)无明显区别。结论　人工耳蜗植入可以作为伴有Mondini畸形的双侧深度感音性聋患者的听觉康复手段 ,植入效果与对照组接近     

人工耳蜗在前庭水管扩大患者中的应用

目的　研究前庭水管扩大患者人工耳蜗植入手术的经验。方法　对 15例 (15耳 )双侧前庭水管扩大伴重度感音神经性聋的患者行多导人工耳蜗植入术 ,并对术后结果进行随访。经面隐窝径路行人工耳蜗植入术 ,在圆窗龛前下方行鼓岬钻孔 ,植入多导人工耳蜗电极。结果　①术中电极完全插入耳蜗 ,其中 6耳出现轻微井喷 ;②术后无面瘫、脑脊液耳漏等并发症 ;③所有患者人工耳蜗植入术后都有听觉 ;④术后听阈与耳蜗结构正常植入患者无明显区别 (自由声场平均听阈 30dBHL) ;⑤术后语训在北京同仁医院或中国聋儿康复中心进行 ,听力及言语能力均有不同程度提高 ,其中 2例患者已经进入正常学校就读。结论　前庭水管扩大伴重度感音神经性聋的人工耳蜗植入与耳蜗结构正常患者植入术后效果基本一致 ,人工耳蜗植入可以作为前庭水管扩大伴有重度感音神经性聋的治疗和康复手段     

人工耳蜗在前庭水管扩大患者中的应用

目的 研究前庭水管扩大患者人工耳蜗植入手术的经验。方法 对15例(15耳)双侧前庭水管扩大伴重度感音神经性聋的患者行多导人工耳蜗植入术，并对术后结果进行随访。经面隐窝径路行人工耳蜗植入术，在圆窗龛前下方行鼓岬钻孔，植入多导人工耳蜗电极。结果 ①术中电极完全插入耳蜗，其中6耳出现轻微井喷；②术后无面瘫、脑脊液耳漏等并发症；③所有患者人工耳蜗植入术后都有听觉；④术后听阈与耳蜗结构正常植入患者无明显区别(自由声场平均听阈30dBHL)；⑤术后语训在北京同仁医院或中国聋儿康复中心进行，听力及言语能力均有不同程度提高，其中2例患者已经进人正常学校就读。结论 前庭水管扩大伴重度感音神经性聋的人工耳蜗植入与耳蜗结构正常患者植入术后效果基本一致，人工耳蜗植入可以作为前庭水管扩大伴有重度感音神经性聋的治疗和康复手段。     

耳蜗骨化与人工耳蜗植入

目的 探讨耳蜗骨化状态下人工耳蜗植入手术的经验及分析术后效果.方法 北京同仁医院自1996年开展多道人工耳蜗植入手术以来,截止到2006年12月已开展手术600余例,遇到双侧耳蜗骨化患者7例.其中4例有明确的脑膜炎病史,3例耳蜗骨化原因不明.3例为耳蜗完全骨化;1例鼓阶完全骨化,前庭阶未受累;1例耳蜗部分骨化;2例耳蜗部分纤维化.总结术前、术中及术后的处理原则、方法及术后康复效果.结果 1例术中出现镫井喷,但比其他内耳畸形术中出现镫井喷的程度轻微;4例电极完全插入耳蜗内,2例植入部分电极,1例家属放弃植入;术后无面神经麻痹、脑脊液瘘等并发症;术后开机调试发现部分患者T值、C值比正常形态耳蜗植入偏高(因病例数量少未做统计学分析),但听阈无明显区别.术后语训听力及言语能力均有不同程度的提高.结论 对于耳蜗部分骨化患者,术中只要将其骨化成分自鼓阶内清除后可顺利植入电极;对于完全骨化患者可以将围绕蜗轴的鼓阶顶壁钻开,将电极嵌入骨槽内即可.脑膜炎后出现重度感音性聋应考虑耳蜗骨化的可能,常规颞骨CT及MRI检查,一旦确诊应尽快手术,以免耳蜗完全骨化.人工耳蜗植入术可以作为伴有耳蜗骨化的深度感音性聋患者的治疗和康复手段.     

耳蜗骨化与人工耳蜗植入

目的探讨耳蜗骨化状态下人工耳蜗植入手术的经验及分析术后效果。方法北京同仁医院自1996年开展多道人工耳蜗植入手术以来，截止到2006年12月已开展手术600余例，遇到双侧耳蜗骨化患者7例。其中4例有明确的脑膜炎病史，3例耳蜗骨化原因不明。3例为耳蜗完全骨化；1例鼓阶完全骨化，前庭阶未受累；1例耳蜗部分骨化；2例耳蜗部分纤维化。总结术前、术中及术后的处理原则、方法及术后康复效果。结果1例术中出现镫井喷，但比其他内耳畸形术中出现镫井喷的程度轻微；4例电极完全插入耳蜗内，2例植入部分电极，1例家属放弃植入；术后无面神经麻痹、脑脊液瘘等并发症；术后开机调试发现部分患者T值、C值比正常形态耳蜗植入偏高（因病例数量少未做统计学分析），但听阈无明显区别。术后语训听力及言语能力均有不同程度的提高。结论对于耳蜗部分骨化患者，术中只要将其骨化成分自鼓阶内清除后可顺利植入电极；对于完全骨化患者可以将围绕蜗轴的鼓阶顶壁钻开，将电极嵌入骨槽内即可。脑膜炎后出现重度感音性聋应考虑耳蜗骨化的可能，常规颞骨CT及MRI检查，一旦确诊应尽快手术，以免耳蜗完全骨化。人工耳蜗植入术可以作为伴有耳蜗骨化的深度感音性聋患者的治疗和康复手段。     

人工耳蜗植入在Mondini畸形患者中的应用

目的探讨Mondini畸形患者人工耳蜗植入术特点及术后言语康复效果。方法回顾性分析2000～2008年于我科确诊的12例Mondini畸形行人工耳蜗植入患者的临床资料,所有患者均经乳突面隐窝进路行人工耳蜗植入术,并将此12例患者植入后听力情况与另12例耳蜗发育正常的人工耳蜗植入者进行比较。结果 12例Mondini畸形患者中10例术中出现井喷,2例术中耳蜗钻孔后见外淋巴液出现不同程度的搏动,但术中电极植入顺利,术后无面瘫及脑脊液漏现象发生,术后听阈与耳蜗结构正常植入患者无明显区别。经过1年以上的言语康复训练,所有患者的听力及言语能力均有不同程度提高。结论人工耳蜗植入可以作为伴有Mondini畸形的双侧极重度感音性聋患者恢复听觉功能的手段。     

多导人工耳蜗的临床应用(附11例报告)

目的：探讨多导人工耳蜗植入对深度感音神经性耳聋患者的治疗效果。方法：对11例深度耳聋患者行MiniSystem22导和Nucleus24导人工耳蜗植入术,其中语前聋6例,语后聋5例。术后1～1．5个月开机调试。结果：11例患者全部恢复了听力;语后聋患者不需要经过唇读或简单的语言训练就能进行对话和打电话,患者能回到有声世界进行正常的生活和工作;对语前聋患者进行评议训练,都有不同程度的进步。结论：深度感音神经性耳聋患者植入22导及24导人工耳蜗后效果良好,尤其对语后聋和年龄较小的儿童效果更好。     

人工耳蜗植入术中脑脊液“井喷”的处理及原因分析

目的 探讨伴内耳畸形患者人工耳蜗植入术中出现脑脊液“井喷”的原因、处理方式及预后。方法 回顾性分析人工耳蜗植入术中出现脑脊液“井喷”的19例患者的临床资料，其中，IP-I畸形10例，IP-III畸形9例，均伴蜗轴或内听道底骨质缺失，所有患者均采用扩大圆窗入路行单侧人工耳蜗植入，术中均见脑脊液经耳蜗开窗口快速大量涌出，待脑脊液压力降低后植入人工耳蜗电极，再采用较大块颞肌严密封堵圆窗造口。结果 19例患者术后植入电极位置正常，所有患者均于术后1个月左右顺利开机，密切随访6～12个月均未出现脑脊液耳鼻漏及脑膜炎。结论 伴蜗轴或内听道底骨质缺损的内耳畸形病例行人工耳蜗植入术时术中可能出现脑脊液“井喷”,采用较大块颞肌严密封堵圆窗造口处，可以减少和避免术后并发症的发生，预后良好。     

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　 2 0 0 1年 1月至 2 0 0 3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Nucleus 2 4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CommonCavity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     

高分辨率螺旋CT对多导人工耳蜗植入的评估

目的 探讨CT检查对人工电子耳蜗植入术前、术后评估的价值。方法 对44例感音性聋患者均采用颞骨轴位高分辨率CT螺旋扫描及内耳三维重建。结果 32例语前聋患者中检出1例(2耳)Mondini畸形Ⅰ型，2例(4耳)Mondini畸形Ⅱ型；12例语后聋患者中检出2例(4耳)慢性化脓性中耳炎，1例(2耳)内耳骨化，1例(2耳)耳硬化症，3例(6耳)大前庭水管综合征。CT检出1例术后植入电极扭结并感染。结论 CT检查对人工耳蜗植入术前病例的选择、术后植入电极的观察以及并发症的检出均具有重要价值。     

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.     

Mondini畸形多道人工耳蜗植入的效果分析

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with Mondini malformation. METHODS: Among 300 patients who received multi-channel cochlear implants from 1996 to 2002 in Beijing Tongren Hospital, 15 patients were diagnosed with Mondini malformation. A retrospective analysis was performed dealing with the surgical techniques, mapping and rehabilitations characteristics after surgery. 15 patients with normal cochlear structure are consider as control group. RESULTS: Gusher is found more common than the normal cochlear implantation, most of them are serious. The electrodes are inserted in the "cochleostomy" in full length of 13 Patients, 2 pairs of electrodes remains outside of "cochleostomy" in 2 patients. No serious complications occurred after implantation. All patients have auditory sensations. The impedance of the electrodes, the T level, C level and the hearing threshold are similar with the normal cochlear implantation group. The results have no significant difference in compare with normal cochlear group(P > 0.05). CONCLUSION: Multi-channel cochlear implantation could be performed safely in patients with Mondini malformation. The primary outcome for patients with Mondini malformation are similar to those with normal cochlear structure following the multi-channel cochlear implantation.     

Cochlear implants in children with enlargement of vestibular aqueduct]

OBJECTIVE: To describe the clinical experiences with multi-channel cochlear implantation in the children with enlargement of vestibular aqueduct. METHOD: Fifteen patients were diagnosed with enlargement of vestibular aqueduct and received multi-channel cochlear implantation at Beijing Tongren Hospital. A cochleostomy was performed for all patients by a standard facial recess approach to anterior inferior to the round window niche. RESULTS: (1) Most of the electrodes were totally inserted in the cochlear, and gusher appeared in 6 implantations. (2) No serious complications occurred after implantation. (3) All patients have auditory sensations after implantations. (4) The hearing threshold in this series were similar to that of the normal cochlear implantation (the average threshold was 30 dB). (5) The rehabilitation concept was developed by Rehabilitation Research Center for Deaf Children in Beijing and Beijing Tongren Hospital. The training sessions included sound detection, word discrimination, auditory comprehension and spoken language skill development. The abilities of speech discrimination and spoken language were improved comparing with that of pre-operation. Two of the patients could go to the normal school after implantations. CONCLUSION: The outcome of hearing rehabilitation for children with enlargement of vestibular aqueduct were similar to those children with "normal" cochlear structure followed the multi-channel cochlear implantation.     

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。     

Audiological performance after cochlear implantation in children with inner ear malformations

OBJECTIVES: To prove that cochlear implantation is a beneficial method of rehabilitation in deaf children with malformations of the inner ear. DESIGN: The evaluation of auditory responses to speech (EARS) test battery was performed on the children in this study after an average implant use of 3 years. RESULTS: Individual results of six children with inner ear anomalies receiving cochlear implants are presented in this study. Three of the patients showed an incomplete partition (Mondini dysplasia), one had a cochlear hypoplasia and two suffered from an intraoperative cerebrospinal fluid leak. The majority of the children in this study are successful implant users. Wherever possible, test scores are included and subjective case reports given. CONCLUSIONS: Results are similar to those in children with normal cochleas, therefore inner ear malformations found in as many as 20% of patients with congenital sensorineural hearing loss are no contraindication for cochlear implantation. Nevertheless, factors influencing the success of implantation are multiple, including a thorough preoperative radiological examination, a well-performed surgery and an individually tailored postoperative rehabilitation programme.     

前庭水管扩大综合征患者的人工耳蜗植入术

目的　评价前庭水管扩大综合征患者人工耳蜗植入术的安全性和可行性。方法　 1995年 5月 1日～ 2 0 0 2年 6月 1日因双耳重 极重度感音神经性聋在北京协和医院接受人工耳蜗植入术的患者 312例中诊断为双耳前庭水管扩大者 10例 (3 2 % ) ,其中语前聋 7例 ,语后聋 3例。回顾性分析这 10例患者的临床资料。结果　 10例患者人工耳蜗电极植入顺利 ,8例耳蜗底回开窗时发生轻度井喷 ,迅速用颞肌筋膜牢固封闭圆窗制止井喷。全部患者术后未出现脑脊液漏、颅内感染、面瘫、中耳炎等并发症。开机 6个月时 8例患者具有开放性言语识别力 ,已进入普通学校 (幼儿园、小学、大学 )就读。另 2例语前聋的幼儿视觉强化测听听阈达 4 0dBHL ,与其他无内耳畸形的全聋儿童术后效果差异无显著性。全聋前语言能力较好的 5例患者术后语言能力明显好于语前聋的患者 ,语言交流基本听不出聋人特有的语音特征。另 5例患者语言均有不同程度的进步。结论　尽管前庭水管扩大患者在人工耳蜗植入术中可能出现井喷 ,但术后未出现并发症且听力 言语康复效果好 ,因此重 极重度聋的前庭水管扩大综合征患者行人工耳蜗植入术是安全可行的