脑膜炎后全聋病人多通道人工耳蜗植入术

目的：评价脑膜炎后全聋病人人工耳蜗植入术的可行性。方法：回顾性分析经我科诊治的3例脑膜炎后全聋病人人工耳蜗植入，术中情况与术后听力学效果。结果：3例脑膜炎后全聋病人均有耳蜗底转轻度骨化，经面神经隐窝进路行人工耳蜗植入术，术中磨除耳蜗底转骨化部分，电极全部植入蜗内，未出现并发症。术后声场听阈与无内耳畸形或耳蜗骨化的病人比较无显著差异。结论：脑膜炎后全聋病人耳蜗骨化发生率高，手术有一定难度，但术后听力效果好，因此脑膜炎后全聋耳蜗轻度骨化时人工耳蜗植入术是可行的。     

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。     

1448例人工耳蜗植入手术中疑难问题的总结

目的通过对1448例人工耳蜗植入手术的总结,讨论人工耳蜗植入术中疑难问题,为提高人工耳蜗植入手术的成功率提供参考。方法1448例人工耳蜗植入手术病例资料来源于1998年1月～2009年3月。术前信息年龄8．5个月～51．2岁,平均年龄为4．57。全部为双耳重度和极重度感音神经性聋,其中语前聋患者1430例、成人语后聋患者18例。术前进行常规检查包括：①听力学检查、影像学检查、小儿肢体智力发育评估。②全麻常规检查。手术方法：①按常规面神经隐窝入路自耳蜗底转鼓阶植入耳蜗电极。②自乳突入路从耳蜗与前庭共同腔后下植入耳蜗电极。③在开放式乳突腔内埋藏电极线后仍于圆窗入路植入耳蜗电极。手术疑难判断方法：①无法按常规面隐窝入路手术者。②由于乙状窦、天盖、外耳道壁等解剖问题不能顺利完成乳突轮廓化者。③由干面神经、鼓索神经、外耳道骨壁的解剖问题使开放面隐窝的空间狭小导致暴露圆窗困难者。④耳蜗或圆窗等骨化或畸形等问题不能按常规自圆窗口部顺利植入耳蜗电极者。结果①手术成功率：1448例人工耳蜗植入手术均～期成功,成功率为100％。②术中疑难问题：术中疑难问题共241例（耳）,占16．64％,其中非常规手术入路10例,乙状窦前移影响手术入路4例,面神经垂直部高位并前移使与鼓素神经或外耳道壁的距离狭小影响圆窗的暴露179例,耳蜗底转骨化影响打开耳蜗鼓阶23例,圆窗骨化无法以圆窗为标记打开耳蜗鼓阶15例,耳蜗海绵状改变判断鼓阶正确位置困难5例,耳蜗与内听道共同腔耳蜗电极在共同腔内正确摆放困难5例。结论对1448例人工耳蜗植入手术总结体会认为,人工耳蜗植入手术中的疑难问题主要表现为：①面神经垂直部过度前移影响圆窗的暴露。②耳蜗骨化或海绵状改变影响开放耳蜗     

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。     

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。     

耳蜗性耳硬化症人工耳蜗植入的应用研究

分析耳蜗性耳硬化症患者的临床特点、人工耳蜗植入术中情况及人工耳蜗植入术后听觉言语康复效果,为该疾病的诊治提供参考。方法分析4例耳蜗性耳硬化症患者病史资料、听力学检查结果及影像学结果,观察人工耳蜗植入手术的术中所见,并定期对4例患者进行听力学及影像学随访。结果①所有患者人工耳蜗电极均经圆窗膜径路完全植入鼓阶。其中1例于术中发现镫骨完全固定,导致术中鼓阶开孔时外淋巴波动不明显;1例发现圆窗膜骨化,术中鼓阶开孔定位困难;其余2例患者镫骨活动好,圆窗结构清晰。所有患者术中电极阻抗检测均正常,且引出标准的神经反应遥测波形;②术后随访1~5年无术后并发症出现,声场测听示平均听阈为40.8 dBHL,平均言语识别率为77.3%,言语及交流能力较术前提高;颞骨高分辨率CT提示双侧内耳病变范围无明显进展。结论耳蜗性耳硬化症进展缓慢,严重时可导致重度/极重度感音神经性聋,当使用助听器无效时,人工耳蜗植入能帮助患者获得较满意的听觉康复效果。     

不同内耳畸形人工耳蜗植入效果分析

目的 就不同内耳畸形人工耳蜗植入特点及效果进行分析,积累经验。方法 1996年1月～2004年5月北京同仁医院共开展多道人工耳蜗植入术410例(410耳),对其中双侧内耳畸形患者82例(82耳)进行回顾性分析,就不同原因内耳畸形的人工耳蜗植入手术特点及术后开机调试特点与耳蜗形态正常的人工耳蜗植入者进行了比较。结果 ①所有内耳畸形患者人工耳蜗植入术后都有听觉;②内耳畸形人工耳蜗术中容易出现井喷;③82例中除2例患者各遗留2对蜗外电极外,其余电极均完全植入蜗内;④术后无面瘫、脑脊液漏等并发症;⑤术后开机调试数值与结构正常植入者接近,无统计学差异(P>0.05);⑥术后经过语训,1年后听力及言语能力均有不同程度的提高。结论 伴有内耳发育畸形的深度感音性聋患者可以行人工耳蜗植入手术,植入效果与耳蜗发育正常患者的人工耳蜗植入效果基本一致,人工耳蜗植入术可以作为伴有内耳畸形的深度感音性聋患者的治疗和康复手段。     

先天性外耳道骨性闭锁患者人工耳蜗植入的手术分析

目的探讨先天性外耳道骨性闭锁行人工耳蜗植入患者的听力学检查、影像学特征及手术径路的选择。方法收集2015年7月—2019年1月诊治的5例先天性外耳道骨性闭锁行人工耳蜗植入术的患者,回顾性分析其病史、听力学检查、影像学特征及手术径路。结果5例患者听力均表现为重度感音神经性聋。影像学表现为外耳道骨性闭锁,伴听骨链畸形,面神经走形异常,均无内耳畸形。5例患者均采用鼓窦径路,其中2例患者经面神经后下植入人工耳蜗。所有患者均为圆窗膜植入,植入电极过程顺利,术后开机反应佳。结论针对外耳道骨性闭锁畸形需行人工耳蜗植入的患者,应采用外耳道骨性闭锁的鼓窦径路的手术方式,开放部分乳突及鼓窦,取出畸形的听骨链,在此基础上,进一步暴露圆窗龛。对于面神经乳突段前移,完全遮挡圆窗龛的患者,则可转经面神经后下暴露圆窗龛。     

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。     

先天性内耳畸形患者多通道人工耳蜗植入的效果观察

目的探讨多通道人工耳蜗在先天性内耳畸形患者植入的效果.方法对16例先天性内耳畸形患者(畸形组,Mondini畸形12例,大前庭导水管综合征4例),2例经前庭窗植入电极,3例经鼓岬植入电极,11例经圆窗龛前上缘植入电极.2例术中发生镫井喷.以10例耳蜗发育正常的植入者作为对照组,进行术后效果对比.结果畸形组与对照组术中和术后并发症差异无显著性意义.术后听阈畸形组多数患者达到30～40 dB HL,与正常组相似,仅少数患者听阈稍高.经统计学分析差异无显著性意义(P>0.05).听觉语言康复效果畸形组多数与对照组接近.结论多通道人工耳蜗植入适用于绝大多数先天性内耳畸形的患者,术后听力及语言康复效果满意.     

内耳畸形与耳蜗骨化患者的耳蜗植入

目的探讨先天性内耳畸形与耳蜗骨化患者耳蜗植入术的有关问题.方法对我院2002年10月～2004年2月间行耳蜗植入术的8例内耳畸形和2例耳蜗骨化的患者进行回顾性分析.结果4例大前庭水管综合征患者术中有外淋巴液搏动;3例Mondini畸形和1例共同腔畸形患者术中出现井喷;1例耳蜗部分骨化患者术中误将电极插入内听道后纠正;另一例耳蜗部分骨化患者植人短电极.1例Mondini畸形患者术后发生少量脑脊液耳鼻漏,保守治疗3月后痊愈,其余患者无并发症.所有患者均成功开机.结论对于内耳畸形或耳蜗骨化的患者,耳蜗植入术前详细的影像学评估,对术中困难的充分估计和正确、规范处理是手术成功的基本条件.     

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。     

Follow-up of cochlear implant use in patients who developed bacterial meningitis following cochlear implantation

Objectives/Hypothesis: The present study is a long‐term follow‐up of speech perception outcomes and cochlear implant use in three cases of meningitis that occurred after cochlear implantation. Study Design: Case series study. Methods: Study was performed on three children implanted with different models of Clarion® devices, two of them with positioner. Recognition and comprehension were assessed via the Italian adaptation of GASP (TAP) test, and phonetically balanced bi‐syllabic words in open‐set. High resolution computed tomography scan acquisition was performed to obtain axial coronal and oblique multiplanar reconstructions of the cochlea. Results: Two patients were affected by enlarged cochlear acqueduct and Mondini malformation the first carrying positioner. One patient had a normal cochlea, and the positioner could have been the main cause of bacterial spread. As a consequence of meningitis the child with normal cochlea and the other with enlarged vestibular acqueduct developed cochlear ossification, increased M‐level and worsening of hearing outcomes. The child with Mondini malformation developed facial nerve stimulation. Contralateral implantation was performed in the first two patients. Conclusion: Bacterial meningitis occurring after cochlear implantation may induce cochlear ossification, facial nerve stimulation, and permanent or temporary loss of implant use. Planned follow‐up with high resolution computed tomography and evaluation of M‐levels could be useful prognostic tools in the management of these patients.     

The influence of post-meningitic obliteration and ossification of the cochlea on cochlear microphonics

Cochlear microphonics are electrical stimulus responses of the inner ear. They are generated by mechanical displacement of the hair cells caused by acoustic stimulation and can be recorded from the cochlear promontory via a needle electrode. In individuals with post-meningitic deafness, fibrous obliteration or ossification of the cochlea may occur. The aim of the present investigation was to establish whether obliteration or ossification leads to any alteration in the extent to which cochlear microphonics can be recorded (as compared with that in patients with a fluid-filled cochlea whose deafness was not post-meningitic), as an indication of changes in the mechanical displacement of the remaining hair cells. Cochlear microphonics in 15 individuals with proven obliteration or ossification of the cochlea were compared with those in 15 control subjects (deaf individuals whose hearing loss was not post-meningitic, and who had a fluid-filled cochlea). Our findings reveal no statistically significant differences between the two groups in the degree to which cochlear microphonics can be recorded. This study does not demonstrate evidence of a change in this property due to ossification or obliteration of the cochlea.     

耳蜗骨化患者人工耳蜗植入术

目的介绍耳蜗骨化患者人工耳蜗植入术中情况与术后效果，探讨耳蜗骨化时人工耳蜗植入术的可行性。方法回顾性分析31例耳蜗骨化患者人工耳蜗植入术中和术后的临床资料。结果1995年5月至2005年7月因重度和极重度感音神经性聋接受人工耳蜗植入术的患者720例，术中诊断耳蜗骨化31例（4．3％），其中男14例，女17例；手术时患者年龄1．4～59．0岁，平均13．2岁。轻度骨化27例，严重骨化4例。全部患者均经乳突一面隐窝进路完成人工耳蜗植入术。术中和术后均未出现并发症。患者术后声场听阈均达30～40dB，与无耳蜗骨化的患者相比差异无统计学意义（P〉0．05）。结论尽管耳蜗骨化时人工耳蜗植入手术难度很大，但耳蜗骨化时经常规乳突一面隐窝进路仍能完成电极植入，且轻度耳蜗骨化时能将电极全部植入蜗内，并且对电极损伤极小，术后听力效果好，因此人工耳蜗植入术对于耳蜗轻度骨化具有可行性。     

脑膜炎后耳蜗骨化患者人工耳蜗植入的临床分析

目的总结因脑膜炎而致严重感音神经性听力损失的人工耳蜗植入经验,探讨其诊疗策略。方法回顾性分析2010年9月—2020年9月于首都医科大学附属北京同仁医院耳鼻咽喉头颈外科因脑膜炎致重度及极重度感音神经性听力损失进行人工耳蜗植入的22例患者资料,其中成人13例,儿童9例。对其术前影像学、手术所见进行分析。结果颞骨HRCT检查中16例(72.7%)诊断耳蜗骨化;6例(27.3%)未诊断,但经手术探查证实存在耳蜗骨化。18例内耳MRI检查中,3例(16.7%)未发现耳蜗信号改变,而经手术探查存在耳蜗骨化。两者结合后检出率为90.9%(20/22),20例(90.9%)可见合并半规管等其他迷路病变。术中未见耳蜗骨化者1例(4.5%),圆窗骨化7例(31.8%),耳蜗底转骨化14例(63.6%)。电极完全植入者18例(81.8%),部分植入者4例（18.2%）。结论脑膜炎后耳蜗骨化可致严重感音神经性听力损失,人工耳蜗是理想的治疗策略,建议尽早植入。术前颞骨HRCT和MRI对确定是否适合耳蜗植入和术前计划至关重要,诊断存在假阴性,但联合检查可大大提高耳蜗骨化的术前诊断阳性率。     

The vienna cochlear implant in patients with obliteration of the cochlea

Twelve deaf patients with obliterated or ossified cochleas received the extracochlear version of the Vienna Cochlear Implant. Four patients, 1 of them a child, developed open speech comprehension. Obliteration of the cochlea could not always be predicted by conventional tomography of the temporal bone. Short duration of deafness, wide dynamic range, and good ability of time resolution (small temporal difference limen [TDL]) are predictors for good postoperative results. Obliteration or ossification of the cochlea per se is no contraindication to cochlear implantation.     

Intraoperative CT-guided cochlear implantation in congenital ear deformity

Abstract Conclusions: Intraoperative computed tomography (iCT)-guided cochlear implantation is practical and effective for correct electrode placement in the cochlea of patients with congenital inner ear and/or complex middle ear malformation. Objectives: The operation in patients with inner ear and/or complex middle ear malformation including abnormal facial nerve course is difficult. This study evaluated the efficacy of cochlear implantation under the guidance of iCT to insure correct electrode placement. Methods: This was a prospective interventional case series. Ten patients with severe to profound sensorineural hearing loss due to ear malformations were enrolled, and iCT was used to confirm the right placement of electrodes. Results: Intraoperative CT was performed three times in one patient, twice in two, and once in the others. Interruption of the surgical process for each iCT until resumption of surgery was 9.64 ± 0.63 min. iCT revealed incorrectly positioned cochlear implants in two patients, which were immediately corrected. There were no reoperations due to misplacement of electrodes. iCT helped locate the cochlea in the middle ear of one patient with an abnormal facial nerve course. The overall intervention rate based on iCT findings was 30%. Level of evidence: level 4.     

Electrode migration after cochlear implantation.

OBJECTIVE: To review the occurrence of electrode migration after cochlear implantation. STUDY DESIGN: Review of public database and retrospective case series. SETTING: Tertiary academic referral center, ambulatory. PATIENTS: Retrospective review of electrode migration reported in association with the US Food and Drug Administration Manufacturer and User Facility Device Experience database and a single-institution case series between 1996 and 2006. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURE: Occurrence of electrode migration. RESULTS: During the period 1996 to 2006, 151 reports of electrode migration were filed in the Manufacturer and User Facility Device Experience database. The most common association with migration was cochlear ossification, although 127 of 151 cases had no known association. During an equivalent period, 637 cochlear implant (CI) procedures were performed at the University of Miami. Of 580 primary CI operations, 2 cases of electrode migration were identified, both associated with reossification of a cochlea that was fully ossified at the first procedure. CONCLUSION: Electrode migration is an underrecognized complication of CI surgery and may be associated with cochlear ossification. Electrode stabilization techniques may reduce the incidence of CI electrode migration in nonossified or incompletely ossified cases.     

Auditory brainstem implant in a child with severely ossified cochlea

OBJECTIVE: The hearing outcome after implanting a severely ossified cochlea has always been less satisfactory than implanting a patent one. The aim of our study is to present a case where brainstem implantation was successfully performed as an alternative to cochlear implantation in a child with bilateral severe ossification of the cochlea. STUDY DESIGN: Case presentation. This study was conducted at Gruppo Otologico, Rome, Italy, a private referral center for neurotology and skull base surgery. METHODS: The subject of our study was a 12-year-old female child with postmeningitic deafness and bilaterally ossified cochleae. This case is the first brainstem implantation performed at our center with the indication of severe ossification of the cochlea. RESULTS: Successful brainstem implantation of a device was carried out, and the hearing of the patient was restored to the degree that she can freely use the telephone after 8 months of implantation. CONCLUSION: Although more cases are needed before establishing the exact outcome of brainstem implantation in cases of deafness in the presence of severe bilateral cochlear ossification, preliminary results show the superiority of brainstem implants to conventional or even customized cochlear implants.