慢性化脓性中耳炎患者的1期和分期人工耳蜗植入

目的：对合并慢性化脓性中耳炎的人工耳蜗植入的手术方式和手术分期进行总结和探讨,为慢性化脓性中耳炎患者的人工耳蜗植入提供参考。方法：回顾分析2009—06—2013-03期间我院行合并慢性化脓性中耳炎的人工耳蜗植入6例语后聋患者的临床资料,对手术分期选择、手术技巧、可能存在的风险及疗效进行分析和总结。结果：6例患者中3例接受1期颞骨岩部次全切除、人工耳蜗植入术。3例1期接受颞骨岩部次全切除,4～6个月后2期行人工耳蜗植入术。6例患者随访时间3～12个月。全部接受人工耳蜗植人手术未出现并发症,获得良好的开放语言识别能力。结论：对于合并慢性化脓性中耳炎的人工耳蜗植入,分期手术作为首选,1期人工耳蜗植入存在潜在的风险,应该慎重采用。手术成功的关键是彻底清理病变,这对手术技巧和显微镜等手术设备提出较高的要求。     

中耳炎性病变患者的人工耳蜗植入

目的探讨伴中耳炎性病变的患者进行人工耳蜗植入的适应证、手术方法和术后并发症的发生情况。方法对1995年5月至2006年2月完成的866例人工耳蜗植入术的临床资料进行回顾性分析。术前或术中发现伴中耳乳突炎性病变患者共41例，其中男17例，女24例，年龄1岁3个月-38岁，平均10岁2个月；左耳人工耳蜗植入18例，右耳植入23例，均为单侧植入。其中慢性分泌性中耳炎13例，隐蔽性中耳炎18例，中耳炎后遗症鼓膜穿孔1例，双侧胆脂瘤中耳炎2例，中耳乳突肉芽肿7例。中耳炎性病变伴内耳、中耳畸形7例。对于双侧胆脂瘤中耳炎乳突根治术后患者，设计采用了带蒂翻转帽状腱膜法保护植入电极和面神经。结果41例患者经 Ⅰ期或分期手术，术后随访时间5个月至6年11个月，平均未发现耳部炎性并发症，装置工作良好。结论慢性非化脓性中耳炎、中耳乳突肉芽肿、中耳炎后遗症伴鼓膜穿孔者可采用Ⅰ期或分期方法行人工耳蜗植入。胆脂瘤中耳炎在彻底去除病变基础上可分期行人工耳蜗植入术。对于乳突根治术后没有足够组织覆盖保护植入电极的病例，可采用带蒂翻转帽状腱膜方法。存在活动性中耳乳突炎症者为手术禁忌证。中耳炎性病变患者人工耳蜗术后应长期随访。     

哈尔滨医科大学附属第二医院耳鼻咽喉头颈外科

耳科开展乳突根治手术和各种类型的鼓室成形手术治疗慢性化脓性中耳炎；颞内段面神经减压术和面神经移植术治疗周围性面瘫：1999年开展了颞颈联合进路颈静脉球体瘤切除术：2002年开展了经迷路后进路听神经瘤切除术，均取得良好疗效。2002年开展了全省第1例人工耳蜗植人手术，并于2004年建立了人工耳蜗植入基地，目前已经为近70例患者成功植入了人工耳蜗，成功率为100％。人工耳蜗植人手术开展的同时也带动了听力学检测技术的发展，能够配合完成人工耳蜗植入术前听力学评估、术中监测及术后开机调试，使人工耳蜗植人工作更加完善。     

22导人工耳蜗植入4例报告

对3例语后聋、1例请前聋患者进行22导人工耳蜗植入，4例耳聋的原因分别为慢性中耳炎、分娩后不明原因、耳毒性药物和先天性聋，术前CT等检查未见耳蜗异常，但术中发现迷路严重骨化和耳蜗畸形各1例。水后1．0～1．5月进行开机调试，均恢复了听力和有满意的语言分辨率。文中对人工耳蜗植入的有关问题进行了讨论。     

人工耳蜗植入术前听神经完整性的评估

目的 探讨、评估人工耳蜗植入候选者术前听神经完整性的检查方法;在对侧耳人工耳蜗植入术前,客观检查耳蜗发育及病变状态的检查方法。方法 对12例人工耳蜗植入候选者进行检查,根据本小组建立的人工耳蜗植入术前耳蜗影像学检查方法、术前听神经完整性检查方法及评估标准、以及术后植入电极位置的早期检查方法,结合11例人工耳蜗植入术后听觉康复疗效进行分析。结果 11例患儿满足本小组制定的人工耳蜗植入遴选标准而接受了多导人工耳蜗植入,包括1例对侧耳(第2耳)人工耳蜗植入。一例患儿因评估结果为听神经发育障碍,未作人工耳蜗植入。所有人工耳蜗植入患儿术后听觉能力皆明显改善。结论 MRI头轴位及听神经轴位和颞骨CT薄层扫描、膜迷路表层实时三维重建及膜迷路实时重建等影像学方法,结合听力学及外耳道电刺激测听反应阈与不适阈差值的电生理方法来评估听神经的完整性,对术前预测人工耳蜗植入后患儿听觉康复的可能疗效,有非常重要的意义。术后早期反斯氏位X线摄片观察蜗内植入电极部位及植入电极形态,可作为人工耳蜗电极植入成功性直观、简捷的评估方法。     

人工耳蜗植入术前的影像学检查

目的 :探讨CT和MRI检查对人工耳蜗植入术前评估的价值。方法 :对 2 6例感音性耳聋患者均采用颞骨轴位高分辨率CT螺旋扫描、MR水成像及内耳三维重建。结果 :1 8例先天性感音性耳聋患儿中检出Mondini畸形Ⅰ型 1例 (2耳 ) ,Ⅱ型 1例 (2耳 ) ,内耳纤维化 1例 (2耳 ) ;5例语后聋患者中检出慢性化脓性中耳炎 1例 (2耳 ) ,内耳骨化 1例 (2耳 )。结论 :对于人工耳蜗植入术的术前评估 ,CT检查具有重要价值 ,必不可少 ,MRI检查是必要的补充。对内耳的三维重建 ,MRI优于CT。     

伴有分泌性中耳炎的人工耳蜗植入适应证与手术时机

目的 探讨伴有分泌性中耳炎的人工耳蜗植入手术适应证与手术时机.方法 对26例伴有分泌性中耳炎的重度或极莆度耳聋患儿行人工耳蜗植入手术,术前对分泌性中耳炎进行必要的非手术治疗,选择手术适应证和手术时机.人工耳蜗植入术中清理分泌性中耳炎病变、通畅引流,术后定期随访,同步进行人工耳蜗植入效果的评估.结果 26例伴有分泌性中耳炎的人工耳蜗植入者均进行单侧耳手术,手术一次成功,其中左耳5例,右耳21例.术后1个月开机调试,26例均有主观电听觉.术后随访13个月～4.3年,分泌性中耳炎痊愈19例,鼓膜形态正常、声导抗检查为A型鼓室压曲线;分泌性中耳炎好转7例,鼓膜轻度浑浊内陷,声导抗吃不开C型鼓室压曲线;无鼓膜穿孔、中耳粘连或分泌性中耳炎急性发作病例.结论 伴有分泌性中耳炎的人工耳蜗植入手术在严格掌握手术适应证的前提下,应及早手术,以争取术后听觉言语康复的最佳时期;通过术前必要的药物治疗、术中的病变处理与术后的治疗以及密切随访,都可以避免并发症的发生,达到人工耳蜗植入的预期效果.     

感音神经性聋并分泌性中耳炎患者一期人工耳蜗植入

目的探讨感音神经性聋并慢性分泌性中耳炎患者一期人工耳蜗植入的可行性。方法对就诊于中山大学孙逸仙纪念医院耳鼻咽喉科的2例慢性分泌性中耳炎并双侧极重度感音神经性聋患者行一期人工耳蜗植入术,观察植入效果及听力言语康复情况。结果 2例患者术中均顺利植入电极,术后随访均无分泌性中耳炎复发,听力言语能力恢复效果好。结论对于合并慢性分泌性中耳炎的感音神经性聋患者,在清除病变的基础上,同期行人工耳蜗植入是可行的。     

人工耳蜗植入术中迷路骨化与内耳畸形的处理

目的：对迷路骨化与内耳畸形患者进行听力康复。方法：人工耳蜗植入术。结果：术中发现内耳迷路骨化与先天性内耳畸形各１例。其中，迷路骨化继发于分娩后未定病因，术中见单侧前庭和耳蜗广泛骨化，后将耳蜗电有植入对侧未骨化的耳蜗；先天天性内耳畸形呈鼓岬骨质增厚，圆窗和卵圆窗骨性封闭并增厚，基底转起始部鼓阶扩张，予成功植入耳蜗电极。结论：人工耳蜗植入术中迷路骨化耳畸形时，应根据具体情况采取适当的措施，以保证手术取     

中耳感染性疾病Ⅰ期和分期人工耳蜗植入病例分析

目的总结中耳感染性疾病患者人工耳蜗植入(Cochlear Implant,CI)的临床经验。方法对2000年12月-2019年3月在解放军总医院行CI伴中耳感染性疾病患者的临床资料进行回顾性研究。对Ⅰ期或Ⅱ手术患者的中耳病变性质和范围、手术方式、术腔填塞物种类、手术疗效以及术后并发症等进行总结分析。结果术后随访9个月-10年,Ⅰ期植入11例,其中,慢性化脓性中耳炎静止期5例,活动期2例,中耳胆脂瘤4例(胆脂瘤范围较小,或有完整包膜)。Ⅱ期植入9例,其中,慢性化脓性中耳炎活动期6例,静止期1例;中耳胆脂瘤2例(胆脂瘤范围较大,或包绕正常组织,不易完整清除)。分期植入组中有1例耳蜗植入术后9年电极脱出。其余病例通过结合病例特点,采用不同手术方式均取得满意的治疗效果。结论对于中耳感染性疾病的患者,人工耳蜗分期植入相对于同期植入更加安全。Ⅰ期植入应尽可能选择病变较轻,病灶局限易完整清理的病例,术式选择应在彻底清除病灶的基础上尽量选择损伤范围较小的术式。     

术中圆窗耳蜗电图对人工耳蜗植入电极安全性评估

目的：分析人工耳蜗电极植入前后圆窗耳蜗电图（R W EcochG）中耳蜗微音电位（CM）阈值的变化,以客观评估人工耳蜗植入手术是否对耳蜗基底膜造成损伤。方法：对40例拟行人工耳蜗植入术的患者在全身麻醉下行R W EcochG测试,分析植入电极前后CM阈值的变化。结果：40例人工耳蜗植入患者中,39例电极植入前后CM阈值相近,且在个别频率植入电极后CM阈值减小5dB;仅有1例患者电极植入后CM阈值比植入前明显增加,术中植入电极时有阻力感。结论：术中RWEcochG测试可以客观评估人工耳蜗植入手术本身是否对耳蜗基底膜造成损伤。     

听神经-听觉通路完整性综合评估法

目的：探讨评估人工耳蜗植入候选者听神经-听觉通路完整性的方法及其临床应用价值。方法：应用本小组建立的听神经-听觉通路完整性综合评估法，对24例双侧耳极重度感音神经性聋的人工耳蜗植入候选者进行了检查和评估。听神经-听觉通路完整性综合评估法由①临床听力学；②放射影像学；③外耳道电刺激测听法；④日常环境声反应评估法；以及⑤言语发育反馈听力评估法等5个方面的检查构成。结果：24例人工耳蜗植入候选者中，有23例符合听神经—听觉通路完整性的标准而施行了人工耳蜗植入术，开机调试后全部人工耳蜗植入的患儿听阈皆降低(≥50dB)；另1例患儿听神经—听觉通路完整性的综合评估结果提示其双侧听神经缺如，避免了无意义的人工耳蜗植入。结论：本小组建立的听神经—听觉通路完整性综合评估法简便可行，结论可靠。听神经—听觉通路的完整性应列为人工耳蜗植入候选者适应证的一项必备条件。     

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。     

Usher type I syndrome in children: genotype/phenotype correlation and cochlear implant benefits

OBJECTIVE: To assess the benefit of cochlear implant in children presenting an Usher type 1 syndrome (speech understanding, speech production intelligibility, academic performance) and to search any correlation between the phenotype and the genotype in this population. MATERIALS AND METHODS: Retrospective case series analysis about 13 implanted Usher type I children. Cochlear implantation was performed from 1995 to 2005. Our population was divided in three groups: group 1 (implantation between 1 and 3 years of age); group 2 (implantation between 4 and 7 years of age) and group 3 (implantation between 14 and 17 years of age). Postoperative speech perception, speech production intelligibility and education settings were evaluated. RESULTS: Molecular genetic analysis was performed in 11 patients and pathogenic mutations were identified in all cases: (mutation in myosin 7A gene in 5 cases; mutation in cadherin 23 gene in 6 cases). Four new mutations 2 in the MYO7A gene and 2 in the CDH23 gene never reported before were found. Walking delay and hearing level were not statistically correlated with the genotype abnormalities found. The speech discrimination skills, the speech production intelligibility and the academic performance were better in the group 1 children than the group 2 children after cochlear implantation. All the children of group 1 but one were in mainstreaming education. Specific language impairment was identified in two children of group 1. The group 3 children could not achieve open-set perceptive tasks after implantation--only closed-set word test can be done and their speech production remained unintelligible after cochlear implantation. CONCLUSION: Molecular analysis of Usher type I syndrome can ascertain the diagnosis in spite of the genetic heterogeneity. In this study, clinical symptoms weren't correlated with genotypic mutations. Speech discrimination skills, speech production quality, and academic performance were correlated with the age at implant.     

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。     

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。     

人工耳蜗植入术后感染的因素及其生物学特点研究

目的:分析人工耳蜗植入术后感染的因素及其生物学特点,探讨如何减少术后感染及感染后的治疗措施。方法:回顾性分析2001-07-2011-10期间我院人工耳蜗植入中心接受耳蜗植入的患者发生术后感染的情况。结果:由同一术者完成的316例人工耳蜗植入手术,术后有5例发生感染,外院术后感染转来1例,共6例,均经治疗后痊愈,其中1例术后8年因植入体电极支撑小柱等因素感染取出植入体。结论:人工耳蜗植入术后感染致病菌多为金黄色葡萄球菌、铜绿假单胞菌等。细菌生物膜的形成是人工耳蜗植入术后感染的关键环节,采用化学制剂清除细菌生物膜,能够控制人工耳蜗术后感染,特别是皮瓣感染。人工耳蜗植入术后发生感染时并不一定非要取出植入体。采用积极围手术期干预,积极治疗术后感染,及时采取有效措施,是人工耳蜗植入术后言语康复的有效保障。     

The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease.

BACKGROUND: Gadolinium-enhanced magnetic resonance imaging (GdMRI) is routinely used in the evaluation and management of suspected retrocochlear pathology such as vestibular schwannoma. However, its value in the evaluation and diagnosis of cochlear pathology associated with sensorineural hearing loss (SNHL) has been less clear. STUDY DESIGN: Retrospective review of case histories and imaging studies of patients with SNHL and cochlear enhancement on GdMRI diagnosed between 1998 and 2000. RESULTS: Five patients with SNHL who required gadolinium administration to establish the diagnosis of endocochlear disease were identified. Diagnosed lesions included an intralabyrinthine schwannoma, intracochlear hemorrhage, radiation-induced ischemic change, autoimmune labyrinthitis, and meningogenic labyrinthitis. In these illustrative cases, the GdMRI demonstrated intrinsic high signal or contrast enhancement within the cochlea and labyrinth in the absence of a retrocochlear mass. In one patient with meningogenic labyrinthitis, cochlear enhancement on MRI led to prompt cochlear implantation before the potential development of cochlear ossification. CONCLUSION: Our experience suggests that GdMRI plays a crucial role in the diagnosis of cochlear pathology associated with sensorineural hearing loss and may directly impact patient management.     

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。     

Exploring factors that affect the age of cochlear implantation in children

Objective

Early access to sound through early cochlear implantation has been widely advocated for children who do not derive sufficient benefit from acoustic amplification. Early identification through newborn hearing screening should lead to earlier intervention including earlier cochlear implantation when appropriate. Despite earlier diagnosis and the trend towards early implantation, many children are still implanted well into their preschool years. The purpose of this study was to examine the factors that affected late cochlear implantation in children with early onset permanent sensorineural hearing loss.

Methods

Data were examined for 43 children with cochlear implants who were part of a group of 71 children with hearing loss enrolled in a Canadian outcomes study. Eighteen (41.9%) of the 43 children were identified through newborn screening and 25 (58.1%) through medical referral to audiology. Medical chart data were examined to determine age of hearing loss diagnosis, age at cochlear implant candidacy, and age at cochlear implantation. Detailed reviews were conducted to identify the factors that resulted in implantation more than 12 months after hearing loss confirmation.

Results

The median age of diagnosis of hearing loss for all 43 children was 9.0 (IQR: 5.1, 15.8) months and a median of 9.1 (IQR: 5.6, 26.8) months elapsed between diagnosis and unilateral cochlear implantation. The median age at identification for the screened groups was 3.3 months (IQR: 1.4, 7.1) but age at implantation (median 15.8 months: IQR: 5.6, 37.1) was highly variable. Eighteen of 43 children (41.9%) received a cochlear implant more than 12 months after initial hearing loss diagnosis. For many children, diagnosis of hearing loss was not equivalent to the determination of cochlear implant candidacy. Detailed reviews of audiologic profiles and study data indicated that late implantation could be accounted for primarily by progressive hearing loss (11 children), complex medical conditions (4 children) and other miscellaneous factors (3 children).

Conclusions

This study suggests that a substantial number of children will continue to receive cochlear implants well beyond their first birthday primarily due to progressive hearing loss. In addition, other medical conditions may contribute to delayed decisions in pediatric cochlear implantation.