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。     

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.     

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。     

内耳畸形语前聋患儿人工耳蜗术后康复效果分析

目的　评估内耳畸形语前聋患儿人工耳蜗植入术后康复结果。方法　回顾性分析术前影像学检查为内耳畸形语前聋人工耳蜗21例,并筛选背景接近的21例正常耳蜗结构语前聋人工耳蜗植入患儿配对组合。对患者术前、术后6个月及术后1年3个阶段听力言语康复效果进行评估。结果　9例前庭导水管扩大综合征和6例Mondini 畸形患者与耳蜗结构正常者相比,听觉言语结果无明显差异。畸形比较严重的1例Mondini和5例共同腔患者与耳蜗正常者相比,听觉言语结果有明显的差异。结论　前庭导水管扩大综合征患者人工耳蜗术后效果与耳蜗发育正常者相似,但对严重的Mondini 畸形和共同腔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,以助于术后制定康复方案。     

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。     

人工耳蜗再植入病例分析

目的 报告人工耳蜗再植入的常见原因、手术方法及再植入术后患者听力语言康复情况.方法 2007年5月～2010年11月北京友谊医院采用面隐窝进路为23例患者行人工耳蜗再植入,分析其再植入原因及手术方法.结果 23例患者均顺利完成人工耳蜗再植入,再植入术后患者听力言语康复效果满意或正在康复机构接受康复.再次植入的原因分别为:电极植入下鼓室1例;植入体部分破碎1例;电极部分脱出1例;电极从外耳道后壁穿出1例,磁铁移位1例;耳蜗底转骨化致植入失败1例;皮瓣或切口感染4例;耳蜗植入体故障7例;原因不明者3例;电极阻抗无限大2例;左侧耳蜗植入后无反应再植入右侧1例.结论 人工耳蜗再植入的原因多样且较复杂,再植入手术需要注意蜗内纤维化和骨化导致电极植入困难的问题.术前准确评估及患者术后避免头部剧烈碰撞是避免人工耳蜗再植入的关键.     

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。     

内耳畸形人工耳蜗植入术后听力言语康复效果分析

目的：使用听觉和言语问卷分级及术后产生听觉的最小电流值（T值）的方法,评估并比较内耳畸形与正常解剖结构语前聋患儿人工耳蜗植入术后的听觉言语康复效果。方法：按术前影像学检查将语前聋人工耳蜗植入患儿分为正常结构组和内耳畸形组,并配对组合,对思儿家长进行问卷形式调查随访,对术后听力及言语康复效果进行评估分析,记录术后1年凋机T值。用秩和检验比较2组听觉行为分级标准（CAP）、言语可懂度分级标准（SIR）结果及T值。结果：人工耳蜗植入患儿家长术前主要担心术后效果不理想及手术并发症的发生,多数认为听力言语康复训练应由医疗机构进行;秩和检验显示：2组CAP及SIR均无显著差异,术后1年调机时产生听觉的T值无显著差异。结论：①内耳畸形患儿人工耳蜗植入术后,经正规康复训练,听力言语康复效果与内耳解剖结构正常植入者相同,人工耳蜗植入术可帮助伴耳蜗畸形的极重度感音神经性聋患者重建听力,重返主流社会;②听力言语康复训练尚有很多方面需要改进。     

EABR辅助31例Mondini畸形人工耳蜗植入及术后效果分析

目的探讨电刺激听觉脑干诱发电位(electrical evoked auditory brainstem responses,EABR)辅助Mondini畸形患者人工耳蜗植入手术的方法及术后效果分析。方法我院自2001年开始为Mondini畸形患者植入人工耳蜗,收集2012年至2015年在我院行人工耳蜗手术的Mondini畸形患者31例,回顾性分析Mondini畸形患者术前听力学特征,术前EABR特点,术中手术特点、术后NRT测试结果及开机调试结果,随访术后听觉言语康复能力CAP(categories of auditory performance)和SIR(speech intelligibility rating)评分。另选择相似耳聋年龄的耳蜗结构正常耳蜗植入患者20例作为正常对照组,测试结果进行对比分析。结果 31例Mondini畸形患者术前听力均为极重度感音神经性聋,其中14例无残余听力,对照组7例无残余听力,术前行EABR测试,引出率100%,有反应者植入不同类型电极的人工耳蜗;术中电极全部植入耳蜗内,其中4例出现脑脊液井喷,颞肌封堵,术后无面瘫、脑脊液漏、脑膜炎等手术并发症;所有Mondini畸形患者术后均获得听觉,开机测试神经反射遥测(neural response telemetry,NRT),引出率41.94%低于对照组95%;开机6月平均T值Mondini组171.37±13.14CL;对照组145.34±17.43CL;动态范围Mondini组33.24±10.73CL,对照组43.55±15.36CL,经统计学分析P<0.05,有显著差异;随访6月~4年,Mondini组CAP平均得分6.13±1.15,SIR平均得分3.05±0.62;对照组CAP平均得分7.12±0.80,SIR平均得分4.03±0.91,经两样本比较t检验,P<0.05有显著性差异。结论 Mondini畸形的双侧极重度感音神经性聋患者可以根据残余听力情况在EABR辅助下行人工耳蜗植入,术后听力言语康复较正常耳蜗结构患者效果稍差,但明显好于术前听觉和言语能力。     

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。     

Nucleus 24 Contour在极重度聋患者中的临床应用

目的探讨Nucleus24Contour在极重度聋患者中的手术置入方法及其术后产生电听觉所需的电流量。方法对使用Nucleus24Contour组及Nucleus24M组患者的手术方法及术后产生电听觉所需要电流量进行比较。结果82例患者中,只有1例患者置入电极28环,其余81例患者32环电极全部置入。31例患者于耳蜗开窗时有少量淋巴液流出,2例耳蜗骨化者耳蜗开窗时未见淋巴液。术后7例患者出现较轻微的并发症。使用Nucleus24Contour组术中测得神经反应测试阈值明显减低,且在第一通道有统计学意义(P<0.05)。结论Nucleus24Contour手术置入容易,创伤小,耗电量少,是极重度感音神经性聋患者新型的、安全有效的置入物。     

内耳畸形聋儿人工耳蜗植入术常见类型及手术并发症

目的 探讨内耳畸形聋儿实施人工耳蜗植入术时常见的类型及并发症。方法 回顾性分析电子耳蜗植入术病历资料170例,对其中的32例双侧内耳畸形患者加以畸形类型及手术并发症总结。结果 ①人工耳蜗植入患儿内耳畸形所占比例(32/170,18.8%)明显高于其他文献报道;②32例内耳畸形中,大前庭导水管23例(占全部畸形数71.3%),大前庭导水管伴其他类型畸形者5例(并发Mondini畸形4例,并发外半规管未发育1例),Mondini畸形2例,Mondini畸形并发外半规管未发育前庭腔扩大1例,耳蜗CT影像疑似为“三叉”无法分类1例;③术中发生严重井喷3例(耳蜗CT影像疑似为“三叉”畸形、Mondini畸形并发外半规管未发育前庭腔扩大1例,及大前庭导水管并发Mondini畸形1例);④耳蜗影像疑似为“三叉”患者,术中发生严重井喷,电极植入困难,4个电极不能植入,术后听力未改善,半年后行对侧耳植入成功;⑤Mondini畸形并发外半规管未发育前庭腔扩大患儿术后半年并发脑脊液耳鼻漏、反复脑膜炎发作,术后1年行手术探查,后治愈。结论 ①人工耳蜗植入常见的内耳畸形包括,大前庭导水管综合征及其相伴发或单发的各类内耳畸形;②内耳畸形非人工耳蜗植入术的绝对禁忌证,但术中严重井喷多见,电极植入不完全多见,术后脑脊液耳鼻漏并发脑膜炎也多发生于畸形耳蜗,术前详细的影像学检查可以对各类畸形进行详细分类,并在术前对手术难度有充分的准备,可以减少相关并发症的发生。     

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。     

Suprameatal approach for cochlear implantation in 45 Chinese children

OBJECTIVE: To investigate the feasibility of applying the suprameatal approach (SMA) for cochlear implantation in Chinese children with profound sensory hearing loss, and to demonstrate a technical modification incorporated in the procedure due to an observed racial difference. STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Forty-five Chinese children (total 47 ears) with profound sensory hearing loss were surgically treated from May 2005 to May 2006. The patients were followed anywhere from 1 month to 20 months post-surgery, with 30 patients being followed for more than 6 months. INTERVENTIONS: All patients received cochlear implantation through the suprameatal approach. In this procedure, the cochleostomy was performed in one stage after the suprameatal tunnel was finished, rather than the two-stage approach described by Kronenberg (who firstly introduced the suprameatal approach). Three patients with low-lying dura (which is considered to be the contraindication for cochlear implantation with SMA) were treated with a further modified surgical approach. RESULTS: Among the 47 ears, full electrode pairs were completely inserted in 45 ears without surgical difficulties, but 1 ear was only fitted with 9 pairs of electrodes because of an ossified cochlea, and another with just 8 pairs of electrodes due to serious cochlear dysplasia. An intraoperative "gusher" occurred in the dysplasia case, and a small piece of temporalis muscle was used, along with biology glue, to seal the cochleostomy and prevent further leakage. In 1 case, the electrode was inserted into the cochlea through the tunnel lateral to the chorda tympani because adhesion had occurred between the incus and chorda tympani. There were no postoperative complications in any case. Thirty cases exhibited better hearing or speech development from cochlear implantation after more than 6 months of follow-up. CONCLUSIONS: The SMA was found to be a simple and safe technique for cochlear implantation in Chinese children. It enables wide exposure of the middle ear, and is especially suitable for cases with a narrow facial recess, an anteriorly located facial nerve, or an ossified cochlea. It is almost impossible to injure the facial nerve or the chorda tympani nerve. The cochleostomy can be performed in one stage in those patients with a normal cochlea. With some modifications, a low-lying dura will not be the absolute contraindication of SMA.     

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.     

人工耳蜗植入术中发生脑脊液“井喷”病例的影像学分析

目的分析人工耳蜗植入术中发生脑脊液“井喷”的原因及影像学特点。方法回顾性分析1998年1月～2011年7月2238例人工耳蜗植入患者中,发生脑脊液井喷的134例患者术前的影像学特征。结果134例人工耳蜗植入术中发生脑脊液“井喷”的病例中,大前庭导水管合并与内耳有骨性交通11例,大前庭导水管伴Mondini畸形合并内听道与内耳骨性交通28例,Mondini畸形伴内听道底与内耳骨性交通88例,耳蜗与内听道共同腔畸形7例。结论伴有内听道底骨缺损与内耳相通的任何内耳畸形患者,人工耳蜗植入术中必然发生脑脊液“井喷”。     

Congenital malformation of the inner ear and pediatric cochlear implantation.

OBJECTIVES: To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears. STUDY DESIGN: Clinical and audiometric evaluation in 13 patients. METHODS: Patient data concerning surgery, postoperative follow-up, and pre- and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included. SETTING: Tertiary referral center. PATIENTS: The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity). RESULTS: Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation. CONCLUSIONS: Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.