"井喷"在内耳畸形人工耳蜗植入术中的发生率

目的：探讨“井喷”在内耳畸形人工耳蜗植入术中的发生率，为有“井喷”现象的人工耳蜗植入术积累临床经验。方法：对680例行人工耳蜗植入术患者行术前高分辨CT检查，显示80例双耳有内耳畸形，其中20例并发内耳道底骨性缺损与内耳相通，行人工耳蜗植入术。结果：20例（25％）并发内耳道底骨性缺损与内耳相通者，术中发生了“井喷”，其中大前庭导水管畸形者5例，Mondini畸形者11例，耳蜗前庭与内耳道共同腔畸形者4例；耳蜗、前庭、外半规管共同腔和前庭、外半规管共同腔畸形者无“井喷”发生。结论：①80例内耳畸形人工耳蜗植入术患者“井喷”的发生率为25％；②内耳畸形人工耳蜗植入术中“井喷”的发生率依次为：耳蜗、前庭与内耳道共同腔畸形，Mondini畸形，大前庭导水管畸形；③单纯前庭、耳蜗、外半规管共同腔畸形及半规管畸形者，人工耳蜗植入术中未发生“井喷”；④80例内耳畸形人工耳蜗植入术中，发生“井喷”者均存在内耳道底骨缺损，与内耳形成共同开放的通道。     

人工耳蜗植入术中因内耳畸形发生“井喷”的危险因素分析

目的 分析人工耳蜗植入术治疗内耳畸形中发生“井喷”的危险因素。方法 回顾性分析湘南学院附属医院耳鼻咽喉头颈外科及中南大学湘雅二医院耳鼻咽喉科2010年1月～2015年10月收治的25例内耳畸形行人工耳蜗植入术患者的病例资料。结果 8例患者术后出现“井喷”,其中植入右侧6例、左侧2例。畸形类别：双侧耳蜗内听道相通伴Mondini畸形或前庭导水管扩大分别为4例、2例,双侧耳蜗内听道不相通+前庭导水管扩大1例,右侧耳蜗内听道相通+左侧Michel畸形右侧Mondini畸形1例。25例患者术后均获得满意听力。结论 内耳畸形中前庭导水管扩大、Mondini畸形和耳蜗听道相通是人工耳蜗植入术中的常见危险因素,术前需行影像学检查,全面评估“井喷”发生风险,降低其发生率,确保手术效果及人工耳蜗听觉建立。     

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”。     

先天性内耳发育畸形患儿的人工耳蜗植入术

为评估先天性内耳发育畸形患儿行人工耳蜗植入的手术及听力学效果 ,回顾分析 1994～ 2 0 0 2年315例人工耳蜗植入手术患儿病例资料。总结 2 8例经颞骨高分辨率CT证实为先天性内耳发育畸形的手术病例 ,分析其CT影像、术中所见、术后并发症、闭合式及开放式言语识别率测试等。结果 :表现为耳蜗底转发育不全 (IP)、前庭导水管扩大(EVA)、前庭扩大综合征的病例 (如Mondini畸形 )以及单独的前庭导水管扩大或部分半规管发育不全的病例 ,均可获得较好的言语识别率。完全性半规管发育不全、单独的耳蜗底转发育不全、耳蜗不发育或空泡状的病例则…     

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.     

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     

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。     

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。     

蜗轴缺失内耳形态学表现与人工耳蜗植入入路的选择

目的：总结人工耳蜗植入手术患者耳蜗蜗轴缺失的类型和人工耳蜗植入手术入路的选择,为掌握人工耳蜗植入手术的适应证提供参考。方法：人工耳蜗植入患者按术前颞骨高分辨CT检查,将存在的耳蜗蜗轴发育缺失分为3种类型：1耳蜗发育不良形成的蜗轴缺失（Mondini畸形）;2耳蜗前庭半规管发育不良（Common cavity畸形）;3内耳道与耳蜗共腔（IP-Ⅲ）畸形。将蜗轴缺失的内耳畸形人工耳蜗植入手术入路分成3种类型：Ⅰ型,常规面神经隐窝入路将圆窗扩大开放入耳蜗内;Ⅱ型,常规面隐窝入路后鼓岬表面开放入畸形的耳蜗腔内,电极沿侧壁摆放;Ⅲ型,乳突侧开放外半规管突入共腔将电极摆放于耳蜗侧的腔内骨壁。结果：1246例内耳畸形中耳蜗蜗轴发育缺失共166例,其中Mondini畸形的215例患者中存在蜗轴缺失135例;Conmon cavity畸形18例,全部存在耳蜗蜗轴缺失;内耳道与耳蜗共腔（IP-Ⅲ）畸形13例,全部存在耳蜗蜗轴缺失。2内耳畸形存在耳蜗蜗轴缺失的人工耳蜗植入手术入路的选择：Ⅰ型136例,其中Mondini畸形123例,内耳道与耳蜗共腔（IP-Ⅲ）畸形13例;Ⅱ型12例,全部为Mondini畸形存在蜗轴缺失患者;Ⅲ型18例,全部为Conmon cavity畸形。33种内耳畸形人工耳蜗植入术后效果：Mondini畸形声场测试平均为65dB,单韵母言语识别为95%,单声母言语识别为25%;Conmon cavity畸形声场测试平均为80dB,单韵母言语识别为60%,单声母言语识别为0;内耳道与耳蜗共腔（IP-Ⅲ）畸形声场测试平均为55dB,单韵母言语识别为100%,单声母言语识别为45%。结论：耳蜗蜗轴缺失的内耳畸形人工耳蜗植入术后患者听觉言语识别效果很差,Conmon cavity畸形最差,Mondini畸形次之,内耳道与耳蜗共腔（IP-Ⅲ）畸形较前两者好。     

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.     

A new classification for cochleovestibular malformations

OBJECTIVE: The report proposes a new classification system for inner ear malformations, based on radiological features of inner ear malformations reviewed in 23 patients. STUDY DESIGN: The investigation took the form of a retrospective review of computerized tomography findings relating to the temporal bone in 23 patients (13 male and 10 female patients) with inner ear malformations. The subjects were patients with profound bilateral sensorineural hearing loss who had all had high-resolution computed tomography (CT) with contiguous 1-mm-thick images obtained through the petrous bone in axial sections. METHODS: The CT results were reviewed for malformations of bony otic capsule under the following subgroups: cochlear, vestibular, semicircular canal, internal auditory canal (IAC), and vestibular and cochlear aqueduct malformations. Cochlear malformations were classified as Michel deformity, common cavity deformity, cochlear aplasia, hypoplastic cochlea, incomplete partition types I (IP-I) and II (IP-II) (Mondini deformity). Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct. RESULTS: Four patients demonstrated anomalies involving only one inner ear component. All the remaining patients had diseases or conditions affecting more than one inner ear component. Eight ears had IP-I, and 10 patients had IP-II. Ears with IP-I had large cystic vestibules, whereas the amount of dilation was minimal in patients with IP-II. The majority of the semicircular canals (67%) were normal. Semicircular canal aplasia accompanied cases of Michel deformity, cochlear hypoplasia, and common cavity. In 14 ears, the IAC had a defective fundus at the lateral end. In two ears the IAC was absent. In all seven cases of common cavity malformations, there was a bony defect at the lateral end of the IAC. In five of them the IAC was enlarged, whereas in two the IAC was narrow. All patients with IP-I had an enlarged IAC, whereas in patients with type II disease, four had a normal IAC and 10 had an enlarged IAC. All cases of IP-II had an enlarged vestibular aqueduct, whereas this finding was not present in any of the cases of IP-I. In all cases, the vestibular aqueduct findings were symmetrical on both sides (simultaneously normal or enlarged). No patient demonstrated enlargement or any other abnormalities involving the cochlear aqueduct. CONCLUSIONS: Radiological findings of congenital malformations in the present study suggested two different types of incomplete partition. Cystic cochleovestibular malformation (IP-I) and the classic Mondini deformity (IP-II). The type I malformation is less differentiated than the type II malformation. Classic Mondini deformity has three components (a cystic apex, dilated vestibule, and large vestibular aqueduct), whereas type I malformation has an empty, cystic cochlea and vestibule without an enlarged vestibular aqueduct. Mondini deformity represents a later malformation, so the amount of dysplasia is much less than in type II. Therefore, it is more accurate and useful for clinical purposes to classify these malformations (in descending order of severity) as follows: Michel deformity, cochlear aplasia, common cavity, IP-I (cystic cochleovestibular malformation), cochlear hypoplasia, and IP-II (Mondini deformity). Only in this way can these complex malformations be grouped precisely and the results of cochlear implantation compared.     

The large vestibular aqueduct syndrome.

The radiographic observation of 50 patients, each having an enlarged (greater than 1.5 mm. diameter) vestibular aqueduct, are analyzed. In addition to the aqueduct other associated inner ear anomalies have been identified in 60% of this population including: enlarged vestibule (14); enlarged vestibule and lateral semicircular canal (7); enlarged vestibule and hypoplastic cochlea (4); and hypoplastic cochlea (4). The large equeduct then presumably represents an arrested phase of inner ear development common to all 50 cases. Only 8 of these cases may fall into the Mondini or Mondini-Alexander classification wherein cochlear abnormalities have been identified. The size of the aqueduct ranged from 1.5 to 8 mm. in the anteroposterior diameter; the clinical incidence is 50 in 3700 consecutive cases referred for inner ear tomography. Bilateral involvement is twice as common as unilateral with a female to male predominance of 3:2. Most cases are associated with congenital hearing losses.     

Clinical review of inner ear malformation

We had 126 patients with inner ear malformation diagnosed with temporal bone computed tomography (CT) scans at Azabu Triology Hospital between 1996 and 2002. We classified cases of inner ear malformation according to Jackler et al. The incidence of inner ear malformation in our series was as follows; 1. Labyrynthine anomalies 61% (isolated lateral semicircular canal dysplasia 56%, compound semicircular canal dysplasia 4%, semicircular canal aplasia 1%), 2. Cochlear anomalies 24%, 3. Enlargement of the vestibular aqueduct 12%, 4. Narrow internal auditory canal 2%, 5. Complete labyrinthine aplasia 1%, 6. Enlargement of the cochlear aqueduct 0%. The most frequent anomaly was isolated lateral semicircular canal dysplasia. We did not detect any significant clinical features in this anomaly. There were 2 patients with cochlear anomalies who had past histories of meningitis. Some patients with enlargement of the vestibular aqueduct had frequent attacks of fluctuating hearing. Clinically it is important to detect patients with inner ear malformation such as cochlear anomalies and enlargement of the vestibular aqueduct usually accompanied by congenital sensorineural hearing loss. For patients with congenital sensorineural hearing loss, we recommend temporal bone CT scan.     

感音神经性聋患者内耳高分辨率CT检查内耳畸形的分类

目的 探讨感音神经性聋患者中通过颞骨高分辨率螺旋CT检查内耳畸形的检出率及分类.方法 回顾性分析近10年来5 398耳感音神经性聋患者的颞骨高分辨率螺旋CT资料,患者年龄2个月~68岁,平均17.8±3.3岁;其中单侧24耳,双侧5 374耳;平均听阈83.90±5.2 dB HL,按听力损失程度分为:轻度170耳,中度1 446耳,重度1 386耳,极重度2 396耳;参照Sennaroglu 2010分类标准分析各类内耳畸形的检出情况.结果 5 398耳感音神经性聋患耳中共检出1 640耳内耳畸形(30.38%,1 640/5 398),其中,耳蜗畸形53.66%(880/1 640),非耳蜗畸形46.34%(760/1 640);880耳耳蜗畸形中,Michel畸形5耳、耳蜗未发育23耳、共同腔畸形6耳、耳蜗发育不全畸形69耳(CH-I 19耳、CH-II 16耳、CH-III 34耳)、耳蜗分隔不全畸形777耳(IP-I 44耳、IP-II 703耳、IP-III 30耳);760耳非耳蜗畸形中,大前庭导水管680耳,单纯前庭、半规管、内听道畸形80耳.与大前庭水管相关畸形共1 383耳(IP-II型 703耳、大前庭水管680耳),占全部内耳畸形的84.33%(1 383/1 640).结论 本组感音神经性聋患者内耳畸形检出率较高,且以大前庭水管相关畸形多见,Sennaroglu2010分类方法有利于各类内耳畸形发病率的流行病学统计.     

多层螺旋CT对先天性内耳发育畸形的诊断价值

目的探讨多层螺旋CT(multi-slice computed tomography，MSCT)对先天性内耳发育畸形的诊断价值。方法对44例先天性感音神经性聋患者做MSCT横断面螺旋方式扫描及多平面重建(multi-planar reformation，MPR)，必要时做单侧重叠放大重建，并利用容积漫游技术(volume rendering technique，VRT)对骨迷路进行三维重建。结果44例患者中25例CT表现正常，19例(36耳)表现为内耳发育畸形。畸形有以下几种：Michel型(1耳次)，共同腔畸形(3耳次)，不完全分隔Ⅰ型(3耳次)，不完全分隔Ⅱ型(Mondini型，5耳次)，前庭及半规管畸形(14耳次)，前庭导水管扩大(16耳次，其中6耳次伴随其他畸形)，内耳道畸形(8耳次均伴随其他畸形)。36耳畸形中33耳MSCT横断面图像和MPR图像、VRT图像均可以清晰的显示畸形的部位和程度，其中VRT图像可以直观、立体地显示畸形的空间形态结构；3耳水平半规管短小畸形患者VRT图像较断面图像更好的显示了畸形的部位和程度。结论MSCT提高了横断面图像以及MPR、VRT图像的空间分辨率。VRT图像直观、立体的显示了骨迷路畸形的空间形态结构，并且可以通过旋转多方位观察畸形的形态。MSCT扫描结合VRT可更加准确的对内耳骨迷路畸形的部位和程度做出诊断，加深了我们对各种畸形的理解，有助于人工耳蜗植入手术计划的制定。     

Cochlear implants in forty-eight children with cochlear and/or vestibular abnormality

CT and MRI scans for 48 children with cochlear and/or vestibular abnormality were classified in decreasing severity; common cavity, Mondini plus enlarged vestibular aqueduct, Mondini dysplasia alone and enlarged vestibular aqueduct alone. No significant relationship between degree of cochlea abnormality and surgical issues (cerebrospinal fluid gusher, depth of insertion, number of electrodes) or speech perception/language outcomes was found. A significant relationship was observed between cerebrospinal fluid gusher and partial electrode insertion, fewer active electrodes and poorer sentence understanding. Optimum language outcomes were associated with younger age at implant.     

Cochlear implantation in children with congenital inner ear malformations

OBJECTIVE/HYPOTHESIS: To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. STUDY DESIGN: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. METHODS: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed- and open-set testing of speech perception were analyzed. RESULTS: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. CONCLUSIONS: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.