CT与磁共振成像在儿童人工耳蜗植入术前研究

目的　评价CT与磁共振成像 (magneticresonanceimaging ,MRI)在儿童人工耳蜗植入术前的诊断作用 ,以及对手术选择的影响。方法　 71例 ( 142耳 )双耳重度耳聋、拟行人工耳蜗植入的患儿进行术前CT与MRI检查。结果　CT与MRI均发现 ,12例 ( 2 2耳 )患者有前庭水管扩大 ( 15 5 % ) ;14例 ( 2 5耳 )患儿有Mondini畸形 ( 17 6% ) ;3例 ( 5耳 )患儿有内耳道扩大 ( 3 5 % ) ;2例 ( 4耳 )患者有可疑内耳道底骨质缺损 ( 2 8% )。MRI发现有 5例 ( 5耳 )患者单侧耳蜗纤维化 ( 3 5 % ) ,而CT未见异常。1例 ( 2耳 )患儿的CT显示面神经裸露 ( 1 4% ) ,MRI正常。结论　人工耳蜗植入术前应该进行CT与MRI检查。对于发现前庭水管综合征、Mondini畸形、内耳道扩张及内耳道底骨质缺损有重要意义。这二种影像学检查结果可以相互补充诊断耳蜗纤维化与面神经裸露。对手术适应证的选择以及保证手术正常进行有重要意义     

CT与磁共振成像在儿童人工耳蜗植入术前研究

目的 评价CT与磁共振成像（magnetic resonance imaging,MRI)在儿童人工耳蜗植入术前的诊断作用，以及对手术选择的影响。方法 71例（142耳）双耳重度耳聋、拟行人工耳蜗植入的患儿进行术前CT与MRI检查。结果 CT与MRI均发现，12例（22耳）患者有前庭水管扩大（15.5%)；14例（25耳）患儿有Mondini畸形（17.6%)；3例（5耳）患儿有内耳道扩大（3.5%)；2例（4耳）患者有可疑内耳道底骨质缺损（2.8%)。MRI发现有5例（5耳）患者单侧耳蜗纤维化（3.5%)，而CT未见异常。1例（2耳）患儿的CT显示面神经裸露（1.4%)，MRI正常。结论 人工耳蜗植入术前应该进行CT与MRI检查。对于发现前庭水管综合征、Mondini畸形、内耳道扩张及内耳道底骨质缺损有重要意义。这二种影像学检查结果可以相互补充诊断耳蜗纤维化与面神经裸露。对手术适应证的选择以及保证手术正常进行有重要意义。     

儿童重度感音神经性聋的CT及MRI影像学观察

目的:探讨CT和MRI在儿童重度感音神经性聋(SNHL)诊断中的价值。方法:对96例重度SNHL患儿进行颞骨高分辨率CT和MRI检查,并进行三维立体成像。结果:15例患儿CT(MRI)显示异常,其中耳蜗不发育(Michel畸形)1例,耳蜗发育不良1例,共同腔畸形1例,Mondini畸形1例,内耳道狭窄及闭锁各1例,蜗神经未发育1例,前庭水管扩大8例;其余81例CT和MRI均未见内耳及内耳道畸形。结论:部分先天性SNHL患儿存在内耳畸形,颞骨高分辨率CT能显示内耳骨迷路畸形,MRI则能显示畸形的迷路内腔及内耳道内前庭耳蜗神经,为制定干预策略及选择治疗方式提供了重要依据。     

内耳畸形伴脑脊液耳漏的高分辨率CT特征

目的:探讨先天性内耳畸形伴脑脊液耳漏的高分辨率CT(HRCT) 特征.方法:回顾性分析11例先天性内耳畸形伴脑脊液耳漏患者的HRCT表现.结果:共11例患者,耳蜗未发育4例,共腔畸形2例,耳蜗未未分隔型3例,耳蜗不全分隔型2例.内耳道底骨质缺损8例.所有患者患侧乳突气化和发育程度与对侧一致,前庭窗处都有软组织影.结论:内耳畸形伴脑脊液耳漏患者常存在内耳道底骨质缺损.前庭窗是脑脊液由内耳进入中耳腔的好发部位.HRCT能为先天性内耳畸形伴脑脊液耳漏患者的诊断及治疗提供可靠依据.     

先天性重度、极重度感音神经性聋患儿内耳及颅脑影像学评估

目的探讨先天性重度、极重度感音神经性聋患儿中内耳畸形及颅脑异常的发病情况,为人工耳蜗植入适应证的选择提供参考。方法对确诊为先天性重度、极重度感音神经性聋的245例患儿进行影像学检查,其中232例行颞骨高分辨率CT检查,204例行内听道MRI检查,196例行常规颅脑MRI检查,分析内耳结构异常及颅脑异常患儿的影像表现。结果行颞骨高分辨率CT扫描的232例中,195例内耳正常,37例内耳异常,占15.95%;行内听道MRI检查的204例中,167例正常,37例异常,占18.14%;行颅脑MRI平扫的196例中,110例正常,86例异常,占43.88%。耳蜗畸形包括:Michel畸形3例4耳,耳蜗未发育4例7耳,耳蜗发育不全2例3耳,不完全分隔Ⅰ型5例8耳,不完全分隔Ⅱ型5例10耳。前庭畸形16例26耳,半规管畸形14例24耳;内听道畸形7例12耳;前庭导水管畸形16例29耳。颅脑异常者包括:脑白质病61例,颅内发育变异9例,脑先天畸形7例,脑损伤、破坏性疾病6例,颅骨异常2例及松果体囊肿1例。结论本组先天性重度或极重度感音神经性聋患儿内耳畸形发生率为15.10%(37/245),颅脑异常发生率为43.88%(86/196),术前常规进行颞骨高分辨率CT、内听道MRI及颅脑MRI检查能够发现内耳及颅脑病变,可作为人工耳蜗植入适应人群的筛选及植入耳选择的参考。     

双内听道的影像学特征

目的 总结双内听道(duplication of internal auditory canal,DIAC)的多层螺旋CT和MRI影像学特点,提高对该病的认识.方法 结合文献回顾性分析4例(5耳)DIAC患者的影像学资料,4例均行多层螺旋CT检查,其中2例同时行MRI检查.结果 多层螺旋CT显示5耳内听道被骨性间隔分为双管状,上部骨管与面神经管相连,下部骨管与前庭和耳蜗相连,其中2耳骨性间隔不完整.5耳上下两管径之和均超过2 mm.3耳可显示蜗神经管狭窄.5耳均合并前庭扩大及外半规管发育不良,2例(2耳)同时合并小耳廓畸形,1耳合并小耳廓畸形、外耳道闭锁及听小骨发育不良.多平面重建(multiplanar reconstruction)、容积再现(volume rendering)图像可全面立体显示骨性间隔及双管结构.MRI显示1耳前庭和蜗神经发育不良、面神经完好,另1耳前庭、蜗神经和面神经均发育不良.结论 多层螺旋CT能清晰显示内听道被骨性间隔分为双管状及伴发畸形,可作为确诊DIAC的依据.MRI能表示其神经发育异常,为电子耳蜗植入对象的选择提供一定帮助.     

伴随耳蜗畸形的面神经管异常

本文通过颞骨 CT扫描来分析与耳蜗畸形有关的面神经管异常。该作者曾报道一例非蒙底尼 (Mondini)型耳蜗畸形的病人伴有面神经管第一段向前内移位 ,也就是迷路段相对于正常位置向前内移位 ,耳蜗在前庭上呈花蕾状憩室 ,并且耳蜗上两转未发育。由于面神经管的发育与耳蜗的发育关系密切 ,该作者推测这例患者耳蜗畸形是面神经移位的原因 ,并检查其它面神经管第一段移位的患者 ,以研究面神经管异常和耳蜗畸形间的关系。从感音神经性聋病人的颞骨 CT扫描中 ,对 15例确诊为面神经管第一段异常的患者进行分析 ,有 7例为双耳 ,共 2 2耳。另外 5例 8…     

内耳道内神经问吻合及位置关系的显微解剖研究

目的 探讨内耳道内神经走行及相互吻合关系.方法 在手术显微镜下对30例成人颞骨标本进行显微解剖学研究,观察神经走行、面神经与前庭神经之间的吻合及耳蜗神经与前庭神经之间的吻合.结果 (1)30例颞骨标本中,37%(11例)的面神经在内耳道内一直在前上位置;63%(19例)的面神经从内耳道底至内耳道口过程中均有围绕前庭神经纵轴不同程度的向前下旋转,旋转范围大致在30°～90°之间,旋转的方向与耳蜗旋转的方向相同. (2)30例标本中,17%(5例)的标本没有发现前庭神经与面神经吻合支;83%(25例)的面神经与前庭神经之间存在吻合支,并且形态和数目存在差异.67%(16例)的吻合支在接近内耳道口部位;33%(8例)的吻合支在接近内耳道底的外1/3的内耳道内.吻合支直径不等,约在0.5～1 mm之间.(3)30例标本中,80%(24例)存在前庭耳蜗神经吻合支.在内耳道外1/3部位.13例标本中可明显看到一些刷状的神经纤维束直接进入球囊,15例在内耳道外1/3段有横行的吻合支,2例标本可以看到有多个吻合支,部分为两种形式共存.仅在内耳道中段有吻合支者1例;20%(6例)的标本未见有明显的前庭耳蜗神经吻合.结论 在内耳道内,神经走行关系及神经之间的吻合均存在着个体差异.熟悉这些关系有助于手术中保护神经功能,以及探讨面神经、前庭及耳蜗疾病之间的关系.     

蜗神经磁共振成像在感音神经性聋中的应用

目的探讨蜗神经磁共振成像在感音神经性聋(SNHL)中的应用价值。方法采用GE TwinExct1.5T磁共振扫描仪对130例(260耳)SNHL患者进行蜗神经磁共振成像及内耳水成像。蜗神经成像序列为斜矢状位FRFSET2加权像,内耳水成像为3DFIESTA序列。结果 90例大于等于18岁的患者中,85例170耳显示蜗神经、迷路正常;1例2耳前庭导水管扩大;1例1耳蜗神经信号缺失;3例6耳蜗神经细小;40例小于18岁的患者中,19例38耳显示蜗神经、迷路正常;2例3耳Michel畸形;6例12耳Mondini畸形;1例2耳共腔畸形;12例24耳前庭导水管扩大;21例畸形中7例14耳蜗神经信号缺失。2例4耳显示内听道狭窄伴蜗神经细小。结论磁共振内耳成像对诊断SNHL有着重要的价值,对判断患者内耳蜗神经发育情况及内耳畸形有着不可替代的作用,是这类患者进行人工耳蜗植入术前必要检查。     

先天性内耳道狭窄的多层螺旋CT和MRI表现

目的 总结先天性内耳道狭窄的多层螺旋CT(multiple slices CT,MSCT)和MRI影像学特点,提高对该病的认识.方法 回顾性分析13例(15耳)先天性内耳道狭窄患者MSCT和MRI检查的影像资料.结果 先天性内耳道狭窄单侧11例,双侧2例.MSCT显示内耳道管腔不同程度狭窄.孤立性内耳道狭窄3耳,合并其他畸形12耳,其中10耳仅合并内耳畸形,1耳同时合并内、中、外耳畸形,另1耳同时合并内、中、外耳畸形及额骨发育畸形.MRI检查15耳均显示前庭蜗神经发育细小;其中7耳蜗神经未显示,7耳蜗神经发育细小,1耳蜗神经显示不清;其中2耳面神经发育细小.容积再现(volume rendering,VR)图像可立体显示内耳道狭窄程度及伴发的内耳畸形.结论 MSCT 可显示内耳道狭窄的程度及伴发畸形,MRI则可显示其神经发育情况.

Abstract：

Objective To investigate multiple slice computed tomography ( MSCT) and magnetic resonance imaging( MRI) features of congenital stenosis of the internal auditory canal (CSIAC) and improve the ability for diagnosis.Method Thirteen cases with fifteen ears were studied.In all cases a MSCT and MRI was performed.Results Eleven cases were unilateral,and 2 cases were bilateral.MSCT could show the narrowness of IAC.Three cases were isolated,but the others were combined with inner ear malformations.One ear had inner,middle and outer ear malformations.One ear had inner,middle,and outer ear malformations with a frontal bone malformation.MRI demonstrated that all of the vestibulocochlear nerves were hypoplastic.The cochlear nerve in seven ears was not present,in seven ears the nerve was thinner,and in the last case it was poorly visualized.The facial nerve in two ears was hypoplastic.Volume rendering( VR) could present the degree of the narrowed internal auditory canals,combined with other inner ear anomalies.Conclusion MSCT will show the degree of the narrow internal auditory canals and combined anomalies,while the MRI can further demonstrate the nerves' development.     

HRCT and MRI findings in X-linked non-syndromic deafness patients with a POU3F4 mutation

Objective

The aim of this study was to analyze HRCT and MRI findings in patients with X-linked non-syndromic deafness and a POU3f4 mutation.

Methods

HRCT and MRI data of four patients (males, 2–19 years old) with a POU3f4 mutation were collected and a retrospective review was performed. Cochlea, internal auditory canal (IAC), vestibule, semicircular canals, vestibular aqueduct, nerve canals in the IAC fundus, stapes and cochlear nerve were evaluated on 2D images (multi-planner reformation, MPR) and cochlear foramen on 3D images (CT virtual endoscopy, CTVE). Ten cases with normal hearing subjected to CT and MR exams served as controls.

Results

Inner ear malformations were bilateral and symmetrical. Cochlear malformation was shown to consist of as a relatively normal outer coat shape, absence of a cochlear modilous, and a direct intercommunication between the IAC and cochlear inner cavity. The lateral portion of the IAC was dilated. A spiral cochlear inner cavity was observed with CTVE images versus a helical cochlear nerve foramen as seen in controls. The labyrinthine facial nerve canal and superior vestibular nerve canal were enlarged. The Bill's bar was hypertrophic and partially pneumatized. A thickened stapes footplate was present and a fissura ante fenestram was absent in seven ears examined. A column shaped stapes was observed in one ear.

Conclusions

The absence of a cochlear modilous with a dilated lateral IAC and thickened stapes footplate were the remarkable features observed with imaging these in X-linked non-syndromic deafness patients with a POU3F4 mutation. Preoperative recognition of the image features in these patients is important because it precludes stapedectomy and indicates the risks in the surgery of cochlear implantation including CSF gusher and electrode insertion into IAC.     

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.     

听骨链CT扫描并三维重建的临床应用

目的:探讨CT仿真内镜(CTVE)显示中耳正常结构的显示方法,评价中耳病变特别是听骨链连接状态的价值及意义,确立显示听骨链及其他中耳结构的位点和方法。方法:选择40例无耳科疾病的志愿者(正常组)和30例疑有中耳疾病的患者(病例组),分别采用德国西门子(Siemens SOMATOM Sensation 16)螺旋CT机Inner Ear扫描程序对患者进行轴位扫描,重建原始图像,用Fly-through软件经选择的A、B、C点入路进行CTVE成像研究。重点显示听骨链连接状态,并对慢性中耳炎的显示结果与手术中探查图像进行对照。结果:正常组CTVE中锤骨、砧骨、鼓岬、面神经管水平段、外半规管隆凸显示率均为100%;而镫骨头、两足弓的显示率在3个显示层面则分别为57.5%、70.0%,97.5%;圆窗、卵圆窗为90.0%、93.0%、97.5%。在中耳炎、外伤、颞骨畸形等情况下可显示听小骨损伤、移位、中断、缺失和畸形。结论:CTVE在显示3个听小骨之间的链接关系(如中断等方面)有一定优势。通过选择恰当的入路,CTVE在显示听小骨及其链接关系以及病理状态方面有相当优势。通过比较CTVE在3个不同显示层面观察镫骨的能力,明确CTVE是断层影像的有利补充。     

婴幼儿中耳异常分析

目的 分析婴幼儿听力学测试和(或)颞骨薄层CT提示中耳异常的病例,探讨婴幼儿中耳异常的听力学和医学诊断.方法 回顾性分析2005年12月至2008年6月在本院儿童听力诊断中心就诊的31例2个月到2岁6个月婴幼儿中耳异常病例的临床资料,比较了听力学和颞骨薄层CT检查的结果.结果 ABR测试31例(62耳)中38耳表现了I波潜伏期延迟,其中双耳延迟13例,单耳延迟12例;15耳ABR没有表现I波潜伏期延迟,但其中6耳CT显示中耳乳突有高密度影;9耳未引出I波.16例完成鼓室导抗图测试,异常17耳,表现为11个月以下11300 Hz导抗图无正峰,12个月以上226 Hz导抗图B型或C型.15例ABR测试和(或)鼓室导抗图测试提示中耳病变者行CT检查,鼓室乳突有高密度影双侧12例,单侧3例.ABR I波延迟,同时CT鼓室乳突有高密度影13耳,ABR I波潜伏期正常,而CT鼓室乳突有高密度影的4耳.没有ABR I波潜伏期延迟而CT无改变者,但ABR、鼓室导抗图和CT间存在不一致现象.结论 婴幼儿声导抗测试和ABR I波潜伏期检查可以检出绝大部分的中耳异常,但仍不能完全反映婴幼儿中耳状况,婴幼儿中耳异常诊治中存在着困惑,有待深入研究.     

先天性小耳畸形全耳廓成形及听力重建术

目的:探讨先天性小耳畸形的临床特点及全耳廓成形及听力重建术的方法和效果。方法:统计、整理2005-01-2010-10期间在我科住院治疗的58例(62耳)小耳畸形患者的资料,本组患者均为先天性耳廓畸形伴外耳道闭锁、听骨链畸形、重度传导性聋。所有患者术前行颞骨CT检查并三维重建,听力学检查。手术分2期进行,一期行耳廓一次成形,外耳道重建,中耳重建手术;二期行耳后植皮,颅耳角再造,对术前、术后的资料进行回顾性分析。结果:耳廓成形加外耳道、中耳重建术后再造耳廓外形良好,耳廓和外耳道口位置接近正常,大部分患者术后听力获得改善。结论:对先天性耳廓畸形并外耳道闭锁的患者,术前应精心设计,根据多层螺旋CT三维重建结果确定听力重建入路及中耳畸形程度,全耳廓成形及听力重建术后可以同时改善耳廓外形和提高听力。     

高分辨率CT容积重建对内耳畸形的诊断价值

目的：通过高分辨率CT（HRCT）容积重建（VR）对正常内耳形态的观察,探讨其对先天性内耳畸形的诊断价值。方法：对10例（20耳）无耳部疾患者（对照组）和7例（11耳）先天性内耳畸形患者（病变组）行HRCT扫描,利用容积漫游技术对内耳骨迷路重建。对照组观察内耳骨迷路的正常结构,病变组观察内耳畸形情况。结果：正常耳VR图像不但显示了内耳骨迷路的细微结构,而且还反映了各结构之间的关系;病变耳立体显示了畸形部位及程度。11耳患耳中Mondini型7耳次;前庭及半规管畸形3耳次;前庭导水管扩大7耳次,其中6耳次伴随其他畸形;内耳道畸形2耳次且均伴随其他畸形。11耳畸形中9耳HRCT横断面图像和平面重建（MPR）冠状位图像、VR图像均可以清晰地显示畸形的部位和程度,其中VR图像可以直观、立体地显示畸形的空间形态结构;2耳水平半规管短小畸形患者VR图像较断面图像更好地显示了畸形的部位和程度。结论：VR三维重建可以立体显示正常内耳骨迷路的形态,直观显示内耳畸形的程度及病变位置,对内耳畸形的诊断具有重要的辅助价值。     

Relative humidity of the external auditory canal in normal and abnormal ears,and its pathogenic effect

Objective: To find the relative humidity of the normal external ear canal and to demonstrate that the relative humidity of the external auditory canal was higher in abnormal ears, i.e. ears commonly associated with chronic discharge. Setting: Secondary health care within the ENT departments of Addenbrooke's and Hinchingbrooke hospitals. Design: A clinical observational study. Participants: Volunteers seen in ENT outpatients. Inclusion criteria for the normal ear group, was a normal ear with no history of infection or previous ear disease. Inclusion criteria for abnormal ear group commonly associated with chronic discharge. Exclusion criteria were the presence of pus or infection within the ear canal. Method: The relative humidity of the external ear canal was measured in normal and abnormal ears. Abnormal ears were grouped as those commonly associated with chronic discharge, mastoid cavities, tympanic perforations, recurrent otitis externa and hearing‐aided ears. Results: The range of relative humidity of normal ears is 40–70%. The humidity in abnormal ears was found to be significantly higher than the humidity in normal ears (P < 0.0001). Conclusion: The raised relative humidity in the external canal may explain the predisposition of certain abnormal ears to chronic discharge and infection. If the humidity of the canal could be brought down to a normal level, this could offer a new therapeutic treatment.     

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.