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。     

45例先天性内耳畸形的高分辨率CT特征

目的：探讨先天性内耳畸形的高分辨率CT(HRCT)特征及其在先天性内耳畸形诊治中的意义。方法：总结45例先天性内耳畸形的临床资料．分析颞骨HRCT所见。结果：45例耳聋多起于幼儿．呈缓慢进行性感音神经性听力减退，波动性者15耳；17耳半规管麻痹。HRCT显示：Michel型3例(4耳)；Mondini型25例(39耳)；不伴内耳畸形的前庭水管扩大13例(23耳)；内耳道发育畸形4例(5耳)。伴中、外耳畸形13耳。结论：HRCT对先天性内耳畸形具有重要的诊断价值，并为临床治疗、特别是人工耳蜗植入术适应证的选择提供了重要的依据。     

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.     

内耳先天性畸形102例临床分析

报道１０２例（２００耳）内耳先天性畸形的临床分析。此类患儿多为双侧重度听力损失或全聋，３／４患儿于一岁以内即被家长发现耳聋，其中４７例（４６％）患儿的母亲有妊娠早期感冒史。采用高分辨内耳ＣＴ检查，其颞骨畸形大致分为五型：（１）Ｍｉｃｈｅｌ型，１６耳，（２）Ｍｏｎｄｉｎｉ型，１５５耳；（３）前庭导水管扩大畸形，１６耳，（４）耳蜗导水管发育畸形，１２耳；（５）内耳道发育畸形，１耳。值得指出的是多数先天     

先天性内耳畸形的影像学诊断及进展

先天性内耳畸形是指内耳胚胎期不同阶段发育障碍导致的内耳结构异常的一组疾病,是儿童感音神经性聋的主要病因,其群体发病率约为1/2 000～1/6 000。内耳分为骨迷路和膜迷路,迷路由前向后分为耳蜗、前庭和半规管。先天性内耳畸形可发生在骨迷路和膜迷路的任何部分,其中约20%为骨迷路畸形,约80%为膜迷路畸形。由于内耳位置深在,结构细小,过去一直是影像学检查的盲区,随着能清     

多层螺旋CT对婴幼儿先天性感音性耳聋的诊断价值

先天性内耳畸形是婴幼儿听力减退的主要病因,其群体发病率约为1/2000～1/6000,其中约20%为骨迷路畸形,80%为膜迷路畸形。骨迷路畸形导致内耳形态结构异常,可被CT及MRI诊断。MSCT扫描结合VRT提高了内耳骨迷路畸形的检出率及诊断准确性,对婴幼儿先天性感音性耳聋的病因诊断具有重要价值。     

Mondini畸形的基础和临床研究

CarloMondini于1791年在对1例罹患先天性聋患儿死后的颞骨解剖时发现其内耳发育畸形，证实其先天性聋的病因为内耳发育畸形。后来这种内耳发育畸形被命名为Mondini畸形。Mondini畸形仅是内耳畸形中的一种类型。经典的内耳畸形分型是采用组织病理学方法将内耳畸形分为Michel、Mondini、Siebenmana—Bing和Scheibe畸形4型。近又有学者采用影像学技术将内耳畸形分为耳蜗、前庭、半规管、内听道、前庭水管和耳蜗小管畸形，其中的耳蜗畸形又分为Michel、耳蜗未发育、共同腔、耳蜗发育不全、不完全分隔Ⅰ型和不完全分隔Ⅱ型（Mondini畸形）。在内耳畸形中Mondini畸形的临床发病率最高，占54．9％～77．5％。Mondini畸形的临床表现不具特征性，主要呈感音神经性聋表现，少数患者可伴眩晕等症状。所以临床对该病常出现诊断不能明确，甚或误诊的情况。为提高对该病的认识，减少临床误诊情况，本文对该病近年来的基础和临床研究进行综合整理并报告如下。     

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…     

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.     

感音神经性聋患儿中先天性内耳畸形的构成、影像学及临床听力学特征

目的:分析婴幼儿、儿童先天性感音神经性聋(SNHL)中先天性内耳畸形的构成、影像学及临床听力学特征。方法:回顾性分析2005-02-2010-01上海交通大学医学院附属上海儿童医学中心耳鼻咽喉科诊治的860例先天性SNHL患儿中,经颞骨高分辨率CT及MRI发现有先天性内耳畸形的125例(225耳)患儿的听力学及影像学资料。结果:860例先天性SNHL患儿中有先天性内耳畸形者占14.5%;累及双侧98例(78.4%),单侧27例(21.6%)。225耳中167耳(74.2%)为极重度聋,36耳(16%)为重度聋,22耳(9.8%)为中度聋。该组内耳畸形中,前庭水管扩大最多见(75.6%),其次为前庭畸形(32%),再次为耳蜗前庭畸形(23.1%)。耳蜗前庭畸形中以Mondini畸形最常见(55.8%),其次为共同腔畸形(28.9%)。累及耳蜗的内耳畸形中极重度聋者明显多于未累及耳蜗的内耳畸形中极重度聋者。结论:对了解中国婴幼儿、儿童先天性SNHL中先天性内耳畸形的构成,对先天性SNHL的病因诊断以及对包括助听器、耳蜗植入等在内的干预策略的制订及其预后有一定意义。     

3D-FIESTA序列在先天性内耳畸形中应用

目的评估3D-FIESTA序列在先天性内耳畸形中的应用价值。方法采用3D-FIESTA序列对38例内耳畸形患者进行扫描,并进行多平面重组。结果 38例（62耳）内耳畸形中耳蜗畸形31耳,前庭畸形28耳,半规管畸形32耳,前庭水管及内淋巴囊扩大21耳,内耳道神经发育异常29耳。结论 3D-FIESTA序列可以清晰显示内耳道及内耳迷路的先天发育异常,具有较高的临床应用价值。     

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.     

Variations in the labyrinthine segment of facial nerve canal revealed by high-resolution computed tomography

Objective

To study variations in the labyrinthine segment of fallopian canal and the associated middle and inner ear malformations.

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。     

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.     

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.     

HRCT imaging characterized of congenital abnormalities of the inner ear in 45 cases]

OBJECTIVE: To explore the high resolution CT (HRCT) image characterized of congenital abnormalities of the inner ear(CAIE), and its value in the diagnosis and treatment of CAIE. METHOD: The clinic data and axial HRCT scans of CAIE in 45 cases were analyzed. RESULT: In 45 CAIE patients, most of them were frequently associated with slowly progressive sensorineural hearing loss in childhood, 15 ears were fluctuating hearing loss. Seventeen ears were unilateral semicircular canal paralysis. HRCT showed that Michel type 3 cases(4 ears), Mondini type 25 cases(39 ears). Large vestibular aqueduct malformation not associated with anomalies of inner ears 13 cases(23 ears), anomalies of internal auditory canal 4 cases (5 ears). Thirteen ears were associated with outer and middle ear malformation. CONCLUSION: HRCT image has the important value in the diagnosis and treatment of CAIE, especially for the excerpt of indication of cochlear implantation.