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。     

普遍新生儿听力筛查中确诊耳聋者颞骨CT的影像学分析

目的：探讨普遍新生儿听力筛查中确诊为耳聋的婴幼儿颞骨高分辨率CT（HRCT）的影像学表现,以及对耳聋原因诊断的作用。方法：2005年1～12月,121400名新生儿参加上海市新生儿听力筛查,1077例2次筛查阳性者转至上海市儿童听力障碍诊治中心进一步行诊断性听力检查,其中184例被确诊为先天性聋的患儿接受颞骨HRCT检查,对此资料进行分析研究。结果：184例先天性聋患儿中颞骨HRCT发现解剖畸形者58例（31．5％）,其中外耳畸形26例（44．8％）,中耳畸形21例（36．2％）,内耳畸形31例（53．4％）。内耳畸形包括Mondini畸形12例,共同腔畸形1例,前庭导水管扩大、不伴耳蜗畸形6例,前庭、半规管畸形10例,内听道异常5例。另外,单纯鼓室积液20例（10．8％）。结论：HRCT检查对婴幼儿耳聋原因的鉴别诊断和治疗有一定帮助。     

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.     

Sensorineural and Conductive Hearing Loss Associated With Lateral Semicircular Canal Malformation

Objective Lateral semicircular canal (LSCC) malformation is one of the most common radiological inner ear malformations. Traditionally, inner ear malformations are thought to be associated with sensorineural hearing loss (SNHL). Recent experience with patients with LSCC malformation suggested that LSCC malformation may be associated with both SNHL and conductive hearing loss (CHL). The auditory phenotype associated with LSCC malformation is not well delineated. The objective of this study is to define the nature of the hearing loss associated with LSCC malformation. Study Design Retrospective review Methods Retrospective review of clinical records, audiological evaluation, and imaging studies. Results Two patients with unilateral and 13 patients with bilateral LSCC malformation were identified. LSCC malformation was associated with CHL in 14% (4 ears), SNHL in 71% (20 ears), normal hearing in 11% (3 ears) and CHL due to atresia in one ear. Hearing loss varied from mild to profound but did not correlate with the severity of LSCC malformation. In bilateral malformation, the hearing loss was asymmetric in half of the cases. Malformation of the posterior limb of the LSCC was always associated with a large vestibular aqueduct. An absent or rudimentary LSCC was invariably associated with a cochlear abnormality. Conclusions LSCC malformation, like other inner ear malformations such as large vestibular aqueduct and X‐linked mixed deafness with perilymph gusher, can be associated with CHL, SNHL, or normal hearing. Children with unexplained conductive hearing loss often undergo exploratory surgery to improve hearing. Given that inner ear malformations may be associated with a pure CHL, it is critical that children undergo computed tomography scan of the temporal bone prior to undergoing exploratory surgery.     

Usefulness of measuring electrically evoked auditory brainstem responses in children with inner ear malformations during cochlear implantation

Conclusions: EABR is a reliable and effective way of objectively confirming device function and implant-responsiveness of the peripheral auditory neurons up to the level of the brainstem in cases of inner ear malformation. Objective: To investigate the usefulness of measuring the intra-operative electrically evoked compound action potential (ECAP) and electrically evoked auditory brainstem response (EABR) in patients with and without congenital inner ear anomalies during cochlear implantation. Method: Thirty-eight consecutive children (40 ears) aged 5 or younger with congenital profound hearing loss. Twenty-four (25 ears) lacked congenital inner ear anomalies. The 14 patients (15 ears) with a malformation had common cavities (four ears), incomplete partition type I (three ears), cochlea hypoplasia type III (three ears), enlarged vestibular aqueduct (four ears), and cochlear nerve canal stenosis (one ear). Main outcome measures are ECAP and EABR responses. Results: Of the 25 ears lacking any malformation, 21, three, and one showed ‘Good’, ‘Variable’, and ‘No’ ECAP responses, respectively, and 24 and one showed ‘Good’ and ‘Variable’ intra-cochlear responses, respectively. Of the 15 ears with a malformation, two showed ‘Good’ ECAP responses, nine had ‘Variable’ ECAP responses, and four showed ‘No’ ECAP responses. Moreover, five showed ‘Good’ EABR responses and 10 showed ‘Variable’ EABR responses.     

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。     

Inner ear anomalies in cochlear implantees: importance of radiologic measurements in the classification.

OBJECTIVE: To classify the inner ear anomalies of cochlear implantees with profound sensorineural hearing loss. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: For the normative data of the inner ear structures, the temporal bone computed tomography of 60 patients (120 ears) with normal bone-conduction threshold (< 15 dB) were used, and the data were applied to 570 ears of 285 cochlear implantees. INTERVENTIONS: Predesignated inner ear structures were measured in temporal bone computed tomography images from the normal and cochlear implantation groups using a computer-based caliper that formed part of a picture archiving and communication system. MAIN OUTCOME MEASURES: The inner ear anomalies were defined when the structures presented visually obvious malformations or the measurements deviated 2 standard deviations from the means in the normative data. RESULTS: The application of normative data to 570 profound SNHL ears resulted in the identification of 293 individual anomalies in 127 anomalous ears. An enlarged vestibular aqueduct was the most common individual anomaly (49 cases), followed by vestibular enlargement (38 cases) and other semicircular canal dysplasia (37 cases). When the individual anomalies were reaccounted according to the more prominent anomaly where multiple anomalies were present in each ear, incomplete partition type II was the most common (34 ears), followed by cochlear hypoplasia (22 ears) and incomplete partition type I (20 ears). CONCLUSION: We suggested a measurement technique for the inner ear structures using computed tomography and derived normative measurements helpful for diagnosing inner ear anomalies. Using these normative data, we classified the inner ear anomalies of profound SNHL ears in cochlear implantees.     

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.     

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。     

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.

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。     

Vestibular hydrops in patients with semicircular canal malformation

ObjectiveTo investigate the vestibular endolymphatic hydrops in patients with semicircular canal malformation.MethodsWe searched 7864 patients who underwent MR Imaging after contrast injection and found 21 patients with semicircular canal malformations. Another 9 non-malformed patients with unilateral hearing loss were randomly included. We asked patients about their medical history and measured the volume of total vestibular fluid space and endolymphatic space. The vestibular volume ratio = endolymphatic space/total fluid space × 100.ResultsHearing loss was observed in 18 of 30 malformed ears and in 7 of 12 non-malformed ears. Statistical analysis showed no association between semicircular canal malformation and hearing loss. In the semicircular canal malformation group, the average vestibular volume ratio (22.6%) in the ears with hearing loss was higher than that in the ears without hearing loss (11.4%). There was no statistically significant difference in the average vestibular %EL in ears with hearing loss between the malformed inner ear group (22.6%) and non-malformed group (28.2%) (P>0.05).ConclusionThere was no correlation between semicircular canal malformation and hearing loss. The mean vestibular hydrops volume ratio of the semicircular canal deformed ears with hearing loss was about 22.6%, which was not different from that of normal ears.     

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.     

Common clinical features of children with enlarged vestibular aqueduct and Mondini dysplasia

OBJECTIVES/HYPOTHESIS: The purpose of the study was to investigate the etiological factors and the audiological data of different types of inner ear malformations, which the authors thought might be helpful in elucidating the inter-relation among malformations and shedding light on pathogenesis. STUDY DESIGN: Retrospective study from 1998 to 2002 at a tertiary care university hospital. METHODS: One hundred sixty consecutive children with a total of 302 affected ears undergoing high-resolution computed tomography of the temporal bone for sensorineural hearing loss were enrolled. The image results were correlated with causes and origins, hearing loss patterns, hearing levels, and audiogram configurations. RESULTS: Inner ear malformation was present in 114 (38%) ears. The most common malformations were enlarged vestibular aqueduct, incomplete partition of cochlea (Mondini dysplasia), large vestibule, and semicircular canal dysplasia, presenting either as isolated finding or in combination. Eighty-four (74%) ears had abnormalities confined to these four malformations; only 30 (26%) ears showed other malformations. Patients with complex of enlarged vestibular aqueduct, Mondini dysplasia, large vestibule, and semicircular canal dysplasia (EMVS complex) demonstrated a significantly higher incidence of fluctuating hearing loss (93%) and a better hearing level compared with those with other malformations. Homogeneity in audiological features among these four malformations was also disclosed. CONCLUSION: The authors identified a distinct clinical entity, the EMVS complex, which is characterized by fluctuating hearing loss and a better hearing level. The authors proposed that malformations belonging to this complex result from a common pathogenetic mechanism.     

迷路炎的影像学

目的 探讨迷路炎的影像学检查方法及其诊断价值.方法 观察27例31耳临床提示为迷路炎同时存在异常影像学表现的病例的高分辨率CT(high resolution CT, HRCT)及MRI图像,总结其内耳在HRCT及MRI图像上的不同改变.结果 进行了HRCT扫描的22耳中,内耳1个或多个结构表现为密度增高者6耳,密度增高且发生变形者8耳,单纯变形者1耳,内耳迷路内腔局部或完全硬化消失者7耳.在22耳中除上述改变外亦有内耳骨质缺损者4耳.进行了HRCT及MRI检查的9耳中,7耳在HRCT上可见内耳不同程度的异常改变,2耳内耳未见异常改变.MRI图像上9耳均可见内耳迷路内腔1个或多个结构T2WI信号减低或消失.行增强扫描的6耳中,4耳可见明显强化,2耳未见强化.31耳中,耳蜗受累30耳,其中仅基底周受累5耳,中顶周受累2耳,耳蜗各周均受累23耳;半规管受累26耳;前庭受累20耳;前庭窗受累18耳;蜗窗受累19耳.结论 HRCT可以较好显示骨迷路迷路炎的异常改变,MRI对于显示迷路内腔有重要价值,二者在迷路炎的影像检查与诊断中的作用是互补的,对迷路炎的临床诊断起着重要的辅助作用.     

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。     

"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。     

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。     

CT检查在非突发性单耳感音神经性聋诊疗中的意义

目的：探讨颞骨高分辨率CT在发现单耳感音神经性聋中的诊疗意义,为合理使用医疗资源提供依据。方法：回顾性分析门诊收集的28例不明时间发现的单耳听力下降的年轻患者病史资料及CT检查结果,并结合相关文献对单耳聋的认识做一综述。结果：研究对象均为单耳重度一极重度的感音神经性聋患者,且外中耳均无异常,颞骨高分辨率CT检查发现9例患者有内耳畸形,阳性发现率为32％,其中单侧的内听道狭窄患者有5例（5／9）,单侧共同腔畸形和mondini畸形各1例,前庭与部分半规管融合的畸形2例。19例患者CT检查未发现明显异常,其中4例患者曾有流行性腮腺炎病史。结论：CT检查在发现单耳聋病因方面有一定的意义,可避免患者频繁就医而浪费医疗资源。