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。     

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.     

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。     

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.     

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     

高分辨率CT多平面重组对迷路瘘管的诊断价值

目的:探讨高分辨率CT(HRCT)多平面重组对半规管的显示能力及对慢性化脓性中耳炎并发迷路瘘管的诊断价值。方法:对86例(正常79耳,病变93耳)慢性化脓性中耳炎患者行HRCT扫描,正常耳行轴位、冠状位及各半规管多平面重组(s-MPR)图像整体显示半规管的形态和结构情况;病变耳观察各半规管的破坏情况,并与手术结果比较。结果:正常耳轴位仅部分能显示外半规管全程(仅显示外半规管68耳),冠状位各个半规管全程均不能完整显示,s-MPR可分别在同一层面上显示各半规管全程(上半规管73耳,外半规管79耳,后半规管77耳)。病变耳轴位显示外半规管瘘5耳,冠状位显示外半规管瘘4耳,两者均显示1耳上半规管瘘;s-MPR显示外半规管瘘6耳,上半规管瘘2耳。手术探查见外半规管破坏6耳,上半规管破坏2耳。结论:s-MPR可以完整显示各半规管全程,对迷路瘘管具有较高的诊断价值。     

15例先天性中耳畸形临床特点及治疗

目的分析单纯中耳畸形的临床特点,探讨先天性单纯中耳畸形的诊断治疗方法。方法先天性中耳畸形患者15例（15耳）。男11例,女4例。年龄11～37岁,平均年龄（20.4±6.21）岁,单侧发病7例,双侧发病8例。平均气骨导差（50.33±7.73）dB HL,均行颞骨薄层CT扫描。显微镜下行鼓室探查及鼓室成形术,根据不同的畸形情况采用相应的听骨链重建技术。结果 15例中11例颞骨CT显示不同程度的听骨链异常。术中进行镫骨足板开窗或足板全切除4例（4耳）、前庭开窗2例（2耳）、外半规管开窗2例（2耳）、镫骨上结构存在、足板活动正常,行IIIa型鼓室成形术7例（7耳）。结论影像学检查对先天性中耳畸形的诊断十分重要,手术是主要治疗手段,多数病例可通过手术重建听骨链,恢复中耳传音功能,提高听力。但手术涉及内耳比例高,病例选择要慎重同时要做好充分术前准备。     

高分辨CT三维容积重建对颞骨骨质破坏的诊断价值

目的：探讨高分辨CT(HRCT)三维容积重建在颞骨骨质破坏中的诊断价值。方法：术前高分辨率轴位或冠状位CT上显示有颞骨骨质破坏或高度怀疑有骨质破坏的15例胆脂瘤型中耳炎患者，应用容积再现(VR)对病变区进行重建，术中观察病变范围并与重建图像比较。结果：与轴位或冠状位CT图像相比，所有重建图像均清晰地显示了骨质的破坏区域，并立体地显示了与周围骨质之间的关系，与手术结果相符合。结论：HRCT三维容积重建对颞骨骨质破坏诊断具有较高价值，并且有利于手术方式的制定。     

迷路后进路内镜手术中内耳道的定位

目的：为迷路后进路处理桥脑小脑角区及内耳门区病变手术中内耳道的定位提供参考依据。方法：4％多聚甲醛固定的成人头颅标本（正常完整颅底）15例（30侧,性别不限）,模拟迷路后进路操作,沿外半规管平面导入0°2．7mm耳内镜,观察内耳道与外半规管的关系。结果：内耳道口中心与外半规管平面一致,面听神经束的走行（内耳道走行）与外半规管平面的关系：①面听神经起始部位于外半规管平面之下者2侧（6．7％）;②面听神经起始部位于外半规管平面者15侧（50．0％）;③面听神经起始部位于外半规管平面之上者13侧（43．3％）。结论：在迷路后进路桥脑小脑角区及内耳道手术中,以外半规管定位内耳道具有直接、准确、对周围组织损伤小的优点,更符合微创外科手术的要求。     

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.     

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.     

Superior semicircular canal dehiscence and enlarged vestibular aqueduct

Superior semicircular canal dehiscence is a well described labyrinthine defect, detect in pediatric population too.We report a case of superior semicircular canal dehiscence, which radiological diagnosis was confirmed by multiplanar reformatted CT images, associated to omolateral enlarged vestibular aqueduct in a 16-year-old female who presented with congenital hearing loss without vestibular symptoms. Both inner ear malformations act as a third mobile window into the labyrinth and cochleo-vestibular symptoms can result from loud sounds or pressure changes. An early diagnosis should be strived for preserve inner ear functions.     

Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss

The objective of this study is to use standardized measurements of the inner ear to see whether there are subtle bony malformations in children with congenital sensorineural hearing loss (SNHL) whose temporal bone computed tomography (CT) are grossly normal. The study includes 45 ears with congenital SNHL and grossly normal temporal bone CT scans and 45 ears with normal inner ear structures and normal hearing. Standardized measurements of the inner ear structures were made on axial temporal bone CT scans. Student’s t test was performed to compare the measurements of the two groups. There were significant differences in the measurements of the bony island width of the superior semicircular canal, bony island width of the lateral semicircular canal and maximal height of cochlea between two groups (P < 0.05). In conclusion, standardized measurements of bony labyrinth of inner ear on temporal bone CT can identify subtle abnormalities of inner ear in patients with congenital SNHL having grossly normal radiological images.     

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.     

Bilateral semicircular canal aplasia with near-normal cochlear development. Two case reports

Congenital malformations of the vestibular labyrinth (pars superior) are rare. We present two patients with computed tomographic findings of bilateral semicircular canal aplasia with normal or near-normal cochleas. Initial bone conduction thresholds were within normal limits, although both patients had significant conductive hearing losses due to congenital middle ear malformations. Bithermal caloric responses were absent in both. To our knowledge these are the first reports of vestibular aplasia concomitant with normal or near-normal cochlear development. These findings conflict with conventional hypotheses that state that inner ear malformations result from arrested development during the normal stages of inner ear embryogenesis.