儿童先天性感音神经性耳聋的高分辨率CT和MRI研究

目的 探讨CT和MRI在儿童先天性感音神经性耳聋中的应用价值.资料与方法 对48例儿童先天性感音神经性耳聋患者进行颞骨高分辨率CT和MRI检查.结果 前庭导水管扩大11例,MRI显示所有病例内淋巴管和内淋巴囊均扩大;单纯Mondini Ⅱ型畸形2例;水平半规管不发育2例;共同腔畸形合并内听道狭窄1例;脑脊液内耳漏2例,MRI显示前庭耳蜗神经畸形;单侧Mondini Ⅰ型畸形合并单侧内听道狭窄1例;双侧内听道狭窄合并前庭半规管发育不良1例;单纯内听道狭窄1例.所有内听道狭窄者MRI均显示其前庭耳蜗神经畸形.其余27例CT和MRI均未见内耳及内听道畸形.结论 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,同时还清晰显示扩大的内淋巴管和内淋巴囊以及内听道内前庭耳蜗神经.因此MRI在评价先天性感音神经性耳聋方面比CT更具优越性.     

儿童人工耳蜗植入术前的影像学评估

分析先天性感音神经性聋患儿人工耳蜗植入术前颞骨高分辨率CT与MRI表现,探讨其应用价值。对78例感音神经性聋患儿行术前的高分辨率CT以及其中68例另行MRI,对61例患儿术后6个月进行听力学评估随访。内耳发育畸形共21例:其中耳蜗不发育1例;MondiniI型1例;单纯MondiniII型1例;前庭导水管扩大14例,其中合并MondiniII型5例,合并半规管发育不良1例;内听道狭窄伴前庭耳蜗神经发育不良1例;半规管发育不良2例,其中1例伴内听道扩大;脑脊液内耳漏1例。术后随访结果提示:耳蜗神经发育不良者听力无明显改善,Mondini畸形者的预后较大前庭导水管综合征者及无特殊畸形者差。CT能显示外耳、中耳及内耳骨迷路解剖,MRI则能显示包括膜迷路在内的内外淋巴液,同时还可显示内听道内前庭蜗神经,而耳蜗神经的存在是CI手术的必要条件,不同类型的内耳畸形与预后之间可能存在一定相关性。     

先天性内耳畸形的CT、MRI表现

目的 :分析先天性内耳畸形的影像学特点。方法 :收集120例(224耳)先天性内耳畸形的临床资料,所有患儿均行CT及MRI扫描,分析其临床特点与影像学表现。结果 :120例中,听力障碍102例(85.00%),面部形态异常14例(11.67%),外耳畸形4例(3.33%)。CT及MRI示耳蜗前庭畸形17例,前庭畸形14例,半规管畸形39例,内听道畸形50例。其中伴前庭导水管和耳蜗导水管畸形87例(72.50%)。结论:内耳畸形是小儿最主要的致聋因素,临床表现多样,CT可观察内耳结构,MRI可判断内耳膜迷路及听神经的发育程度,为内耳畸形的临床诊断、分类及制订治疗方案提供影像学依据。     

顺序和针对性图像分析方法诊断内耳畸形

目的:探讨内耳畸形的颞骨CT诊断和术前评价意义。方法:采用顺序性和针对性图像观察方法回顾性分析26例内耳畸形的颞骨CT表现.与原诊断报告的描述进行比较,并进行一致性检验。结果:26例(共52侧耳)内耳畸形患者中耳蜗发育不全15例、前庭导水管扩大19例、半规管发育不良和前庭扩大11例,这4种畸形单发或联合者占所有骨迷路畸形的80%。原诊断报告中主要遗漏了半规管发育不良4侧耳、前庭导水管扩大7侧耳、耳蜗发育不全6侧耳。采用顺序性图像观察方法,两位阅片者诊断内耳畸形的Kappa值为0.97;与原诊断报告的Kappa值分别为0.51和0.55。人工耳蜗植入术前评价发现耳蜗及前庭与内听道底部骨质缺损17侧耳、半规管骨壁缺损2侧耳、面神经管迷走6侧耳。结论:高分辨力颞骨CT扫描对详细了解内耳畸形的程度、部位及类型具有重要意义。采用顺序性观察方法可避免内耳畸形的漏诊;针对性的评价对充分做好人工耳蜗植入术前准备具有指导意义。     

MSCT和MRI对人工耳蜗植入术前的评价

目的:探讨MSCT和MRI对人工耳蜗植入术前的评价方法及其临床价值。方法:对43例拟行人工耳蜗植入术的患者行MSCT和MRI检查,分析其影像学表现,指导临床选择适应证和植入耳侧。结果:43例中20例MSCT和MRI均未发现异常,23例(42耳)MSCT和MRI均发现有异常,其中先天性内耳发育畸形20例39耳:①MicheⅠ型1耳,表现为内耳结构完全缺如;②共同腔畸形3耳,耳蜗和前庭融合呈一囊状结构;③不完全分隔Ⅰ型(IP-Ⅰ型)3耳,耳蜗和前庭均呈囊状,两者轮廓可以区分;④Mondini型5耳,耳蜗仅1.5圈,中圈和顶圈融合,前庭扩大;⑤前庭与半规管畸形13耳:耳蜗正常,前庭扩大,半规管短小、粗大或缺如;⑥前庭导水管扩大14耳,前庭导水管扩大呈喇叭口状或与总脚相通。骨化性迷路炎3例3耳,MSCT表现为内耳迷路内密度不同程度增高,MRI表现为迷路信号减低。MRI显示蜗神经缺如5耳,蜗神经细小2耳。结论:术前MSCT和MRI检查相互补充,应作为常规术前检查。MSCT在显示中耳结构和立体显示内耳畸形的形态方面优于MRI,可指导手术方案的制定和判断预后,MRI对于评价听神经是否正常有重要价值。     

磁共振内耳成像在先天性内耳发育畸形中的诊断应用

目的:探讨磁共振内耳成像在先天性内耳发育畸形中的临床应用价值。方法:18例先天性感音神经性耳聋患者行MRI扫描并进行后处理,主要方法包括多平面重组(MPR)、最大密度投影(MIP)及客积再现技术(VRT)。结果:18例34耳存在内耳畸形。1例2耳Michel畸型;2例3耳共同腔畸形;4例8耳Mondini畸型;8例16耳前庭导水管扩大;3例5耳显示内听道畸形。MRI MPR、MIP及VRT重组图像可以清晰地显示内耳畸形的部位和程度。结论:磁共振内耳成像能清楚、立体显示正常膜迷路和内听道内神经结构,对于内耳畸形的诊断及人工耳蜗植入术前评价有重要价值。     

蜗神经管狭窄与蜗神经发育相关性研究

目的探讨蜗神经管狭窄与蜗神经发育的相关性。方法回顾性的分析51例(102耳)感音性耳聋(SNHL)患儿的颞骨HRCT及内耳MRI表现。对各种内耳畸形进行分类;从中选取41例(82耳)内听道正常并耳蜗存在的患儿,依据HRCT中蜗神经管(CNC)管径大小分三组,a:>1.8mm,b:1.8～1.5mm,c:<1.5mm,同时根据MRI表现将蜗神经(CN)分正常及发育不良,分析蜗神经管狭窄与蜗神经发育相关性。结果 51例患儿中14例出现不同程度内耳的畸形;41例82耳中53耳CNC>0.18mm,其中2耳蜗神经发育不良;15耳CNC 1.8～1.5mm,2耳蜗神经发育不良;14耳CNC<0.15mm,12耳蜗神经发育不良。所得数据进行Spearman相关性分析,相关系数r=0.641,各组蜗神经发育不良分别占3.7%、13.3%、85.7%。结论蜗神经管狭窄与蜗神经发育呈中度正相关性,蜗神经管越窄,提示合并蜗神经发育不良准确性越高,CNC<0.15mm,强烈提示蜗神经发育不良的存在,在HRCT诊断中要给予重视,但蜗神经管的狭窄可以有正常的蜗神经,因此人工耳蜗植入术前HRCT、MRI均是不可缺少的检查。     

Waardenburg综合征(附一例报告并文献复习)

目的:提高对Waardenburg综合征的临床、影像学表现的认识及诊断水平。方法:报告我院1例确诊为Waardenburg综合征Ⅰ型的临床及影像学资料,结合文献复习、总结Waardenburg综合征的临床特点及影像学表现。结果:双侧先天性感音神经性聋伴虹膜异色,内眦外移,鼻根增宽是本例的临床特征。CT示双侧耳蜗仅11/2周,骨螺旋板缺如,底周扩大,中及上周融合;双侧前庭扩大;双侧外半规管增粗,右上半规管形成不全;双侧内听道增宽。MRI示双侧耳蜗、前庭及左侧上半规管呈囊状扩张,双侧内耳道扩大,右侧耳蜗神经及前庭下神经显示不清,左侧耳蜗神经明显变细(发育不良)。结论:CT及MRI对检出Waardenburg综合征的内耳畸形有重要价值。Waardenburg综合征有特征性虹膜异色及先天性重度感音神经性聋临床征象。     

多层CT对先天性内耳畸形的诊断价值

目的：探讨多层CT（MDCT）对先天性内耳畸形的诊断价值。材料和方法：对50例感音神经性耳聋（SNHL）患者行MDCT螺旋扫描，原始数据采集完后，按层厚0．625mm，重建间隔0．3mm，单侧FOV9．6cm放大重建，再传至工作站行多平面重建（MPR）并利用最小密度投影（MinIP）、容积漫游（VR）及其叠加技术等后处理方法观察内耳结构。结果：50例感音神经性耳聋患者中有20例37耳先天性内耳畸形，其中耳蜗未发育1耳、共同腔畸形1耳、不完全分隔Ⅰ型1耳、不完全分隔Ⅱ型即Mondini畸形8耳、前庭和半规管畸形22耳、前庭导水管扩大20耳、内耳道畸形8耳，伴内耳道底发育不全3耳、外耳道骨性闭锁2耳、面神经管异常4耳、前庭窗闭锁2耳、蜗窗闭锁1耳。结论：MDCT螺旋扫描结合后处理技术能清楚显示先天性内耳畸形的部位和程度并明确其分类，为电子耳蜗的植入提供依据。     

POU3F4基因突变所致X连锁的非综合征型耳聋患者影像学表现

目的回顾性分析POU3F4基因突变所致X连锁的非综合征型耳聋患者HRCT和MRI表现及特征。方法收集4例POU3F4基因突变所致X连锁的非综合征型耳聋患者的HRCT和MRI资料,在二维图像上(多平面重组图像)上回顾性评价耳蜗、内听道、前庭、半规管、前庭导水管、内听道底神经管、镫骨和耳蜗神经,在三维图像上(CT仿真内镜图像)评价耳蜗神经孔。收集10例听力正常的患者影像资料作为对照组。结果患者内耳畸形是双侧对称性的。耳蜗畸形表现为耳蜗外形大致正常,蜗轴缺如,内听道与耳蜗内腔直接沟通;内听道外侧部扩张;CTVE直接显示螺旋形耳蜗内腔而非对照组所示的螺旋形蜗神经孔。面神经管迷路段和上前庭神经管扩张。Bill嵴肥大,部分气化。7耳发现镫骨底板增厚和镫骨前间隙消失,1耳镫骨呈柱状。结论蜗轴缺如、内听道外侧部扩张及镫骨底板增厚是本组POU3F4基因突变所致X连锁的非综合征型耳聋患者的典型影像学特征。此类患者术前检查可指导治疗方法的选择,应避免镫骨底板切除术而行人工电子耳蜗植入术,同时,影像学检查可预测电子耳蜗植入术中并发症,如脑脊液漏和电极进入内听道。     

Pediatric sensorineural hearing loss, part 1: Practical aspects for neuroradiologists

SNHL is a major cause of childhood disability worldwide, affecting 6 in 1000 children. For children with prelingual hearing loss, early diagnosis and treatment is critical to optimizing speech and language development, academic achievement, and social and emotional development. Cross-sectional imaging has come to play an important role in the evaluation of children with SNHL because otolaryngologists routinely order either CT or MR imaging to assess the anatomy of the inner ears, to identify causes of hearing loss, and to provide prognostic information related to potential treatments. In this article, which is the first in a 2-part series, we describe the basic clinical approach to imaging of children with SNHL, including the utility of CT and MR imaging of the temporal bones; we review the most recent proposed classification of inner ear malformations; and we discuss nonsyndromic congenital causes of childhood SNHL.     

Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes

This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. Previously, we discussed the clinical work-up of children with hearing impairment, the classification of inner ear malformations, and congenital nonsyndromic causes of hearing loss. Here, we review and illustrate the most common syndromic hereditary and acquired causes of childhood SNHL, with an emphasis on entities that demonstrate inner ear abnormalities on cross-sectional imaging. Syndromes discussed include BOR syndrome, CHARGE syndrome, Pendred syndrome, Waardenburg syndrome, and X-linked hearing loss with stapes gusher. We conclude the article with a review of acquired causes of childhood SNHL, including infections, trauma, and neoplasms.     

先天性感音神经性耳聋患儿的CT病因分析

目的：探讨先天性感音神经性聋儿患者的CT诊断价值及可能的病因分析。方法：收集120例先天性感音神经性耳聋患儿高分辨率CT图像,对内耳结构行MPR重组最佳显示层面,观察其形态结构、大小、位置有无异常。结果：120例患者中有21例（33耳）内耳发育畸形,其中共同腔畸形2例（3耳）,耳蜗发育不全3例（4耳）,耳蜗不完全分隔Ⅱ型9例（17耳）,半规管畸形7例（10耳）。结论：多层螺旋CT能准确显示先天性骨性内耳畸形的部位和程度,为先天性感音神经性聋儿的病因分析及临床制定正确的治疗方案提供重要的参考依据。     

Review of congenital inner ear abnormalities on CT temporal bone

The aetiology of profound hearing loss in children is complex and multifactorial. Congenital inner ear abnormality is a major cause of hearing loss in children. CT temporal bone imaging is the modality of choice in the investigation of hearing loss. Recognising the congenital abnormalities of the inner ear guides the clinician's management of the condition. This pictorial essay illustrates the congenital abnormalities of the inner ear on high resolution CT temporal bone images and correlation with developmental arrest during embryology.     

Evaluation of labyrinthine fistula by MR cisternography

Sensorineural hearing loss (SNHL) can develop after trauma and determination of its etiology is crucial for treatment planning. Computed tomography (CT) is the first-line imaging method in evaluation of trauma victims; however, its value is limited in visualization of perilymphatic fistula or in assessment of cochlear implant candidates. Herein, we present imaging findings of a patient with traumatic bilateral transverse fractures of temporal bones and resultant SNHL. This patient had a fistula between inner ear and subarachnoid space which was detected at non-contrast-enhanced MR cisternography (NCE-MRC) and contrast-enhanced MR cisternography (CE-MRC) scans. To the best of our knowledge, this is the first case in the literature. In conclusion, in patients with transverse fractures of temporal bone, in presence of otic capsule involvement at CT images, the inner ear structures and surrounding organs should be carefully evaluated via NCE-MRC. In case of a suspicion of a fistula, CE-MRC can help in early diagnosis and can also help to visualize the leakage location, which is of paramount importance for correct treatment and management of such cases.     

Ear malformations: what do radiologists need to know?

Ear malformations represent 50% of ear, nose and throat malformations. Ear malformations cause conductive hearing loss (CHL) and/or sensorineural hearing loss (SNHL) with a significant childhood disability worldwide. Early accurate diagnosis and treatment are mandatory to enhance language and speech development. Understanding the embryology of the ear explains the outcome of ototoxic prenatal insult according to the affected gestational age and the incidence of association among inner, middle, and external ear malformations. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations of the temporal bone are used in the evaluation of ear malformations. In this review article, the spectrum of ear malformations is discussed in detail with hints on the ear embryology, the ear radiological anatomy, and radiological determinant factors of operative reconstruction of ear anomalies.     

儿童先天性耳聋的颞骨高分辨CT研究

目的:了解我国儿童先天性耳聋颞骨畸形的发生率及特点,为临床提供必要的依据。方法:175例听力筛查结果提示听力障碍的婴儿及其他5岁以下先天性耳聋的儿童。采用颞骨螺旋CT扫描,然后进行冠状面MPR重组。结果:CT共检出颞骨畸形61例,其中,外耳畸形24例(36耳),中耳畸形16例(20耳),内耳畸形34例(60耳),其中13例为合并畸形;单纯鼓室积液20例(32耳);其余94例颞骨无异常。约35%患者颞骨有畸形,55%患儿颞骨结构正常。结论:颞骨高分辨CT横断面螺旋扫描和冠状面MPR重组,能很好显示儿童先天性耳聋的颞骨解剖异常,鼓室积液可能是造成婴幼儿听力障碍的重要原因。     

Large vestibular aqueduct in distal renal tubular acidosis. High-resolution MR in three cases

High-resolution MR of the inner ear performed in 3 consecutive pediatric patients affected by distal renal tubular acidosis (dRTA) and progressive sensorineural hearing loss (SNHL) revealed enlarged vestibular aqueducts (LVA) (bilateral in 1 case and unilateral in 2). LVA is associated to sporadic, progressive SNHL, often secondary to minor head trauma and activities involving the Valsalva maneuver.We believe that the presence of LVA may have contributed to the onset of SNHL and its progression in our patients, and therefore want to stress the importance of morphological studies of the inner ear in patients affected by dRTA and SNHL.     

Systematic approach by computed tomography and magnetic resonance imaging in cochlear implantation candidates in Suez Canal University Hospital

Objectives

To create a systematic approach using computed tomography (CT) and magnetic resonance imaging (MRI) findings to facilitate identifying the etiology of hearing loss, evaluating the anatomy for surgery, and predicting complications.