蜗神经发育不良患者的人工耳蜗植入

目的:观察蜗神经发育不良的患儿人工耳蜗植入术后的效果,探讨术前HRCT和MRI对蜗神经发育不良的诊断及评估价值。方法:回顾性分析10例蜗神经发育不良患儿的术前影像学资料及人工耳蜗植入术后对声音的反应和语训效果。结果:3例(6耳)HRCT提示双侧内耳道狭窄,耳蜗、前庭和半规管发育正常,MRI提示蜗神经发育不良。7例(14耳)HRCT提示双侧内耳道正常,其中1例IP-Ⅰ型,伴前庭半规管发育不良,6例双侧耳蜗、前庭和半规管发育正常;7例MRI提示蜗神经发育异常。10例患儿均行人工耳蜗植入,经过12～32个月的随访,3例学会简单语言,MAIS量表、CAP分数及SIR分级较前提高;1例虽能讲话,但口齿含糊;4例对声音有反应,仅能说"爸爸"、"妈妈"等单音节词;2例无反应(1例取出)。10例蜗神经发育不良患儿同其他蜗神经发育正常儿童相比,术后康复情况明显落后。结论:对蜗神经缺陷患儿,术前仅做颞骨HRCT检查易漏诊,结合内耳道MRI(含内耳道斜矢状位重建)已成必须。若发现疑似病例,应慎重筛选,告知行人工耳蜗植入效果不佳的巨大可能性,严格掌握手术指征。     

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。     

蜗神经缺陷的MRI表现

高分辨率磁共振成像(MRI)能清晰显示内耳道(IAC)神经解剖和内耳结构,IAC内有面神经(Fn)和前庭蜗神经的三条分支,即蜗神经(Cn)、前庭上神经(Vsn)和前庭下神经(Vin)。正常情况下,与IAC轴向垂直的斜矢状面成像时能显示IAC内的四条神经,Fn     

HRCT指导下人工耳蜗植入术相关的解剖研究

目的:在HRCT扫描重建的引导下,模拟人工耳蜗植入入路主要步骤解剖,作相关的解剖和HRCT数据测量对照以便为人工耳蜗植入术前影像学评估提供指导.方法:通过6例儿童颞骨标本解剖,模拟人工耳蜗主要步骤解剖,解剖前后作HRCT横轴位和斜矢状位扫描,观察测量手术解剖和HRCT之间的关系.结果:解剖测量面神经隐窝宽度在蜗窗水平为(3.13±0.34)mm,在前庭窗水平宽度为(4.12±0.44)mm,HRCT测量面神经隐窝宽度在蜗窗水平为(3.20±0.38)mm,在前庭窗水平为(4.14±0.47)mm,斜矢状位HRCT可使面神经垂直段、水平段全程显示.解剖测量数据与HRCT测量数据差异无统计学上意义(P>0.05).结论:横轴位面神经到外耳道后壁的距离、斜矢状位面神经到蜗窗距离是反映面神经位置关系的很重要的参数,面神经垂直段 、外耳道后壁、蜗窗是测量的重要解剖标志,术前作HRCT人工耳蜗植入相关测量可用来指导临床手术.     

耳蜗神经发育不良听神经病谱系障碍患者听力学分析

目的分析中国听神经病谱系障碍患者蜗神经的发育情况,进一步探讨中国听神经病谱系障碍患者的相关影像学特征。方法本研究以19例行内听道斜矢状位高分辨核磁共振成像的听神经病谱系障碍患者为研究对象,进行详细的病史采集和听力学检测。纯音测听检测、声导抗检测、听性脑干反应、DPOAE检测、言语识别率检测等,回顾性分析听神经病谱系障碍患者的蜗神经影像学特征,并探讨蜗神经发育异常患者的听力学特征。结果本研究的19例听神经病谱系障碍患者中,共发现4例蜗神经发育异常的患者,所占比例为21.05(4/19)。4例患者在内听道斜矢状位MRI上均表现为双侧蜗神经细小。其中,2例患者有耳聋家族史,1例患者伴有共济失调障碍。4例蜗神经发育异常的患者听力曲线类型各异:2例患者表现为全频中度下降的平坦型听力曲线,1例表现为中重度低频上升型听力曲线,另外1例表现为中重度的高频下降型听力曲线。4人(8耳)的言语识别率均较差,除1耳外,其余7耳的言语识别率均低于40%。结论对于听神经病谱系障碍的患者,若条件允许的情况下,应常规行内听道斜矢状位高分辨核磁共振成像,以评价蜗神经粗细及发育情况,对于了解听神经病谱系障碍患者的病变部位和病因。     

1例耳蜗前庭神经畸形患者耳蜗植入术前的功能性磁共振评估并文献复习

目的:探讨功能性磁共振(fMRI)评价耳蜗前庭神经畸形患者是否有人工耳蜗植入指征的可能性。方法:对1例15岁双侧耳聋患者,在人工耳蜗植入前进行听力学、影像学检查及fMRI综合评估。结果:纯音测听和听觉脑干反应显示患者为双侧极重度感音神经性聋,瞬态诱发性耳声发射双侧均未引出。颞骨薄层CT显示内耳畸形(不全分隔Ⅰ型),MRI斜矢状位重建显示耳蜗前庭神经畸形。fMRI显示左耳给声,1000Hz100dBHL,右侧听皮层被激活;右耳给声,双侧听皮层均未见激活。左侧人工耳蜗植入术后1个月开机,患者对声音有反应。结论:结合听力学和影像学检查,fMRI可评估人工耳蜗植入候选者,尤其是在影像学检查显示患者耳蜗前庭神经发育畸形时,更是一种有效的评估方法。     

细菌性脑膜炎致聋患者内耳影像学观察及人工耳蜗置入术

目的 :了解细菌性脑膜炎后内耳MRI影像学的变化 ,及对人工耳蜗置入术的影响。方法 :细菌性脑膜炎致聋患者 5例 (10耳 )行听性脑干诱发反应 (ABR)和内耳MRI检查 ,实施人工耳蜗置入术。结果 :MRI成像检查 ,耳蜗膜迷路形态异常 5耳 ,前庭形态异常 3耳 ,半规管形态异常 8耳 ;10耳的平均听阈为 (10 2 .0± 7.1)dBHL ,手术耳的平均听阈为 (98.0± 5 .7)dBHL ,非手术耳的平均听阈为 (10 6 .0± 6 .5 )dBHL ;实施人工耳蜗置入术时 3耳没能将电极全部插入 ;发病到发现听力下降平均为 (15 .8± 15 .0 )d ,小儿较成人发现听力下降时间延迟。结论 :细菌性脑膜炎可引起不同程度的内耳形态异常 ,人工耳蜗置入术前进行MRI检查可以发现内耳形态异常的程度 ,利于正确选择术耳及手术的顺利进行     

267蜗神经异常的磁共振成像及临床意义

高分辨率磁共振成像(MRI)能清晰显示内耳道内神经解剖和内耳结构,本文介绍蜗神经的胚胎学、解剖学、MRI的扫描方法、蜗神经异常的影像学表现及病因,讨论蜗神经高分辨率MRI在感音神经性聋的诊断和人工耳蜗植入术的临床意义.     

蜗神经异常的磁共振成像及临床意义

高分辨率磁共振成像(MRI)能清晰显示内耳道内神经解剖和内耳结构，本介绍蜗神经的胚胎学、解剖学、MRI的扫描方法、蜗神经异常的影像学表现及病因，讨论蜗神经高分辨率MRI在感音神经性聋的诊断和人工耳蜗植入术的临床意义。     

耳蜗神经发育异常的小儿听神经病

目的 探讨耳蜗神经发育异常的小儿听神经病特征.方法 分析一例耳蜗神经发育异常的听神经病小儿的听力学检测资料、高分辨CT(HRCT)及高分辨磁共振成像(HRMRI)内耳扫描的结果.结果 视觉强化测听右耳最大声强级未引出,左耳听力曲线呈重度低频上升型.鼓室导抗图右、左耳分别为A与As型,双耳声反射均未引出.双侧ABR均未引出.DPOAE经前后三次检测,右耳除个别低频或中频频点未引出外,其余频率正常引出,左耳除个别低频频点及一次伴两中频频点未引出外,其余频率可引出.HRCT显示右侧内听道较左侧稍狭窄,HRMRI内听道斜矢状位显示右侧耳蜗神经信号缺失,左侧耳蜗神经信号细小.结论 耳蜗神经发育异常可呈现听神经病的听力学特征,提示耳蜗神经发育异常可为听神经病病变部位及病因之一.HRMRI内听道斜矢状位扫描技术对显示耳蜗神经异常具有重要意义.     

Magnetic resonance imaging versus computed tomography in pre-operative evaluation of cochlear implant candidates with congenital hearing loss

Recent reports indicate that the cochlear nerve may be absent in some cases of congenital sensorineural hearing loss. The aim of this prospective study was to determine the incidence of cochlear nerve anomaly in cochlear implant candidates with congenital hearing loss using magnetic resonance imaging (MRI). Twenty-seven patients with congenital profound bilateral sensorineural hearing loss who were being evaluated for the cochlear implant procedure were studied. These patients had high-resolution computerized tomography (CT), through the petrous bone in axial sections. MRI examinations consisted of T1 and turbo spin echo (TSE) T2-weighted 3 mm axial images, and additional 3D Fourier Transform T2-weighted TSE sequences obtained on three different planes (axial, perpendicular and parallel to the internal auditory canal (IAC) i.e. oblique sagittal and coronal, respectively) for the purpose of cochlear nerve demonstration. Results showed that all of the 14 patients with normal CT of the temporal bone, had four distinct nerves in the distal part of the IAC on TSE-MRI. Thirteen patients demonstrated various bony malformations of the cochleovestibular system on CT. MRI revealed the absence of the cochleovestibular nerve in four patients where the IAC was very narrow or completely absent on CT. One patient with severe Mondini malformation who had an enlarged IAC demonstrated an isolated absent cochlear nerve.     

Value of computed tomography in the evaluation of children with cochlear nerve deficiency.

OBJECTIVE: To assess the predictive value of high-resolution computed tomography (HRCT) in the evaluation of children with cochlear nerve deficiency (CND). STUDY DESIGN: Retrospective review of medical records. SETTING:: Tertiary referral center, hospital setting. PATIENTS: Nineteen children (31 ears) with CND. INTERVENTIONS: Magnetic resonance imaging (MRI), HRCT, and audiologic evaluation. MAIN OUTCOME MEASURES: Comparisons of the morphology of the internal auditory canal (IAC), the bony cochlear nerve canal (BCNC) as seen on HRCT, and audiologic data. RESULTS: Of 12 ears with MRI evidence of an absent cochlear nerve (CN) and a normal-size IAC, all had a patent BCNC as revealed by HRCT. Four of these ears failed auditory stimulation after cochlear implantation, confirming clinically significant CND. Of 15 ears with a narrow IAC and a single nerve visible on MRI, 2 (13.3%) had a normal-size BCNC, 4 (26.7%) were narrow, and 9 (60.0%) were absent. One ear with a narrow IAC, normal BCNC, and a single nerve as revealed by MRI has benefited from cochlear implantation. CONCLUSION: Using BCNC patency, as revealed by HRCT, as a means of identifying CND would miss all cases of absent CNs in the setting of a normal-size IAC. Thus, MRI should be the primary modality for imaging children with severe to profound sensorineural hearing loss. When MRI demonstrates a single nerve within a narrow IAC, the addition of HRCT can further identify more than half of these cases as involving absent CNs because of an absent BCNC. In a subset of patients, CN status remains indeterminate.     

MRI surveillance following concurrent cochlear implantation in cases of vestibular schwannoma resection

ObjectiveCochlear nerve preserving translabyrinthine vestibular schwannoma (VS) resection enables concurrent cochlear implantation. Implantation in patients with VS raises important concerns including the ability to undergo postoperative magnetic resonance imaging (MRI) monitoring of residual tumor growth or tumor recurrence, specifically with a retained magnet. We aim to assess the feasibility of MRI monitoring and the impact on image quality with retained cochlear implant (CI) magnets.MethodsRetrospective review of post-operative head MRI scans in CI recipients with a retained CI magnet, after cochlear nerve preserving translabyrinthine excision of VS. The ability to visualize the ipsilateral and contralateral internal auditory canal (IAC) and cerebellopontine angle (CPA) were assessed.ResultsA total of eight surveillance head MRI were performed in six patients. In one case, in which the receiver was positioned lower, the view of the ipsilateral IAC and CPA was distorted. In all other cases, the views of both the ipsilateral and contralateral IAC and CPA were overall unimpaired.DiscussionImaging artifact only very rarely impedes adequate visualization of the ipsilateral IAC or CPA in CI recipients. In anticipation of the need for further IAC and CPA imaging, it would be advisable to place the receiver in an exaggerated superior-posterior position to further decrease obscuring artifact. Thus, serial monitoring of VS tumors can be performed safely with preservation of image quality with a retained receiver magnet.ConclusionsWhen placing the CI receiver-stimulator farther posterior-superiorly, excellent visualization of the IAC and CPA can be accomplished without significantly impairing the image quality.     

蜗神经管狭窄患者人工耳蜗植入术后听觉康复效果分析

目的评估极重度感音神经性聋伴蜗神经管狭窄患者人工耳蜗植入术后的听觉康复效果。方法回顾分析10例极重度感音神经性聋伴蜗神经管狭窄患者的临床资料,包括听力学、影像学、术中神经反应测试及术后听觉康复效果评估结果。结果10例患者术耳人工耳蜗植入前听性脑干反应、40Hz听觉事件相关电位、畸变产物耳声发射均未引出,颞骨薄层CT示不同程度的蜗轴与内听道之间的蜗神经管狭窄。术中神经反应测试2例患者有3个电极可引出神经反应波形,1例患者有2个电极可引出神经反应波形,6例患者有1个电极可以引出神经反应波形,1例患者术中未引出神经反应波形。8N患者术后超过2年,其3个月、6个月、9个月、1年、2年时的平均单元音识别率分别为60．3％、60．4％、60．3％、60．2％、60．2％,单辅音识别率分别为19．0％、19．1％、19．1％、19．2％、19．2％。2例患者术后仅3个月,其平均单元音、单辅音识别率分别为60．1％、18．2％。结论10例极重度感音神经性聋伴蜗神经管狭窄患者术前无残余听力,术中神经反应测试只有部分电极可以引出神经反应波形,术后听觉康复效果较差。     

Functional magnetic resonance imaging may avoid misdiagnosis of cochleovestibular nerve aplasia in congenital deafness

OBJECTIVE: To investigate a narrow internal auditory canal (IAC) syndrome using functional magnetic resonance imaging (fMRI) of the auditory cortex. STUDY DESIGN: The study design was a case report. The follow-up period lasted 18 months. SETTING: The study was carried out in the audiology clinic of an ear, nose, and throat department and in the department of pediatric neuroradiology at a university hospital. MAIN OUTCOME MEASURES: Age-appropriate observational audiometry, objective audiovestibular tests, computed tomography (CT), magnetic resonance imaging (MRI), and (fMRI) of the auditory cortex were performed to analyze in detail the profound deafness of a young child. RESULTS: Audiovestibular examination demonstrated both measurable hearing and normal vestibulo-ocular reflex, and CT showed narrow IACs combined with normal labyrinths. Axial MR images completed by sagittal sections perpendicular to the IAC delineated a single nerve that was initially supposed to be the facial nerve. No cochleovestibular nerve was identified. However, fMRI performed with the patient under general anesthesia demonstrated activation of the primary auditory cortex during 1-kHz monaural stimulation on the left side. CONCLUSIONS: The absence of cochleovestibular nerve on MR studies cannot exclude connections between the inner ear and the central auditory pathways. This might be caused by a lack of spatial resolution of anatomical MR studies. The single nerve delineated within the IAC might also carry both facial and cochleovestibular fibers. Functional MRI can assess the cortical response to acoustic stimuli when aplasia of the cochleovestibular nerve is suspected. This case study illustrates a novel and atypical presentation of cochlear nerve dysplasia.     

Internal auditory canal stenosis in congenital sensorineural hearing loss

OBJECTIVE: Sensorineural hearing loss (SNHL) due to internal auditory canal (IAC) stenosis with hypoplasia of the cochleovestibular nerve is a rare disorder. The diagnosis of the IAC stenosis requires both high resolution computed tomography scan (HRCT) and magnetic resonance imaging (MRI). METHODS: A retrospective review over 6 years in an academic tertiary referral center was performed. RESULTS: Six patients with congenital SNHL were diagnosed with congenital IAC stenosis. Four had unilateral and two had bilateral IAC stenosis after imaging. MRI showed hypoplastic vestibulocochlear nerve in all cases. CONCLUSIONS: This paper highlights the importance of imaging in diagnosing IAC stenosis and detecting the presence of cochleovestibular nerve in cases of congenital SNHL.     

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     

内听道狭窄的人工耳蜗植入术效果观察并文献复习

摘要：目的通过观察一例内听道狭窄患者人工耳蜗植入术后的远期康复效果,结合文献复习探讨该类畸形的手术选择指标。方法收集患儿术前听力学资料以及颞骨薄层CT资料,分别于术后半年、2年、4年时进行声场测试、MAIS量表评分、言语识别率测试分析患者康复疗效。并以内听道狭窄、蜗神经管狭窄、蜗神经孔狭窄以及蜗神经发育不良为关键词搜索与人工耳蜗康复的相关文献。结果半年时声场测试500、1 000、2 000、4 000 Hz分别为35、40、40、45 dBHL,2年时MAIS量表评分24分,4年时言语识别率80%,患儿于术后3年时言语理解能力进步明显,康复效果优于文献搜索结果。结论存在内听道狭窄的患者,人工耳蜗植入前对影像学及听力测试结果的综合分析非常重要,选择耳蜗神经发育相对较好侧以及助听效果较好侧手术,同时需坚持术后康复治疗。     

Cochlear implantation in cases with incomplete partition type III (X-linked anomaly)

Incomplete partition-type III anomaly (X-linked deformity) is no common finding in a prospective candidate for cochlear implantation. In this paper, the problems about the cochlear implantation in cases with incomplete partition-type III anomaly (X-linked deformity) and profound sensorineural hearing loss is discussed. High-resolution multidedector computed tomography (MDCT) and magnetic resonance imaging were performed preoperatively in all patients. MDCT revealed that there was bulbous dilatation at the lateral ends of internal auditory canals (IAC) in all patients. There were also enlargements of labyrinthine segments of facial and superior vestibular nerve canals. Patients with the basal turns of cochlea incompletely separated from IAC were also presented. Patients with IP-type III (X-linked deformity) and profound SNHL were implanted. Standard transmastoid-facial recess approach was used and cerebrospinal gusher was encountered after the cochleostomy in all cases. Postoperative performance was very good in all patients. Two patients had complications, which are facial nerve stimulation and device failure. Both patients were reimplanted. Cochlear implantation is a good choice in the patients with IP-type III. However, this anomaly may have special potential risk than the other inner ear abnormalities. Therefore, the surgeon should be aware of them and must be ready to inform the patient and parents. An erratum to this article can be found at