大前庭水管综合征患者人工耳蜗植入效果分析

目的：分析大前庭水管综合征（large vestibular aqueduct syndrome,LVAS）患者人工耳蜗植入的疗效。方法回顾性分析37例行人工耳蜗植入术的LVAS患者（LVAS组）的临床资料,另选择临床资料相匹配37例耳蜗结构正常的人工耳蜗植入患者作为对照组,分别于人工耳蜗植入术前和术后3、6、9、12个月评估其言语识别率、听觉行为分级（categories of auditory performance,CAP）和言语可懂度分级（speech intelligibility rating, SIR）。结果①37例LVAS患者术中有5例（5耳）出现严重井喷,20例（20耳）耳蜗钻孔后出现外淋巴液不同程度波动,对照组均未出现“井喷”现象;两组电极均成功植入;两组术后均无面瘫、脑脊液漏、脑膜炎等并发症;②开机调试时两组电极阻抗值、电极反应阈值（T值）及最大舒适阈值（C值）差异无统计学意义（P＞0．05）,术后3、6、9、12个月两组的言语识别率、CAP、SIR评分均随着康复时间的延长而逐渐提高,在相同的康复时间段,两组之间各项康复指标差异无统计学意义（P＞0．05）;③术中发生“井喷”与未发生“井喷”的LVAS患者之间术后康复效果差异也无统计学意义（P＞0．05）。结论人工耳蜗植入可作为LVAS患者听觉康复的手段,其听觉康复效果与内耳发育正常的人工耳蜗植入患者接近。     

电诱发听性脑干反应和电诱发镫骨肌反射阈值在内耳畸形儿童人工耳蜗植入术后调机中的运用

目的　探讨内耳畸形小儿人工耳蜗植入手术后,植入体电诱发听性脑干反应（electrically evoked auditory brainstem responses,EABR）、电诱发镫骨肌反射阈值（electrically evoked stapedius reflex threshold,ESRT）的变化特点及规律,以指导术后设备调试。方法　将88例澳大利亚Cochlear Nucleus24型人工耳蜗植入手术患儿分为耳蜗形态正常组与内耳畸形组,测试手术后1年内不同时期EABR和ESRT值,术后1年运用行为测试法检测主观阈值（T值）和最大舒适阈（C值）,分析特点及变化规律。结果  内耳畸形组患儿术后不同时期EABR和ESRT阈值较正常组高（P＜0.05）,两组EABR和ESRT阈值变化趋势相同,总的趋势是低频值较低,高频值较高,术后1年EABR和ESRT阈值逐渐增高;两组EABR与T值显著相关,ESRT与C值显著相关。结论　内耳畸形组人工耳蜗植入手术后EABR和ESRT阈值变化规律及特点与正常组患儿相同,阈值可用于指导内耳畸形人工耳蜗植入者手术后设备的调试。     

大前庭水管综合征与内耳结构正常患儿人工耳蜗术后心理物理测试比较

目的:探讨大前庭水管综合征患儿人工耳蜗植入术后阈值、舒适阈和电极阻抗的特点,为这类患儿进行人工耳蜗植入术的可行性提供依据。方法:将131例语前聋患儿,根据CT检查结果分为A和B两组,A组111例,内耳结构正常;B组20例,双侧前庭导水管扩大,但不伴有其他内耳结构异常。所有患儿均使用澳大利亚Cochlear公司生产的Nucleus24M型人工耳蜗。术后4周左右安装体外设备并进行心理物理测试,使用与患儿年龄相应的方法进行阈值和舒适阈的测试;使用R116和R126软件自动测试电极阻抗。结果:经独立样本t检验,两组各电极的阈值和舒适阈的差异均无统计学意义(均P>0.05);两组心理物理测试值即阈值和舒适阈,在开机时和开机后1、2、3周,2、3、6、9个月及1年的差异无统计学意义(均P>0.05)。两组各电极阻抗之间的差异无统计学意义(均P>0.05);两组电极阻抗在开机时和开机后1、2、3周,2、3、6、9个月及1年的差异无统计学意义(均P>0.05)。结论:大前庭水管综合征患儿与内耳结构正常患儿人工耳蜗植入术后的差异无统计学意义,为大前庭水管综合征患儿制定术后调试计划及设置各电刺激参数时,均可采用与内耳结构正常患儿相同的方法。     

内耳发育畸形患者人工耳蜗植入术后的疗效分析

目的分析内耳发育畸形患者行人工耳蜗植入术后的疗效。方法回顾性分析2011年1月~2017年7月在西南医科大学附属医院行人工耳蜗植入术的30例内耳发育畸形患者的临床资料,其中大前庭水管综合征(large vestibular aqueduct syndrome,LVAS) 8例,Mondini畸形（不完全分隔Ⅱ型）7例,同时伴有LVAS和Mondini畸形10例,内听道狭窄5例。随机选取同时期临床资料相匹配的30例耳蜗结构正常的人工耳蜗植入患者作为对照组。采用听觉行为分级标准和言语可懂度分级标准进行评估。比较两组患者的术前听力、术中情况以及术后听觉言语康复情况。结果3例LVAS患者在术中耳蜗钻孔后出现外淋巴液不同程度的外涌,1例Mondini畸形患者术中出现脑脊液井喷现象。两组患者术后均获得有意义的听觉反应,术后的听觉言语能力相比较,差异无统计学意义（P>0.05）。同组患者,术后3,6,9,12个月的听觉言语能力均较各自术前均有明显提高（P<0.05）。结论内耳发育畸形患者不是人工耳蜗植入术的绝对禁忌证,其术后言语康复效果与内耳结构正常者无明显差异,但手术难度比内耳结构正常者要大,术前应根据不同的畸形类型制定个性化的手术方案,以减少并发症的发生。     

内耳畸形人工耳蜗植入术后听力言语康复效果分析

目的：使用听觉和言语问卷分级及术后产生听觉的最小电流值（T值）的方法,评估并比较内耳畸形与正常解剖结构语前聋患儿人工耳蜗植入术后的听觉言语康复效果。方法：按术前影像学检查将语前聋人工耳蜗植入患儿分为正常结构组和内耳畸形组,并配对组合,对思儿家长进行问卷形式调查随访,对术后听力及言语康复效果进行评估分析,记录术后1年凋机T值。用秩和检验比较2组听觉行为分级标准（CAP）、言语可懂度分级标准（SIR）结果及T值。结果：人工耳蜗植入患儿家长术前主要担心术后效果不理想及手术并发症的发生,多数认为听力言语康复训练应由医疗机构进行;秩和检验显示：2组CAP及SIR均无显著差异,术后1年调机时产生听觉的T值无显著差异。结论：①内耳畸形患儿人工耳蜗植入术后,经正规康复训练,听力言语康复效果与内耳解剖结构正常植入者相同,人工耳蜗植入术可帮助伴耳蜗畸形的极重度感音神经性聋患者重建听力,重返主流社会;②听力言语康复训练尚有很多方面需要改进。     

伴大前庭水管畸形患者的人工耳蜗植入疗效的初步Meta分析北大核心CSCD

目的通过比较大前庭水管畸形与内耳解剖结构正常的耳聋患者的人工耳蜗植入术后康复效果,进一步明确人工耳蜗植入术对大前庭水管畸形患者听力的改善。方法通过检索2003-2018发表在PubMed、中国知网、维普医学、万方数据等数据库上关于大前庭水管畸形和内耳结构正常的耳聋患者术后康复效果的病例对照研究,使用Revman5.3软件对原始数据进行Meta分析。结果 Meta分析结果显示大前庭水管畸形组较内耳结构正常组更容易发生术中井喷及淋巴液波动(Z=6.28,P<0.05),但在术后听力(Z=0.22,P=0.82)、平均听阈(Z=0.91,P=0.36)及言语康复(Z=0.30,P=0.76),术后电极反应T值(Z=0.92,P=0.36),电极最佳舒适阈值(Z=1.90,P=0.06)等电极参数调试上两者无统计学差异。结论人工耳蜗植入术对伴大前庭水管畸形耳聋患者是有效的,术后康复可以达到和内耳结构正常组相似的效果,电极参数调试和设置也可以参照内耳结构正常组。     

小儿大前庭水管综合征的人工耳蜗植入

目的 探讨应用人工耳蜗植入法治疗大前庭水管综合征(large vestibular aqueduct syndrome，LVAS)的效果。方法 3例LVAS患儿及12例无外耳、中耳及内耳畸形的重度以上感音神经性聋患儿均进行了人工耳蜗植入，并进行回顾性分析，比较两组患儿的手术植入情况及开机时映射调试情况。结果 LVAS患儿植入术中有1例出现“井喷”现象，开机调试时的非听刺激症要多些。非LVAS患儿手术中无明显异常，且电极全部植入；LVAS患儿中开机调试时C-Tlevel差值比无畸形组的小，但在开机1个月时二组之间无显著性差异；二组的补偿听阈在开机1个月时也无显著性差异。结论 人工耳蜗植入对LVAS患儿同样适用、有效。     

内耳畸形小儿人工耳蜗植入术后神经反应遥测值的研究

目的　探讨小儿内耳畸形人工耳蜗植入手术后,植入体不同电极神经反应遥测（neural response telemetry,NRT）的变化特点及规律。方法　对36例澳大利 亚Cochlear Nucleus 24型内耳畸形小儿人工耳蜗植入手术患儿,测试手术中及手术后1年NRT值,并分析其特点及变化规律。结果　患儿术中及开机后1年内同一时间高、中、低频段电极NRT值有显著差异（P<0.05）,低频值较低,高频值较高,总的趋势是术中至术后1年NRT值逐渐增高。结论　内耳畸形小儿人工耳蜗植入手术后NRT值随时间逐渐增高,高、中、低频之间差异大,故调机时要注意各频率之间差异,选取高、中、低频代表性电极测试,以使患者语言感知达到最佳状态。     

声诱发的镫骨肌反射阈确定人工耳蜗植入者最大舒适阈

目的研究经人工耳蜗引出的声诱发的镫骨肌反射阈确定人工耳蜗植入者开机状态下的最大舒适阈值。方法选择11例Nucleus C124型人工耳蜗植入者,其中4例为双侧大前庭水管综合征,1例共同腔内耳畸形,这5例内耳畸形的患者神经反应遥测值(neural response telemetry,NRT)阈值很高或几乎测不出。分别测试其激活状态下C值的平均值(real-MCL)水平和声诱发(白噪声,110dB)的镫骨肌反射阈(acoustically evoked stapedius reflex threshold,ASRT)(单位：电流级CL)。结果real-MCL与ASRT值(CL)有很好的一致性,经统计学分析两者比较无显著性差异(P＞0.05),并且在开机的初期ASRT值随real-MCL值的升高而升高。结论人工耳蜗植人者的声诱发的镫骨肌反射阈能较好的反映激活状态下患者的最大舒适阈值,该方法操作较其他方法简单,结果可靠,可尝试用于确定小儿人工耳蜗植入者的最大舒适阈。     

内耳畸形语前聋患儿人工耳蜗术后康复效果分析

目的　评估内耳畸形语前聋患儿人工耳蜗植入术后康复结果。方法　回顾性分析术前影像学检查为内耳畸形语前聋人工耳蜗21例,并筛选背景接近的21例正常耳蜗结构语前聋人工耳蜗植入患儿配对组合。对患者术前、术后6个月及术后1年3个阶段听力言语康复效果进行评估。结果　9例前庭导水管扩大综合征和6例Mondini 畸形患者与耳蜗结构正常者相比,听觉言语结果无明显差异。畸形比较严重的1例Mondini和5例共同腔患者与耳蜗正常者相比,听觉言语结果有明显的差异。结论　前庭导水管扩大综合征患者人工耳蜗术后效果与耳蜗发育正常者相似,但对严重的Mondini 畸形和共同腔等内耳畸形者行人工耳蜗植入时术前应准确评估畸形的程度及伴发的畸形,以助于术后制定康复方案。     

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。     

Advanced techniques in magnetic resonance imaging in the evaluation of the large endolymphatic duct and sac syndrome

The purpose of this report is to compare temporal bone computed tomography (CT) to high-resolution magnetic resonance (MR) imaging using a novel thin-section fast spin echo (FSE) pulse sequence in identifying and characterizing patients with large vestibular aqueduct syndrome. Sixteen patients with sensorineural hearing loss and a CT diagnosis of large vestibular aqueduct(s) underwent high-resolution fast spin echo magnetic resonance imaging with dual, 3-in phased array receiver coils centered over the external auditory canals. Magnetic resonance imaging parameters included axial and oblique sagittal fast spin echo with an effective slice thickness of 1 mm contiguous. Thirty-eight patients with 76 normal inner ears who underwent MR imaging using this technique had their endolymphatic duct measured. MR alone identified the enlarged endolymphatic sac seen along with the large endolymphatic duct in all cases. Three cases (five inner ears) with enlarged bony vestibular aqueducts on CT showed no evidence of endolymphatic duct or sac enlargement on MR. MR alone identified a single case of mild cochlear anomaly in conjunction with an enlarged endolymphatic duct and sac. In the normal population the size of the normal endolymphatic duct at its midpoint measured from 0.1 to 1.4 mm. Thin-section, high-resolution fast spin echo MR imaging of the inner ear may be superior to CT in the evaluation of patients with the large vestibular aqueduct syndrome.     

磁共振内耳水成像在大前庭水管综合征诊断中的应用

目的：研究磁共振内耳水成像技术在大前庭水管综合征诊断中的作用。方法：应用三维高级快速自旋回波序列横断扫描大前庭水管综合征患者的整个颞骨岩部,将所有原始图像传给网上工作站,应用最大密度投影法将图像进行三维重建,获内耳立体像。结果：患者的磁共振内耳水成像表现为：后颅窝乙状窦前方、内听道后方硬脑膜外的高信号强度结构膨大,呈长条形,边缘光整。患者内淋巴囊骨内部分中点的最大宽度为2．470mm,远大于MRI诊断内淋巴囊扩大的标准。结论：磁共振内耳水成像是诊断大前庭水管综合征的有效方法,临床上可以用此技术做最终诊断。     

Clinical investigation and mechanism of air-bone gaps in large vestibular aqueduct syndrome

OBJECTIVES: Patients with large vestibular aqueduct syndrome (LVAS) often demonstrate an air-bone gap at the low frequencies on audiometric testing. The mechanism causing such a gap has not been well elucidated. We investigated middle ear sound transmission in patients with LVAS, and present a hypothesis to explain the air-bone gap. METHODS: Observations were made on 8 ears from 5 individuals with LVAS. The diagnosis of LVAS was made by computed tomography in all cases. Investigations included standard audiometry and measurements of umbo velocity by laser Doppler vibrometry (LDV) in all cases, as well as tympanometry, acoustic reflex testing, vestibular evoked myogenic potential (VEMP) testing, distortion product otoacoustic emission (DPOAE) testing, and middle ear exploration in some ears. RESULTS: One ear with LVAS had anacusis. The other 7 ears demonstrated air-bone gaps at the low frequencies, with mean gaps of 51 dB at 250 Hz, 31 dB at 500 Hz, and 12 dB at 1,000 Hz. In these 7 ears with air-bone gaps, LDV showed the umbo velocity to be normal or high normal in all 7; tympanometry was normal in all 6 ears tested; acoustic reflexes were present in 3 of the 4 ears tested; VEMP responses were present in all 3 ears tested; DPOAEs were present in 1 of the 2 ears tested, and exploratory tympanotomy in 1 case showed a normal middle ear. The above data suggest that an air-bone gap in LVAS is not due to disease in the middle ear. The data are consistent with the hypothesis that a large vestibular aqueduct introduces a third mobile window into the inner ear, which can produce an air-bone gap by 1) shunting air-conducted sound away from the cochlea, thus elevating air conduction thresholds, and 2) increasing the difference in impedance between the scala vestibuli side and the scala tympani side of the cochlear partition during bone conduction testing, thus improving thresholds for bone-conducted sound. CONCLUSIONS: We conclude that LVAS can present with an air-bone gap that can mimic middle ear disease. Diagnostic testing using acoustic reflexes, VEMPs, DPOAEs, and LDV can help to identify a non-middle ear source for such a gap, thereby avoiding negative middle ear exploration. A large vestibular aqueduct may act as a third mobile window in the inner ear, resulting in an air-bone gap at low frequencies.     

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.     

The large vestibular aqueduct syndrome

It has long been known that abnormally large vestibular aqueducts may accompany congenital malformations of the cochlea and semicircular canals. Recently, enlargement of the vestibular aqueducts as the sole radiographically detectable inner ear anomaly has been recognized as a distinct pattern of congenital inner ear malformation. Pathogenesis of the large vestibular aqueduct syndrome probably stems from an early derangement in the embryogenesis of the endolymphatic duct. This anomaly appears to be relatively common in children with sensorineural hearing loss and is probably significantly underdiagnosed. Hearing loss is typically bilateral and progressive, with stepwise rather than fluctuant hearing decrements often triggered by relatively minor head trauma. A review of 17 patients (33 ears) revealed an average hearing level at presentation of 57 dB with a speech discrimination score of 66%. Considerable variability exists in hearing level among affected ears, ranging from normal hearing (4%) to profound deafness (39%). In 12 patients (23 ears) with an average long-term follow-up of 7.3 years, the hearing loss progressed by an average of 25 dB, with a drop of 29% in speech discrimination over the period of observation. An endolymphatic to subarachnoid shunt was performed on seven ears in an effort to stabilize hearing. Four of these ears had a substantial immediate postoperative drop in hearing. For this reason, endolymphatic sac surgery is not recommended for patients with this deformity.     

Vestibular Disturbance in Patients with Large Vestibular Aqueduct Syndrome (LVAS)

Large vestibular aqueduct syndrome (LVAS) is a common inner ear anomaly responsible for some unusual vestibular and audiological symptoms. The gross appearance of CT scan of the inner ear is generally normal. However, precise measurement of the inner ear components reveals abnormal dimensions, which may account for accompanying auditory or vestibular dysfunction. It has been reported that sudden increase in cerebrospinal fluid pressure can cause further deterioration of hearing due to transmission of pressure to the inner ear through the enlarged vestibular aqueduct. However, vestibular function is not often studied. In this report, audiovestibular function of 10 patients with large vestibular aqueducts was analysed and compared with the severity of the radiological deformity. The literature was reviewed and typical findings were discussed to emphasize varying aspects of audiovestibular function. It was found that some patients with LVAS have some spontaneous or provoked vestibular disturbance such as vertigo after watching revolving objects. The mean value of electronystagmographic abnormality in patients with hearing loss is greater than in patients with normal hearing. However, there is no statistical correlation between the level of hearing loss, electronystagmographic abnormality and severity of radiological deformity.     

Vestibular disturbance in patients with large vestibular aqueduct syndrome (LVAS)

Large vestibular aqueduct syndrome (LVAS) is a common inner ear anomaly responsible for some unusual vestibular and audiological symptoms. The gross appearance of CT scan of the inner ear is generally normal. However, precise measurement of the inner ear components reveals abnormal dimensions, which may account for accompanying auditory or vestibular dysfunction. It has been reported that sudden increase in cerebrospinal fluid pressure can cause further deterioration of hearing due to transmission of pressure to the inner ear through the enlarged vestibular aqueduct. However, vestibular function is not often studied. In this report, audiovestibular function of 10 patients with large vestibular aqueducts was analysed and compared with the severity of the radiological deformity. The literature was reviewed and typical findings were discussed to emphasize varying aspects of audiovestibular function. It was found that some patients with LVAS have some spontaneous or provoked vestibular disturbance such as vertigo after watching revolving objects. The mean value of electronystagmographic abnormality in patients with hearing loss is greater than in patients with normal hearing. However, there is no statistical correlation between the level of hearing loss, electronystagmographic abnormality and severity of radiological deformity.