听神经病患者耳蜗电图-SP、AP幅度分析

目的分析听神经病患者耳蜗电图中-SP、AP的幅度,探讨该病患者-SP/AP比值增高的原因.方法用银球电极放置在鼓膜后下方,记录听神经病患者和正常人的耳蜗电图,分别测量两组中-SP、AP的幅度.结果听神经病患者-SP幅度与正常组无明显差异(P＞0.05),而AP幅度两组存在极显著差异(P＜0.001).结论听神经病患者-SP/AP比值增高的原因是由于AP幅度的明显降低而造成.     

听神经病患者的耳蜗电图特征

目的:了解听神经病患者的耳蜗电图特征。方法:用外耳道银球电极记录听神经病组和正常对照组的耳蜗电图,比较两者的-SP波幅差异,统计患耳AP出现的情况。结果:听神经病患者-SP波的引出率为100％,并且其波幅与正常对照组的差异有显著性意义(P<0.01)。AP波幅较小,但引出率高达84％。结论:听神经病患者的-SP可引出且波幅增大。     

隐匿性听力损失和耳蜗带状突触病变——对听觉损害与保护的新考量

隐匿性听力损失(Hidden Hearing Loss,HHL)的基础和临床研究是近年来耳科学领域的热点课题。基于动物模型的研究发现耳蜗带状突触病变可能是隐匿性听力损失发生的重要原因。尽管已经获得了很多证据,然而目前在HHL致病机制研究上仍存在诸多疑点,各种诱发因素在病变过程中所起的作用也尚不明晰。临床上,目前还缺乏系统的检测手段对隐匿性听力损失做出诊断。本期专辑收录文章在一定程度上反映了国内部分医疗机构在这个领域中的研究现状和思考,从中可以看出,在隐匿性听力损失和耳蜗带状突触病变的研究上还有很多问题亟待解决。     

听神经病和梅尼埃病患者耳蜗电图振幅比与面积比差异分析

目的分析听神经病(Auditory Neuropathy/Auditory Neuropathy Spectrum Disorder, AN/ANSD)与梅尼埃病(Meniere's disease,MD)患者交替短声诱发的耳蜗电图各项参数差异与特征。方法对正常听力对照组10人(20耳)及听神经病19人(38耳)、梅尼埃病16人(单侧14例,双侧2例)行耳蜗电图检查,刺激声为交替短声100dBnH。对照组较AN和MD多做2组强度分别为80、90dBnHL。统计分析应用SPSS(版本25.0),MD组症状耳与非症状耳采取配对t检验;正常对照组、MD组、AN三组对比采取方差分析。数据非正态分布或方差不齐时行非参数检验。结果 MD症状耳与非症状耳、正常对照组与AN、MD的平均听阈均存在统计学差异;正常对照组不同强度100dBnHL与80dBnHL在SP和AP振幅与潜伏期之间有显著性差异;正常对照组、梅尼埃症状耳组、AN三组SP、AP同时引出率分别为100%、100%、87%。MD组14耳症状耳与非症状耳仅SP振幅与SP/AP振幅比有显著性差异;正常对照组、AN组、MD三组比较,除外SP振幅,其余参数均有显著统计学差异(P <0.05)。以正常对照组SP/AP振幅比95%置信区间上限0.31,SP/AP面积比上限1.47,MD症状耳阳性率振幅比、面积比均为83.33%,非症状耳振幅比及面积比阳性率分别为35.71%及57.14%;AN振幅比及面积比阳性率均为100%。结论 AN与MD患者振幅比和面积比的比值均增高,其差异源于AN患者AP振幅异常降低或消失;MD患者在高强度刺激时AP振幅随听力损失降低但SP振幅不变或增高。ECochG辅助诊断单侧MD自身对照更有意义;SP/AP振幅比、面积比是辅助诊断梅尼埃病和听神经病的有效手段。     

内毛细胞传入神经突触病变和遗传性听功能障碍

近年来的研究表明,耳蜗内毛细胞传入神经突触（cochlear ribbon synapse）在听觉系统中发挥着十分关键的作用。本文介绍了OTOF,SLC17A8,Diaphanous homolog 3（DIAPH3）和SMAD4等目前研究比较深入的几个基因缺陷所导致的遗传性听功能障碍得分子病理机制。上述基因缺陷导致耳聋的一个共同机制是基因缺陷首先破坏了耳蜗内毛细胞传入神经突触的结构和功能。在表型上,这些基因缺陷动物共同表型出了听神经病的临床特征：脑干诱发电位（auditory brainstem respons,ABR）不能引出,畸变产物耳声发射（distortion product acoustic emission,DPOAE）和耳蜗电位（cochlear microphonics,CM）正常或基本正常。形态学研究证明了功能检测的结构及动物耳蜗毛细胞和突触后听神经的形态结构都是正常或基本正常的。对这些基因缺陷致聋机制的研究具有重要的临床价值,特别是对今后开展针对上述基因缺陷耳聋儿童的人工耳蜗植入治疗具有指导意义。     

听神经病/听觉失同步化患者的交替短声诱发耳蜗电图

目的 分析听神经病/听觉失同步化(auditory neuropathy/auditory desynchronization.AN/AD)患者交替短声诱发的耳蜗电图特征,探索耳蜗电图在确定听神经病,听觉失同步化病变部位时的应用价值.方法 AN/AD组患者14人,共28耳,所有患者听性脑干反应(ABR)波形缺失或严重分化异常,耳声发射正常:听力正常对照组28人,共35耳.对两组受试者行耳蜗电图(ECochG)检查,使用交替极性短声作为刺激信号.观测AN/AD组和对照组ECochG的波形,并对比:(1)CAP-N1(复合动作电位N1波)的峰潜伏期;(2)-SP(总和电位)和CAP绝对幅度;(3)-SP和CAP幅度比值;(4)CAP反应周值.结果 对照组全部引出分化良好的CAP和-SP.AN/AD组ECochG波形可分为四种类型:(1)可同时引出-SP、CAP,占60.7%;(2)仅有-SP引出,未见CAP引出,占10.7%;(3)仅有CAP引出,占3.6%;(4)-SP、CAP均未引出,占25%.AN/AD组与对照组的CAP潜伏期(P=0.052)无统计学差异;ANIAD组的CAP绝对幅度低于对照组(P<0.001),-SP(P=0.045)绝对幅度、-SP/CAP幅度比(P<0.001)和阈值(P<0.001)高于对照组.结论 在ABR引不出或分化较差时,耳蜗电图是一种比较可靠的评估外周听觉神经功能的方法,在AN/AD的诊断中能够发挥重要作用.     

人工耳蜗植入术中圆窗耳蜗电图评价耳蜗残余听功能

目的应用术中圆窗耳蜗电图评估极重度感音神经性聋患者耳蜗残余听功能。方法20例患者全麻下人工耳蜗植入手术过程中,行术中圆窗耳蜗电图测试,测出的复合动作电位（CAP）阈值与术前纯音测听或其他听力测试之阈值进行比较。结果20例患者术中圆窗耳蜗电图测试测出的复合动作电位（CAP）阈值与术前1、2、4kHz听力测试阈值分别有较好的相关系数（0．20429,0．04076,0．38163）。结论术中圆窗耳蜗电图可以较准确客观地评估极重度感音神经性聋患者耳蜗残余听功能,且是人工耳蜗植入术前听力学评估方法的有意义的补充。     

耳蜗电图配合甘油试验对梅尼埃病的诊断意义

对５５例眩晕患者以耳蜗电图配合甘油试验观察耳蜗电图的变化，结果３３例梅尼埃病（ＭＤ）患者甘油试验前耳蜗电图优势ＳＰ／ＡＰ占７３％，甘油试验阳性率为５２％，服甘油后优势ＳＰ／ＡＰ比值降至正常或减少１５％以上，从而进一步证实优势ＳＰ是内淋巴积水的表现；以耳蜗电图配合甘油试验较纯音测听的甘油试验诊断ＭＤ更为客观。     

耳蜗电图和ABR在听神经病诊断中的应用研究

目的:分析比较耳蜗电图和ABR测试在听神经病(AN)诊断中的作用。方法:AN组为AN患者16例(32耳),对照组为感音神经性听力损失患者20例(26耳)。对2组受试者行交替极性短声刺激耳蜗电图和ABR测试。分别观测AN组和对照组的波形,并进行以下对比:①比较AN组患者的ABR和耳蜗电图波形引出率;②比较AN组与对照组耳蜗电图的复合动作电位(CAP)潜伏期和绝对幅度,以及总和电位(SP)与CAP的幅度比值。结果:AN组32耳中有25耳引出-SP或CAP,耳蜗电图总体引出率为78.1%。AN组的CAP潜伏期短于对照组(P<0.05),CAP绝对幅度低于对照组(P<0.05),-SP/CAP幅度比值高于对照组(P<0.01)。AN组仅5耳引出不完整的ABR波形,引出率为15.6%,且波形分化较差。ABR引出率明显低于耳蜗电图(P<0.01)。对照组全部引出分化良好的耳蜗电图和ABR波形。结论:耳蜗电图和ABR在AN的诊断中各自发挥重要作用。AN患者听觉信息的时间整合作用受到损害而强度感受机制受影响较小。     

均衡噪声阈值检测法研究耳蜗死区在感音神经性聋患者耳蜗中的分布

目的探讨在感音神经性聋患者中是否存在耳蜗死区及其分布情况。方法采用均衡噪声阈值检测法检测82例(130耳)感音神经性聋患者和25例(50耳)正常听力者是否存在耳蜗死区,并比较耳蜗死区与性别、年龄、耳别、听力损失程度以及病程的关系。结果 82例感音神经性聋患者中有41.46%(34/82)存在耳蜗死区,正常听力者中无耳蜗死区,两者差异具有统计学意义(P<0.01)。耳蜗死区的存在在性别、年龄和耳别之间无明显差异,而不同听力损失程度、病程的感音神经性聋患者耳蜗死区的检出率差异有统计学意义(P<0.05),听力损失越重,病程越长,检出率越高。33.85%(44/130)受试耳存在耳蜗死区,其中高频区耳蜗存在死区者占23.08%(30/130),低频区耳蜗存在死区者占2.31%(3/130),高低频区同时存在耳蜗死区者占8.46%(11/130),高频耳蜗死区检出率远高于低频区。结论在感音神经性聋患者存在耳蜗死区中,耳蜗死区的存在与听力损失程度及病程有关,听力损失程度越重、听力受损时间越长,存在耳蜗死区的可能性越大。     

Electrocochleography in auditory neuropathy

Auditory neuropathy (AN) is a disorder characterized by the absence or the severe impairment of the auditory brainstem responses (ABRs) together with the preservation of otoacoustic emissions and/or cochlear microphonic (CM). We recorded transtympanic electrocochleography (ECohG) evoked by 0.1 ms clicks in one young adult and in four children having distortion product otoacoustic emissions and absent ABRs. In all but one patient CM and summating potential (SP) were present with normal threshold, and their amplitudes appeared comparable to or higher than the values obtained from subjects with normal hearing. The compound action potential (CAP) was absent in two patients while in one subject CM and SP were followed by a highly desynchronized neural activity. A broad CAP was found in two children and the threshold appeared clearly elevated in one of them, while it showed only a mild elevation in the other. No correlation was found between CAP and behavioral thresholds. These results suggest that ECohG can be useful in AN diagnoses since it is the only reliable tool in evaluating the auditory peripheral function in the presence of a desynchronized ABR.     

耳蜗死区对感音神经性听力损失患者言语识别能力的影响

目的 探究耳蜗死区在感音神经性听力损失(sensorineural hearing loss,SNHL)患者中的存在情况及其对言语识别能力的影响.方法 采用纯音听阈测试筛选出41例(81耳)感音神经性听力损失患者,经均衡噪声阈值测试将患者分为有耳蜗死区组(35耳)和无耳蜗死区组(46耳),分别进行言语识别阈(SRT)和言语识别率(SDS)测试,分析81耳耳蜗死区的分布及其对言语识别能力的影响.结果 41例(81耳)感音神经性听力损失患耳中有35耳(43.21％,35/81)存在耳蜗死区,其中轻度SNHL患耳耳蜗死区检出率为0(0/11),中度SNHL患耳耳蜗死区检出率为24.1％(7/29),重度SNHL患耳耳蜗死区检出率为66.7％(24/36),极重度SNHL患耳耳蜗死区检出率为80.0％(4/5),不同听力损失程度耳耳蜗死区检出率差异有统计学意义(P＜0.05);高频耳蜗死区(16耳)明显多于低频耳蜗死区(8耳),但两者的言语识别能力差异无统计学意义(P＞0.05);有耳蜗死区患者的言语识别阈及言语识别率分别为61.63±16.76 dB HL,86.35％±12.03％,无耳蜗死区的患者分别为75.54±9.56 dBHL,64.97％±20.84％,二者间差异有统计学意义(P＜0.05).结论 听力损失越重,耳蜗死区检出率越高;高频耳蜗死区较低频常见,且存在耳蜗死区的感音神经性听力损失患者言语识别能力明显低于无耳蜗死区的患者.     

听神经病病损部位分析

目的：探讨一组以言语听力下降为主的听力损失的命名、临床特点、病损部位。方法：分析１１例低频或以低频为主的感音神经性听力损失患者的临床症状、纯音测听、声导抗测试、听性脑干反应、耳蜗电图、畸变产物耳声发射～ＣＴ和（或）ＭＲＩ资料。结果：本组病例具有下列矛盾现象;主诉进行性听力闰退,尤其听不清语言,纯音听阈呈上升型听力图,听性脑干反应引不同或明显异常,甚经４０００Ｈｚ纯音听琢怕主观阈严重镫骨肌反引不出;     

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。     

听神经病患者人工耳蜗植入后听觉言语康复效果分析

目的评价重度或极重度听力损失听神经病(Auditory neuropathy,AN)患者人工耳蜗植入术后的听觉言语康复效果。方法用纯音听阈、听觉行为分级标准(Categorise of Auditory Performance,CAP)、言语可懂度分级标准(speech Intelligibility Rating,SIR)、有意义听觉整合量表(meaningful auditor integration scale,MAIS),对行人工耳蜗植入术的18例重度或极重度听力损失语前聋AN患者、4例重度或极重度听力损失语后聋AN患者进行术后效果调查,并与非综合症且无明显病因的重度或极重度感音神经性聋的人工耳蜗植入患者比较,用SPSS13.0进行统计学分析。结果 11例AN患者术后平均助听听阈(250Hz、500 Hz、1000 Hz、2000 Hz、4000 Hz)为(38.64±6.36)d B HL。AN组语前聋患者术后发声情况得分、对声音的自发性觉察能力得分与对照组语前聋患者术后得分相比,差异均无统计学意义(P>0.05),而对声音的理解能力得分、MAIS总得分与对照组语前聋患者术后得分相比,差异有统计学意义(P<0.05),AN组语前聋患者对声音的理解能力得分、MAIS总得分均低于对照组语前聋患者得分。AN组语后聋患者术后CAP评分、SIR评分与对照组语后聋患者术后评分相比,差异无统计学意义(P>0.05)。结论重度或极重度听力损失语前聋AN患者人工耳蜗植入术后的发声情况、对声音的自发性觉察能力与耳蜗形态正常且无任何已知综合症的重度或极重度感音神经性聋语前聋患者术后无明显差异,但是重度或极重度听力损失语前聋AN患者人工耳蜗植入术后对声音的理解能力明显低于耳蜗形态正常且无任何已知综合症的重度或极重度感音神经性聋语前聋患者术后对声音的理解能力。重度或极重度听力损失语后聋AN患者人工耳蜗植入术后听觉言语能力与耳蜗形态正常且无任何已知综合症的重度或极重度感音神经性聋语后聋患者术后无明显差异。     

Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss

Auditory neuropathy is a challenging disorder and needs special habilitative/rehabilitative approach. This study aimed to detect its prevalence among infants and young children with severe to profound hearing loss. 112 infants and young children with age ranged 6-32 months were examined and diagnosed as having severe to profound hearing loss and were referred for hearing aid fitting. Those infants were reassessed in our centers for detecting cases with auditory neuropathy. The study group was subjected to immittancemetry, behavioral observation audiometry, ABR and cochlear microphonics.

Results

15 patients were found to have auditory neuropathy according to our criteria for diagnosis.

Conclusions

The prevalence of AN in the study group was 13.4%. CM were recommended to be tested routinely during ABR assessment whenever abnormal results are obtained.     

Cochlear microphonics in sensorineural hearing loss: lesson from newborn hearing screening

The diagnostic dilemma surrounding the presence of cochlear microphonics (CM) coupled with significantly elevated auditory brainstem response (ABR) thresholds in babies failing the newborn hearing screening is highlighted. A case report is presented where initial electo-diagnostic assessment could not help in differentiating between Auditory Neuropathy/Auditory Dys-synchrony (AN/AD) and sensorineural hearing loss (SNHL). In line with the protocol and guidelines provided by the national Newborn Hearing Screening Programme in the UK (NHSP) AN/AD was suspected in a baby due to the presence of CM at 85 dBnHL along with click evoked ABR thresholds of 95 dBnHL in one ear and 100 dBnHL in the other ear. Significantly elevated thresholds for 0.5 and 1kHz tone pip ABR fulfilled the audiological diagnostic criteria for AN/AD. However, the possibility of a SNHL could not be ruled out as the 85 dBnHL stimuli presented through inserts for the CM would have been significantly enhanced in the ear canals of the young baby to exceed the threshold level of the ABR that was carried out using headphones. SNHL was eventually diagnosed through clinical and family history, physical examination and imaging that showed enlarged vestibular aqueducts. Presence of CM in the presence of very high click ABR thresholds only suggests a pattern of test results and in such cases measuring thresholds for 0.5 and 1 kHz tone pip ABR may not be adequate to differentiate between SNHL and other conditions associated with AN/AD. There is a need for reviewing the existing AN/AD protocol from NHSP in the UK and new research to establish parameters for CM to assist in the differential diagnosis. A holistic audiological and medical approach is essential to manage babies who fail the newborn hearing screening.