急性低频感音神经性聋的临床研究

目的探讨急性低频感音神经性聋（acute low-frequency sensorineural hearing loss,ALHL）的临床特征和疗效,提高对该疾病的认识和鉴别能力。方法回顾性分析42例（42耳）ALHL的临床表现、听力学检测结果和2～5年的随访情况,总结ALHL的临床发病特征。结果本组42例（42耳）ALHL呈急性发病,年龄27～44岁。男16例（38.10%）,女26例（61.90%）。20例（20耳）伴低调耳鸣,17例（17耳）伴耳部堵闷,11例（11耳）伴听觉过敏现象,6例（6耳）伴有头昏。0.125～1kHz平均听阈为（38.74±4.62）dBHL,2～8kHz平均听阈为（13.52±3.86）dBHL,治疗后上述平均听阈分别为（21.05±9.74）dBHL和（19.85±9.44）dBHL。所有病人的听性脑干反应（auditory brainstem response,ABR）各波潜伏期及波间期正常。畸变产物耳声发射（distortion product otoacoustic emissions,DPOAE）在低频区引出率为16.67%,高频区引出率为85.71%。结论ALHL以单侧发病为主,常伴低调耳鸣、耳部堵闷及胀满感。该病多见于青年女性。纯音听阈以低频听阈升高为特征,ABR正常。病变可能位于耳蜗顶周。     

感音神经性聋患儿的听功能综合评估

目的探讨听力测试组合（ABR＋ASSR＋声场环境中的行为测听）在感音神经性聋患儿残余听力评估中的应用价值。方法48名（96耳）感音神经性聋患儿中能配合纯音测听的患儿19人（38耳）设为PTA组,进行纯音测听及ASSR检测;不能配合纯音测听的患儿29人（58耳）设为BA组,进行声场环境中的行为测听（behavior audiometry,BA）、ABR及ASSR检测。结果①PTA组0．5、1、2、4kHz各频率ASSR反应阈与纯音听阈显著相关（P〈0．01）,各频率相关系数分别为0．75、0．76、0．76、0．83,建立本实验室的ASSR—PTA直线回归方程;②BA组23耳ABR无反应但仍可引出ASSR,而ASSR无反应耳ABR均未能引出;BA组29例患儿中ASSR检测反应较好耳（29耳）在0．5～4kHz四个频率上,ASSR可测得83个反应阈值,行为测听可测得89个反应阈值,综合ASSR和行为测听可以得到96个反应阈值。结论与单项听力测试方法相比,听力测试组合（行为测听＋ABR＋ASSR）能为更小年龄、听力损失更重的患儿进行残余听力的评估,同时能对双侧耳间听力差异、各频率的听力损失程度进行评估,为听力损失病变的定位判断提供参考。     

低频感音神经性听力损失的病因分析

目的分析低频感音神经性听力损失的病因,以避免漏诊和误诊。方法对56例低频感音神经性听力损失患者详细了解其病史,进行仔细的耳科常规检查及纯音听阈（PTT）、声导抗、听性脑干反应（ABR）、畸变产物耳声发射（DPOAE）、耳蜗电图（ECochG）及CT和/或MRI检查,综合分析各项结果。结果在56例患者中病因不明的急性低频感音神经性听力损失38例,梅尼埃病9例,听神经病6例,听神经瘤1例,多发性硬化1例,小脑半球旁蛛网膜囊肿1例。结论低频听阈升高的上坡型感音神经性听力损失可见于多种疾病。对低频感音神经性听力损失应采用多项组合的听力学检测方法进行检查和综合分析,必要时辅以CT和/或MRI检查可以及时、有效地作出可靠的诊断和鉴别诊断。     

不同程度感音神经性听力损失儿童ASSR、click-ABR反应阈与行为听阈的相关性

目的 探讨不同程度感音神经性听力损失儿童听性稳态反应(ASSR)、短声听性脑干反应(click-ABR)与行为阈值的相关性及在听障儿童听力评估中的价值.方法 回顾性分析2019年1月至2019年12月确诊的46例(92耳)感音神经性听力损失儿童的ASSR、click-ABR及行为听阈结果,其中,轻度听力损失4耳,中度听...     

多频稳态诱发电位(ASSR)的临床应用研究(36例极重度感音神经性耳聋幼儿及32例成人感音神经性耳聋患者ASSR测试结果分析)

目的旨在探讨ASSR与ABR在极重度感音神经性耳聋幼儿及成人感音神经性耳聋患者测试中的临床应用价值.方法对36例(72耳)小于3岁极重度感音神经性耳聋幼儿分别行ASSR和ABR测试;对32例(64耳)成人感音神经性耳聋患者分别行ASSR和电测听测试.结果①极重度感音神经性耳聋幼儿ABR均未引出V波,而ASSR在0.5 kHz、1 kHz、2 kHz、4 kHz的引出率分别为66.67%、86.11%、88.89%、94.44%,ASSR在0.5 kHz、1 kHz、2 kHz、4 kHz的阈值均数、标准差分别为82.56±9.26 dB HL、90.31±6.94 dB HL、88.12±7.93 dB HL、88.62±8.12 dB HL.②对成人感音神经性耳聋患者0.5 kHz、1 kHz、2 kHz、4 kHz ASSR测试阈值与电测听语频听阈(dB HL)进行配对两两比较的t检验,各组P值均大于0.05,无显著性差异.结论ASSR有助于极重度感音神经性耳聋幼儿残余听力的客观评估,尤以高频听阈为佳;ASSR与电测听在感音神经性耳聋诊断上有良好的一致性.     

听神经病

目的介绍一种特殊的感音神经性听力疾患-听神经病,探讨其临床特征及听力学特点.方法报道5例听神经病患者,2例为成人;3例为儿童.记录患者的临床资料,并对患者进行纯音测听、脑干电反应测听、耳声发射、耳蜗电图及语言辨别率等听力学检查.结果5例均主诉听力下降,听力学检查纯音听阈为轻、中度感音神经性聋,与纯音测听不相符的语言辨别率明显下降,不能引出脑干诱发电位(ABR),耳蜗电图基本正常,畸变产物耳声发射(DPOAE)基本正常.提示外毛细胞功能正常,病变可能在听神经.结论听神经病是一种主诉听力下降,纯音听阈为轻、中度感音神经性聋,不能引出脑干诱发电位(ABR),畸变产物耳声发射正常的听力疾患,临床上应与其他感音神经性聋区别.     

急性低频感音神经性听力损失的听力学检查特征及意义

目的明确各项听力学检查在急性低频感音神经性听力损失诊断及研究中的意义,以指导该病的诊断和治疗。方法对2008年7月至2010年5月在本科就诊的急性低频感音神经性听力损失患者的临床听力学检查进行了回顾性分析。结果在所有15例患者中有9人(60.0%)有明显诱发因素。在随访期间,有8例(53.3%)出现复发。男女两性无诱发因素和复发率差异。8例患者进行了耳蜗电图的检查,有5例(62.5%)-SP/AP(总合电位/动作电位)检查异常。结论纯音测听、耳蜗电图、DPOAE(畸变产物耳声发射)、ABR(听性脑干电位)在急性低频感音神经性听力损失诊断及研究中意义较大。其发病很可能与生活压力事件相关,病因可能是膜迷路积水、自身免疫性机制和自主神经功能紊乱等因素的综合作用。     

听神经病的临床及基础研究

关于“听神经病”的命名经历几个不同的认识阶段。我国学者顾瑞将其称之为“中枢性低频感音神经性听力减退”,依据是：①纯音听力正常或轻度减退而言语听力显著减退;②镫骨肌反射不能引出或反射衰减阳性;③多数耳蜗电图（electrocochleogram,ECochG）可引出SP,AP振幅降低或消失;④听性脑干反应（auditory brainstemre．sponse,ABR）引不出或不能重复,特别是不能引出Ⅰ～Ⅴ波,提示病变在听觉传导的中枢通路。提出“Ⅰ型传入神经元病”概念,其依据的是患者有双侧进行性听力损失,严重的言语听力障碍,纯音测听以低频为主的上升型听力图,言语测听和ABR改变比纯音听阈改变严重,耳声发射多正常且幅度增大,不被对侧抑制。     

单侧听神经病的临床听力学特征分析[论著]

目的　分析单侧听神经病在单侧感音神经性听力损失中的发病率及其听力学特征。方法　回顾性分析2008年6月～2020年6月广州开发区医院耳鼻咽喉科489例单侧感音神经性听力损失患者,统计单侧听神经病在单侧感音神经性听力损失中的发病率,比较单侧听神经病和单侧一般感音神经性听力损失的纯音听阈,分析单侧听神经病的声导抗、畸变产物耳声发射（DPOAE）、听性脑干反应（ABR）、视频眼震电图（video-nystagmography,VNG）、内听道MRI检查。结果　489例单侧感音神经性听力损失 患者中单侧听神经病16例（3.27%）,右侧9例,左侧7例;男6例,女10例;年龄9～32岁,平均21岁;平均病程为2.6年。单侧听神经病纯音听阈多为重度以上听力损失,16例单侧听神经病患耳重度或极重度听力损失12例,中重度平坦型听力损失3例,高频下降型听力损失1例。单侧听神经病患者与单侧一般感音神经性听力损失患者的纯音听阈在125、250、500、1000、2000、4000、8000 Hz频段比较,差异均有统计学意义（P＜0.05）。DPOAE双耳均能引出,但患耳对侧声抑制效应消失;ABR结果显示14例患耳100 dB nHL强度刺激下未引出任何波形,2例患耳90 dB nHL强度刺激下仅引出V波;VNG结果显示3例患耳单侧半规管功能减退,2例患耳出现对侧优势偏向;8例行内听道MRI检查中3例患耳听神经缺如。结论　单侧听神经病临床并不罕见,与一般单侧感音神经性听力损失纯音听阈相比具有明显差异。临床工作中对单侧感音神经性听力损失要完善全面听力学检查,特别是完善影像学MRI检查,对诊断及鉴别诊断均具有重要意义,尽量避免单侧听神经病漏诊。     

大前庭水管综合征患者的听力学及前庭诱发肌源性电位检测的特点

目的:探讨大前庭水管综合征(LVAS)患者的听力学、前庭诱发肌源性电位(VEMP)检测的特点及诊断意义。方法:对30例(60耳)LVAS患者行纯音测听、声导抗、ABR、畸变产物耳声发射(DPOAE)、VEMP及冷热实验检测,分析其诊断意义。结果:30例(60耳)LVAS患者均呈进行性、波动性听力下降,16例在听力波动性下降时伴眩晕。纯音测听主要呈感音神经性聋,但47耳(94.0%)在中耳功能正常的情况下于低频250、500Hz分别出现(43±17)dBHL及(33±18)dBHL骨气导差,呈混合性聋。ABR检测18耳(64.3%)于(3.06±0.52)ms引出声诱发短潜伏期负反应。42耳VEMP振幅均值为(147.10±107.55)μV,19耳反应阈为75dBnHL,7耳反应阈为65dBnHL。结论:根据进行性波动性听力减退、感音神经性聋在中耳功能正常的情况下出现低频骨气导差,ABR测试引出声诱发短潜伏期负反应及VEMP呈现高振幅低阈值的特征性提示,有助于临床医生得出LVAS的初步印象,可进一步行影像学检查以确诊。     

单侧人工耳蜗植入术前后双耳残余听力变化的初步研究

目的 通过观察单侧人工耳蜗植入术后儿童双耳残余听力情况,分析其变化趋势,探讨各种可能的影响因素。 方法 将29例单侧人工耳蜗植入的重度极重度感音神经性耳聋儿童纳入研究,根据术前颞骨CT分为A组(有大前庭导水管综合征)和B组(非大前庭导水管综合征),分别于术前、术后1周、开机时、术后半年定期进行裸耳纯音测听,比较植入耳残余听力保存情况及非植入耳残余听力的变化。 结果 患儿术前双耳均不同程度存在残余听力,术后均定期行纯音测听或行为测听,各频率仍部分存有残余听力,主要分布在低频区,且随频率升高其残余听力保留率逐渐下降,各频率残余听力保留率之间差异有统计学意义(Wald χ2=16.980, P=0.001);植入耳与非植入耳各频率残余听力保留率之间差异有统计学意义(Wald χ2=10.031, P=0.002);术前后各时间节点残余听力保留率之间差异无统计学意义(Wald χ2=3.384, P=0.336); AB两组之间残余听力保留率之间差异无统计学意义(Wald χ2=0.906, P=0.341)。 结论 人工耳蜗植入对植入耳残余听力的保留主要分布在低频区;随着植入时间的延长,残余听力可以基本保持;少部分患儿行单侧植入后对非植入耳残余听力有影响;前庭导水管扩大症患儿行单侧耳蜗植入术后,非植入耳残余听力在短期内可有波动。     

浅睡眠状态下的行为观察测听

目的 分别测试低龄儿双耳的听力状况.方法 对327名婴幼儿进行浅睡眠状态下的行为观察测听,并与click声刺激ABR结果进行比较;其中有58名婴幼儿一段时间后再进行游戏测听和纯音测听.结果 浅睡眠状态下的行为观察测听在2 kHz和4 kHz的平均反应阈与ABR的反应阈相当,但比跟踪测得的同频率纯音听阈值高约5～10 dB HL.结论 浅睡眠状态下的行为观察测听可分别测得低龄儿的双耳听反应阈.     

Auditory steady state response in pediatric audiology

The main issue regarding pediatric audiology diagnosis is determining procedures to configure reliable results which can be used to predict frequency-specific hearing thresholds.AimTo investigate the correlation between auditory steady-state response (ASSR) with other tests in children with sensorineural hearing loss.MethodsProspective cross-sectional contemporary cohort study. Twenty-three children (ages 1 to 7; mean, 3 years old) were submitted to ASSR, behavioral audiometry, click audiometry brain stem response (ABR), tone burst ABR, and predicting hearing level from the acoustic reflex.Resultsthe correlation between behavioral thresholds and ASSR was (0.70- 0.93), for the ABR tone burst it was (0.73 -0.93), for the ABR click it was (0.83-0.89) only at 2k and 4 kHz. The match between the ASSR and the hearing threshold prediction rule was considered moderate.Conclusionthere was a significant correlation between the ASSR and audiometry, as well as between ABR click (2k and 4 kHz) and for the ABR tone burst. The acoustic reflex can be used to add information to diagnosis in children.     

Evaluation of residual hearing in cochlear implants candidates using auditory steady-state response

Conclusion: The correlations between behavioral and auditory steady-state response (ASSR) thresholds were significant at 500, 1000, 2000, and 4000 Hz. ASSR presented high sensitivity and specificity in the detection of residual hearing in cochlear implant candidates when compared with warble-tone audiometry. Objectives: To assess residual hearing in cochlear implant candidates by comparing the electrophysiological thresholds obtained in dichotic single-frequency ASSR with behavioral thresholds at 500, 1000, 2000, and 4000 Hz. Methods: This was a comparative study between ASSR and warble-tone audiometry thresholds in 40 cochlear implant candidates (80 ears) before cochlear implantation with bilateral severe-to-profound sensorineural hearing loss. Results: Thresholds were obtained in 62.5% of all frequencies evaluated in warble-tone audiometry and in 63.1% in the ASSR. ASSR sensitivity was 96% and specificity was 91.6%. Mean differences between behavioral and ASSR thresholds did not reach significance at any frequencies. Strong correlations between behavioral and ASSR thresholds were observed in 500, 1000, and 2000 Hz and moderate in 4000 Hz, with correlation coefficients varying from 0.65 to 0.81. On 90% of occasions, ASSR thresholds were acquired within 10 dB of behavioral thresholds.     

Auditory brain stem responses of kernicterus infants

Auditory brainstem evoked responses (ABRs) and behavioral audiometry in 25 infants with kernicterus were studied to determine the level of the lesion causing their hearing disorders. ABR thresholds, peak latencies of wave I and V, and interwave latency of wave V-I were measured; behavioral audiometric thresholds were determined through conditioned orientation reflex audiometry (COR).Eighty-eight per cent of infants with kernicterus who showed ABR threshold elevation with respect to age-matched normals, were found to show ABR abnormalities associated with peripheral hearing loss, and 84% of these were found to have COR threshold elevation.The ABR abnormalities were threshold elevation of wave V, prolonged latency of wave I and V, and the absence of ABRs. However, no brainstem lesion pattern was found in our cases.Our results suggested that at least some lesions which produce hearing disorders in kernicterus occur in the cochlea or auditory nerve.     

Auditory steady-state responses to multiple simultaneous stimuli in children with functional or sensorineural hearing loss

The goal of our study was to identify the role of auditory steady-state responses for hearing assessment in patients with functional hearing loss. The study design was to compare auditory steady-state response thresholds and standard pure-tone audiometry thresholds between patients with functional or sensorineural hearing loss. Subjects comprised 16 patients (24 ears) with functional hearing loss and 17 patients (24 ears) with sensorineural hearing loss. Differences and correlations between auditory steady-state response thresholds and standard pure-tone audiometry thresholds at 500, 1,000, 2,000 and 4,000 Hz were evaluated. In children with functional hearing loss, pure-tone audiometry thresholds and auditory steady-state response thresholds were significantly different at all frequencies and were not significantly correlated. In patients with sensorineural hearing loss, pure-tone audiometry thresholds and auditory steady-state response thresholds did not differ significantly at any frequencies and were significantly correlated. Auditory steady-state responses may have principal role in the assessment of auditory brainstem acuity, particularly at low frequencies in patients with functional hearing loss.     

Auditory brainstem responses in young children with Down's syndrome

Auditory brainstem evoked responses (ABRs) were studied in 37 Japanese infants and children with Down's syndrome to determine the level of the lesion causing their unresponsiveness to sound stimuli. ABR thresholds, peak latencies of waves I and the peak interval latency of wave I-V were measured. For comparison with ABR thresholds, behavioral audiometry was performed. Thirty-two percent of the patients (12 cases) with Down's syndrome showed no responses by ABR as well as behavioral audiometry. The other ABR abnormalities with respect to age-matched controls were classified into 6 types: shorter wave I latencies (2 cases, 5%), shorter wave V latencies (7 cases, 19%), shorter wave I-V intervals (8 cases, 22%), wave I prolongation (13 cases, 35%), wave V prolongation (5 cases, 14%) and prolongation of wave I-V peak interval (no case, 0%). Our results demonstrate a high incidence of hearing loss at middle and inner ear levels, and suggest some anomaly within the auditory brainstem in Japanese cases of Down's syndrome.     

Comparison between Brainstem-Evoked Response Audiometry and Behavioral Audiometry in 270 Infants and Children

The authors refer their results obtained with brainstem-evoked response audiometry (BSERA) and behavioral audiometry in a group of 270 children (aged from 6 months to 12 years) divided into subjects with (group A) and without (group B) reliable audiograms. In group A an agreement between the two methods was found in 77% of cases and a serious discrepancy in 5%. In group B an agreement was found only in 34% of cases, while a serious discrepancy was found in 20% of the children. BSERA was useful because many diagnostic mistakes were avoided. The authors think that BSERA is a reliable technique, as their data show, but in at least 4 cases they found that BSERA gave a hearing level much higher than that obtained with behavioral audiometry or that observed in the children's responses to everyday sounds. Since click gives only partial information of the tonal field and because it is possible to make technical mistakes, the authors believe that BSERA must not be used as the only diagnostic test but that it should be part of a test battery which includes, at least, behavioral audiometry and impedance audiometry.     

感音神经性聋儿多频稳态诱发电位及短纯音听性脑干反应与行为测听的相关性

目的 比较多频稳态诱发电位（MASSR）、短纯音听性脑干反应（Tb—ABR）与感音神经性聋儿行为测试听阈的差值．研究MASSR和Tb—ABR反应阈与行为听阈之间是否存在相关性以及在不同听力损失聋儿、不同的频率之间的差异。方法 对60名感音神经性聋儿分别测试MASSR和Tb—ABR反应阈和行为听阈，评价MASSR反应阈、Tb—ABR反应阈与行为听阈的相关性。结果 MASSR反应阈、Tb—ABR反应阈和行为听阈之间均有较高的相关性。二者在频率为2、4kHz时，对行为听阈的预测具有相似的、较高的准确性；但在频率为0．5、1kHz时，MASSR的准确性较Tb—ABR高。结论 MASSR和Tb—ABR均可用作感音神经性聋儿言语频率客观听阈的预测，为低龄儿童及难以检测行为听力的患儿提供诊断依据。