单侧听神经病11例听力学特点分析

目的：研究单侧听神经病（UAN）的听力学特点，以提高对UAN的认识。方法：回顾性分析11例UAN患者的听力学资料，并与双侧AN及正常组进行对照研究。结果：AN患者340例。其中UAN11例，占AN患者3．2％。左耳5例，右耳6例；男4例。女7例，男女之比为1．00：1．75；年龄5～35岁。平均20岁；病程平均3．1年。患者均诉单侧听力减退，听不清说话6例。伴耳鸣4耳，无眩晕发作。纯音听力图下降型5耳（45．4％），低频上升型4耳（36．4％），平坦型1耳（9．1％），盆型1耳（9．1％）；听力损失极重度聋6耳（54．5％），中重度聋3耳（27．3％），中度聋2耳（18．2％）。纯音听阈各频率均值UAN组明显高于双侧AN组，健侧听力图正常。言语识别率患耳差，健耳正常。声导抗患侧声反射消失，健侧引出，1例双耳同侧及交叉部分频率引出。听性脑干反应（ABR）患耳自波Ⅰ起均未引出（〉100dBnHL），健耳正常引出。畸变产物耳声发射（DPOAE）引出双侧均正常或基本正常，同正常对照组相比，DP—gram幅值和SNR值在全频（0．5～8．0kHz）降低（P〈0．05）.以中、高频显著（P〈0．01），而低频较双侧AN组明显降低（P〈O．01）。4例UAN健耳的对侧声抑制DP-gram幅值下降．患耳的对侧声抑制效应减弱或消失，甚至在5kHz幅值增加较健耳及正常组显著（P〈0．05）。7例行眼震电图（ENG）检查，患耳半规管功能减退及麻痹各1例；5例行前庭诱发性肌源性电位（VEMP）检查，双侧未引出1例，引出4例，其中患耳幅值降低2例。结论：UAN的听力学特点与双侧AN基本相同，但UAN以下降型听力图和极重度聋较多见，DPOAE幅值与双侧AN比较全频降低。健耳对侧声抑制明显，患耳对侧声抑制效应减弱或消失。需注意与一般单侧感音神经性聋相鉴别。DPOAE及ABR对诊断、鉴别诊断具重要意义。     

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。     

耳鸣患者的畸变产物耳声发射测试

目的探讨耳鸣患者畸变产物耳声发射（DPOAE）的临床特征，了解畸变耳声发射检测在耳鸣患者中的临床应用价值。方法对42例耳鸣患者进行DPOAE测试及纯音听阈检测，并进行对比。结果耳鸣伴听力下降患者的畸变产物耳声发射在相关频率幅值下降或未引出，二者有相关性；耳鸣伴听力正常患者畸变产物耳声发射均出现高频段幅值下降或缺失。结论对纯音听阈正常的耳鸣患者DPOAE可用于发现早期耳蜗病变，对听力损失者可有助于客观预估听力损失程度。     

儿童单侧听神经病附三例分析

目的 探讨儿童单侧听神经病的听力学特征。方法 对3例单侧听神经病患儿进行纯音听阈(PTT)、声反射(AR)、听性脑干反应(ABR)和畸变产物耳声发射(DPOAE)检查，并对其结果进行综合分析。结果 3例患儿的PTT均呈1侧耳听力正常，另1侧耳听力丧失。健耳的同侧和交叉AR能引出，患耳的同侧和交叉AR未能引出。健耳的ABR各波潜伏期正常，患耳的ABR各波未能引出。双耳DPOAE各频率反应幅值正常。结论：DPOAE正常或基本正常，PTT、AR和ABR异常是听神经病的重要特征。单侧听神经病的听力学特征与双侧听神经病基本一致。对儿童表现为单侧感音神经性聋者宜进行系统的听力学检查，以期作出正确的诊断。     

听神经病的临床与听功能特征

目的:探讨听神经病的临床与听功能特征。方法:总结分析54例听神经病患者的临床资料、听力学测试及电生理检查情况。结果:纯音听力图呈上升型70耳,覆盆型25耳,平坦型5耳,下降型4耳;低频、中频及高频平均阈值为(67.63±15.30,43.61±16.28,32.25±14.80)dB HL。声导抗鼓室图全部正常,77耳镫骨肌声反射消失,31耳声反射阈部分增高。听性脑干反应(ABR)全部未引出。畸变产物耳声发射(DPOAE)正常引出,26例行对侧声抑制未受影响。16例言语识别率差,与纯音听阈不成比例。23例颞骨CT或MRI未见异常。10例伴有周围神经病。结论:ABR自波Ⅰ起缺失而DPOAE正常引出,言语分辨力差与纯音听阈不成比例,镫骨肌声反射及OAE交叉抑制异常,纯音听力图多呈上升型以低频损失为主,是听神经病听功能的重要特征。提示病损主要位于耳蜗内听神经纤维。应与一般的感音神经性聋和中枢性聋相鉴别。     

听神经病患者纯音听力和耳声发射表现特征分析

目的 探讨听神经病的病损部位及发病机制。方法 常规检测听神经病患者（10例，20耳）和正常听力者（10例，20耳）的纯音听力及DPOAE，并对结果进行比较和统计学分析。结果 听神经病组20耳中18耳纯音测听呈低频下降型听力损失，另2耳呈鞍型听力曲线，低频（250．500、1000Hz）听力损失一般大于40dB。畸变产物耳声发射均可引出，各频率耳声发射幅值与正常对照组差异无统计学意义，纯音听力与相应频率耳声发射出现矛盾。结论 听神经病的病损部位可能位于耳蜗传人通路，神经冲动非同步化或同步化受损是听神经病的发病机制之一。     

听性脑干反应与畸变产物耳声发射检测在听神经病诊断中的表现和意义

目的分析听神经病患者的听性脑干反应与畸变产物耳声发射特征,探讨该类听力学检测方法在听神经病诊断中的意义。方法回顾性研究本科确诊为听神经病的患者37例,比较分析其听性脑干反应与畸变产物耳声发射检测结果,探索其诊断意义。结果本组患者中,3例5耳（双耳病变2例,单耳病变1例）可引出V波,阈值70～90dB SPL,其余患耳ABR各波均未引出（刺激声强〉100dB SPL）。无论纯音听阈损失程度轻重如何,所有患者的DPOAE均全部引出。在各个频率点上,DPOAE的DP—gram幅值左、右耳间的差异均无统计学意义（P〉0．10）。结论听神经病的主要病变部位可能位于听神经传入通路,或伴有脑干内侧橄榄耳蜗系统的传出神经通路病变。     

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.     

椎基底动脉短暂缺血性眩晕患者畸变产物耳声发射的频谱时间序列分析

为探讨畸变产物耳声发射（DPOAE）对椎基底动脉短暂缺血性眩晕（VBTIV）患者进行定位诊断的可能性，利用ILO－92耳声发射仪对21例VBTIV患者进行了纳音听阈、声反射阈及DPOAE测试。结果示，21例VBTIV患者纯音听阈、声反射阈均无明显改变；而DPOAE的平均幅值下降，变异系数增大，DPOAE的平均幅值与变异系数结合发现VBTIV患者中36．8％属于异常。提示部分VBTIV患者存在用常规听力学测试方法难以发现的耳蜗功能损害，DPOAE的频谱时间序列分析有可能为证实并鉴别VBTIV是属于中枢性还是周围性提供依据。     

原发性高血压患者听觉功能早期损害的临床研究

目的：探讨高血压对听觉功能早期损害的听力学特征,为临床研究和防治耳聋提供参考。方法：将68例（136耳）原发性高血压患者分为无眼底动脉硬化的高血压A组35例（70耳）,及伴有眼底动脉硬化的高血压B组33例（66耳）;另选30例（60耳）年龄、性别匹配且无高血压,听力正常者为对照组,分别进行纯音听阈、畸变产物耳声发射（DPOAE）等听力学测试。结果 高血压B组2000～8000Hz纯音听阈提高（P〈0．05）,高血压A组的纯音听阈与对照组比较差异均无统计学意义（P〉0．05）;高血压A、B组的DPOAE反应幅值下降（P〈0．01）,仅高血压B组4000Hz的DPOAE检出率下降（P〈0．05）。结论：高血压会影响患者的听觉系统,即使患者主观上无明显的听力下降,但听觉功能可能已出现早期改变。     

Effects of Early Auditory Deprivation and Stimulation on Auditory Brainstem Responses in the Rat

The purpose of this study was to investigate the effect of auditory sound deprivation or stimulation on auditory brainstem responses (ABRs) during the maturation period of the rat auditory system. At postnatal day (PND) 21, 40 newborn Norway Brown male rats were categorized into 3 groups: (i) an auditory deprivation group in which a bilateral average conductive hearing loss of 27 dB was induced; (ii) an auditory activation group exposed to 65-90 dB sound pressure level; and (iii) a control group. ABR recordings were made on PND 84. In order to compare group differences in interpeak latency (IPL), sensation level (SL), defined as stimulus intensity above threshold, was used. IPL measurements and analysis were restricted to the 20-60 dB SL range. No differences were observed in the IPLs of peaks I-IV between the three groups. Small, but not statistically significant, differences in mean estimated IPLs of peaks I-IV were shown in the ranges >50 dB SL and <25 dB SL. Possible confounding factors explaining the apparent discrepancy between these results and those of other animal studies are reviewed.     

Auditory training program for Arabic-speaking children with auditory figure-ground deficits

ObjectiveListening to speech in noise makes up a great challenge for school children with auditory processing disorders mainly those with deficit in auditory figure ground (AFG) ability. These children are candidates for auditory training programs targeting AFG such as noise-desensitization programs. This work aimed to develop a new training material in Arabic language targeting this ability.MethodsA noise-desensitization semi-formal training program was developed and standardized on normal children in a pilot study preceding the main one. Seventeen school children with AFG deficit were submitted to the program for eight weeks then reevaluated.ResultsThe paired sample t-test revealed significant improvement of all trained children after training period in their psychophysical and electrophysiological results. The electrophysiological threshold of signal to noise ratio decreased from −5.3 dB to −11.3 dB after training.ConclusionThe newly developed training material revealed efficacy in managing children with AFG deficit. The other affected auditory abilities improved also because of the multi-ability tapping character of the program.     

听觉事件相关电位同侧与对侧记录的比较

目的 比较同侧与对侧听觉事件相关电位（auditory event-related potential,AWRP）的反应特性，探讨AERP在二侧听皮层发源上的神经电生理特点和机制及其临床意义。方法 选择正常青年人，采用同侧与对侧导联同时记录的方法记录AERP，分析二种记录条件下AERP各波潜伏期，幅值和波形的特性。结果 同侧与对侧记录的AERP在潜伏期和幅值上无显著性差异，但同侧记录的AERP波形明显优于对侧记录的曲线，表现为波形曲线光滑，波峰明显易辨，杂波成份少。结论 AERP在二侧听皮层的神经发源基本上是对称的，但同侧的反应怀明显优于对侧，可能与AERP的发源有部分皮层下成份的参与，听觉神经通路的双侧传导以及内侧橄榄耳蜗系统的对侧抑制效应有关，临床应用时应考虑到AERP发源部位和成份多元化的影响因素。     

与中枢听觉处理障碍相关的下丘功能及机制探讨

下丘（inferior colliculus,IC）是听觉传导通路上的重要中继站,在声源定位、时域、频率和声音强度分析中均起着重要的作用,而这些功能障碍正是中枢听觉处理障碍（central auditory processing disorder,CAPD）的发病基础。本文将重点介绍下丘听觉处理功能及机制,特别是与CAPD发病机制相关的功能改变,探讨CPAD的发病机制。     

Auditory Pathway Function after Vestibular Schwannoma Surgery

We studied seven patients before and after vestibular schwannoma surgery. Four patients became unilaterally profoundly deaf and three patients preserved their hearing. Cortical responses were recorded with a 122-channel whole-scalp SQUID neuromagnetometer using tone-burst stimuli to the healthy ear. Brainstem auditory evoked potentials (BAEPs) were measured using alternating clicks. Ten healthy volunteers served as a control group. In patients, preoperative cortical response latencies and strengths did not differ significantly from those of controls. However, 6 months after the operation the latency was, on average, 7 ms longer than preoperatively over both hemispheres. BAEPs were in the normal range both before and after the operation. These results suggest that unilateral lesion in peripheral auditory pathways also affects cortical reactivity to stimuli presented to the non-affected ear, possibly reflecting altered binaural interaction in the auditory pathways.     

Effectiveness of computer-based auditory training for adult users of cochlear implants

Abstract

Cochlear implantation is effective at restoring partial hearing to profoundly deaf adults, but not all patients receive equal benefit. The present study evaluated the effectiveness of a computer-based self-administered training package that was designed to improve speech perception among adults who had used cochlear implants for more than three years. Eleven adults were asked to complete an hour of auditory training each day, five days a week, for a period of three weeks. Two training tasks were included, one based around discriminating isolated words, and the other around discriminating words in sentences. Compliance with the protocol was good, with eight out of eleven participants completing approximately 15 hours of training, as instructed. A significant improvement of eight percentage points was found on a test of consonant discrimination, but there were no significant improvements on sentence tests or on a test of vowel discrimination. Self-reported benefits were variable and generally small. Further research is needed to establish whether auditory training is particularly effective for identifiable sub-groups of cochlear-implant users.

Sumario

La implantación coclear es efectiva para restaurar desde una alteración parcial de la audición hasta una sordera profunda en adultos, pero no todos los pacientes reciben el mismo beneficio. El presente estudio evalúa la efectividad de un paquete de entrenamiento computarizado y auto-administrado que se diseñó para mejorar la percepción del lenguaje en adultos que han utilizado implantes cocleares por más de tres años. Se le pidió a once adultos que completaran una hora de entrenamiento auditivo cada día, cinco dóas por semana, por un peróodo de tres semanas. Se incluyeron dos tareas de entrenamiento, una basada en la discriminación de palabras aisladas y la otra en discriminación de palabras en medio de frases. El cumplimiento del protocolo fue bueno, con ocho de quince participantes que completaron aproximadamente 15 horas de entrenamiento, como se instruyó. Se encontró una mejoría significativa de ocho puntos porcentuales en una prueba de discriminación de consonantes, pero no existió una mejoría significativa en las pruebas de frases o en la prueba de discriminación de vocales. Los beneficios auto-reportados fueron variables y generalmente pequeños. Se requiere investigación adicional para establecer si el entrenamiento auditivo es particularmente efectivo en subgrupos identificables de usuarios de implantes cocleares.     

Evaluation of inner hair cell and nerve fiber loss as sufficient pathologies underlying auditory neuropathy

Auditory neuropathy is a hearing disorder characterized by normal function of outer hair cells, evidenced by intact cochlear microphonic (CM) potentials and otoacoustic emissions (OAEs), with absent or severely dys-synchronized auditory brainstem responses (ABRs). To determine if selective lesions of inner hair cells (IHCs) and auditory nerve fibers (ANFs) can account for these primary clinical features of auditory neuropathy, we measured physiological responses from chinchillas with large lesions of ANFs (about 85%) and IHCs (45% loss in the apical half of the cochlea; 73% in the basal half). Distortion product OAEs and CM potentials were significantly enhanced, whereas summating potentials and compound action potentials (CAPs) were significantly reduced. CAP threshold was elevated by 7.5 dB, but response synchrony was well preserved down to threshold levels of stimulation. Similarly, ABR threshold was elevated by 5.6 dB, but all waves were present and well synchronized down to threshold levels in all animals. Thus, large lesions of IHCs and ANFs reduced response amplitudes but did not abolish or severely dys-synchronize CAPs or ABRs. Pathologies other than or in addition to ANF and IHC loss are likely to account for the evoked potential dys-synchrony that is a clinical hallmark of auditory neuropathy in humans.     

Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder

Objective

To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation.

Methods

Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12–84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS).

Results

All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz) of aided hearing thresholds ranged from 17.5 to 57.5 dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition.

Conclusion

Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months.     

中枢听处理障碍的评估与诊断现状

本文介绍了听处理障碍的定义与临床特征,列出了听处理障碍目前常用的评估和诊断方法,并对听处理障碍研究的后继发展进行了展望,为初步了解听处理障碍的定义、评估与诊断方法提供了学习基础.