听神经瘤致聋机理—14例（16耳）听神经瘤病人听力测试结果分析

目的：探讨听神经瘤听力损失的病理生理机制。方法：对14例（16耳）听神经瘤患者行纯音听阈、阻抗、听性脑干反应（ABR）、诱发耳声发射测试（EOAE）及CT和（或）MRI扫描。EOAE能引出的4耳还检测其传出抑制功能，ABR不能检测且EOAE不能引出的重度聋（听力损失大于80dB)有5耳行鼓岬刺激试验（PST）。结果：16耳听神经瘤中2耳（12．50％）听力损失源于神经性损害；6耳（37．50％）蜗性损害；8耳（50％）蜗－神经性损害。能引出EOAE的4耳均有传出功能障碍。结论：EOAE可评价听神经瘤的耳蜗功能形态；ABR结合PST能分析听神经瘤的蜗后神经功能。听神经瘤的听觉病理可同时或单独发生于听外周的耳蜗水平、第Ⅷ对颅神经（传入神经）水平和橄榄核耳蜗传出神经水平。     

听神经瘤的耳声发射观察

目的 观察和分析听神经瘤的耳声发射特点,为评估听神经瘤患者的耳蜗功能和选择保护听力的术式提供参考依据.方法 对20例（22耳）听神经瘤患者行纯音听阈、阻抗、听性脑干反应（auditory brainstem response,ABR）、诱发性耳声发射（evoked otoacoustic emissions,EOAE）测试及CT和（或）MRI扫描,能引出EOAE的瘤耳检测其自发性耳声发射（spontaneous otoacoustic emissions,SOAE）和传出抑制功能.结果 28.57%听神经瘤耳能引出EOAE,按其畸变产物耳声发射（distortion product otoacoustic emissions,DPOAE）特点分为三型：①“耳蜗型”3耳;②“非耳蜗型”2耳;③“混合型”1耳;“非耳蜗型”耳能引出强大的SOAE;能引出EOAE的6耳均有内侧橄榄耳蜗传出系统功能障碍.结论 EOAE可精确分析听神经瘤患者的耳蜗（外毛细胞）功能,部分听神经瘤病人存在“离断耳”现象.耳声发射（otoacousticemissions,OAE）在诊断重度感音神经性聋（包括听神经瘤病人）方面有一定潜能.     

关于听神经病之我见

关于听神经病的定义 ,现在国内外学者有了较为一致的看法 ,即临床表现为不同程度的听力损失 ,听功能检查示诱发性耳声发射 (evokedotoacousticemission ,EOAE)正常、而听性脑干反应 (auditorybrainstemresponse ,ABR)缺失或严重异常的疾病[1]。其突出的临床特点为比纯音听阈下降更为严重的言语分辨率的损害[2 ]。关于听神经病的病变部位和发病机制还需要进一步探讨。国内顾瑞等 1992年报道一类以低频感音神经性聋为主要特征的听力损失患者 ,其各种OAE反应可表现接近正常 ,与纯音听力不符 ,同时可伴有ABR等的异常 ,认为这种临床表现反…     

听神经瘤手术的听力保护

探讨听神经瘤手术的听力保护.方法报告两例经乙状窦后进路、内镜辅助和术中用CAP和ABR连续适时监测下切除听神经瘤的结果.结果例1 MRI示内听道口外瘤体直径约1.5cm,术前语频纯音听阈平均在30dB,能引出ABR各波.术中以ABR适时监测,完全切除瘤体后仍可引出清楚的ABR Ⅰ、Ⅲ、Ⅴ波,术后无面瘫.术后7个月复诊,纯音听阚及ABR波间期恢复术前水平.例2为双侧听神经瘤.MRI示左右听神经瘤直径分别为4cm和5cm.一侧肿瘤切除后间隔4个月行另一侧听神经瘤切除术.为保留第二次手术耳听力,术中用CAP监测.术中虽未能保留蜗神经,但切除肿瘤后仍可记录到CAP.结论听神经瘤手术中的CAP监测结果无法评判术后能否保留听力,而ABR则能够及时反映听神经及其通路的功能状态,肿瘤切除后引出ABR Ⅰ、Ⅲ、Ⅴ波可有望保存术前听力.     

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。     

397例听神经瘤听力特征分析

目的 分析听神经瘤患者听力学特征,为筛查听神经瘤提供参考依据。方法 回顾性分析2019年8月—2022年4月诊治的397例单侧听神经瘤患者临床资料,所有患者均行纯音测听、声导抗测试、言语识别率(SDS)、畸变产物耳声发射(DPOAE)、听性脑干诱发电位(ABR)和颅脑增强MRI。结果 以典型症状就诊者312例,非典型症状就诊者85例。以突发性聋就诊者25例;患耳听力正常者54例,包含大型甚至特大型肿瘤,99例全聋者亦包含内听道和中型肿瘤。5例全聋者言语识别能力未完全消失。DPOAE一致型245例,耳蜗型72例,蜗后型79例。ABR阳性率98.0%(389/397),8例ABR波形正常者中3例为双侧非对称听力损失。结论 非典型症状就诊者占比21.4%,以突发性聋就诊、听力正常或ABR正常者不能排除听神经瘤;DPOAE和ABR可作为筛查听神经瘤的重要检查方法。     

听神经病的听功能状态分析

目的 :探讨听神经病的听功能状态及病损部位。方法 :分析 6 5例听神经病患者的临床资料、纯音测听、声导抗测试、听性脑干反应 (ABR)、4 0Hz听觉相关电位及OAE检查结果。结果 :听神经病的低频听力损失源于蜗后的传入、传出神经及听性脑干受损 ,表现为声反射、传出抑制、ABR异常及诱发性OAE与纯音听阈不呈平行关系 ,与之相对应 ,低频区的外毛细胞处于失抑制的超常活动状态 ,表现为低频区SOAE增强、TEOAE反应幅值及DPOAE幅值升高 ;听神经病的高频听力损失源于耳蜗的外毛细胞损害 ,表现为高频区DPOAE幅值与纯音听阈呈一致性下降 ;听神经病的中频听力损失最轻或接近正常 ,表现为 2kHz附近的纯音听阈和DPOAE幅值均接近于正常。结论 :听神经病的传入、传出系统及耳蜗水平均有不同程度的功能障碍 ,其病损部位主要在耳蜗传入、传出神经 ,向上可侵及脑干 ,向下可侵及耳蜗     

小儿听神经病的误诊和漏诊

目的:探讨小儿听神经病的临床表现。方法:对一组年龄为4～68个月,听力学检查表现为听神经病患儿的临床资料进行回顾性研究。受试者的入选标准为短声听觉脑干反应(ABR)严重异常而畸变产物耳声发射(DPOAE)和(或)耳蜗微音电位(CM)正常。分析其短声ABR、DPOAE、行为听力测试、声导纳以及内耳MRI等结果。结果:①40例听力学表现为双侧听神经病,8例为单侧;②68.2%耳(60/88)在短声ABR的最大输出强度100dBnHL无反应,而有些耳仅在很高的测试强度有波形分化较差的Ⅴ波;③所有耳均可记录到CM,而只有41.5%能记录到DPOAE;④该组患儿的行为听力结果各种各样,为从轻度到极重度的听力损失;⑤4例经内耳MRI诊断为蜗神经发育不全。结论:在听力学表现为听神经病的儿童:①CM的诊断敏感度较DPOAE高;②各种客观听力测试无法预估行为听力;③需作内耳MRI检查,以除外蜗神经发育不全。     

蜗神经发育不良患者的听力学特征分析

目的探讨蜗神经发育不良(cochlear nerve deficiency,CND)患者的听力学特征。方法以30例(54耳)(男14例,女16例,年龄6月~29岁,平均4.9岁)内听道斜矢状位磁共振成像示蜗神经发育不良的患者为研究对象,回顾性分析患者的纯音测听或行为测听、声导抗、耳声发射、听性脑干反应(ABR)、听性稳态反应等听力学检测结果及颞骨高分辨CT(HRCT)结果,分析蜗神经发育异常者的听力学特征。结果 30例54耳蜗神经发育不良患者中,双侧24例(80%),单侧6例(20%),其中蜗神经缺如33耳,蜗神经细小21耳。54耳中,共有25耳(46.29%)伴内耳畸形,而单侧蜗神经发育不良者的对侧6耳中,50%(3耳)伴内耳畸形。纯音测听或行为测听和听性稳态反应结果均提示蜗神经发育不良患耳呈重度-极重度听力损失,且蜗神经缺如耳较蜗神经细小耳的听力损失程度更重。11耳(20.37%,11/54)蜗神经发育不良耳可在80~100 dB nHL高强度声刺激下引出听性脑干反应;6例双侧蜗神经发育不良者(20.0%,6/30)中7耳(1例双耳,5例单耳)全部频率或部分频率引出DPOAE,但其中5耳未引出ABR,2耳分别于100和90 dB nHL刺激声强下引出ABR。结论蜗神经发育不良患者常表现为重度-极重度听力损失,近一半伴内耳畸形,部分患耳可引出DPOAE,而ABR严重异常或缺失。     

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。     

蜗后性听力减退29例病因分析

目的 探讨蜗后性低频听力减退的发病原因。方法 复习1988～2002年29例原因明确的蜗后性低频听力减退患者的临床特点和听力学检查结果。结果 头部外伤、听神经瘤、周围神经病、遗传性聋、多发性硬化和脑干疾病均可表现为蜗后性低频听力减退,其共同的特点是：低频听力减退,诱发性耳声发射正常或至少在部分频率正常,不受对侧噪声抑制,引不出镫骨肌反射,听性脑干反应(auditory能超群brainstem response,ABR)异常。结论 以耳声发射正常和ABR异常为主要特征的听功能障碍是一组症状群,可为不同病因引起的听神经和(或)听性脑干病变的表现,建议对这一组听力学表现称之为蜗后性低频听力减退。     

自发性耳声发射与耳蜗传出调控的关系探讨

OBJECTIVE: To study the relationship between spontaneous otoacoustic emissions(SOAE) and efferent control of cochlea and their clinical significance. METHODS: SOAE, transient evoked otoacoustic emissions (TEOAE), distortion product otoacoustic emissions (DPOAE) and contralateral white noise (60 dB SPL) suppression of TEOAE and DPOAE experiments were conducted in 312 ears of 95 patients with retrocochlear impairment and/or MOCS dysfunction and 64 normal young adults. RESULTS: MOCS dysfunction was shown in 126 ears of 65 patients (130 ears) with auditory neuropathy, 2 ears of 2 patients with unilateral acoustic neuroma, 4 ears of 2 patients with hyperacusis, 14 ears of 26 patients(48 ears) with normal hearing level in unilateral or bilateral tinnitus. Stronger EOAE could be recorded in total 146 ears with MOCS dysfunction at any pure tone hearing level. SOAE could be recorded in 126 of 146 ears (86.3%) with MOCS dysfunction and 44 of 128 ears (34.3%) with normal hearing. SOAE of ears with MOCS dysfunction was mainly at frequencies from 0.693 to 3.055 kHz and SOAE of normal ears was at frequencies from 1.135 to 2.746 kHz. Average value of maximum amplitude of SOAE spectrum (-3.4 +/- 6.4) dB SPL was significantly greater than that in normal ears (-6.8 +/- 7.8) dB SPL (P < 0.01). The major frequency range of SOAE (0.693-3.055 kHz) in MOCS dysfunction ears was essentially consistent with that of efferent suppression in normal ears (0.7-3 kHz). CONCLUSION: The modulation of the cochlear active mechanisms by MOCS mainly presents in the low- and mid-frequency regions, these frequencies correspond to the frequency range of SOAE. Stronger SOAE indicates pathophysiological significance. There is a clear clinical relationship between SOAE and the efferent modulation of the cochlea.     

中枢性低频听力减退的听力学分析

目的 加深对双侧中枢性低频听力减退的认识。方法 复习１９８８～２０００年１０１例原因不明的双侧感音神经性低频听力减退患者的听力学检查结果。结果 其共同的特点是：言语听力不成比例地差于纯音听力,诱发性耳声发射正常,不受对侧噪声抑制,引不出镫骨肌声反射,听性脑干反应（ａｕｄｉｔｏｒｙ ｂｒａｉｎｓｔｅｍ ｒｅｓｐｏｎｓｅ,ＡＢＲ）异常,－ＳＰ／ＡＰ振幅比大于０．４４。结论 此类原因不明的低频听力减退患者听力学表现提示：耳蜗外毛细胞功能正常,与声反射、听传出抑制、ＡＢＲ波Ⅲ相关的脑干听觉神经径路为功能障碍的主要部位,可伴有耳蜗或传入神经功能障碍。建议使用“中枢性低频听减退”的名称。     

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。     

27例听神经病患者的临床特征分析

目的总结探讨听神经病（auditory neuropathy,AN）患者的临床特点和听力学特征。方法回顾性分析27例听神经病患者,并总结其临床资料、纯音听阈、声导抗测试、听性脑干反应与畸变产物耳声发射结果。结果 27例患者中,25例表现为双耳渐进性听力减退,1例表现为渐进性左侧听力下降,1例为查体发现。其中23例伴间断性或持续性耳鸣,3例伴头晕。纯音听力图示上升型感音神经性听力损失。21例患者镫骨肌反射同侧及交叉声反射均未引出,6例镫骨肌反射阈值提高。27例听神经病患者听性脑干反应53耳自波Ⅰ起全部未引出（〉100 dB SPL）。畸变产物耳声发射全部可引出。结论听神经病是一临床表现特殊的感音神经性聋,其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;听神经病的病因及发病机制需进一步探讨研究。     

Audiovestibular findings and location of an acoustic neuroma

Forty-one patients with unilateral acoustic neuroma (AN) were diagnosed by magnetic resonance imaging (MRI) between 1992 and 1997. All cases were analyzed with respect to tumor location and the results of audiometric examinations, auditory brainstem response (ABR) testing, and electronystagmography (ENG). Tumor location was determined by MRI and cases were divided into intracanalicular and extracanalicular sites. Intracanalicular tumors were significantly smaller than the extracanalicular ones The pure-tone hearing thresholds were better in ears with intracanalicular lesions than in those with extracanalicular ones. Respectively, speech reception thresholds were 33 dB and 45 dB, and speech discrimination scores 79% and 65%. ABR was abnormal in 98% of ANs, but was insufficient for determining tumor location. The ENG pursuit test was more frequently normal and the caloric side difference was smaller in ears with intracanalicular than extracanalicular AN. These findings show that the results of pure-tone and speech audiometry and ENG are better in ears with intracanalicular AN, while ABR results are similar regardless of tumor location. Received: 11 August 1999 / Accepted: 16 November 1999     

Click evoked otoacoustic emissions in neonatal screening

Seven hundred and twenty-three neonates under intensive care have been tested by evoked otoacoustic emissions (EOAE) and the auditory brain stem response (ABR) to investigate the use of EOAE as a test for hearing impairment. Three hundred and thirty-one have had follow-up tests to the age of at least 2 years. The EOAE test has been found to be practical and quick to perform. The proportion of NICU infants producing a recordable EOAE is 80%, and the sensitivity and selectivity to the ABR result in the period up to 3 months post due date is 93 and 84%, respectively. These figures are high enough and the reduction in time compared to ABR is sufficient for the EOAE to be considered as the primary screen. The follow-up data show mixed results with both false positives and false negatives present. The incidence of severe hearing impairment is close to that expected from retrospective studies at 2 in 331 (1 bilateral, 1 unilateral). Firm conclusions on the sensitivity of EOAE to long-term hearing impairment await the results from larger numbers of infants and further follow up data.     

Auditory neuropathy characteristics in children with cochlear nerve deficiency

OBJECTIVE: To describe a group of children exhibiting electrophysiologic responses characteristic of auditory neuropathy (AN) who were subsequently identified as having absent or small cochlear nerves (i.e., cochlear nerve deficiency). DESIGN: A retrospective review of the clinical records, audiological testing results, and magnetic resonance imaging (MRI) studies. Fifty-one of 65 children with AN characteristics on auditory brain stem response (ABR) testing had MRI available for review. Nine (18%) of these 51 children with ABR characteristic of AN have been identified as having small (N = 2; 4%) or absent (N = 7; 14%) cochlear nerves on MRI. RESULTS: Of the nine children with cochlear nerve deficiency, five (56%) were affected unilaterally and four (44%) bilaterally. Eight of nine presented after failing a newborn infant hearing screening, whereas one presented at 3 yr of age. On diagnostic ABR testing, all 9 children (9 of 13 affected ears; 69%) had evidence of a cochlear microphonic (CM) and absent neural responses in at least one ear. In the unilateral cases, AN characteristics were detected in all affected ears. In bilateral cases, at least one of the ears in each child demonstrated the AN phenotype, whereas the contralateral ear had no CM identified. Only one ear with cochlear nerve deficiency had present otoacoustic emissions as measured by distortion-product otoacoustic emissions. In children with appropriate available behavioral testing results, all ears without cochlear nerves were identified as having a profound hearing loss. Only 4 (31%) of the 13 ears with cochlear nerve deficiency had a small internal auditory canal on MRI. CONCLUSIONS: Children with cochlear nerve deficiency can present with electrophysiologic evidence of AN. These children frequently refer on newborn screening examinations that use ABR-based testing methods. Similar to other causes of AN, diagnostic ABR testing will show a CM with absent neural responses. Given that 9 (18%) of 51 children with available MRI and electrophysiologic characteristics of AN in our program have been identified as having cochlear nerve deficiency makes this a relatively common diagnosis. These findings suggest that MRI is indicated for all children diagnosed with AN. Moreover, electrophysiologic evidence of unilateral AN in association with a profound hearing loss should make the clinician highly suspicious for this problem. Although children with cochlear nerve deficiency who have a small nerve may benefit from cochlear implantation or amplification, these interventions are obviously contraindicated in children with completely absent cochlear nerves.