听神经病患者最大言语识别率与纯音听阈的相关性分析

目的 分析听神经病患者最大言语识别率与纯音测听之间的相关性,探讨听神经病患者与言语识别率不成比例的临床意义.方法 对106例(212耳)经纯音测听、声导抗、畸变产物耳声发射、听件脑干反应测试确诊为听神经病的患者,行最大言语识别率测试,并与不同程度损失及不同类型听力曲线进行分类、分型比较.依据损失分出轻度、中度、中重度和重度;依据听力曲线分为平坦型、低频上升Ⅰ型、低频上升Ⅱ型、山型、谷型、不典型.统计数据应用SPSS 11.0对不同程度的损失、最大言语识别率采用方差分析和相关性分析.结果 106例(212耳)具有相同听力损失或相同类型听力曲线的听神经病患者,可表现出不同程度的最大言语识别率,在听神经病患者群体水平整体评估,最大言语识别率百分比与全频听力阈值旱负相关(r=-0.602;P<0.01),另外,听力损失程度较接近的听力曲线类型,高频听力损失程度轻者其最大言语识别率也相对较好;106例听神经病患者中有26例(52耳)患者双耳分别记录逐步递增的6个刺激声级的言语识别率曲线,其中平均阈上10 dB出现的最大言语识别率频次最高.结论 听神经病患者最大言语识别率在个体问存在明显差异,相同的听力损失,可以出现不同的最大言语识别率;但在群体水平上最大言语识别率与阈值有一定相关性,即听力阈值越大,言语识别率百分比数值越小,且当听神经病患者听力损失在同一水平时,其最大言语识别率程度与听神经病患者听力曲线类型相关.     

听神经病与前庭上、下神经损害的关系

目的观察听神经病(auditory neuropathy,AN)患者伴发前庭上、下神经损害的特点,分析其与AN的关系。方法检测70例AN患者的眼震电图(electronystagmography,ENG)及前庭诱发肌源性电位(vestibular evoked myo-genic potential,VEMP),观察VEMP波形、潜伏期及振幅,并与对照组比较,分析前庭神经受损与AN患者听力损害程度、年龄及病程的关系。结果70例(139耳)AN患者中VEMP引出38耳(27.3%),未引出101耳(72.7%),ENG冷热试验半规管功能正常106耳(76.3%),异常33耳(23.7%),两种检测结果差异有显著统计学意义(P<0.01)。VEMP引出的38耳中,左、右耳的潜伏期均值P13为24.54±5.388及25.17±6.068ms,N23为31.38±5.766及32.02±6.058ms,对照组P13为17.04±1.781及17.49±2.291ms;N23为25.05±2.864及25.81±3.273ms,两组差异有显著统计学意义(P<0.01);左、右耳的振幅均值为60.16±34.732及43.83±31.288μV,对照组为147.16±50.026及135.66±54.737μV,两组差异有显著统计学意义(P<0.01)。AN患者VEMP是否引出与听力损害程度有相关性(P<0.05),与年龄及病程无相关性(P>0.05)。AN患者ENG的半规管功能是否正常与病程有相关性(P<0.05),与听力损害程度及年龄无相关性(P>0.05)。结论AN患者伴发前庭下神经损害的几率远大于前庭上神经,表现为VEMP波形未引出或潜伏期延长、振幅低;VEMP是否引出与听力损害程度有相关性,ENG的半规管功能异常与病程有相关性,提示AN患者听神经病变极易累及前庭下神经及其终器球囊;VEMP与ENG联合应用可对AN伴发前庭神经病变的范围作出较全面可靠的评估,应作为AN诊断常规检查项目之一。     

听神经病对前庭功能的影响

听神经病是一种以双耳中度低频感音神经耳聋为临床症状,但ABR反应严重异常、耳声发射正常的病症。其相关的前庭功能检测的报道与关注在学术界仍较少,本文旨对听神经病所伴有的前庭功能异常进行文献回顾,从前庭生理学及与其相关的前庭功能检测手段进行总结和概述。期望为听神经病患者的诊治与前庭功能评估提供参考。     

非综合征型听神经病患者前庭上下成分功能障碍定位研究与频率特征

目的 探讨听神经病伴发前庭功能障碍的可能性,并分析其部位和频率特征。 方法 以眼肌前庭诱发肌源性电位(oVEMP)、颈肌前庭诱发肌源性电位(cVEMP)、视频头脉冲试验(vHIT)、头脉冲抑制试验(SHIMP)和冷热试验对20例(40耳)非综合征型听神经病患者进行前庭功能评估,对统计学处理结果进行客观分析。 结果 20例患者(40耳)接受oVEMP和cVEMP测试,oVEMP异常率为85%,cVEMP异常率为95%,差异无统计学意义。11例(22耳)接受vHIT和SHIMP测试,外、上、后半规管vHIT和SHIMP的异常率分别为14%、18%、9%和9%,组间差异无统计学意义。19例患者行冷热试验,异常率为74%。11例接受vHIT和SHIMP测试的患者,冷热试验异常率为82%,显著高于vHIT和SHIMP异常率,差异有统计学意义。 结论 听神经病患者存在前庭功能障碍,前庭上下成分(包括感受器和前庭上下神经)受累概率相当,耳石器和半规管均可受累,各半规管受累概率相当,半规管功能损伤主要累及低频。     

早期梅尼埃病患者前庭诱发肌源性电位的特征

目的 建立听力室前庭诱发肌源性电位（VEMPs）潜伏期、耳间潜伏期差值、振幅比及耳间不对称率等参数的正常值,分析早期梅尼埃病患者的VEMPs特征。方法 随机选取听力正常的健康成人39例,用于建立VEMPs正常值范围并用作健康对照;对单侧发病的早期梅尼埃病患者37例,行双侧VEMPs检测,分析其VEMPs特征。结果 37例单侧发病的早期梅尼埃病患者中,患侧未能引出者11例,双侧未能引出者4例,另外15例患侧VEMPs的异常表现可为p13或n23潜伏期延长、耳间潜伏期差值延长、振幅增强或减弱等,总异常率为81.08%。结论 当VEMPs的耳间潜伏期差值表现为延长、振幅表现为增强或减弱时,对早期梅尼埃病的诊断有显著意义。     

眼肌和颈肌前庭诱发肌源性电位在外周性前庭传导通路疾病诊断中的应用

目的:观察外周性前庭损害患者眼肌前庭诱发肌源性电位(oVEMP)和颈肌前庭诱发肌源性电位(cVEMP)的引出情况并探讨其临床诊断价值。方法:选择2011-03-2012-03期间在我院临床诊断为外周前庭损害,并接受门诊和(或)住院治疗的患者13例(14耳),进行双耳气导短纯音诱发的oVEMP和cVEMP检测,观察两种电位的引出情况,分析前庭上成分(椭圆/前庭上神经传入通路)和前庭下成分(球囊/前庭下神经传入通路)机能受损的状况。结果:13例(14耳,双侧1例)外周性眩晕患者包括:耳带状疱疹3例(3耳),听神经瘤3例(4耳),Ⅶ+Ⅷ颅神经牵拉伤1例(1耳),前庭神经炎2例(2耳),梅尼埃病3例(3耳),单侧内听道发育不全1例(1耳)。总体oVEMP正常引出2耳(正常引出率为14.3%),cVEMP正常引出3耳(正常引出率21.4%)。结论:外周性眩晕患者前庭耳石器及其传导通路机能受损的情况可以通过临床oVEMP和cVEMP检测进行客观评价,其表现形式根据病变波及的范围与程度不同各异。     

听神经病对前庭功能的影响

目的探讨听神经病患者的前庭功能,以了解听神经病患者的平衡能力。方法对32例听神经病患者及正常对照组患者行前庭功能检查,包括眼震电图检查和静态姿势描记图检查,并将2组的结果进行比较。结果听神经病患者的平衡能力减低,眼震电图检查异常者6例(18.75%),姿势描记图检查结果明显异常,睁、闭眼状态下,重心晃动的轨迹长度与晃动速度与正常对照组相比较,差异有显著性意义(P<0.05)。结论在今后的临床工作中,对听神经病患者不妨加行前庭功能检查,以进一步了解其第Ⅷ颅神经的功能状态,尤其是静态姿势描记图,该测试快速、简单,结果客观可靠。     

Tullio现象与Ramsay Hunt综合征的前庭诱发的肌源性电位

目的应用前庭诱发的肌源性电位(vestibular evoked myogenic potentials,VEMPs),观察Tullio现象与Ramsay Hunt综合征的电位特点,为诊断提供客观依据.方法Tullio现象与Ramsay Hunt综合征各1例,通过已经建立的VEMPs检查方法观察两者的电位引出情况.结果正常人VEMP刺激声在阈上85dB nHL可引出VEMPs.本文Tullio现象患者在刺激强度降至39dB nHL时仍可引出.而健侧在69dB nHL时就已不能引出.Ramsay Hunt综合征在阈上105dBHL右侧不能引出VEMP;而左侧可以引出.结论VEMPs可用来了解前庭下神经的功能状态.由于VEMPs的检查特点,可用于动态观察前庭神经病变后的恢复情况.     

听神经病并发的前庭及肢体神经损害

目的：观察听神经病患者前庭神经和四肢末梢;周围神经的受累情况,加深对听神经病的总体认识。方法：对28例青少年听神经病患者行前庭功能和神经传导速度检查并分析其结果：:28列听神经病中,有22例外周前庭功能受损（78．57％）,11例肢体末梢神经受损（39．29％）,冷热试验诱发性眼震反应分下列三种类型：（1）双侧正常6例（占21．43％）,（2）双侧减弱20例（占71．43％）;（3）单侧减弱2例（占7．4％）。神经传导速度检查结果分四种类型：（1）运行,感觉均正常17例（占60．71％）;（2）运动,感觉均异常4例（占14．29％）;（3）单独运动异常4例（占14．29％）;（4）单独感觉异常3例（占10．71％）。结论：影响听神经的病理过程也可以影响其它颅内或颅外的周围神经（如前庭神经和肢体神经）,听神经病既可以单发也可以并发多种周围神经病。     

听神经病并发的前庭及肢体神经损害

目的 :观察听神经病患者前庭神经和四肢末梢周围神经的受累情况 ,加深对听神经病的总体认识。方法 :对2 8例青少年听神经病患者行前庭功能和神经传导速度检查并分析其结果。结果 :2 8例听神经病中 ,有 2 2例外周前庭功能受损 (78.5 7% ) ,11例肢体末梢神经受损 (39.2 9% )。冷热试验诱发性眼震反应分下列三种类型 :1双侧正常 6例 (占2 1.43% ) ;2双侧减弱 2 0例 (占 71.43% ) ;3单侧减弱 2例 (占 7.4% )。神经传导速度检查结果分四种类型 :1运动、感觉均正常 17例 (占 6 0 .71% ) ;2运动、感觉均异常 4例 (占 14.2 9% ) ;3单独运动异常 4例 (占 14.2 9% ) ;4单独感觉异常 3例 (占 10 .71% )。结论 :影响听神经的病理过程也可以影响其它颅内或颅外的周围神经 (如前庭神经和肢体神经 ) ,听神经病既可以单发也可以并发多种周围神经病。     

听神经病伴发前庭下神经损害

OBJECTIVE: To investigate if auditory neuropathy have inferior vestibular nerve (IVN) lesion and to explore the relation between AN and the IVN lesion by vestibular evoked myogenic potentials (VEMPs). METHODS: VEMPs were observed in 13 patients with auditory neuropathy. And the relation among the duration, hearing threshold of lower frequency and speech discrimination score with VEMPs were observed. RESULTS: Fifty-four percent patients in auditory neuropathy had abnormal VEMPs. They took the form of lower amplitude and no response. The statistical analysis showed that the abnormality of VEMPs had no correlation with lower frequency hearing loss, the duration and speech discrimination score. CONCLUSIONS: The IVN dysfunction may coexist with auditory neuropathy, having lesion in the IVN. However, there was no significant relation between the severity of AN and VEMPs, which meant that AN and inferior vestibular neuropathy had their independence to some extent.     

Influence of aging over 10 years on auditory and vestibular functions in three patients with auditory neuropathy

The influence of aging on hearing and vestibular function in patients with auditory neuropathy has not been investigated. The purpose of this study was to investigate how hearing and vestibular function in this disease change with aging. The subjects were three female patients with auditory neuropathy. We checked their hearing and vestibular function by speech discrimination tests, ABR, ECochG, DPOAE, caloric test, damped-rotational chair test, and VEMPs. In all three patients, speech discrimination ability and vestibular function markedly declined with aging. However, speech language understanding and higher brain function were less affected by aging.     

Sacculo-collic pathway dysfunction accompanying auditory neuropathy

Conclusions. In a patient with bilateral auditory neuropathy (AN), the vestibular-evoked myogenic potential (VEMP) was probably absent because of a neuropathy involving the inferior vestibular nerve and/or its end organ, the saccule. Our result can therefore be interpreted as a concomitant unilateral sacculo-collic neuropathy. We suggest the use of more precise terms to characterize AN patients with involvement of different parts of the inner ear and its innervations. We encourage detailed vestibular assessment in patients with AN in order to assess the co-existence of any symptomatic or asymptomatic vestibular disorder. Information such as that provided in this report will be valuable for clinicians caring for this group of patients. Objective. AN is a disorder characterized by the absence or severe impairment of auditory brainstem responses in the presence of normal cochlear outer hair cell function as revealed by otoacoustic emissions (OAEs) and/or electrocochleography (ECoG). A variety of processes and etiologies are thought to be involved in its pathophysiology. In most literature reports the auditory profile of patients with AN is discussed. However, the extent of vestibular involvement, especially that involving the saccule, is not known. We performed vestibular tests to assess the status of the saccule in a patient with AN. Material and methods. One patient with AN was studied. The patient was a right-handed 21-year-old female with chief complaints of hearing loss and speech perception difficulty. Results. The auditory test results were consistent with the diagnosis of AN, i.e. absent auditory brainstem responses, moderate hearing loss, an inappropriately profound speech discrimination score and the presence of OAEs and measurable cochlear microphonics on ECoG. On neurological examination, gait and balance tests were normal. Ice-water caloric testing induced a sensation of dizziness in both ears. Short tone-burst VEMPs showed no response on left-ear stimulation and a biphasic response with normal latency and amplitude on right-ear stimulation.     

Sacculo-collic pathway dysfunction accompanying auditory neuropathy

CONCLUSIONS: In a patient with bilateral auditory neuropathy (AN), the vestibular-evoked myogenic potential (VEMP) was probably absent because of a neuropathy involving the inferior vestibular nerve and/or its end organ, the saccule. Our result can therefore be interpreted as a concomitant unilateral sacculo-collic neuropathy. We suggest the use of more precise terms to characterize AN patients with involvement of different parts of the inner ear and its innervations. We encourage detailed vestibular assessment in patients with AN in order to assess the co-existence of any symptomatic or asymptomatic vestibular disorder. Information such as that provided in this report will be valuable for clinicians caring for this group of patients. OBJECTIVE: AN is a disorder characterized by the absence or severe impairment of auditory brainstem responses in the presence of normal cochlear outer hair cell function as revealed by otoacoustic emissions (OAEs) and/or electrocochleography (ECoG). A variety of processes and etiologies are thought to be involved in its pathophysiology. In most literature reports the auditory profile of patients with AN is discussed. However, the extent of vestibular involvement, especially that involving the saccule, is not known. We performed vestibular tests to assess the status of the saccule in a patient with AN. MATERIAL AND METHODS: One patient with AN was studied. The patient was a right-handed 21-year-old female with chief complaints of hearing loss and speech perception difficulty. RESULTS: The auditory test results were consistent with the diagnosis of AN, i.e. absent auditory brainstem responses, moderate hearing loss, an inappropriately profound speech discrimination score and the presence of OAEs and measurable cochlear microphonics on ECoG. On neurological examination, gait and balance tests were normal. Ice-water caloric testing induced a sensation of dizziness in both ears. Short tone-burst VEMPs showed no response on left-ear stimulation and a biphasic response with normal latency and amplitude on right-ear stimulation.     

Involvement of peripheral vestibular nerve in individuals with auditory neuropathy

The vestibulocochlear nerve is a sensory nerve that serves the organs of hearing and equilibrium. Neuropathies of the nerve, particularly auditory neuropathy may be caused by primary demyelination or by axonal diseases. In disorders affecting the cochlear nerve, it is probable that the vestibular nerve is involved as well. There are isolated reports of the involvement of the inferior vestibular nerve (using vestibular-evoked myogenic potentials) in individuals with AN. However, there is a dearth of information on the involvement of the superior vestibular nerve and other functions such as optokinetic, saccade and vestibulo-occular reflex. A total of three subjects diagnosed as having auditory neuropathy, underwent an extensive vestibular assessment consisting of clinical tests of stability (Romberg, Fukuda stepping test), administration of dizziness questionnaire developed by Maryland hearing and balance centre, cervical vestibular-evoked myogenic potentials and a standard electronystagmography test battery. In the present study, the entire subject population assessed showed hypofunctional caloric responses and absent VEMPs. Two out of the three subjects were asymptomatic of vestibular dysfunction. On the clinical tests of stability, two subjects showed deviations to the right, while one subject performed normally. Thus, the present study indicates a possible involvement of peripheral vestibular nerve involvement in individuals with auditory neuropathy.     

Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing AIDS or cochlear implants

OBJECTIVE: To evaluate speech perception skills in children with auditory neuropathy (AN)/auditory dyssynchrony (AD)-type hearing loss managed with either hearing aids or cochlear implants. STUDY DESIGN: Prospective data collection in 3 subject groups: AN/AD children fitted with bilateral amplification, AN/AD children fitted with cochlear implant (in 1 or both ears), and a matched control group of implanted children with sensorineural hearing loss. MAIN OUTCOME MEASURE: Open-set monosyllabic words (consonant-nucleus-consonant). RESULTS: Of the 10 implanted AN/AD children, 9 demonstrated significant speech discrimination (consonant-nucleus-consonant phoneme score > or =55%). Similar results were obtained for the aided AN/AD group. Findings for both AN/AD subject groups were poorer than those of the implanted sensorineural cohort. CONCLUSION: Cochlear implantation can offer useful hearing in subjects with AN/AD-type hearing loss. However, expectations for this group may need to be lower than for patients with peripheral (cochlear) loss.     

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。     

Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy

Auditory neuropathy (AN) is a hearing disorder characterized by the preservation of outer hair cell function despite the absence of auditory brainstem responses. The pathophysiology and etiology of this condition remain unknown. Recent studies have shown that some patients with AN benefit significantly from cochlear implantation. These patients have all been native speakers of Western languages. A 3-year-old Mandarin-speaking boy was referred to our center because of speech delay. After a series of audiological surveys, retro-cochlear lesion was impressed. During the 2-year period of rehabilitation, poor speech discrimination out of proportion to aided hearing thresholds led to the diagnosis of auditory neuropathy. Because of the limited benefit from amplification, he received a cochlear implant. Significant improvement of speech perception skills assessed by a Mandarin auditory perception test was noted shortly after implantation. The post-implantation performance in this Mandarin-speaking child was consistent with that of reports for implantees speaking Western languages. For Mandarin-speaking children with AN who fail to benefit from conventional treatment, cochlear implantation may be a good alternative choice.     

Perceptual characterization of children with auditory neuropathy

OBJECTIVE: To characterize the perceptual abilities of a group of children with auditory neuropathy (AN)-type hearing loss, correlating results on a range of psychophysical tasks with open-set speech perception performance. DESIGN: Frequency resolution, temporal resolution and frequency discrimination ability was assessed in a group of 14 children with AN. Data also were obtained from a cohort of matched subjects with sensorineural hearing loss, and from a group of normally hearing children. RESULTS: Frequency resolution (notched noise masking) results for the AN subjects were equivalent to those of the normal-hearing subjects reflecting the "normal" outer hair cell function that characterizes the AN condition. Temporal resolution (TMTF) findings were, however, abnormal in many AN subjects and the degree of temporal disruption was correlated with speech discrimination (CNC) score. Frequency discrimination ability (for both fixed and frequency modulated stimuli) was also affected in those children with poor temporal resolution. CONCLUSIONS: The findings of this study indicate that the perceptual profiles of children with AN are quite different from those with sensorineural hearing loss. Where subjects in the latter group presented with impaired frequency resolution and normal temporal processing, the AN subjects typically showed normal frequency resolution and varying degrees of temporal disruption. The severity of this temporal abnormality, which appeared to affect both temporal resolution/amplitude modulation detection and the temporal aspects of frequency discrimination (such as phase locking), was strongly correlated to speech perception performance.     

Vestibular Function in Auditory Neuropathy

Auditory neuropathy is characterized by mild-to-moderate pure-tone hearing loss, poor speech discrimination out of proportion with this loss, absent or abnormal auditory brainstem responses and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We followed three patients in our clinic whom we classified as auditory neuropathy patients. These patients also complained of balance disorders and we report our auditory and vestibular system analyses of these patients. The data presented herein include results of audiometric tests (serial pure-tone audiometry and speech discrimination tests), otoacoustic emissions, auditory-evoked brainstem responses and vestibular function tests (clinical tests of balance, electronystagmography, damped rotation tests and vestibular-evoked myogenic potentials). In all patients, pure-tone audiometry revealed mild-to-moderate sensorineural hearing loss, markedly poor speech discrimination scores and absent auditory-evoked brainstem responses, all in the presence of normal otoacoustic emissions. Balance tests (caloric tests and damped rotation test) were abnormal. Saccades, smooth pursuit eye movements and optokinetic nystagmus were normal in all patients. Neurological and motor system evaluations were normal in all patients. These three auditory neuropathy patients manifest a disorder of cochlear nerve function in the presence of normal outer hair cell activity. They additionally manifest a disorder of the vestibular nerve and its end organs. We conclude that, in patients with isolated auditory neuropathy, the vestibular branch of the VIIIth cranial nerve and its innervated structures may also be affected. We suggest the use of the term "cochlear neuropathy" to characterize those patients with involvement of only the auditory branch of the VIIIth cranial nerve and its innervation.