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。     

一家系遗传性进行性感音神经性聋耳声发射测试分析

目的 :探讨耳声发射 ( OAE)测试对遗传性进行性感音神经性聋的诊断意义。方法 :对一家系 6代中 5 4例 ( 10 8耳 )和 5 0例正常人进行了纯音测听、瞬态诱发耳声发射 ( TEOAE)和畸变产物耳声发射 ( DPOAE)测试 ,了解其耳蜗功能。结果 :本家系纯音听阈 >2 0 d B HL组中 ,84%出现 TEOAE反应幅度下降或消失 ,82 %出现DPOAE振幅下降或消失 ;纯音听阈正常组中 ,75 %出现 TEOAE反应幅度下降 ,6 4%出现 DPOAE某个频率的振幅下降或缺失 ;对照组 TEOAE和 DPOAE的检出率均为 10 0 %。结论 :OAE测试有助于遗传性进行性感音神经性聋的早期诊断     

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

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.     

母系遗传性聋家系的纯音测听及畸变产物耳声发射测试的相关分析

目的：探讨母系遗传非综合征性聋的听力学特征及畸变产物耳声发射测试对其听改变早期诊断的优越性。方法：对六个母系遗传家系成员共102人进行纯音测听、DPOAE评估其听力，比较纯音测听与DPOAE检测耳蜗早期病变的灵敏性。结果：纯音测听为感音神经性聋的38例中，PTA≥45d B HL的18例，DPOAE反应缺失；PTA＜45dB HL的20例，DPOAE高频或高、中频振幅下降或缺失；纯音测听正常者中4例DPOAE显示高频或高中频振幅下降。结论：母系遗传非综合征性聋的听力损害为双侧、对称性、进行性感音神经性聋，早期表现高频损害，DPOAE可在纯音听阈改变之前，发现早期耳蜗轻微的病理变化，对早期诊断及遗传咨询有较大指导意义。     

热休克蛋白—70免疫印迹测定在免疫相关与非相关性内耳疾病的诊断意义

为评价热休克蛋白 - 70的 Western免疫印迹测定在自身免疫性内耳疾病诊断中的意义 ,该作者应用Western免疫印迹法对 5 3例特发性渐进性感音性听力损失、突发性耳聋和梅尼埃病病人血液中的热休克蛋白 - 70进行了测定 ,其中特发性渐进性感音性听力损失2 4例 ,突发性耳聋 19例 ,梅尼埃病 10例 ,治疗前抽取血液标本并进行全身检查、纯音测听及 MRI检查 ,对应用皮质激素无禁忌的 4 7例给予甲基强地松龙治疗 ,用量 1mg/ kg/天 ,逐渐减量至 2 1天。检测结果显示 ,Western免疫印迹测定仅在 5例 (9.4 % )病人检测到热休克蛋白 - 70抗体 ,其阳性和…     

机械纺织工人畸变产物耳声发射的临床研究

目的研究畸变产物耳声发射(distortion product otoacoustic emissions,DPOAE)在监测和早期发现噪声性聋(noise induced hearingloss,NIHL)方面的应用价值。方法检测160名(285耳)机械纺织工人(其中噪声性聋组125例224耳,有噪声接触史但纯音听阈正常者为对照组,共35例61耳)和75名听力正常人(正常对照组)的鼓室导抗图、镫骨肌反射、纯音听阈及DPOAE,比较三组的DPOAE幅值和引出率。结果①噪声性聋组DPOAE幅值及引出率均较正常对照组明显下降(P<0.05);②对照组与正常对照组比较,对照组的DPOAE幅值在4kHz处、引出率在3~6kHz处明显下降(P<0.05);③随着接触噪声工龄的延长,DPOAE引出率逐渐下降(P<0.05)。结论DPOAE可以客观地监测和早期发现噪声性聋,较纯音测听更有意义。     

听神经病患者的临床听力学特点

目的探讨听神经病患者的临床听力学特点。方法自2003年1月～2007年5月共收集到22例听神经病患者的病例。总结听神经病患者的病史、纯音测听、鼓室曲线图、镫骨肌声反射、言语测听、听性脑干反应（auditory brainstem response，ABR）、耳蜗电图（electrocochleogram，ECochG）、畸变产物耳声发射（distortion product otoacoustic emission，DPOAE）及对侧白噪声抑制试验特点。其中10例患者行颅脑核磁共振成像（magnetic resonance imaging，MRI）检查。结果男性患者9例，女性患者13例，发病年龄为10～20岁，表现为进行性双耳听力下降，7例患者伴有耳鸣，最突出的表现是言语识别率差，无噪声接触史和耳毒性药物应用史，无家族史。22例患者听力曲线包括上升型13例，平坦型7例，高频听力下降型2例；鼓室曲线图均为A型；镫骨肌声反射均消失。所有听神经病患者ABR不能引出；全部病例均可记录到DPOAE，但不能被对侧白噪声抑制。耳蜗电图显示17例患者-SP／AP〉0．4，5例患者动作电位（action potential，AP）消失或振幅很小，颅脑MRI检查无异常发现。结论听神经病患者听力学检查有以下共同特点：①双耳进行性听力下降，青少年发病，言语辨别能力甚差，听力损失程度不同，多为轻度到重度，少数为极重度，纯音听力大多为低频下降为主的感音神经性聋，一部分表现为平坦型、甚至高频下降型；②声导抗为“A”型鼓室曲线，镫骨肌声反射引不出；③DPOAE均可引出，耳声发射对侧抑制消失；④ABR引不出或严重异常；⑤影像学检查无任何阳性发现。听神经病的确定目前仍停留于功能性诊断，尚不能确定其病因、病变部位，详尽的听力学检查、典型的听力学表现有助干临床诊断。     

听神经病谱系障碍患者听力学特征随访研究

目的分析听神经病谱系障碍患者(auditory neuropathy spectrum,ANSD)首诊与复诊的听力学特征,预测ANSD患者临床听力学转归,并建立有效的随访方案。方法对1997年4月至2014年3月间确诊为ANSD的22例(40耳)患者进行随访,随访期1-17年,对比分析患者首诊及末次复诊的听力学特征。听力学检查包括:纯音测听、言语识别率测试(SDS)、畸变产物耳声发射测试(DPOAE),声导抗测试,听性脑干反应测试(ABR)。结果对比患者首诊与末次复诊听力学特点:1不同随访期组间两两比较,纯音听阈未见明显变化(P>0.05);2随访期1-3年组与随访期>7年组间言语识别率有统计学意义(P<0.05);3首诊患者的DPOAE均正常或大部分正常引出,复诊中低频DPOAE的引出率降低,其中随访期为1-3年的患者全频DPOAE引出率下降;4镫骨肌反射引出率由20%下降为5%;5ABR波形无明显变化,均为2耳(5%)引出V波。结论 1随着随访期的延长,ANSD患者言语识别率、DPOAE引出率、镫骨肌反射引出率下降,部分患者将表现出感音神经性耳聋的临床听力学特征。患者中主诉有言语识别障碍的,仍有可能为ANSD;2随访方案仍需继续完善,以更好地为患者提供帮助,并在一定程度上提高复诊率。     

听神经病谱系障碍患者的临床及听力学特征分析

目的探讨听神经病谱系障碍(auditory neuropathy spectrum disorder,ANSD)的临床和听力学特征。方法分析14例(22耳)ANSD患者的临床资料及听力学检查结果,14例均行声导抗测试、听性脑干反应(auditory brainstem re-sponse,ABR)测试及畸变产物耳声发射(distortion product otoacoustic emission,DPOAE)测试,9例行纯音听阈测试,6例行听性稳态反应(auditory steady-state response,ASSR)测试。5例行颞骨薄层CT检查,9例行MRI检查。结果 14例(22耳)患者中,双侧ANSD 8例,单侧ANSD 6例。9例(14耳)行纯音测听的患者听力图以低频损失为主呈上升型7耳(占50%),覆盆型2耳(占14.29%),平坦型1耳(占7.14%),下降型1耳(占7.14%),全聋型3耳(占21.43%)。低频组(250Hz、500Hz)的平均阈值较中(1k Hz、2k Hz)、高频组(4k Hz、8k Hz)明显升高。声导抗鼓室图均为A型,镫骨肌反射消失。11例(6例单侧ANSD,共16耳)ABR未引出;其余3例(共6耳)ABR严重异常。14例DPOAE均正常引出。6例(9耳)ASSR结果均为极重度聋。结论 ANSD的临床表现具有明显的个体差异,这种个体差异产生的原因目前尚无法确定。对疑似ANSD的感音神经性聋患者,应行全面的听力学检查以明确诊断,对确诊的患者应定期随访。     

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。     

Sudden hearing loss and autoimmune inner ear disease

This case report describes the audiologic and medical diagnostic evaluations, results, and treatment options in a patient with a classic presentation of immune-mediated sensorineural hearing loss, commonly called autoimmune inner ear disease (AIED). It reviews findings of the basic battery, immittance audiometry, transient otoacoustic emissions, and auditory brainstem response measures and medical findings over more than 2 years. AIED generally causes asymmetric bilateral sensorineural hearing loss with atypical configuration. Although hearing loss is generally fluctuant, the overall pattern is usually rapid progression, particularly in the absence of early medical intervention. Word recognition is usually disproportionately poor. In our case, otoacoustic emissions and auditory brainstem responses suggest both cochlear and retrocochlear involvement and may initially appear to be inconsistent with pure-tone thresholds. Audiologists must be familiar with AIED because early identification is critical. Additionally, an immunologic basis may be a factor in other disorders, including many cases of Meniere's disease.     

Auditory function in women with autoimmune inner ear diseases and their offspring

Objective

The precise cause of congenital sensorineural hearing loss (CSNHL) is unclear in many cases. In a previous study we found that offspring from guinea pigs with autoimmune sensorineural hearing loss (ASNHL) exhibited signs of SNHL. Here we studied women with autoimmune inner ear diseases (AIED) and their offspring. Our aim was to determine if autoimmune damage may be one of the causes of CSNHL.

Methods

Thirty-eight pregnant women with AIED were recruited. Thirty-three had ASNHL; one with autoimmune delayed endolymphatic hydrops (ADEH) and four with autoimmune Meniere's disease (AIMD). The following were assessed in all women: audiogram, auditory brain stem response (ABR), otoacoustic emission (OAE), vestibular function test and presence of inner ear antigens. The following were assessed in offspring from these women: OAE, ABR and presence of inner ear antigens.

Results

Five of the 38 children born to women with AIED had SNHL (an incidence much higher than normal). OAEs were not inducible in these children shortly after birth or within 46-100 days after birth. Abnormal ABR findings were apparent in these five children and inner ear antigens were detected in three of the five children (the mother's of these children were also positive for inner ear antigens).

Conclusions

These preliminary findings suggest that the prevalence of congenital ASNHL may be increased in offspring born to women with AIED.     

实验性自身免疫性内耳病耳蜗热休克蛋白70的表达

目的　探讨自身免疫性内耳病模型动物耳蜗热休克蛋白的表达。方法　利用同种异体内耳抗原免疫豚鼠 ,以建立自身免疫性内耳病 (autoimmuneinnereardisease ,AIED)动物模型 ,并应用免疫组织化学及原位杂位技术 ,研究热休克蛋白 (heatshockprotein ,hsp) 70在正常对照组及AIED模型动物实验组耳蜗中的表达情况。结果　对照组豚鼠螺旋神经节细胞中存在hsp70样蛋白基础表达 ;实验组 2 8耳中 10耳 (35 7% )听阈提高≥ 10dB ;此组动物螺旋神经节细胞和血管纹、螺旋韧带hsp70及其mRNA表达明显增强。结论　以粗制膜迷路抗原免疫豚鼠 ,听阈提高动物hsp70在螺旋神经节细胞和血管纹、螺旋韧带合成增加。     

Validity of the Western blot immunoassay for heat shock protein-70 in associated and isolated immunorelated inner ear disease

OBJECTIVE: To assess the validity of the Western blot immunoassay for heat shock protein-70 (hsp-70) for diagnosis of autoimmune inner ear disease. STUDY DESIGN: Retrospective study of 53 patients affected by sudden deafness (n = 19), idiopathic progressive sensorineural hearing loss (n = 24), and Meniere's disease (n = 10) who were treated from 1995 to 1999. The clinical course and response to corticosteroid were evaluated. METHODS: A purified hsp-70 antigen from bovine kidney cell line was used for the Western blot immunoassay. RESULTS: Only five patients (9.4%) showed anti--hsp-70 antibodies: Two presented a sudden sensorineural hearing loss (sudden deafness group), two showed an idiopathic progressive sensorineural hearing loss (idiopathic progressive sensorineural hearing loss group), and one was affected by fluctuating hearing loss (Meniere's disease group). A systemic autoimmune condition was observed in 29.1% of patients with idiopathic progressive sensorineural hearing loss. CONCLUSIONS: The low sensitivity of Western blot immunoassay for patients affected by idiopathic progressive sensorineural hearing loss and Meniere's disease may result from either the long time elapsed from the hearing loss and vertigo to the initial examination or from the increased percentage of cases of systemic autoimmune disease present in patients with idiopathic progressive sensorineural hearing loss. More studies to detect the immune-mediated inner ear disease in Western blot immunoassay-negative patients are required.     

Expression of heat shock protein 70 in immune response of the guinea pig inner ear]

OBJECTIVE: To explore whether the immune response of the inner ear could induce heat shock protein (hsp) 70 in guinea pig cochlea. METHODS: A model of autoimmune inner ear disease (AIED) was established by systemically immunizing the guinea pig with the homologous crude inner ear antigen (CIEAg). The immunized cochleae and normal control cochleae were examined for the expression of hsp70 with techniques of immunohistochemistry and in situ hybridization. RESULTS: In the control animals, the expression of the hsp70-like protein appeared only in the spiral ganglion, whereas in the cochleae with CIEAg immunization, strong expression of the hsp70-like protein and its mRNA appeared in the spiral ganglion as well as in the stria vascularis and the spiral ligament. The hearing thresholds were significantly increased in 10 out of 28 cochleae (35.7%) with CIEAg immunization. CONCLUSION: The results suggest that the immune response of the inner ear can induce the expression of hsp70 in the guinea pig cochlea.     

Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies

Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58 kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing's syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68 kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss.     

Cochlear versus retrocochlear presbyacusis: clinical correlates

This study reviews audiologic findings on cases of hearing-impaired elderly adults who were matched on the basis of peripheral hearing loss and who were divided into cochlear and retrocochlear groups on the basis of auditory brain stem response (ABR) test results. ABR test results were compared to other audiologic test results that included: (1) word-recognition scores and performance-intensity functions of phonetically balanced word lists; and (2) performance-intensity functions of Synthetic Sentence Identification lists with ipsilateral competing messages. The results of this study indicate that the retrocochlear group performed less favorably than the cochlear group on speech measures known to be sensitive to a retrocochlear site of disorder. This study further emphasizes the complexity of presbyacusis by demonstrating that peripheral hearing loss, alone, cannot account for the totality of the auditory problems that are encountered by elderly listeners.     

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。     

Autoimmune mouse antibodies recognize multiple antigens proposed in human immune-mediated hearing loss.

HYPOTHESIS: Autoimmune diseased mice with hearing loss will have autoantibodies against the various cochlear antigens proposed in clinical autoimmune inner ear disease. BACKGROUND: Serum antibodies of patients with hearing loss recognize several proteins that are proposed as possible antigenic targets in the ear. This often leads to a clinical diagnosis of autoimmune inner ear disease, although it is not clear how these antibodies cause inner ear disease. Therefore, to better understand the relationship of autoantibodies and ear disease, an examination was made of serum autoantibodies in the MRL/MpJ-Fas(lpr) autoimmune mouse with hearing loss. Similar antibody patterns in the mouse would provide an animal model in which to investigate potential autoimmune mechanisms of this clinical ear disorder. METHODS: Sera from MRL/MpJ-Fas(lpr) autoimmune mice and normal C3H mice were tested by the enzyme-linked immunosorbent assay technique for reactivity against various reported cochlear antigens: heat shock protein 70 (bovine, human, bacterial), laminin, heparan sulfate proteoglycan, cardiolipin, and collagen types II and IV. RESULTS: The autoimmune mouse sera showed significantly greater antibody reactivity against all of the antigens when compared with normal mouse sera. CONCLUSIONS: Serum antibodies from autoimmune mice recognized several putative autoantigens reported for patients with hearing loss, suggesting that comparable antigen-antibody mechanisms might be operating. However, the recognition of multiple antigens did not identify any one as being the specific target in autoimmune hearing loss. The correlation of antibodies in the MRL/MpJ-Fas(lpr) autoimmune mouse and human studies indicates this animal model should aid further investigations into potential cochlear antigens in autoimmune hearing loss.