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听神经病的临床听力学表现及其影响因素
听神经病(auditory neuropathy,AN)的临床及研究越来越引起人们的关注,但由于AN的影像学检查常是阴性,这为其发病部位和机理的研究带来不少困难.所以,目前对AN的定义,命名及诊断仍基于听功能的表现.本文根据我们所做的研究工作结合文献报道,从病理生理学角度分析其听力学表现特征及其影响因素,以期对AN的发病部位和机理有进一步的认识.
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建立听神经病诊治标准势在必行
20世纪80年代,耳科医师和听力学家既已注意到临床上有些患者可听到声音但听觉理解能力异常为特征的听功能障碍性疾病[1].1996年Starr 等[2]首次将其命名为听神经病(auditory neuropathy).
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Objectives: Auditory neuropathy (AN) is a sensorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic emissions (OAEs). Many risk factors are thought to be involved in its etiology and pathophysiology. Three Chinese pedigrees with familial AN are presented herein to demonstrate involvement of genetic factors in AN etiology. Methods: Probands of the above - mentioned pedigrees, who had been diagnosed with AN, were evaluated and followed up in the Department of Otolaryngology Head and Neck Surgery, China PLA General Hospital. Their family members were studied and the pedigree diagrams were established. History of illness, physical examination,pure tone audiometry, acoustic reflex, ABRs and transient evoked and distortion- product otoacoustic emissions (TEOAEs and DPOAEs) were obtained from members of these families. DPOAE changes under the influence of contralateral sound stimuli were observed by presenting a set of continuous white noise to the non - recording ear to exam the function of auditory efferent system. Some subjects received vestibular caloric test, computed tomography (CT)scan of the temporal bone and electrocardiography (ECG) to exclude other possible neuropathy disorders. Results: In most affected subjects, hearing loss of various degrees and speech discrimination difficulties started at 10 to16 years of age. Their audiological evaluation showed absence of acoustic reflex and ABRs. As expected in AN, these subjects exhibited near normal cochlear outer hair cell function as shown in TEOAE & DPOAE recordings. Pure- tone audiometry revealed hearing loss ranging from mild to severe in these patients. Autosomal recessive inheritance patterns were observed in the three families. In Pedigree Ⅰ and Ⅱ, two affected brothers were found respectively, while in pedigree Ⅲ, 2 sisters were affected. All the patients were otherwise normal without evidence of peripheral neuropathy at the time of this writing. Conclusions: In this study, patients with feature of non- syndromic hereditary auditory neuropathy were identified in three Chinese families.Pedigree analysis indicates autosomal recessive inheritances in the pedigrees. The observed inheritance and clinical audiologic findings are different from those previously described for non-syndromic low-frequency sensorineural hearing loss. This information should facilitate future molecular candidate genes screening for understanding the mechanism of AN.
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Objective The main purpose of the present study was to describe the vestibular function in patients with auditory neuropathy (AN), and to assess their ability to maintain balance. Methods Vestibular function tests were performed on 32 patients with AN and 36 normal subjects including electronystagmopraphy(ENG) and static postrography(SPG). The results from the two groups were compared. Results Equilibrium function in patients with AN, was abnormal, compared to normal subjects. Conclusion Vestibular function tests, espe-cially static postrography, should be performed on patients with AN.
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听神经病谱系障碍儿童的听觉能力
通过对比听神经前反应(耳声发射/耳蜗微音电位)和神经反应(听性脑干反应)的结果来诊断小儿听神经病,已经是一件相对简单的事情.然而,如何评价受累小儿的听觉能力,并据此采取合适的干预策略仍然是一个重大挑战.
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听神经病谱系障碍个性化的处理
这次会议上大家达成共识,将听神经病/听觉同步不良更名为听神经病谱系障碍(ANSD),这是一种需要多专业合作才可以为患者提供更好服务的典型疾病.如果听力学家、言语语言病理学家、耳鼻咽喉科医生、神经科医生、早期干预工作者以及聋儿的特殊教育家都能够理解ANSD患者所感知的是独特的时间处理混乱、类似于静止的言语信号,那么在此基础上才能够达成很好的合作效果(Zeng et al,1999).虽然这些患者异常的听觉系统表现为ABR反应消失而耳声发射正常,其听觉系统的影响和发展轨迹有着很多不确定性,对于这些患者,我们的目标是"将他们培养成一名受过良好教育的纳税人",正像我的一位患者的父亲概括的那样.
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婴幼儿听神经病谱系障碍诊断及处理指南——婴幼儿听神经病诊断及处理指南研讨会制订(2008年,意大利,科莫)
"婴幼儿听神经病诊断及处理指南研讨会"是Yvonne Sininger博士在科罗拉多州儿童医院Bill Daniael儿童听力中心的儿童听力学进展讲座(Advances in Children's Hearing Lecture)的基础上发展而来的.讲座中Sininger博士阐述了关于听神经病诊断和处理方面的新进展,也讨论了关于这个复杂多变疾病存在的许多问题和争论.在这充满智慧、鼓舞人心的讲座之后,我和她开始考虑是否能召开一次国际性的研讨会,邀请各方面专家来分享信息,并希望能够总结出一个实用的指南,指导临床医生识别、诊断和处理患有该病的婴幼儿.
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儿童听神经病谱系障碍的处理:助听器
听性脑干反应(ABR)无反应,但对声音的行为反应又可记录到,两种结果互相矛盾的报告,发表已超过25年(Davis and Hirsch,1979;Worthington and Peters 1980;Kraus et al.,1984).但直到听力学发展到可以将它与感音神经性耳聋鉴别后,这种现称之为"听神经病/听觉同步不良(AN/AD)"的疾病才能够被确诊.20世纪90年代中期,Starr对10名听性脑干反应缺失或异常,而耳蜗微音电位和耳声发射反映耳蜗外毛细胞功能正常的患者进行了研究(Starr et al.,1996),患者年龄从4到49岁不等,诊断听觉障碍时并无神经系统问题,然而,其中8名陆续出现了周围神经病变,3名确诊为进行性神经性肌萎缩(Chareot Marie Tooth disease),因而创立了听神经病(AN)一词来描述这类归因于听神经病变的听力损失(Starr et al.,1996).Starr等报告后才引起人们对该病的注意.
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儿童听神经病谱系障碍的处理:人工耳蜗植入
本中心目前正致力于听神经病(AN)患者植入人工耳蜗的研究,研究对象为47名已植入了人工耳蜗的AN儿童,其中大部分(32例)为单侧植入,另外的15例为双侧植入.首两例植入者是双胞胎,病因均为Otoferlin基因的变异(Varga,2003).从1998年至2007年底,我们已完成了413例成人和210例儿童人工耳蜗植入手术.
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《婴幼儿听神经病谱系障碍诊断及处理指南》解读
2008年在意大利科莫举办的新生儿听力筛查国际会议上,就婴幼儿听神经病诊断及处理指南和相关热点问题召开了专题研讨会,会后出版了英文版的"Guidelines for Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder".2011年在北京听力论坛上,由中华医学会耳鼻咽喉头颈外科学分会听力学组组织国内外专家对该指南进行了充分讨论,会后组织完成了该指南的中文翻译,中文版<婴幼儿听神经病谱系障碍诊断及处理指南>已经发表于<听力学及言语疾病杂志>2012年第一期.该指南详细描述了对听神经病诊治认识的发展历史及存在的各种问题,对相关领域学者具有非常重要的指导意义.现就2011年北京听力论坛讨论的热点问题,对该指南进行解读分析如下.