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The sound and the fly: Drosophila in biomedical research on human hearing 声音与苍蝇:人类听觉生物医学研究中的果蝇
Pub Date : 2012-05-18 DOI: 10.3109/1651386X.2012.685222
M. Capovilla
Deep biochemical and cellular similarities exist between Drosophila and vertebrates in the development and function of all organs. Despite their morphological differences, it is considered ever more likely that the hearing sensory cells have a common evolutionary origin and that consequently the molecular mechanisms that govern their functions are at least partially conserved. Thanks to transgenesis techniques and to the rich collection of mutant stocks well characterized from genetic, molecular and physiological points of view, Drosophila provides the means to test in vivo vertebrate gene functions in known metabolic and cellular pathways. In addition, the search for sequence homologies between human and Drosophila genes has allowed the identification of many disease genes in the human auditory system. These results indicate that Drosophila is a good model for the study of the genes that cause deafness in humans, and offers the possibility of using the numerous and sophisticated genetic tools of the insect to decipher how mechanical signals are elaborated by the auditory system. In this review, contributions to biomedical research from studies on Drosophila mutants are briefly summarized following a concise account on the fruitfly life cycle, tools and available resources. Particular attention is paid to studies on the genetic control of hearing mechanisms in the insect. The acquired knowledge will help confront some vertebrate hearing diseases on a molecular basis.
果蝇和脊椎动物在所有器官的发育和功能上存在着深刻的生化和细胞相似性。尽管它们在形态上存在差异,但更有可能的是,听觉感觉细胞有一个共同的进化起源,因此,控制它们功能的分子机制至少部分是保守的。由于转基因技术和从遗传、分子和生理角度充分表征的丰富突变体,果蝇提供了在已知代谢和细胞途径中测试体内脊椎动物基因功能的手段。此外,寻找人类和果蝇基因之间的序列同源性使得人类听觉系统中许多疾病基因的鉴定成为可能。这些结果表明,果蝇是研究导致人类耳聋的基因的一个很好的模型,并提供了利用昆虫大量和复杂的遗传工具来破译听觉系统如何阐述机械信号的可能性。在这篇综述中,简要总结了果蝇突变体研究对生物医学研究的贡献,并简要介绍了果蝇的生命周期、工具和可用资源。特别关注昆虫听觉机制的遗传控制研究。所获得的知识将有助于在分子基础上对抗一些脊椎动物听力疾病。
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引用次数: 0
What is the evidence that 1Hz rTMS positively affects chronic tinnitus? 1Hz rTMS对慢性耳鸣有积极影响的证据是什么?
Pub Date : 2012-05-18 DOI: 10.3109/1651386X.2012.684497
Natasha Phillips, Amr El Refaie
Objective: To investigate relevant long-term studies which assess the use of 1Hz repetitive transcranial magnetic stimulation (rTMS) in participants with chronic tinnitus and so determine its benefits as a possible treatment option. Study design: A systematic review was developed to conclude the research question. The inclusion involved double-blind randomized sham-controlled clinical trials conducted over six months using 1Hz rTMS. The search strategy included the use of databases such as Pubmed, Cochrane Library, OVID Medline, CINAHL, Google Scholar, University of Bristol MetaLib and the University of Bristol library catalogue. Results: Three suitable studies were obtained and fulfilled the inclusion criteria. The results indicated that rTMS appears to have a beneficial psychological and physiological long-term effect spanning six months, although a correlation between a particular participant trait and overall success has not been found due to high inter-subject variability. Conclusion: Despite the possible benefits of rTMS with chronic tinnitus, further research is still required to fully determine the long-term benefits of rTMS after six months and potentially standardize rTMS into an evidence-based tinnitus management tool.
目的:调查相关的长期研究,评估1Hz重复经颅磁刺激(rTMS)在慢性耳鸣患者中的应用,从而确定其作为一种可能的治疗选择的益处。研究设计:采用系统综述来总结研究问题。纳入包括使用1Hz rTMS进行的为期6个月的双盲随机假对照临床试验。搜索策略包括使用Pubmed、Cochrane Library、OVID Medline、CINAHL、Google Scholar、University of Bristol MetaLib和University of Bristol图书馆目录等数据库。结果:获得3项合适的研究,均符合纳入标准。结果表明,rTMS似乎具有持续6个月的有益心理和生理长期影响,尽管由于受试者之间的高度差异,尚未发现特定参与者特征与总体成功之间的相关性。结论:尽管rTMS对慢性耳鸣可能有益处,但仍需要进一步的研究来充分确定rTMS在6个月后的长期益处,并有可能将rTMS标准化为循证耳鸣管理工具。
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引用次数: 2
Spontaneous otoacoustic emissions in individuals with auditory neuropathy spectrum disorder 听神经病变谱障碍患者的自发性耳声发射
Pub Date : 2012-02-10 DOI: 10.3109/1651386X.2012.657776
V. Avilala, Dhanya Mohan, A. Barman
Objective: The objective of the study was to determine the prevalence of spontaneous otoacoustic emissions (SOAEs) in individuals with auditory neuropathy spectrum disorder (ANSD), and to examine the spectral distribution of the SOAE responses recorded compared with normal hearing individuals. Study design: SOAEs were recorded from 30 individuals with normal hearing and 30 individuals diagnosed as having auditory neuropathy spectrum disorder (ANSD). The recorded responses were analysed to determine the prevalence and spectral distribution of the SOAE responses from both the groups. Results and discussion: The prevalence of SOAEs in individuals with ANSD was higher than for normal hearing individuals; however, the difference was not statistically significant. Individuals with ANSD also exhibited a greater number multiple SOAEs than their normal counterparts, which is statistically significant. The SOAEs recorded from individuals with ANSD were mostly located in the lower frequency region, i.e. below 1500 Hz. On the other hand, many of the normal hearing individuals exhibited SOAEs more at higher frequencies, i.e. above 1500 Hz. Conclusion: The findings of the present study suggest that individuals with ANSD exhibit subtle abnormalities in the cochlea or medial olivocochlear system (MOCS).
目的:本研究的目的是确定自发性耳声发射(SOAE)在听神经病变谱障碍(ANSD)患者中的患病率,并检查与正常听力个体相比,所记录的SOAE反应的频谱分布。研究设计:从30名听力正常的个体和30名被诊断为听觉神经病变谱系障碍(ANSD)的个体中记录soae。对记录的反应进行分析,以确定两组SOAE反应的患病率和频谱分布。结果与讨论:听力障碍患者的soae患病率高于听力正常人群;然而,差异无统计学意义。与正常个体相比,患有ANSD的个体也表现出更多的多重soae,这在统计学上是显著的。从ANSD个体记录的soae大多位于较低的频率区域,即1500 Hz以下。另一方面,许多听力正常的人在更高的频率(即1500 Hz以上)表现出更多的soae。结论:本研究结果表明,ANSD患者在耳蜗或内侧耳蜗系统(MOCS)表现出轻微的异常。
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引用次数: 4
The role of communication partners in the audiological enablement/rehabilitation of a person with hearing impairment: an overview 交流伙伴在听力障碍患者听力学启用/康复中的作用:综述
Pub Date : 2012-02-10 DOI: 10.3109/1651386X.2012.655914
Vinaya Manchaiah, D. Stephens, F. Zhao, S. Kramer
Objective: Hearing impairment is known to have various effects upon both the person with hearing impairment (PHI) and their communication partners (CPs). In addition, CPs are reported to play an important role in making the decision to seek a consultation and the acceptance of intervention by the PHI. The overall aim of this paper is to provide a comprehensive overview of the role of the CP in the audiological enablement/rehabilitation of the PHI keeping clinical practice in focus. Method: A literature review was conducted using a number of resources including electronic databases, books and websites. Results: An overview of the literature was presented in the following sections: 1) Factors influencing the audiological enablement/rehabilitation of the PHI; 2) Effect of the PHI's hearing impairment on their CPs; 3) CPs’ influence on their PHI's audiological enablement/rehabilitation; 4) Positive experiences reported by CPs of the PHI; 5) Models to represent CPs within the social network context of the PHI; and 6) CP involvement in the audiological enablement/rehabilitation. This paper also identifies gaps in the literature and provides recommendations for further research. Conclusion: It is clear that involvement of the CP in the audiological enablement/rehabilitation can result in mutual advantages for both the PHI and their CPs.
目的:听力障碍对听障者(PHI)和他们的交流伙伴(CPs)都有不同的影响。此外,据报道,CPs在决定寻求咨询和接受PHI干预方面发挥着重要作用。本文的总体目的是提供一个全面概述CP在PHI的听力学启用/康复中的作用,以保持临床实践的重点。方法:利用电子数据库、图书、网站等多种资源进行文献综述。结果:文献综述如下:1)影响腓骨外缘听力学使能/康复的因素;2) PHI听力障碍对其CPs的影响;3) CPs对PHI听力学使能/康复的影响;4) PHI的CPs报告的积极经验;5)在PHI的社会网络背景下表示CPs的模型;6) CP参与听力学使能/康复。本文还指出了文献中的差距,并为进一步研究提供了建议。结论:很明显,CP参与听力学使能/康复对PHI和它们的CP都有好处。
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引用次数: 35
A biophysical approach to the study of structure and function of connexin channel nanopores 连接蛋白通道纳米孔结构和功能研究的生物物理方法
Pub Date : 2012-02-10 DOI: 10.3109/1651386X.2012.658652
M. Bortolozzi, F. Mammano
Abstract Objective: Connexins are transmembrane proteins that form intercellular junctional channels in vertebrates and are known or suspected to be involved in a wide variety of biological processes including cardiac development and function, hearing, haematopoesis, regeneration, lens transparency, fertility, immune system function and protection from oxidative stress. Connexin mutations can cause developmental and physiological defects, and link to various diseases. In particular, defective permeation of cAMP or inositol-1,4,5-trisphosphate (InsP3) through connexin channels is associated with peripheral neuropathies and deafness, respectively. Here we present a method to estimate the permeability of single-gap junction channels to second messengers. Study design: Using HeLa cells that overexpressed wild-type human connexin 26 (HCx26wt) as a model system, we combined measurements of junctional conductance and fluorescence resonance energy transfer (FRET) emission ratio of biosensors selective for cAMP and InsP3. Results: The unitary permeabilities to cAMP (47 ± 15 × 10–3 μm3/s) and InsP3 (60 ± 12 × 10–3 μm3/s) were similar, but substantially larger than the unitary permeability to Lucifer Yellow (LY; 7 ± 3 × 10–3 μm3/s), an exogenous tracer. Conclusion: This method permits quantification of defects of metabolic coupling and can be used to investigate interdependence of intercellular diffusion and cross-talk between diverse signalling pathways.
目的:连接蛋白是一种跨膜蛋白,在脊椎动物中形成细胞间连接通道,已知或怀疑参与多种生物过程,包括心脏发育和功能、听力、造血、再生、晶状体透明性、生育、免疫系统功能和氧化应激保护。连接蛋白突变可引起发育和生理缺陷,并与多种疾病有关。特别是,cAMP或肌醇-1,4,5-三磷酸(InsP3)通过连接蛋白通道的渗透缺陷分别与周围神经病变和耳聋有关。本文提出了一种估算单间隙结通道对第二信使的磁导率的方法。研究设计:以过表达野生型人连接蛋白26 (HCx26wt)的HeLa细胞为模型系统,结合对cAMP和InsP3选择性生物传感器的结导和荧光共振能量转移(FRET)发射比的测量。结果:对cAMP(47±15 × 10-3 μm3/s)和InsP3(60±12 × 10-3 μm3/s)的单一渗透率相似,但明显大于对路西法黄(LY;7±3 × 10-3 μm3/s),外源示踪剂。结论:该方法可以量化代谢偶联的缺陷,并可用于研究不同信号通路之间细胞间扩散和串扰的相互依赖性。
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引用次数: 0
Local drug delivery for inner ear therapy 内耳局部给药治疗
Pub Date : 2012-02-10 DOI: 10.3109/1651386X.2012.657777
Enrique Perez, Dana R. Libman, T. R. Van De Water
This review of local drug delivery for inner ear therapy covers the topics of: noise-induced hearing loss (NIHL); vibration-induced hearing loss (VHL); cisplatin ototoxicity; aminoglycoside ototoxicity; and mechanical trauma-induced hearing loss that can occur during the process of cochlear implantation. The cellular, biochemical and molecular mechanisms involved in the causation of the hearing losses that result from exposure to these diverse traumas to the cochlea and its auditory sensory epithelium are explored as well as the efficacy of different drug therapies in their ability to either prevent or lessen the damage to the cochlear sensory epithelium and ameliorate the level of hearing loss. This review concludes with a section that explores future strategies for unique methods of drug delivery to the cochlea (e.g. biorelease from hydrogels via the round window membrane) and the development of novel inner ear therapies (e.g. short interfering ribonucleic acids, siRNAs) to conserve hearing against trauma associated losses and/or to restore hearing (e.g. stem cell therapy) following trauma-initiated losses of hair cells and hearing.
本文综述了内耳局部给药治疗的主题:噪声性听力损失(NIHL);振动性听力损失(VHL);顺铂耳毒性;氨基糖苷类耳毒性;机械性外伤性听力损失可能发生在人工耳蜗植入过程中。本文探讨了不同耳蜗及其听感觉上皮损伤导致听力损失的细胞、生化和分子机制,以及不同药物治疗在预防或减轻耳蜗感觉上皮损伤和改善听力损失水平方面的疗效。本综述的最后一节探讨了未来独特的耳蜗药物递送方法的策略(例如通过圆窗膜从水凝胶释放生物)和新型内耳疗法的发展(例如短干扰核糖核酸,sirna),以保护听力免受创伤相关损失和/或恢复听力(例如干细胞治疗)外伤引起的毛细胞和听力损失。
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引用次数: 0
Does reduced frequency selectivity in children with mild to moderate sensorineural hearing loss affect frequency discrimination in Mismatch Negativity and Pitch Pattern Sequence Tests? 轻度至中度感音神经性听力损失儿童的频率选择性降低是否影响错配阴性和音高模式序列测试的频率区分?
Pub Date : 2012-02-10 DOI: 10.3109/1651386X.2012.657339
M. Shabana, A. Shalaby, A. Dabbous, Abir Abd-El-Meneim Emara
Objective: Sensorineural hearing loss (SNHL) results in reduced sensitivity, abnormal growth of loudness, reduced frequency selectivity and reduced temporal resolution (1). Our aim was to study frequency discrimination abilities in children with SNHL using the psychophysical Pitch Pattern Sequence Test (PPST) and an electrophysiological measure, the Mismatch Negativity Test (MMN). Methods: This is a cross-sectional study that included 90 children, ages ranging from 6 - 12 years, divided into a study group of 60 children with mild to moderate SNHL and a control group of 30 normal hearing children. Both groups were subdivided into 3 subgroups according to age. Results: Subgroups of children with SNHL showed statistically significant poorer scores on the PPST than their well-matched controls. Although MMN can be elicited in children with mild to moderate degree of SNHL, its latency was prolonged. The older control subgroups did not show any statistically significant better scores on the PPST; MMN latencies were longer with increased duration of hearing loss. Degree of hearing loss, gender and side had no effect on PPST or MMN. There was no statistically significant correlation between the results of the PPST and MMN. Conclusion: SNHL affects frequency discrimination abilities demonstrated in poor scores on the PPST and prolonged MMN latency.
目的:感音神经性听力损失(SNHL)导致敏感性降低、响度异常增长、频率选择性降低和时间分辨率降低(1)。我们的目的是通过心理物理音高模式序列测试(PPST)和电生理测量失配阴性测试(MMN)来研究SNHL儿童的频率识别能力。方法:这是一项横断面研究,包括90名年龄在6 - 12岁之间的儿童,分为轻至中度SNHL儿童60名研究组和听力正常儿童30名对照组。两组患者按年龄又分为3个亚组。结果:SNHL儿童亚组在PPST上的得分低于匹配良好的对照组,具有统计学意义。轻至中度SNHL患儿虽可诱发MMN,但其潜伏期延长。年龄较大的对照组在PPST上的得分没有统计学上的显著提高;MMN潜伏期随着听力损失持续时间的增加而延长。听力损失程度、性别和侧耳侧对PPST和MMN无影响。PPST结果与MMN结果无统计学意义相关。结论:SNHL影响频率识别能力,表现为PPST评分差和MMN潜伏期延长。
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引用次数: 0
Giorgio Grisanti
Pub Date : 2011-12-01 DOI: 10.3109/1651386x.2011.628847
E. Arslan
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引用次数: 0
Does hearing aid experience alter un-aided auditory perception? 助听器经历是否改变了非辅助听觉感知?
Pub Date : 2011-11-14 DOI: 10.3109/1651386X.2011.625673
M. Shabana, A. Shalaby, A. Dabbous, Abir Abd-El-Meneim Emara
Abstract Objective: The aim of this study was to assess the ability of hearing aid (HA) experience to improve central auditory skills by making use of auditory plasticity. The aim would be achieved through studying the unaided auditory perception of a time compressed sentence test (TCST), patterning tests (pitch pattern test (PPT), duration pattern test (DPT)), and an auditory fusion test (AFT) in children with and without previous hearing aid usage. Study design: This was a cross-sectional study that included a study group of 19 children with mild to moderate sensorineural hearing loss (SNHL) having HA experience. Their ages ranged from six to 12 years and were compared to a control group of 27 well-matched children without previous hearing aid experience. Results: The children with SNHL having HA experience showed better scores than their controls in the psycho-physical tests, but this was statistically significant only on the PPT and the AFT at 500 Hz; at 4000Hz. The results were not significant for the DPT or TCST. PPT in both ears improved significantly with increased age of the children having HA experience; TCST at 60% in both ears and AFT at 4 kHz improved significantly with increased duration of HA use. AFT at 4 kHz improved significantly by early onset of HA use. Conclusion: HA experience results in improvement of un-aided auditory perception on some psycho-physical tests for children with mild to moderate SNHL, which emphasizes the need for early and regular use of amplification.
摘要目的:本研究旨在评估助听器(HA)体验利用听觉可塑性提高中枢听觉技能的能力。本研究通过对使用助听器和未使用助听器的儿童进行时间压缩句测试(TCST)、模式测试(音调模式测试(PPT)、持续时间模式测试(DPT))和听觉融合测试(AFT)的独立听觉感知进行研究。研究设计:这是一项横断面研究,包括19名有HA经历的轻度至中度感音神经性听力损失(SNHL)的儿童。他们的年龄从6岁到12岁不等,并与27名没有助听器经验的儿童组成的对照组进行了比较。结果:有HA经历的SNHL患儿在心理生理测试中得分高于对照组,但仅在500 Hz的PPT和AFT上有统计学意义;在4000赫兹。DPT和TCST的结果不显著。双耳PPT随HA经历儿童年龄的增加而显著改善;随着HA使用时间的延长,双耳60%的TCST和4 kHz的AFT显著改善。早期HA使用显著改善了4 kHz时的AFT。结论:HA经验可改善轻至中度SNHL儿童的一些心理生理测试的非辅助听觉,这强调了早期和定期使用扩音的必要性。
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引用次数: 0
Frequency of hearing loss in a series of rural communities of five developing countries located along the Silk Road 丝绸之路沿线五个发展中国家一系列农村社区的听力损失频率
Pub Date : 2011-11-14 DOI: 10.3109/1651386X.2011.616282
G. Girotto, N. Pirastu, A. Gasparini, P. D'Adamo, P. Gasparini
Abstract Objective: Hearing loss (HL) affects millions of people worldwide. While epidemiological data from Western countries are already available, this information is still lacking for many developing countries and rural communities. Here we report, for the first time, a study on the frequency of HL in a series of rural communities located along the Silk Road. Study design: Four hundred and ninety-six subjects (236 males and 260 females ranging from eight to 84 years of age) selected in non-random, convenience samples from rural communities belonging to Terra Madre Organization have been enrolled: 228 from Georgia and Azerbaijan (Caucasus region), 151 from Uzbekistan and Kazakhstan (Central Asia), and 117 from Pair (Tajikistan). Subjects underwent pure-tone audiometry at 0.25, 0.5, 1, 2, 4, 6, and 8 kHz; pure-tone average (PTA) values were determined. Results: The overall HL frequency, ranging from 9% to 18%, in the investigated communities located along the Silk Road is higher than that reported for WHO European region countries but comparable with that reported for WHO South-East Asian region countries and other developing countries. Interestingly, with regard to the impact of age, two different disease behaviours have been identified in all the tested communities – one for males and the other for females. In females HL starts at all PTAs between 30 and 40 years of age. In contrast, in males age starts to affect hearing at high frequencies in young adulthood (20–25 years old), but later (50–60 years old) at low and medium frequencies. Conclusion: Despite the difficulty in reaching rural communities, mainly located in remote places, and the need to perform further studies using a larger sample size, recent data provide new information and will contribute to a better definition of HL worldwide frequency.
摘要目的:听力损失(HL)影响着全球数百万人。虽然来自西方国家的流行病学数据已经可用,但许多发展中国家和农村社区仍然缺乏这方面的信息。本文首次对丝绸之路沿线一系列农村社区的HL发病率进行了研究。研究设计:从Terra Madre组织所属农村社区的非随机方便样本中选择了496名受试者(236名男性和260名女性,年龄从8岁到84岁不等):228名来自格鲁吉亚和阿塞拜疆(高加索地区),151名来自乌兹别克斯坦和哈萨克斯坦(中亚),117名来自Pair(塔吉克斯坦)。受试者在0.25、0.5、1、2、4、6和8 kHz进行纯音测听;测定纯音平均值(PTA)。结果:丝绸之路沿线调查社区的HL总体发病率为9% - 18%,高于世卫组织欧洲区域国家报告的发病率,但与世卫组织东南亚区域国家和其他发展中国家报告的发病率相当。有趣的是,关于年龄的影响,在所有接受测试的社区中发现了两种不同的疾病行为——一种是男性,另一种是女性。在女性中,HL开始于30至40岁之间的所有pta。相比之下,男性年龄在青年时期(20-25岁)开始影响高频听力,但在较晚时期(50-60岁)开始影响低频和中频听力。结论:尽管很难到达主要位于偏远地区的农村社区,并且需要使用更大的样本量进行进一步的研究,但最近的数据提供了新的信息,并将有助于更好地定义HL的全球频率。
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引用次数: 16
期刊
Audiological medicine
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