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| 1 | 隐性听力损失的一种新机制——短暂性听神经脱髓鞘显示文摘以往认为,听阈正常的人听觉处理能力是正常的(即使是从噪声暴露导致的暂时性阈移中恢复过来的听阈正常者),其所出现的听力缺陷问题被归因为中枢问题。但是,最新的动物和临床研究表明,中等强度的噪声暴露或者年龄因素能够导致一种新型的外周听力损失,即隐性听力损失(hidden hearing Loss,HHL)。 | 盛海斌 黄治物 Wan G Corfas G | 2017 | 听力学及言语疾病杂志2017，25，4： | 20 |
| 2 | 听力障碍筛查量表用于老年人群听力筛查分析显示文摘目的探讨中文版老年听力障碍筛查量表(the hearing handicap inventory for the elderly-screening version,HHIE-S)用于老年人群听力筛查的可行性及实用性。方法随机选取840例年龄大于60周岁的老年人,先自行填写HHIE-S量表,然后在工作人员指导下再次填写,对比指导前后HHIE-S量表的得分,计算指导后量表的信度Cronbach'sα值,判断量表的可行性;所有填表者均进行0.5、1.0、2.0、4.0kHz纯音听阈测试,计算平均纯音听阈(PTA)值,比较筛查目标听力损失分别设定为PTA>25dB HL、PTA>40dB HL、PTA>60dB HL时,HHIE-S得分>8分与>10分及指导前后所填量表的灵敏度、特异度、阳性预测值、假阳性率及假阴性率,并根据ROC曲线及曲线下面积值(AUC),判断三种目标听力损失时量表的实用性。结果 840例老年人指导前后HHIE-S量表平均得分分别为7.6±10.6和10.8±16.7分(P<0.01),指导后量表的Cronbach'sα值为0.85;以HHIE-S>8分作为听力障碍判断标准时较HHIE-S>10分灵敏度高,假阴性率低(P<0.05);指导后且以HHIE-S>8分时PTA>25dB HL、PTA>40dB HL及PTA>60dB HL三种听力损失条件下ROC曲线下面积值分别为0.70±0.03、0.84±0.01及0.88±0.02。结论适当指导后,HHIE-S量表用于老年人听力筛查得分>8分即可认为存在听力障碍,目标听力损失为PTA>40dB HL时,HHIE-S量表的实用性强,汉化版HHIE-S量表可作为老年人群听力障碍有效的筛查方式。 | 胡娴亭 黄治物 陈建勇 梅玲 李蕴 任燕 唐职健 沈敏 张勤 吴皓 | 2014 | 听力学及言语疾病杂志2014，22，3： | 18 |
| 3 | 耳蜗突触病变和隐性听力损失显示文摘临床上常遇到这样一类患者,常规纯音听力测试显示其听阈正常,但存在一定程度的阈上听觉感知缺陷,如:嘈杂环境中言语分辨能力下降等,这种听觉功能的损伤被称为隐性听力损失(hidden hearing loss,HHL)。英国早在1989年就有一项大范围的调查研究显示,26%的成年人在嘈杂环境中对言语听觉有较大困难, | 盛海斌 黄治物 吴皓 | 2016 | 听力学及言语疾病杂志2016，24，6： | 12 |
| 4 | 线粒体DNA突变与遗传性聋显示文摘遗传性聋是临床上最常见的先天性疾病之一,目前,全世界范围内约有3.6亿耳聋患者,其在发达国家新生儿中的发病率约为3‰[1]。耳聋可以由基因突变和环境因素(包括耳毒性的氨基糖苷类抗生素的应用)造成。遗传性聋分为非综合征型聋(non-syndromic hearing loss,NSHL)和综合征型聋(syndromic hearing loss,SHL),线粒体DNA突变是造成遗传性聋的一个重要原因; | 王芳 刘星辰 郭玉芬 | 2016 | 听力学及言语疾病杂志2016，24，4： | 11 |
| 5 | Occupational Noise Exposure and Worker’s Health in China显示文摘The World Health Organization(WHO)released the World Report on Hearing on March 2,2021.The WHO estimated that by 2050,nearly 2.5 billion(1 in 4 people)would be living with some degree of hearing loss,and at least 700 million of whom would require rehabilitation services(1).The risk factors that cause hearing loss not only include occupational but also non-occupational factors,such as,high-level exposure of noise and ototoxic chemicals at workplaces,age. | Xin Sun | 2021 | China CDC weekly2021，3，18： | 11 |
| 6 | 突发性耳聋中医病因病机探析显示文摘1994年De Kleyn[1]给出了突发性耳聋(sudden sensorineural hearing loss,SSHL,以下简称突聋)的定义。突聋是指突然出现的单(双)侧感音神经性耳聋,甚至伴有耳鸣、耳内闷塞感、眩晕、恶心呕吐症状,可在3d以内发生,并至少出现连续3个频率上最少30dBd的听力损失,是耳鼻喉科常见疾病,任何年龄均可发病,其发病率约为2~20/10万人,实际发病率更高(这是由于部分突聋患者有自愈情况),高发年龄为50~60岁,男女比例为1:1,且单侧多于双侧。 | 刘琴 姚行齐 | 2016 | 中西医结合研究2016，8，4： | 11 |
| 7 | The potential of miR-183 family expression in inner ear for regeneration,treatment,diagnosis and prognosis of hearing loss显示文摘miRNA-183 family, in normal biology, is expressed in a harmonious and stable manner in the neurosensory organs and cells. Studies have also shown that miRNA-183 family, in different pathways, affects the neurosensory development, maintenance, survival and function. In addition, it has potential neuroprotective effects in response to neurosensory destructive stimulations. miRNA-96 mutation causes hereditary deafness in humans and mice, and therefore affects the inner ear activity and its maintenance. Certain roles have been identified for mi R-96 in the maintenance and function of the inner ear. The comparison of the target genes of family-183 in transcriptomes of newborn and adult hair cells shows that hundreds of target genes in this family may affect development and maintenance of the ears. Identifying the genes that are regulated by miRNA-183 family provides researchers with important information about the complex development and environmental regulation of the inner ear, and can offer new approaches to the maintenance and regeneration of hair cells and auditory nerve. | Mohammad-Reza Mahmoodian-sani Ameneh Mehri-Ghahfarrokhi | 2017 | Journal of Otology2017，12，2： | 11 |
| 8 | GJB2 mutation spectrum in deaf population in a typical southeastern area of China显示文摘Mutations in GJB2 gene are the most frequently found mutations in patients with nonsyndromic hearing impairment. However, the spectrum and prevalence of mutations in this gene vary among different ethnic groups. In China, 30,000 infants are born with congenital hearing impairment annually. In order to provide appropriate genetic testing and counseling to the families, we investigated the molecular etiology of nonsyndromic deafness in 103 unrelated school children attending Nantong School for the Deaf and Mute in Jiangsu Province, China. The coding exon of the GJB2 gene was PCR amplified and sequenced. Sixty two GJB2 mutant alleles were identified in 35.9% (37/103) of the patients. Twenty five patients carried two pathogenic mutations and 12 patients carried one mutant allele. The 235delC was the most common mutation accounting for 69.4% (43/62) of GJB2 mutant alleles. The GJB2 mutant alleles accounted for 30.1% (62/206) of all chromosomes responsible for nonsyndromic hearing impairment. Testing of the 3 most prevalent deleterious frame shift mutations in this cohort detected 100% of all GJB2 mutant alleles. These results demonstrate that an effective genetic testing of GJB2 gene for patients and families with nonsyndromic hearing impairment is possible. | DAI Pu1*, YOU Yi-wen2*, CUI Jing-hong2*, YU Fei1, HAN Bing 1, KANG Dong-yang1, YUAN Hui-jun1, HAN Dong-yi1, 1. Department of Otolaryngology, PLA General Hospital, Beijing, People’s Republic China, 100853 2. Department of Otolaryngology, Nantong University Affiliated Hospital, Nantong, Jiangsu Province, People’s Republic China, 226001 *Pu Dai, Yiwen You, Jinghong Cui contribute equally to this paper | 2006 | Journal of Otology2006，1，2： | 10 |
| 9 | Head-related transfer function database and its analyses显示文摘Based on the measurements from 52 Chinese subjects (26 males and 26 females), a high-spatial-resolution head-related transfer function (HRTF) database with corre- sponding anthropometric parameters is established. By using the database, cues relating to sound source localization, including interaural time difference (ITD), interaural level difference (ILD), and spectral features introduced by pinna, are analyzed. Moreover, the statistical relationship between ITD and anthropometric parameters is estimated. It is proved that the mean values of maximum ITD for male and female are significantly different, so are those for Chinese and western sub- jects. The difference in ITD is due to the difference in individual anthropometric parameters. It is further proved that the spectral features introduced by pinna strongly depend on individual; while at high frequencies (f≥ 5.5 kHz), HRTFs are left-right asymmetric. This work is instructive and helpful for the research on bin- aural hearing and applications on virtual auditory in future. | XIE BoSun ZHONG XiaoLi RAO Dan LIANG ZhiQiang | 2007 | Science China(Physics,Mechanics & Astronomy)2007，50，3： | 10 |
| 10 | 单侧聋患者人工耳蜗植入的进展显示文摘既往未将双耳非对称性听力损失(asymmetric hearing loss,AHL)的极端案例-单侧听力损失(unilateral hearing loss,UHL)或单侧聋(single side deafness,SSD)纳入人工耳蜗植入的适应症。近年来有人开始尝试为单侧聋伴有严重耳鸣的患者患耳植入人工耳蜗,结果发现不但可以有效地减轻甚至消除耳鸣,而且还发现适应后可以与健耳听力很好地整合,从而达到双耳聆听的效果。 | 银力(综述) 高珊仙(综述) 屠文河(综述) 曹永茂(综述) 平利川(综述) 龙墨(综述) 傅前杰(审校) 高志强(审校) | 2017 | 听力学及言语疾病杂志2017，25，2： | 10 |
| 11 | The Effect of PCDH15 Gene Variations on the Risk of Noise-induced Hearing Loss in a Chinese Population显示文摘导致噪音的听觉损失(NIHL ) 是在环境、基因的因素之间的相互作用引起的复杂疾病。这研究调查了在 protocadherin 的基因可变性是否联系了 15 (PCDH15 ) 在一张中国人口位于增加的危险性下面到 NIHL 的发展。结果证明与 rs11004085 的 TT 遗传型相比, CT/CC 遗传型与 NIHL 的增加的风险被联系[调整机会比率(或)= 2.64;95% 信心间隔(CI ) :1.14-6.11, P = 0.024 ] 。另外,在 rs11004085 和 rs978842 之间的重要相互作用基因变化和噪音暴露在高级暴露组被观察(P < 0.05 ) 。而且,当与噪音暴露的高水平结合了时,风险 haplotype TAGCC 被观察(P < 0.05 ) 。因此,我们的学习证实在 PCDH15 的基因变化在人修改危险性到 NIHL 开发。 | XU Xiang Rong WANG Jing Jing YANG Qiu Yue JIAO Jie HE Li Hua YU Shan Fa GU Gui Zhen CHEN Guo Shun ZHOU Wen Hui WU Hui LI Yan Hong ZHANG Huan Ling ZHANG Zeng Rui JIN Xian Ning | 2017 | Biomedical and Environmental Sciences2017，30，2： | 9 |
| 12 | OSBPL2-disrupted pigs recapitulate dual features of human hearing loss and hypercholesterolaemia显示文摘Oxysterol binding protein like 2(OSBPL2), an important regulator in cellular lipid metabolism and transport, was identified as a novel deafness-causal gene in our previous work. To resemble the phenotypic features of OSBPL2 mutation in animal models and elucidate the potential genotypephenotype associations, the OSBPL2-disrupted Bama miniature(BM) pig model was constructed using CRISPR/Cas9-mediated gene editing, somatic cell nuclear transfer(SCNT) and embryo transplantation approaches, and then subjected to phenotypic characterization of auditory function and serum lipid profiles. The OSBPL2-disrupted pigs displayed progressive hearing loss(HL) with degeneration/apoptosis of cochlea hair cells(HCs) and morphological abnormalities in HC stereocilia, as well as hypercholesterolaemia. High-fat diet(HFD) feeding aggravated the development of HL and led to more severe hypercholesterolaemia. The dual phenotypes of progressive HL and hypercholesterolaemia resembled in OSBPL2-disrupted pigs confirmed the implication of OSBPL2 mutation in nonsydromic hearing loss(NSHL) and contributed to the potential linkage between auditory dysfunction and dyslipidaemia/hypercholesterolaemia. | Jun Yao Huasha Zeng Min Zhang Qinjun Wei Ying Wang Haiyuan Yang Yajie Lu Rongfeng Li Qiang Xiong Lining Zhang Zhibin Chen Guangqian Xing Xin Cao Yifan Dai | 2019 | Journal of Genetics and Genomics2019，46，8： | 7 |
| 13 | Value of section plane,MPR,and 3D-CTVR techniques in the fine differential diagnosis of ossicular chain in the case of conductive hearing loss with intact tympanic membrane显示文摘Aim: To assess the quality of high-resolution CT section planes(HRCT), multi-planar reformation(MPR) and 3-dimensional volume rendered computer tomography(3D-CTVR) were here used in the fine differential diagnosis of ossicular chain in the case of conductive hearing loss with intact tympanic membrane.Methods: Here, 17 cases of otosclerosis and 22 cases of ossicular chain deformity were selected. All patients had normal external ear canals,intact tympanic membranes, conductive hearing loss, type A tympanograms, and negative Gelle's tests. The respective radiological reports of the status of the ossicles via 3 protocols were compared to surgical findings. The quantitative assessments of the representation of different segments of the ossicular chain were based on a 3-point scoring system.Results: MPR and CTVR imaging both showed the integrity of whole ossicular chain well. MPR and CTVR imaging were found to be superior to section planes with respect to showing the superstructure of the stapes and malformations(P > 0.05).Conclusion: CTVR and MPR imaging were found to be better able to show the whole ossicular chain in the conductive hearing loss with normal tympanic membranes. Furthermore, the use of these techniques can have profound contributive value in the differential diagnosis of otosclerosis and ossicular chain absence or malformation. | Yang Liu Feng Yang QiaoHui Lu Danheng Zhao | 2017 | Journal of Otology2017，12，2： | 7 |
| 14 | 线粒体DNA突变与遗传性耳聋显示文摘1概述
遗传性耳聋是指由于人类个体基因(包括核基因及线粒体基因)异常所致的耳聋。它可以分为非综合征型遗传性耳聋(non-syndromic hearing impair-ment)和综合征型遗传性耳聋(syndromic hearing im-pairment)两大类。综合征型遗传性耳聋通常为染色体结构或功能异常引起的一类同时伴有多种其他临床表现或疾病的耳聋,这类患者约占遗传性耳聋的30%。 | 张雪溪 张杰 陈敏 郝津生 杨扬 张亚梅 | 2015 | 中华耳科学杂志2015，13，3： | 7 |
| 15 | The genetic basis of deafness in populations of African descent显示文摘Hearing loss is the most common sensorineural disorder worldwide and is associated with more than1000 mutations in more than 90 genes. While mutations in genes such as GJB2(gap-junction protein β 2)and GJB6(gap-junction protein β 6) are highly prevalent in Caucasian, Asian, and Middle Eastern populations, they are rare in both native African populations and those of African descent. The objective of this paper is to review the current knowledge regarding the epidemiology and genetics of hearing loss in African populations with a focus on native sub-Saharan African populations. Environmental etiologies related to poor access to healthcare and perinatal care account for the majority of cases. Syndromic etiologies including Waardenburg, Pendred and Usher syndromes are uncommon causes of hearing loss in these populations. Of the non-syndromic causes, common mutations in GJB2 and GJB6 are rarely implicated in populations of African descent. Recent use of next-generation sequencing(NGS) has identified several candidate deafness genes in African populations from Nigeria and South Africa that are unique when compared to common causative mutations worldwide. Researchers also recently described a dominant mutation in MYO3α in an African American family with non-syndromic hearing loss. The use of NGS and specialized panels will aid in identifying rare and novel mutations in a more cost-and timeeffective manner. The identification of common hearing loss mutations in indigenous African populations will pave the way for translation into genetic deafness research in populations of African descent worldwide. | Jason R.Rudman Rosemary I.Kabahuma Sara E.Bressler Yong Feng Susan H.Blanton Denise Yan Xue-Zhong Liu | 2017 | Journal of Genetics and Genomics2017，44，6： | 7 |
| 16 | 新生儿及婴幼儿听力损失显示文摘随着新生儿听力筛查在世界范围内的开展,新生儿听力损失(hearing loss)已成为围产医学、儿科学、儿童保健及听力学界等极为关注的课题,现就国内外新生儿听力损失的发病状况、相关高危因素及其病因等内容加以回顾。 | 聂文英 吴汉荣 戚以胜 | 2004 | 中国妇幼保健2004，19，8X： | 6 |
| 17 | 神经纤维瘤病2型的研究显示文摘神经纤维瘤病2型(neurofibromatosis type 2,NF2)为常染色体显性遗传疾病,是由位于22号常染色体上的基因突变所造成,临床上以双侧听神经瘤为主要特征。本病临床少见,诊断和治疗较复杂,预后较差。本文主要介绍NF2的病因、临床表现、诊断及治疗的研究进展。 | 陈广理 陈沛 龚树生 | 2006 | 国际耳鼻咽喉头颈外科杂志2006，30，5： | 6 |
| 18 | MYO15A基因突变在非综合征型聋中的研究进展显示文摘耳聋分为遗传性聋和非遗传性聋,其中大约有一半以上为遗传性聋,非综合征型聋(nonsyndromic hearing impairment,NSHI)约占遗传性聋的70%,常染色体隐性非综合征型聋(ARNSHL)在NSHI中约占75%-85%,临床多表现为双耳学语前非进行性重度-极重度感音神经性聋。 | 任晓菲 朱姝 朱宝生 李云龙 | 2017 | 听力学及言语疾病杂志2017，25，4： | 6 |
| 19 | 我国听力障碍儿童康复救助策略显示文摘据第2次全国残疾人抽样调查显示,我国有0~6岁听力残疾儿童13.7万人,每年新增2.3万人[1]。 | 胡向阳 | 2012 | 中国医学文摘(耳鼻咽喉科学)2012，27，6： | 5 |
| 20 | Safety and outcomes of cochlear implantation in the elderly:A review of recent literature显示文摘Global demographic changes related to longevity are leading to increasing numbers of the elderly,for whom hearing loss is a significant cause of morbidity and disability.Once met with reticence,severely hearing impaired older adults are increasingly being considered for cochlear implantation(CI).Significant data indicate that CI in the elderly population is safe,well-tolerated,and effective.Risks from CI surgery and anesthesia are low and generally comparable to rates in other age groups.Outcomes studies regarding CI in older adults have shown excellent improvements to speech perception,quality of life,and even cognition.Overall,currently available data suggests that advanced age should not,in itself,be considered a barrier to implantation.This review paper will highlight selected articles from recent medical literature regarding the safety and efficacy of CI in the elderly population. | Zao Yang Maura Cosetti | 2016 | Journal of Otology2016，11，1： | 5 |
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