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中华耳科学杂志（英文版）

中华耳科学杂志（英文版）

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Journal of Otology
Journal of Otology is a newly created specialty journal dedicated to clinical and basic research issues in the field of otology. The journal is sponsored by the Chinese PLA General Hospital, a leading medical center in China in research and patient care, and managed by the Department of Otolaryngology at Chinese PLA General Hospital, based in Beijing, China. Designed to be a platform for clinicians and scientists involved in hearing and balance to exchange ideas and share their findings, our goal is to promote high-level research and continuous improvement in the care for patients with hearing and balance disorders.
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  • 发文量：419
  • 被引量：42
  • H指数：1
  • 开始收录时间：2006(1)
  • CNKI综合因子：0.434
  • 维普期刊影响因子： 0.49
  • 万方期刊影响因子：0.28
  • 创刊时间：2006
  • 国内刊号/CN：11-4883/R
    国际刊号/ISSN：1672-2930
  • 发行周期：半年刊
    邮发代号：-
  • 主管单位：解放军总医院
    主办单位：解放军总医院耳鼻咽喉科研究所
中华耳科学杂志（英文版）-联系信息
  • 主编：Shiming YANG
  • 电话：010-68286148
  • 邮箱：zhek301@163.com
  • 地址：北京复兴路28号
  • 邮编：100853
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中华耳科学杂志（英文版） >2023年第4期

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  • 摘要：目的 研究华东地区非综合征性大前庭导水管耳聋患者SLC26A4基因的突变情况,以进一步了解其突变谱,为阐明EVA的分子机制和指导基因诊断提供依据.方法 通过巢式聚合酶链式反应扩增目的片段和Sanger测序对135例明确诊断的散发非综合征性EVA耳聋患者SLC26A4基因的外显子及侧翼序列进行变异检测.结果在135例EVA耳聋患者中,明确为SLC26A4双等位基因突变致病的占74.07％(100/135),单等位基因突变的占7.41％(10/135),18.52％(25/135)的患者未检测到任何SLC26A4基因突变.共检出51种SLC26A4基因突变,其中,c.919-2A>G突变和p.H723R突变最常见,分别占总检出突变的50％(105/210)和10.48％(22/210).结论与既往报道一致,c.919-2A>G突变为中国人群SLC26A4基因的最常见变异.有相当一部分患者未检出SLC26A4双等位基因突变,提示可能有其他致病因子参与EVA的发生,有待进一步研究阐明.
  • 摘要：目的 探讨突发性耳聋患者的精神心理特点及相关因素分析.方法 用症状自评量表(self-reporting inventory 90,SCL-90)对1000例突发性耳聋患者的精神心理状态评分,然后与中国常模行对照研究.采用视觉模拟量表(visual analogue scale,VAS)对与突发性耳聋相关的耳鸣、眩晕、失眠、耳堵塞感和精力下降5项主观症状进行评分,将其评分和心理学评分行相关性分析.对突聋患者作组内比较,分析不同性别、年龄、病程、文化程度与精神心理障碍的关系.以SPSS 19.0软件对数据进行统计学分析.结果 突聋患者心理有轻度损害者716名(71.6％),中度损害者102名(10.2％).患者的抑郁(t=4.10,P=0.01)、人际关系敏感(t=4.46,P=0.00)、躯体化(t=4.21,P=0.00)、焦虑(t=3.03,P=0.02)和其他(睡眠、饮食,t=4.65,P=0.00)共5项因子高于中国常模.对5项主观症状的评分和心理学评分行相关性分析发现,耳鸣、耳堵塞感与人际关系敏感呈正相关趋势,失眠、耳堵塞感、精力下降与抑郁呈正相关趋势,耳鸣、失眠、精力下降与焦虑呈正相关趋势,眩晕、失眠、耳堵塞感与其他(睡眠、饮食)呈正相关趋势.突聋组SCL-90组内比较显示:女性的人际关系敏感(Z=-3.09,P=0.00)、抑郁(Z=-2.30,P=0.02)、焦虑(Z=-2.11,P=0.04)、其他(睡眠、饮食,Z=-2.82,P=0.01)评分高于男性;年龄更小者的敌对(Z=-2.27,P=0.01)和恐怖(Z=-2.08,P=0.04)评分更高;病程更长者的人际关系敏感(Z=-2.12,P=0.02)和焦虑(Z=-3.01,P=0.01)评分更高;学历更低者的偏执(Z=-2.25,P=0.02)评分更高.结论 突发性耳聋患者有轻度和中度的心理健康损害;人际关系敏感、抑郁、焦虑和其他(睡眠、饮食)随病情的加重而严重;女性、年龄更小者、病程更长者、学历更低者是相对的高危人群.
  • 摘要：目的探究自动听性脑干反应(automatic auditory brainstem response,AABR)及耳声发射(otoacoustic emisson,OAE)联合听力筛查在不同病情、胎龄新生儿中的临床意义.方法本研究选取2015年1月～2017年6月我院新生儿病区收治的846例新生儿作为研究对象,按其入院时的病情分为:危重新生儿组(危重新生儿有听损高危因素的416例)、普通新生儿组(普通性疾病排除听损高危因素的430例)、健康对照组(健康体检新生儿178例);另外按新生儿胎龄分为足月儿组(胎龄≥37周,体重≥2500g的402例)、早产儿组(胎龄<37周但>28周,体重<2500g的444例).不同组别的新生儿均在病情稳定情况下接受AABR、OAE检测,统计不同组别新生儿在三种筛查模式(仅测试AABR、仅测试OAE、联合测试AABR及OAE)下的听力筛查未通过率.结果危重新生儿组、普通新生儿组和正常新生儿组经OAE测试后的未通过率分别为11.78％、7.93％和6.87％,经AABR测试后的未通过率分别为12.38％、8.36％和7.03％,经OAE联合AABR测试后的未通过率分别为17.67％、13.45％和7.89％,OAE、AABR、OAE联合AABR的未通过率在每组之间比较,差异均有统计学意义(χ2=10.82,11.39,20.29,P均﹤0.05),危重新生儿组OAE、AABR、OAE联合AABR的未通过率均分别高于其他两组(OAE:与普通新生儿组比较χ2=7.16,P﹤0.05,与正常新生儿组比较x2=6.87,P﹤0.05;AABR:与普通新生儿组比较χ2=7.32,P﹤0.05,与正常新生儿组比较χ2=7.44,P﹤0.05;OAE联合AABR:与普通新生儿组比较χ2=5.62,P﹤0.05,与正常新生儿组比较χ2=19.07,P﹤0.05);危重新生儿、普通新生儿组的OAE、AABR、OAE联合AABR测试后的未通过率比较,差异均有统计学意义(危重新生儿组χ2=14.57,普通新生儿组χ2=18.46,P﹤0.05),且危重新生儿、普通新生儿组OAE联合AABR明显高于单独OAE测试和单独AABR测试(危重新生儿:与OAE比较χ2=11.49,P﹤0.05,与AABR比较χ2=9.11,P﹤0.05;普通新生儿:与OAE比较χ2=14.02,P﹤0.05,与AABR比较χ2=11.50,P﹤0.05);足月儿组、早产儿组经OAE测试后的未通过率分别为6.23％、10.59％,经AABR测试后的未通过率分别为13.64％、19.14％,经OAE联合AABR测试后的未通过率分别为15.64％、21.85％,OAE、AABR、OAE联合AABR的未通过率分别在两组之间比较,差异有统计学意义(χ2=5.17,4.56,5.24,P均﹤0.05),且早产儿组均高于足月儿组(P>0.05);每组的OAE、AABR、OAE联合AABR未通过率比较,差异均有统计学意义(足月儿组χ2=19.55,早产儿组χ2=21.56,P﹤0.05)),OAE联合AABR的未通过率均高于单独OAE、单独AABR,且差异具有统计学意义(足月儿组:与OAE比较χ2=18.43,P﹤0.05,与AABR比较χ2=3.13,P﹤0.05;早产儿组:与OAE比较χ2=20.72,P﹤0.05,与AABR比较χ2=2.95,P﹤0.05).结论OAE联合AABR筛查模式可对单一方法筛查模式进行有效补充,在危重新生儿及早产儿中的筛查未通过率较高,应积极在早产儿及危重新生儿群体中推广应用.
  • 摘要：分泌性中耳炎(otitis media with effusion,OME)是以中耳积液及听力下降为主要特征的疾病,同中耳的化脓性炎症不同的是其通常不伴有耳部急性感染症状.本病发病机制复杂,与咽鼓管功能障碍、感染及颅面部畸形等多种因素相关,治疗手段也因病因不同而方式多样.本文就分泌性中耳炎的发病机制及其治疗方式的新进展作一综述.
  • 摘要：目的了解新生儿听力损失的发病情况.方法回顾性分析了2013年至2014年在我院出生的3592例新生儿听力筛查的结果,其中包括正常足月儿2891例,早产儿166例,低体重儿61例,轻中度窒息儿69例,以及宫内窘迫儿405例,采用瞬态诱发性耳声发射筛查仪进行初复筛.对于复筛仍未通过者,再行耳声发射、声导抗、听性脑干反应(ABR)检查,进行听力学综合评估后确诊.结果出生后48～72小时接受初筛3592例,初筛率达到99.42％.初筛未通过人数457例,其中单侧未通过299例,双侧未通过158例,初筛未通过率平均13.26％.产后42 d应复筛人数为457例,实际复筛人数360例,实际复筛率78.77％,复筛未通过人数79例,复筛未通过率21.94％,正常足月儿听力初筛通过率为90.10％(2601/2891),早产儿为72.8％(121/166),低体重儿为75.4％(46/61),轻、中度窒息儿为69.5％(48/69),宫内窘迫儿为83.9％(340/405).确诊听力损失的患儿15例,正常足月儿共5例,占这类新生儿的0.17％,听力障碍高危因素儿(早产儿、低体重儿及轻、中度窒息儿、宫内窘迫儿)10例,占该类新生儿的1.43％.结论早产儿、低体重儿及轻、中度窒息儿、宫内窘迫儿听力初筛的通过率明显低于正常足月儿,且确诊为听力损失的患儿所占比例显著高于正常新生儿.
  • 摘要：2018年3月24日,首都医科大学附属北京儿童医院成功举办“京津冀儿童听力诊断中心2018年第一季度学术研讨会”。学术研讨会由首都医科大学附属北京儿童医院耳鼻咽喉头颈外科主任张杰教授致欢迎词,儿童听力诊疗中心主任刘海红教授担任主持。本次学术交流有两大鲜明特色,即主题报告专家阵容强大,亲自做学术报告的教授较多。来自北京市卫计委老年妇幼康复处的主任科员金英楠、北京妇幼保健院潘迎主任、北京市六家儿童听力诊断中心——首都医科大学附属北京儿童医院、解放军总医院、首都医科大学附属北京同仁医院、北京协和医院、北京大学第三医院、中国听力语言康复研究中心以及北京大学第一医院、北京市各区妇幼保健院、天津市妇女儿童保健中心、石家庄妇幼保健院、秦皇岛妇幼保健院的专家及团队等120余人参加了本次学术交流。
  • 摘要：Objective:Electrocochleography (ECochG) is valuable to diagnose Meniere's disease objectively. The aim of the present study was to provide preliminary normative data for ECochG among Asian adults. The influences of ethnicity (Malay versus Chinese) and gender on ECochG results were also studied. Methods:Twenty-two Malay adults (10 men and 12 women) and twenty Chinese participants (10 men and 10 women) aged between 20 and 49 years participated in this study. Extratympanic ECochG (ET-ECochG) was recorded according to standard non-invasive procedure. Summating potential (SP) amplitude, action potential (AP) amplitude and SP/AP ratio were analyzed accordingly. Results:ET-ECochG results were found to be comparable between left and right ears (p>0.05). No notable differences in ET-ECochG results were found between Malay and Chinese groups (p > 0.05). No significant influence of gender on ET-ECochG outcomes was also noted (p>0.05). The derived normative data for Asian adults (84 ears) are consistent with previous reports. Conclusion: The present study provides preliminary normative data for ET-ECochG among Asian adults. The ECochG components do not appear to be influenced by either ethnicity or gender. The derived normative data can be used for clinical applications and as the reference for future studies involving Asian population. Copyright ? 2017, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license.
  • 摘要：Aminoglycosides (AmAn) are widely used for their great efficiency against gram-negative bacterial infections. However, they can also induce ototoxic hearing loss, which has affected millions of people around the world. As previously reported, individuals bearing mitochondrial DNA mutations in the 12S rRNA gene, such as m.1555A>G and m.1494C>T, are more prone to AmAn-induced ototoxicity. These mutations cause human mitochondrial ribosomes to more closely resemble bacterial ribosomes and enable a stronger aminoglycoside interaction. Consequently, exposure to AmAn can induce or worsen hearing loss in these individuals. Furthermore, a wide range of severity and penetrance of hearing loss was observed among families carrying these mutations. Studies have revealed that these mitochondria mutations are the primary molecular mechanism of genetic susceptibility to AmAn ototoxicity, though nuclear modifier genes and mitochondrial haplotypes are known to modulate the phenotypic manifestation.
  • 摘要：Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations (right side, incomplete partition type I [IP-I];left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditory—vestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear. Copyright ? 2017, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license.
  • 摘要：Electrically evoked compound action potential (eCAP) amplitudes elicited at suprathreshold levels were assessed as a measure of the effectiveness of cochlear implant (CI) stimulation. Twenty-one individuals participated;one was excluded due to facial stimulation during eCAP testing. For each participant, eCAPs were elicited with stimulation from seven electrodes near the upper limit of the individual's electrical dynamic range. A reduced-channel CI program was created using those same seven electrodes, and participants performed a vowel discrimi-nation task. Consistent with previous reports, eCAP amplitudes varied across tested electrodes;the profiles were unique to each individual. In 6 subjects (30%), eCAP amplitude variability was partially explained by the impedance of the recording electrode. The remaining amplitude variability within subjects, and the variability observed across subjects could not be explained by recording electrode impedance. This implies that other underlying factors, such as variations in neural status across the array, are responsible. Across-site mean eCAP amplitude was significantly correlated with vowel discrimination scores (r2=0.56). A single eCAP amplitude measured from the middle of the array was also significantly correlated with vowel discrimination, but the correlation was weaker (r2=0.37), though not statistically different from the across-site mean. Normalizing each eCAP amplitude by its associated recording electrode impedance did not improve the correlation with vowel discrimination (r2=0.52). Further work is needed to assess whether combining eCAP amplitude with other measures of the electrode-neural interface and/or with more central measures of auditory function provides a more complete picture of auditory function in CI recipients.
  • 摘要：Introduction Types of paper Contributions falling into the following categories will be considered for publication: original research papers, reviews, letters to the editor.Please ensure that you select the appropriate article type from the list of options when making your submission.Authors contributing to special issues should ensure that they select the special issue article type from this list. Before You Begin Ethics in Publishing For information on Ethics in Publishing and Ethical guidelines for journal publication see http://www.elsevier.com/publishingethics and http://www.elsevier.com/ethical guidelines.
  • 摘要：Objective:To test the feasibility of measuring fine temporal bone structures using a newly established cone-beam computed tomography (CBCT) system. Materials and methods: Six formalin-fixed human cadaver temporal bones were imaged using a high-resolution CBCT system that has 900 frames and copper+aluminum filtration. Fine temporal bone structures, including those of the facial nerve canal and vestibular structures, were identified and measured. Results:The fine structures of the middle ear, including the tympanic membrane, tendon of the tensor tympani, cochleariform process of the semicanal of the tensor tympani, pyramidal eminence, footplate of the stapes, full path of the facial nerve within the temporal bone, supra-labyrinthine space, semicircular canals, pathway of the subarcuate canal, and full path of the vestibular aqueduct, were clearly demonstrated. The vestibular aqueduct has a midpoint width of 0.4 ± 0.0 mm and opercular width of 0.5 ± 0.1 mm (mean ± SD). The length of the internal acoustic meatus was 10.6 ± 1.2 mm (mean ± SD), and the diameter of the internal acoustic meatus was 3.7 ± 0.3 mm (mean ± SD). Conclusion:This novel high-resolution CBCT system has potentially broad applications in the diagnosis of inner ear disease and in monitoring associated pathological changes, surgical planning, navigation for the ear surgery, and temporal bone training. Copyright ? 2017, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license.
  • 摘要：The development and plasticity of central auditory system can be influenced by the change of peripheral neuronal activity. However, the molecular mechanism participating in the process remains elusive. Brain-derived neurotrophic factor (BDNF) binding with its functional re-ceptor tropomyosin receptor kinase B (TrkB) has multiple effects on neurons. Here we used a rat model of auditory deprivation by bilateral cochlear ablation, to investigate the changes in expression of BDNF and TrkB in the auditory cortex after auditory deprivation that occurred during the critical period for the development of central auditory system. Reverse transcription-quantitative polymerase chain reaction (RT-qPCR) and immunohistochemistry methods were adopted to detect the mRNA and protein expression levels of BDNF and TrkB in the auditory cortex at 2, 4, 6 and 8 weeks after surgery, respectively. The change in the expression of BDNF and TrkB mRNAs and proteins followed similar trend. In the bilateral cochlear ablation groups, the BDNF-TrkB expression level initially decreased at 2 weeks but increased at 4 weeks followed by the reduction at 6 and 8 weeks after cochlear removal, as compared to the age-matched sham control groups. In conclusion, the BDNF-TrkB signaling is involved in the plasticity of auditory cortex in an activity-dependent manner. Copyright ? 2017, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license.
  • 摘要：Hearing loss is one of the most frequent health issues in industrialized countries. The pathogenesis and molecular mechanisms of hearing loss are still unclear. Histone deacetylases (HDACs) are emerging as key enzymes in many physiological processes, including chromatin remodeling, regulation of transcription, DNA repair, metabolism, genome stability and protein secretion. Recent studies indicated that HDACs are associated with the development and progression of hearing loss. Dysfunction of HDACs could promote the oxidative stress and aging in the inner ear. In light of considering the current stagnation in the development of therapeutic options, the need for new strategies in the treatment of hearing loss has never been so pressing. In this review, we will summarize the reported literatures for HDACs in hearing loss and discuss how HDAC family members show different performances for the possibility of process of diseases development. The possibility of pharmacological intervention on hearing loss opens a novel path in the treatment of hearing loss.
  • 摘要：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.
  • 摘要：Introduction:Middle ear volume (MEV) is a clinically relevant parameter across middle ear diseases. MEV values between these techniques have never before been tested for agreement in ears with perforated tympanic membranes (TMs). Methods:Middle ears were identified from 36 patients ranging 18-89 years of age with TM perforations who underwent tympanometry and temporal bone computed tomography (CT) between 2005 and 2015. MEVs calculated by both tympanometry and three-dimensional volume reconstruction (3DVR) were analyzed for agreement using Bland Altman plots. The differences between tympanometric and 3DVR MEV values for each given middle ear were characterized across MEV quartiles (1=smallest;4=largest) and across increasing states of middle ear disease using Kruskal-Wallis and Wilcoxon testing with Bonferroni correction. Results: Bland Altman plots demonstrated significant disagreement between MEV measurement techniques. Differences between tympano-metric (T) and 3DVR MEV values were significantly greater with increasing average (i.e. (T+3DVR)/2)) MEV per linear regression (p < 0.0001). Significance was demonstrated between fourth and first average MEV quartiles (p = 0.0024), fourth and second quartiles (p=0.0024), third and first quartiles (p=0.0048), and third and second quartiles (p=0.048). Absolute MEV difference was not significantly different across varying states of middle ear disease (p=0.44). Conclusion:Statistically and clinically significant disagreement was demonstrated between tympanometric and 3DVR MEV values. Studies that vary in MEV estimation techniques may be expected to demonstrate significantly different results. These preliminary results suggest that cli-nicians should endeavor to seek further confirmation when interpreting high tympanometric MEV values.
  • 摘要：Introduction Types of paper Contributions falling into the following categories will be considered for publication: original research papers, reviews,letters to the editor. Please ensure that you select the appropriate article type from the list of options when making your submission.Authors contributing to special issues should ensure that they select the special issue article type from this list.Before You Begin Ethics in Publishing For information on Ethics in Publishing and Ethical guidelines for journal publication see http://www.elsevier.com/publishingethics and http://www.elsevier.com/ethical guidelines.Conflict of interest All authors are requested to disclose any actual or potential conflict of interest including any financial, personal or other relationships with other people or organizations within three years of beginning the submitted work that could inappropriately influence, or be perceived to influence, their work. See also http://www.elsevier.com/conflictsofinterest.
  • 摘要：Objective:Establish normative data for tympanic electrocochleography (TM ECochG) parameters in normal hearing adults without Ménière's disease's (MD) symptoms. Describe TM ECochG variables that help to distinguish normal from MD ears. Material and methods:We enrolled 100 subjects (N=200 ears), 59 females, aged between 19 and 71 years from 09/2010 to 04/2014. Inclusion criteria:normal otomicroscopy, hearing thresholds≤25 dB nHL from 250 to 4000 Hz, normal tympanogram, no symptoms of MD according to the AAO-HNS 1995 criteria and Gibson's score<7. We excluded subjects with dizziness, aural fullness or other symptoms of endolymphatic hydrops. The following parameters were analyzed: SP/AP amplitude ratio, SP/AP area ratio and the difference between AP latency with rarefaction and condensation stimuli. Results:There was no significant difference between right and left ears (Intraclass correlation coefficient<0.6). SP/AP amplitude ratio varied between 0.084 and 0.356 and SP/AP area ratio between 0.837 and 1.671 (percentiles 5 and 95). The AP latency difference to rarefaction and condensation clicks was between 0.0 and 0.333 ms. Conclusion:Normative data for TM ECochG parameters were established in 100 normal hearing subjects without MD. These data can be used to distinguish normal from pathological findings and in follow-up of MD patients.
  • 摘要：Alport syndrome is an X-linked syndrome that results in nephritis, renal failure, sensorineural hearing loss, and eye deficits. As a result of sensorineural hearing loss, these individuals are likely to experience difficulties in the area of speech and language. While studies in the past have examined the speech and language characteristics of children with syndromic sensorineural hearing loss, to our knowledge there are no previous studies to have documented the speech and language characteristics of these children on a long-term basis. The current study addresses this limitation by reporting speech, language, hearing, and function of twin brothers with X-linked Alport syndrome across a seven-year period. Information was collected by examining the medical records of the participants as well as through a verbal interview with the participants' guardian. Results revealed that the participants' hearing abilities gradually deteriorated over the seven-year period which affected their speech and language development as well. The kidney function tests revealed significant presence of hematuria (blood in the urine) as well as pro-teinuria (protein in the urine) suggesting chronic kidney dysfunction. This longitudinal study demonstrates the functional relationship between the kidneys and the cochlea, although they appear to be independent of one another. As individuals with Alport syndrome exhibit systemic complications, interdisciplinary collaboration is essential among health care providers including audiologists, speech-language pathologists, nephrologists, and ophthalmologist to promote evidence-based practice.
  • 摘要：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 miR-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.
  • 摘要：Middle ear surgery is usually performed using a surgical microscope. Initially, in otorhinolaryngology practice, endoscopes were used for paranasal sinus surgeries. It was only later that they were applied in the area of otology. In otologic surgeries, endoscopes were first used to visualize the middle ear, before being used to assist with visualization of instruments during cholesteatoma surgeries, although they are still not used alone in various otologic surgeries. As in other surgical fields, there is also a trend towards minimally invasive intervention in the field of otorhinolaryngology. Smaller incisions performed under the guidance of endoscopes are preferred over conventional large incisions. Using this approach, improved outcomes can be achieved and postoperative morbidities can be reduced. In addition, the outcomes of grafts performed using the endoscopic approach are similar to that achieved by the microscopic approach. Therefore, endoscopic ear surgery implementations are becoming increasingly popular.
  • 摘要：Objectives: To evaluate audiological and vestibular functions via basic audiological evaluation, otoacoustic emissions and videonystagmography (VNG) in vitiligo patients. Material and methods: 30 vitiligo patients (8 acrofacial and 22 mixed types) as the study group and 30 normal healthy adults as the control group were included in the study. All participants were submitted to basic audiological evaluation, transient and distortion product otoacoustic emmisions (TEOAEs and DPOAEs), vestibular assessment including history taking, office tests and videonystagmography (VNG) and dermatological assessment to determine type, percent of body surface area involvement and duration of vitiligo. Results: This study showed statistically significant difference between control and study groups in pure tone audiometry (PTA) and otoacoustic emissions results. Fifty percent of vitiligo patients had peripheral vestibular disorders (10 vestibular neuritis and 5 posterior canal benign paroxysmal positional vertigo. Conclusion: The results in this study showed that 50％ of vitiligo patients suffered from peripheral vestibular disorders in addition to auditory affection. Vitiligo patients require routine monitoring for auditory and vestibular functions for early identification and monitoring of changes as the disease progress.
  • 摘要：Many patients with sensorineural hearing loss have a precipitous high-frequency loss with relatively good thresholds in the low frequencies. This present paper briefly introduces and compares the basic principles of four types of frequency lowering algorithms with emphasis on nonlinear frequency compression (NLFC). A review of the effects of the NLFC algorithm on speech and music perception and sound quality appraisal is then provided. For vowel perception, it seems that the benefits provided by NLFC are limited, which are probably related to the parameter settings of the compression. For consonant perception, several studies have shown that NLFC provides improved perception of high-frequency consonants such as /s/ and /z/. However, a few other studies have demonstrated negative results in consonant perception. In terms of sentence recognition, persistent use of NLFC might provide improved performance. Compared to the conventional processing, NLFC does not alter the speech sound quality appraisal and music perception as long as the compression setting is not too aggressive. In the subsequent section, the relevant factors with regard to NLFC settings, time-course of acclimatization, listener characteristics, and perceptual tasks are discussed. Although the literature shows mixed results on the perceptual efficacy of NLFC, this technique improved certain aspects of speech understanding in certain hearing-impaired listeners. Little research is available on speech perception outcomes in languages other than English. More clinical data are needed to verify the perceptual efficacy of NLFC in patients with precipitous high-frequency hearing loss. Such knowledge will help guide clinical rehabilitation of those patients.
  • 摘要：Objectives: Recent studies have introduced middle ear volume (MEV) as a novel determinant of perforation-induced conductive hearing loss (CHL) in a mechanism driven by trans-tympanic membrane pressure differences. The primary aims of this preliminary report are to: 1) correlate CHL with perforation size; 2) describe the relationship between CHL and MEV; and 3) compare CHL across a range of cholesteatoma involvement. Design: A retrospective pilot study was performed in 31 subjects with audiometry indicative of conductive hearing loss, temporal bone CT scans, and no prior middle ear surgery. Perforation size and MEV were analyzed with respect to CHL in a cohort of 10 perforated ears with no cholesteatoma. CHLs were compared in 3 groups defined by extent of cholesteatoma involvement. Results: Ears with large and small perforations showed mean ABG values of 32.0 ± 15.7 dB and 16.0 ± 16.4 dB, respectively. A direct rela-tionship was observed between MEV and CHL for ears with large perforations across all frequencies, whereas this relationship for small per-forations was frequency-dependent. Finally, a statistically significant increase in CHL was found across ears with increasing cholesteatoma involvement at 1000 Hz (x2(2) = 9.786, p = 0.008), 2000 Hz (x2(2) = 8.455, p = 0.015), and 4000 Hz (x2(2) = 8.253, p = 0.016). Conclusions: These pilot data suggest that greater perforation-induced conductive hearing losses may be associated with larger perforation sizes and cholesteatoma. The correlation between MEV and CHL may require additional study.
  • 摘要：Aims: The goal of the present study is to summarize our experience on surgical management of retraction pockets (RP) as a preventive tool against cholesteatomas. Methods: Twenty-five ears have been followed up for a mean period of 6.16 ± 4.35 years (from 1 to 17 years). The sample presented a mean age of 47.56 ± 19.11 years (from 16 to 73 years). All patients underwent cartilage graft surgery. Furthermore 10 (40％) underwent tympanoplasty (TPL) type Ⅰ, 14 (56％) TPL type Ⅱ and 1 (4％) TPL type V. Results: Eleven ears (44％) showed cholesteatoma: all these cases were stage Ⅲ according to Charachon staging, and stage Ⅳ or Ⅴ according to Gersdorff classification. Twelve patients (48％) showed erosion of the ossicular chain. Of these, five were associated with cholesteatoma and seven only with retraction. The recurrence rate of cholesteatoma was 12％. None of the patients with a stage Ⅱ or Ⅲ RP (according to Gersdorff classification) developed cholesteatoma. The recurrence of RP was 0％. In regards to literature review, seven references were selected. These studies showed a success rate ranging from 79.1％ to 88％, while recurrences of RP varied from 6.4％ to 13％. Only one study specified a recurrence rate of cholesteatoma of 28％. Conclusions: Surgical treatment of stages Ⅱ and Ⅲ RP is an effective tool to prevent cholesteatoma formation. The presence of keratin accumulation and cholesteatoma at the RP (stages Ⅳ and Ⅴ, according to Gersdorff) are the real predictors of poor prognosis.
  • 摘要：Most adults with auditory processing disorder (APD) often have trouble at work. It is possible that these listening problems in adults with APD influence their mental health. Thus, we have to take the subject's personality and mental status into consideration when supporting subjects with APD. However, studies on APD in adults are comparatively rare. In this study, we aimed to evaluate the relationships between the results of auditory tests, APD symptoms, and psychological status in subjects with APD. This study included 22 adults who complained of listening problems in everyday life, and they underwent auditory processing tests and were questioned regarding their psychological characteristics. From the results of the auditory processing tests, all subjects showed low scores on at least one test; therefore, they were suspected of having APD. Especially, the scores were low on the auditory memory test. Furthermore, subjects with APD show relatively similar psychological characteristics to each other. However, there was no significant relationship between the results of the auditory processing tests and psycho-logical characteristics. We have to take notice of a subject's psychological state when they perceive their listening difficulties as a large-scale problem and feel anxious as a result. For this purpose, we should take psychological characteristics into consideration at the time of the medical examination interview before audio-psychological testing.
  • 摘要：This article reviews the effectiveness of intratympanic corticosteroids for vertigo control in Ménière's disease at 2-years follow-up according to the guidelines expressed by the American Academy of Otolaryngology-Head & Neck Surgery. Despite the increased use of intratympanic corticosteroids for vertigo control in Ménière's disease there is debate as to their effectiveness, particularly compared to gentamicin. Even so, after just a single course of injections, corticosteroids can reliably provide complete vertigo control (Class A) at 2-years in about 50％ of cases as indicated in a recent double-blind randomized controlled clinical trial (Patel et al., 2016). But the effectiveness of intratympanic corticosteroids truly increases when treatment is provided 'as-needed', whereby complete vertigo control is established in up to 91％ of cases. On the basis of available literature, there is good evidence to recommend the use of intratympanic steroid treatment for vertigo control in Ménière's disease, but patients must be monitored for non-response. The rationale for treating patients as-needed and the possible reasons for corticosteroid non-response are discussed.
  • 摘要：Objective: A common cavity deformity (CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studies paying attention to common cavity. Our group is engrossed in observing the improvement of auditory and verbal abilities in children who have received cochlear implantation (CI), and comparing these targets between children with common cavity and normal inner ear structure. Material and methods: A retrospective study was conducted in 12 patients with profound hearing loss that were divided into a common cavity group and a control group, six in each group matched in sex, age and time of implantation, based on inner ear structure. Categories of Auditory Performance (CAP) and speech intelligibility rating (SIR) scores and aided hearing thresholds were collected and compared between the two groups. All patients wore CI for more than 1 year at the Cochlear Center of Anhui Medical University from 2011 to 2015. Results: Postoperative CAP and SIR scores were higher than before operation in both groups (p < 0.05), although the scores were lower in the CCD group than in the control group (p < 0.05). The aided threshold was also lower in the control group than in the CCD group (p < 0.05). Conclusion: Even though audiological improvement in children with CCD was not as good as in those without CCD, CI provides benefits in auditory perception and communication skills in these children.

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