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Research 2024年第1期2023卷 721-733页

作者： Miao Liu Haiqing Zhang Zhichao Wang Tingting Mo Xuefeng Lai Yaling He Minghui Jiang Meian He Weijia Kong Tangchun Wu Xiaomin Zhang Department of Occupational and Environmental Health Ministry of Education Key Laboratory of Environment and Healthand State Key Laboratory of Environmental Health(Incubating)School of Public HealthTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubeiChina Department of Otorhinolaryngology Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubeiChina Department of Epidemiology and Biostatistics School of Public HealthTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubeiChina

Evidence available on the independent and combined associations of sleep duration,bedtime,and genetic predisposition with hearing loss was lacking.The present study included 15,827 participants from the Dongfeng–Tongji cohort study.Genetic risk was characterized by polygenic risk score(PRS)based on 37 genetic loci related to hearing loss.We conducted multivariate logistic regression models to assess the odds ratio(OR)for hearing loss with sleep duration and bedtime,as well as the joint association and interaction with PRS.Results showed that hearing loss was independently associated with sleeping≥9 h/night compared to the recommended 7 to9:00 p.***.to 10:00 p.***.compared to those with bedtime>10:00 p.***.to 11:00 p.m.,with estimated ORs of 1.25,1.27,and 1.16,respectively.

关键词： Middle hearing fen

Novel heterozygous USH1C mutation impacts hair cell mechanotransduction and causes progressive hearing loss

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Science Bulletin 2024年第2期69卷 167-172页

作者： Yanyan Jia Gaogan Jia Luo Guo Nan Song Yu-Meng Wang Luoying Jiang Yilai Shu Yan Chen Shujia Zhu Huawei Li Wenyan Li ENT Institute and Otorhinolaryngology Department of Eye&ENT Hospital State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain ScienceFudan UniversityShanghai 200031China Institutes of Biomedical Sciences Fudan UniversityShanghai 200032China NHC Key Laboratory of hearing Medicine(Fudan University) Shanghai 200031China The Institutes of Brain Science and the Collaborative Innovation Center for Brain Science Fudan UniversityShanghai 200032China Shanghai Center for Brain Science and Brain-Inspired Intelligence Technology Shanghai 201210China Institute of Neuroscience State Key Laboratory of NeuroscienceCAS Center for Excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghai 200031China Department of Cardiology and Institute for Developmental and Regenerative Cardiovascular MedicineXinhua Hospital Affiliated to Shanghai Jiaotong University School of MedicineShanghai 200092China

hearing loss is one of the most common sensory disorders worldwide,affecting approximately 466 million people,including 34 million children[1].Genetic mutations accounts for approximately 60%of inherited hearing loss cases[2,3].These genetic changes result in a wide variety of clinical manifestations,ranging from nonsyndromic hearing loss(NSHL)to over 400 syndromes involving hearing loss and from mild adult-onset hearing loss to profound congenital deafness,covering the entire spectrum of Mendelian inheritance.

关键词： mec hearing approximate

Impact of auditory stimulation nursing protocol on auditory response in cerebrovascular stroke patients

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Journal of Integrative Nursing 2024年第2期6卷 111-116页

作者： Mohammed ElSayed ZAKY Hanan Ahmed AL SEBAEE Nagat El Morsy IBRAHIEM Heba Ahmed MOHAMMED Mohammad Edrees MOHAMMAD Department of Medical Surgical Nursing Faculty of NursingCairo UniversityCairoEgypt Department of Neurology Faculty of MedicineCairo UniversityCairoEgypt

Objectives:The objective of the study was to explore the impact of auditory stimulation protocol on auditory response in cerebrovascular stroke(CVS)patients.Materials and Methods:A pre-posttest nonequivalent control group quasi-experimental study was conducted at Al Mainal Hospital at Al Kars Einy Hospital,Cairo University,among 60 CVS patients,and the sample was randomly assigned through a coin to allocate subjects to study or control group(30 for each group).Auditory stimulation techniques were applied three times per week for the study group for two consecutive weeks.Patients in the control group received only routine hospital care during the study period.The Glasgow Coma Scale(GCS)score for assessing the neurological function and level of arousal and the auditory subscale score of Western Neuro Sensory Stimulation Profile(WNSSP)for assessing auditory response were recorded and compared between the two groups before and after the intervention.Results:There were statistically significant differences between the study group and the control group regarding third reading of GCS score(11.5±0.3 vs.10.6±0.9,t=4.676,P=0.000).The scores of all auditory subscales of WNSSP in the study groups increased after implementing the auditory stimulation protocol(Phearing responses in individuals with CVS.

关键词： Auditory stimulation techniques hearing response nursing management stroke

Absence of Serpinb6a causes progressive hair cell apoptosis and hearing loss in mice

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Journal of Genetics and Genomics 2023年第2期50卷 122-125页

作者： Cheng Cheng Jieyu Qi Liyan Zhang He Li Jie Lu Siyu Li Zhong Zhang Yue Qiu Chen Zhang Lulu Jiang Chaorong Yu Xia Gao Phillip I.Bird Renjie Chai Department of Otolaryngology Head and Neck Surgery Affiliated Drum Tower Hospital of Nanjing University Medical SchoolJiangsu Provincial Key Medical Discipline(Laboratory)No.321 Zhongshan RoadNanjingJiangsu 210008China Research Institute of Otolaryngology No.321 Zhongshan RoadNanjingJiangsu 210008China State Key Laboratory of Bioelectronics School of Life Sciences and TechnologyJiangsu Province High-Tech Key Laboratory for Bio-Medical ResearchSoutheast UniversityNanjingJiangsu 210096China Department of Otolaryngology-Head and Neck Surgery First Affiliated Hospital of Wenzhou Medical UniversityWenzhouZhejiang 325015China Northern Jiangsu People’s Hospital Affiliated to Yangzhou University YangzhouJiangsu 225009China Beijing Key Laboratory of Neural Regeneration and Repair Capital Medical UniversityBeijing 100069China Suzhou Otovia Therapeutics Inc. SuzhouJiangsu 215021China Department of Biochemistry and Molecular Biology Monash Biomedicine Discovery InstituteMonash UniversityClaytonVictoria 3800Australia State Key Laboratory of Bioelectronics Department of Otolaryngology Head and Neck SurgeryZhongda HospitalSchool of Life Sciences and TechnologyAdvanced Institute for Life and HealthJiangsu Province High-Tech Key Laboratory for Bio-Medical ResearchSoutheast UniversityNanjingJiangsu 210009China Co-Innovation Center of Neuroregeneration Nantong UniversityNantongJiangsu 226001China Department of Otolaryngology Head and Neck Surgery Sichuan Provincial People’s HospitalUniversity of Electronic Science and Technology of ChinaChengduSichuan 610072China Institute for Stem Cell and Regeneration Chinese Academy of ScienceBeijing 100086China

hearing impairment constitutes a global public health problem with severe social,political,and economic consequences,and the World Health Organization estimates that about 466 million individuals globally have some degree of hearing loss.Genetic factors account for about half of all deafness cases.Currently,more than 100 genes are known to be involved in hearing loss,but the underlying molecular mechanisms behind these deafness genes have not been fully characterized.The main clinical treatment for deafness is hearing aids and cochlear implants,but their efficacy depends heavily on the quantity and quality of residual hair cells(HCs)and spiral ganglion neurons and has only limited auditory effects.

关键词： deafness auditory hearing

Preventing autosomal-dominant hearing loss in Bth mice with CRISPR/CasRx-based RNA editing

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Signal Transduction and Targeted Therapy 2022年第4期7卷 1258-1270页

作者： Ziwen Zheng Guo Li Chong Cui Fang Wang Xiaohan Wang Zhijiao Xu Huiping Guo Yuxin Chen Honghai Tang Daqi Wang Mingqian Huang Zheng-Yi Chen Xingxu Huang Huawei Li Geng-Lin Li Xiaoxiang Hu Yilai Shu ENT Institute and Department of Otorhinolaryngology Eye&ENT HospitalState Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain ScienceFudan UniversityShanghai 200031China Institutes of Biomedical Sciences Fudan UniversityShanghai 200032China NHC Key Laboratory of hearing Medicine(Fudan University) Shanghai 200032China State Key Laboratory of Agrobiotechnology China Agricultural UniversityBeijing 100193China College of Biological Sciences China Agricultural UniversityBeijing 100193China School of Life Science and Technology Southeast UniversityNanjing 210096China Department of Otolaryngology-Head and Neck Surgery Graduate Program in Speech and Hearing Bioscience and Technology and Program in NeuroscienceHarvard Medical SchoolBostonMA 02115USA Eaton-Peabody Laboratory Massachusetts Eye and Ear InfirmaryBostonMA 02114USA School of Life Science and Technology ShanghaiTech UniversityShanghai 200031China CAS Center for Excellence in Molecular Cell Science Shanghai Institute of Biochemistry and Cell BiologyChinese Academy of SciencesUniversity of Chinese Academy of SciencesShanghai 200031China Institutes of Brain Science and the Collaborative Innovation Center for Brain Science Fudan UniversityShanghai 200031China

CRISPR/RfxCas13d(CasRx)editing system can specifically and precisely cleave single-strand RNAs,which is a promising treatment for various disorders by downregulation of related gene expression.Here,we tested this RNA-editing approach on Beethoven(Bth)mice,an animal model for human DFNA36 due to a point mutation in Tmc1.We first screened 30 sgRNAs in cell cultures and found that CasRx with sgRNA3 reduced the Tmc1^(Bth)transcript by 90.8%,and the Tmc1 wild type transcript(Tmc1^(+))by 44.3%.We then injected a newly developed AAV vector(AAV-PHP.eB)based CasRx into the inner ears of neonatal Bth mice,and we found that Tmc1^(Bth)was reduced by 70.2%in 2 weeks with few off-target effects in the whole transcriptome.Consistently,we found improved hair cell survival,rescued hair bundle degeneration,and reduced mechanoelectrical transduction current.Importantly,the hearing performance,measured in both ABR and DPOAE thresholds,was improved significantly in all ages over 8 weeks.We,therefore,have validated the CRISPR/CasRx-based RNA editing strategy in treating autosomal-dominant hearing loss,paving way for its further application in many other hereditary diseases in hearing and beyond.

关键词： CRISPR/Cas editing hearing

A novel splicing variant in the TMC1 gene causes non-syndromic hearing loss in a Chinese family

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Chinese Medical Journal 2022年第21期135卷 2631-2633页

作者： Beiping Zeng Hongen Xu Yongan Tian Qianyu Lin Haifeng Feng Zhifeng Zhang Siqi Li Wenxue Tang BGI College and Henan Institute of Medical and Pharmaceutical Sciences Zhengzhou UniversityZhengzhouHenan 450052China Precision Medicine Center Academy of Medical ScienceZhengzhou UniversityZhengzhouHenan 450052China The Second Affiliated Hospital of Zhengzhou University ZhengzhouHenan 450014China Department of Otorhinolaryngology Head and Neck SurgeryThe First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan 450052China The Third People’s Hospital of Zhengzhou ZhengzhouHenan 450000China Department of Physiology and Neurobiology Academy of Medical ScienceZhengzhou UniversityZhengzhouHenan 450001China

To the Editor:hearing loss is the most common sensory disorder in humans.There is one case of congenital deafness among every 1000 newborns,and in 50%of cases,the deafness is hereditary.Deafness exhibits high genetic heterogeneity.To date,over 110 non-syndromic deafness genes have been identified(https://***/).Lots of those genes can cause both autosomaldominant hearing loss(ADNSHL)and autosomal-recessive non-syndromic hearing loss(ARNSHL)andTMC1(encoding the transmembrane channel-like 1)is one of them.TMC1(OMIM:606706)is a member of the TMC family located at 9q21.13.The protein contains 760 amino acids and has six transmembrane regions.TMC1 is expressed in the inner and outer hair cells of the cochlea.A TMC1 mutation was first shown to cause deafness in 2002.[1]The prevalence of TMC1 variants ranged from 3.4%(19/557)among Pakistani ARNSHL families to 8.1%(7/86)in Turkish families.To date,around 20 hearing loss families associated withTMC1 variants have been reported in China.

关键词： deafness congenital hearing

hearing loss and cognitive decline among older adults with atrial fibrillation:the SAGE-AF study

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Journal of Geriatric Cardiology 2020年第4期17卷 177-183页

作者： Wei-Jia WANG Darleen Lessard Hawa Abu David D.McManus Tanya Mailhot Jerry H.Gurwitz Robert J.Goldberg Jane Saczynski Division of Cardiovascular Medicine Department of MedicineUniversity of Massachusetts Medical SchoolWorcesterMAUSA Department of Population and Quantitative Health Sciences University of Massachusetts Medical SchoolWorcesterMAUSA Department of Pharmacy and Health System Sciences Northeastern UniversityBostonMAUSA Meyers Primary Care Institute University of Massachusetts Medical SchoolWorcesterMAUSA

Objective To examine the association between hearing loss and cognitive function cross-sectionally and prospectively among older adults with atrial fibrillation(AF).Methods Patients with AF≥65-year-old(n=1244)in the SAGE(Systematic Assessment of Geriatric Elements)-AF study were recruited from five internal medicine or cardiology clinics in Massachusetts and Georgia.hearing was assessed by a structured questionnaire at baseline.Cognitive function was assessed by Montreal Cognitive Assessment(MoCA)at baseline and one year.Cognitive impairment was defined as score≤23 on the MoCA.The associations between hearing loss and cognitive function were examined by multivariable adjusted logistic regression.Results Participants with hearing loss(n=451,36%)were older,more likely to be male,and have depressive symptoms than patients without hearing loss.At baseline,528(42%)participants were cognitively impaired.Individuals with hearing loss were significantly more likely to have cognitive impairment at baseline[adjusted odds ratio(OR)=1.37,95%confidence interval(CI):1.05–1.81].Among the 662 participants who did not have cognitive impairment at baseline and attended the one-year follow-up visit,106(16%)developed incident cognitive impairment.Individuals with,versus those without,hearing loss were significantly more likely to develop incident cognitive impairment at one year(adjusted OR=1.68,95%CI:1.07–2.64).Conclusions hearing loss is a prevalent but under-recognized factor associated with cognitive impairment in patients with AF.Assessment for hearing loss may be indicated among these patients to identify individuals at high-risk for adverse outcomes.

关键词： Atrial fibrillation Cognitive function hearing

New US Rules Promise to Unlock hearing Aid Availability

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Engineering 2022年第7期8卷 7-9页

作者： Mitch Leslie Senior Technology Writer

One day in late 2022 or early 2023,a customer somewhere in the United States will make history by purchasing the first pair of hearing aids sold over the counter(OTC).For more than 40 years,these devices have been available in the United States and many other countries mainly through audiologists or other hearing professionals.But updated regulations scheduled for enactment later this year by the Food and Drug Administration(FDA),the US agency that oversees drugs and medical devices,will allow retailers to sell hearing aids directly to consumers[1].

关键词： drugs Promise hearing

The Role of the Kurtosis Metric in Evaluating the Risk of Occupational hearing Loss Associated with Complex Noise—Zhejiang Province,China,2010−2019

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China CDC weekly 2021年第18期3卷 378-382页

作者： Meibian Zhang Xiangjing Gao Wei Qiu Xin Sun Weijiang Hu Occupational Health and Radiation Protection Institute Zhejiang Provincial Center for Disease Control and PreventionHangzhouZhejiangChina Auditory Research Laboratory State University of New York at PlattsburghPlattsburghNew YorkUSA National Institute of Occupational Health and Poisoning Control China Center for Disease Control and PreventionBeijingChina.

Summary What is already known about this topic?Occupational noise-induced hearing loss(NIHL)has been the second most common occupational disease in China.Noise energy is the main risk factor for occupational NIHL.Evidence shows the temporal structure of noise(as indicated by kurtosis metric)contribute to the development of NIHL.However,the role of the kurtosis metric in evaluating the risk of occupational NIHL associated with complex noise has been rarely reported.

关键词： China Occupational hearing

hearing evaluation after successful myringoplasty

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Journal of Otology 2017年第4期12卷 192-197页

作者： Mohammed Radef Dawood Otolaryngology Department College of Medicine Mustansiriyah University Baghdad Iraq

Objectives: To assess postoperative hearing level, and factors that may have influence hearing improvement after myringoplasty.Methods: Twenty six cases of successful myringoplasty were included in this prospective study. Patient parameters including age, gender, size and site of the perforation, mastoid status, and etiology were evaluated. hearing levels were assessed as the mean air conduction(AC), and airbone gap(ABG) at 500, 1000, and 2000 Hz, and their relation with aforementioned parameters were analyzed.Results: The mean AC hearing gain was 22.373 dB and mean ABG reduction was 20.733 dB. The maximum AC hearing gain was 25.93 dB for subtotal perforation and 26.24 dB for big central perforation, and the maximum ABG reduction was 25.63 dB for subtotal perforation and 24.20 for big central perforation. Mean AC hearing gain was 23.01 dB, 22.72 dB, and 21.39 dB for 500, 1000, and 2000 Hz, respectively, and mean ABG reduction was 21.52 dB, 20.79 dB, and 19.86 dB for 500, 1000, and 2000 Hz, respectively. Patient age, gender, mastoid status and etiology did not seem to have any bearing on postoperative hearing improvement.Conclusion: While patient parameters do not seem to correlate with hearing improvement following myringoplasty, the size and location of perforation appear to have an impact on postoperative hearing outcomes. Most hearing improvement appears to occur at 500 Hz.

关键词： Myringoplasty hearing loss Tympanic membrane