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Hearing Disorders: HELP
Articles by Jing Wang
Based on 19 articles published since 2010
(Why 19 articles?)
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Between 2010 and 2020, Jing Wang wrote the following 19 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Review Toward Cochlear Therapies. 2018

Wang, Jing / Puel, Jean-Luc. ·INSERM UMR 1051, Institute for Neurosciences of Montpellier , Montpellier , France ; and University of Montpellier, Montpellier , France. ·Physiol Rev · Pubmed #30156495.

ABSTRACT: Sensorineural hearing impairment is the most common sensory disorder and a major health and socio-economic issue in industrialized countries. It is primarily due to the degeneration of mechanosensory hair cells and spiral ganglion neurons in the cochlea via complex pathophysiological mechanisms. These occur following acute and/or chronic exposure to harmful extrinsic (e.g., ototoxic drugs, noise...) and intrinsic (e.g., aging, genetic) causative factors. No clinical therapies currently exist to rescue the dying sensorineural cells or regenerate these cells once lost. Recent studies have, however, provided renewed hope, with insights into the therapeutic targets allowing the prevention and treatment of ototoxic drug- and noise-induced, age-related hearing loss as well as cochlear cell degeneration. Moreover, genetic routes involving the replacement or corrective editing of mutant sequences or defected genes are showing promise, as are cell-replacement therapies to repair damaged cells for the future restoration of hearing in deaf people. This review begins by recapitulating our current understanding of the molecular pathways that underlie cochlear sensorineural damage, as well as the survival signaling pathways that can provide endogenous protection and tissue rescue. It then guides the reader through to the recent discoveries in pharmacological, gene and cell therapy research towards hearing protection and restoration as well as their potential clinical application.

2 Review MYH9-related disease: description of a large Chinese pedigree and a survey of reported mutations. 2014

Zhang, Shufang / Zhou, Xueya / Liu, Shengnan / Bai, Tingting / Zhang, Yingai / Wang, Jing / Wang, Shunlan / Zhang, Xuegong / Wang, Binbin. · ·Acta Haematol · Pubmed #24643058.

ABSTRACT: We describe a large four-generational Chinese pedigree segregating MYH9 -related disease caused by a V1516L mutation. The clinical findings supported previously established genotype-phenotype correlations, and also demonstrated interindividual variability of disease manifestations even within the same family. The same mutation was previously reported in another Chinese pedigree but resulting from a different DNA substitution. Analyzing the patterns of previously reported mutations revealed a limited spectrum of pathogenic variants. The implications of this finding are discussed.

3 Review Molecular and cellular mechanisms of loss of residual hearing after cochlear implantation. 2013

Jia, Huan / Wang, Jing / François, Florence / Uziel, Alain / Puel, Jean-Luc / Venail, Frédéric. ·INSERM, Institute for Neurosciences of Montpellier, University Montpellier I, Montpellier, France. ·Ann Otol Rhinol Laryngol · Pubmed #23472314.

ABSTRACT: OBJECTIVES: We describe the various molecular and cellular pathways that lead to early and delayed loss of residual hearing after cochlear implantation. METHODS: We performed a systematic review using the Medline database with the key words cochlear implant, residual hearing, inflammation, apoptosis, and necrosis. RESULTS: The mechanisms underlying the loss of residual hearing after cochlear implantation are multiple. Early hearing loss may be provoked by the surgical access to the inner ear spaces and by trauma caused by insertion of the electrode array. After the initial trauma, an acute inflammatory response promotes elevated levels of cytokines and reactive oxygen species, which in turn promote sensory cell loss by apoptosis, necrosis, and necrosis-like programmed cell death. Treatments that counteract such an inflammatory reaction, production of reactive oxygen species, and apoptosis are effective at preventing hair cell degeneration. However, delayed hearing loss appears to be a consequence of chronic inflammation with development of fibrotic tissue. The mechanisms that lead to fibrosis are poorly understood, and standard antiinflammatory drugs are insufficient for preventing its development. CONCLUSIONS: Cochlear implantation is followed by an inflammatory response involving several pathways that lead to either short-term or long-term sensory hair cell degeneration. Future studies should focus on revealing the precise molecular mechanisms induced by cochlear implantation to allow the discovery of new targets for the effective prevention and treatment of loss of residual hearing.

4 Review [The diversity of aging models]. 2012

Galas, Simon / Château, Marie-Thérèse / Pomiès, Pascal / Wang, Jing / Menardo, Julien / Puel, Jean-Luc / Hugnot, Jean-Philippe / Verdier, Jean-Michel / Devau, Gina. ·Université Montpellier 1, CNRS UMR 5237, équipe biotechnologies du vieillissement, 1919 route de Mende, 34293 Montpellier Cedex 5, France. simon.galas@univ-montp1.fr ·Med Sci (Paris) · Pubmed #22480654.

ABSTRACT: Most of the signalling pathways involved in aging regulation have been recently found well conserved at various levels throughout the evolution. Taking this into account, a diversity of model organisms, including worms, rodents, and lemurs as well, allows to address different questions: how to understand the interactions between genetic and environmental factors while challenging theories of aging, to preserve hearing integrity, to fight against senescence of neural stem cells, or to explore brain fitness from gene expression to cognitive and social behavior? Here are the main issues that can be considered, stressing the complementarities of the models. The differentiation of aging physiological aspects from those induced by age-related pathologies will also be specified. By emphasizing recent ability of technologies to promote new aging insights, we discuss towards a better understanding of mechanisms governing aging.

5 Article Petrous bone cholesteatoma: our experience of 51 patients with emphasis on cochlea preservation and use of endoscope. 2019

Gao, Zhen / Gao, Gang / Zhao, Wei-Dong / Jia, Xian-Hao / Yu, Jing / Dai, Chun-Fu / Chen, Bing / Chi, Fang-Lu / Wang, Jing / Yuan, Ya-Sheng. ·a Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital , Fudan University , Shanghai , People's Republic of China. · b NHC Key Laboratory of Hearing Medicine (Fudan University) , Shanghai , People's Republic of China. · c Department of Otolaryngology, Head and Neck Surgery , Second Affiliated Hospital of Nantong University , Nantong , People's Republic of China. ·Acta Otolaryngol · Pubmed #31050582.

ABSTRACT:

6 Article ROS-Induced Activation of DNA Damage Responses Drives Senescence-Like State in Postmitotic Cochlear Cells: Implication for Hearing Preservation. 2019

Benkafadar, Nesrine / François, Florence / Affortit, Corentin / Casas, François / Ceccato, Jean-Charles / Menardo, Julien / Venail, Frederic / Malfroy-Camine, Bernard / Puel, Jean-Luc / Wang, Jing. ·INSERM - UMR 1051, Institut des Neurosciences de Montpellier, 80 rue Augustin Fliche, 34295, Montpellier, France. · Université Montpellier, 34295, Montpellier, France. · INRA, UMR 866 Différenciation Cellulaire et Croissance, 34060, Montpellier, France. · MindSet Rx, Arlington, MA, USA. · INSERM - UMR 1051, Institut des Neurosciences de Montpellier, 80 rue Augustin Fliche, 34295, Montpellier, France. jing.wang@inserm.fr. · Université Montpellier, 34295, Montpellier, France. jing.wang@inserm.fr. ·Mol Neurobiol · Pubmed #30693443.

ABSTRACT: In our aging society, age-related hearing loss (ARHL) has become a major socioeconomic issue. Reactive oxygen species (ROS) may be one of the main causal factors of age-related cochlear cell degeneration. We examined whether ROS-induced DNA damage response drives cochlear cell senescence and contributes to ARHL from the cellular up to the system level. Our results revealed that sublethal concentrations of hydrogen peroxide (H

7 Article How body composition influences hearing status by mid-childhood and mid-life: The Longitudinal Study of Australian Children. 2018

Wang, Jing / Sung, Valerie / Lycett, Kate / Carew, Peter / Liu, Richard S / Grobler, Anneke / Zubrick, Stephen R / Olds, Tim / Wake, Melissa. ·Murdoch Children's Research Institute, Parkville, VIC, Australia. · Department of Paediatrics, The University of Melbourne, Parkville, VIC, Australia. · Department of General Medicine, Royal Children's Hospital, Parkville, VIC, Australia. · Department of Audiology and Speech Pathology, The University of Melbourne, Parkville, VIC, Australia. · Telethon Kids Institute, The University of Western Australia, West Perth, WA, Australia. · Centre for Child Health Research, The University of Western Australia, Perth, WA, Australia. · Alliance for Research in Exercise, Nutrition and Activity (ARENA), University of South Australia, Adelaide, SA, Australia. · Murdoch Children's Research Institute, Parkville, VIC, Australia. melissa.wake@mcri.edu.au. · Department of Paediatrics, The University of Melbourne, Parkville, VIC, Australia. melissa.wake@mcri.edu.au. · Department of Paediatrics & The Liggins Institute, The University of Auckland, Grafton, Auckland, New Zealand. melissa.wake@mcri.edu.au. ·Int J Obes (Lond) · Pubmed #30026592.

ABSTRACT: BACKGROUND: Hearing loss is a disabling condition whose prevalence rises with age. Obesity-a risk factor common to many non-communicable diseases-now appears to be implicated. We aimed to determine: (1) cross-sectional associations of body composition measures with hearing in mid-childhood and mid-life and (2) its longitudinal associations with 10-year body mass index (BMI) trajectories. METHODS: Design & Participants: There were 1481 11-12-year-old children and 1266 mothers in the population-based cross-sectional CheckPoint study nested within the Longitudinal Study of Australian Children (LSAC). Anthropometry (CheckPoint): BMI, fat/fat-free mass indices, waist-to-height ratio; LSAC wave 2-6-biennial measured BMI. Audiometry (CheckPoint): Mean hearing threshold across 1, 2 and 4 kHz; hearing loss (threshold > 15 dB HL, better ear). ANALYSIS: Latent class models identifying BMI trajectories; linear/logistic regression quantifying associations of body composition/trajectories with hearing threshold/loss. RESULTS: Measures of adiposity, but not fat-free mass, were cross-sectionally associated with hearing. Fat mass index predicted the hearing threshold and loss in children (β 0.6, 95% confidence interval (CI) 0.3-0.8, P < 0.001;, odds ratio (OR) 1.2, 95% CI 1.0-1.4, P = 0.05) and mothers (β 0.8, 95% CI 0.5-1.2, P < 0.001; OR 1.2, 95% CI 1.1-1.4, P = 0.003). Concurrent obesity (OR 1.5, 95% CI 1.1-2.1, P = 0.02) and waist-to-height ratio (WHtR) ≥ 0.6 (OR 1.6, 95% CI 1.2-2.3, P = 0.01) predicted maternal hearing, with similar but attenuated patterns in children. In longitudinal analyses, mothers', but not children's, BMI trajectories predicted hearing (OR for severely obese 3.0, 95% CI 1.4-6.6, P = 0.01). CONCLUSIONS: Concurrent adiposity and decade-long BMI trajectories showed small, but clear, associations with poor hearing in mid-life women, with emergent patterns by mid-childhood. This suggests that obesity may play a role in the rising global burden of hearing loss. Replication and mechanistic and body compositional studies could elucidate possible causal relationships.

8 Article Mechanisms of Hearing Loss in a Guinea Pig Model of Superior Semicircular Canal Dehiscence. 2018

Tong, Bu-Sheng / He, Zi-Yu / Ding, Chen-Ru / Yang, Juan-Mei / Wang, Jing / Han, Zhao / Huang, Yi-Bo / Gao, Na / Jia, Xian-Hao / Chi, Fang-Lu / Ren, Dong-Dong. ·ENT Institute and Otorhinolaryngology Department, Affiliated Eye and ENT Hospital, Fudan University, Shanghai, China. · Department of Otorhinolaryngology of the First Affiliated Hospital, Anhui Medical University, Hefei, China. · Shanghai Auditory Medical Center, Shanghai, China. · Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. ·Neural Plast · Pubmed #29853836.

ABSTRACT: Defective acoustic transmission in the cochlea is closely related with various auditory and vestibular symptoms. Among them, semicircular canal dehiscence (SCD) with a defective semicircular bone is typical. Currently, the pathogenesis of SCD is usually explained by the third window hypothesis; however, this hypothesis fails to explain the variability in the symptoms and signs experienced by superior SCD (SSCD) patients. We evaluated the mechanism of hearing loss in a guinea pig model of bony dehiscence with various sizes and locations along the superior semicircular canal. Auditory brainstem responses (ABRs) and laser Doppler velocimetry were used to measure hearing loss and vibration changes before and after fenestration, as well as after restorative patching. ABR thresholds at low frequencies (e.g., 1000 Hz) increased after fenestration and decreased back to the normal range after we repaired the defect. Energy leakage from the surgically introduced third window was detected in the range of 300-1500 Hz, accompanied by increased vibration at the umbo, stapes head, and the dehiscence site, while decreased vibration was observed at the round window membrane in the same frequency range. After the patching procedure, the deviant vibrations were recovered. The degree of postfenestration energy leakage was proportional to the size of fenestration and the proximity of the fenestration site to the oval window. These results suggest that the bony fenestration of the superior semicircular canal mimics the hearing loss pattern of patients with SSCD. The decrease in perilymph wave impedance likely accounts for the auditory changes.

9 Article Metabolic syndrome is associated with hearing loss among a middle-aged and older Chinese population: a cross-sectional study. 2018

Han, Xu / Wang, Zhichao / Wang, Jing / Li, Yaru / Hu, Hua / Hu, Yujuan / Zhao, Xueyan / Zhan, Yue / Yuan, Jing / Wei, Sheng / Liang, Yuan / Zhang, Xiaomin / Guo, Huan / Yang, Handong / Wu, Tangchun / Kong, Weijia / He, Meian. ·a Institute of Occupational Medicine and the Ministry of Education Key Lab of Environment and Health , School of Public Health, Huazhong University of Science and Technology , Wuhan , China. · b Department of Otorhinolaryngology , Union Hospital, Tongji Medical College, Huazhong University of Science and Technology , Wuhan , China. · c Dongfeng Central Hospital , Dongfeng Motor Corporation and Hubei University of Medicine , Shiyan , Hubei , China. ·Ann Med · Pubmed #29693425.

ABSTRACT: BACKGROUND: Although the association of metabolic syndrome (MetS) and hearing loss has been evaluated, findings are controversial. This study investigated this association in a Chinese population. METHODS: A cross-sectional study including a total of 18,824 middle-aged and older participants from the Dongfeng-Tongji Cohort study was conducted. Hearing loss was defined as the pure-tone average (PTA) of frequencies 0.5, 1.0, 2.0, and 4.0 kHz >25 decibels hearing level (dB HL) in the better ear and graded as mild (PTA 26-40 dB HL), moderate (PTA >40 to ≤60 dB HL), and severe (PTA >60 dB HL). MetS was defined according to the International Diabetes Foundation (IDF) criteria of 2005. Association analysis was performed by logistic regression. RESULTS: After adjustment for potential confounders, participants with MetS showed higher OR of hearing loss (OR, 1.11; 95% CI: 1.03-1.19). The MetS components including central obesity (OR, 1.07; 95% CI: 1.01-1.15) and hyperglycemia (OR, 1.12; 95% CI: 1.04-1.20) were also positively associated with hearing loss. Low HDL-C levels were also associated with higher OR of moderate/severe hearing loss (OR, 1.21; 95% CI: 1.07-1.36). CONCLUSIONS: The MetS, including its components central obesity, hyperglycemia, and low HDL-C levels were positively associated with hearing loss. Key messages Studies indicated that cardiovascular disease and diabetes might be risk factors of hearing loss. However, few efforts have been made to establish a direct relationship between metabolic syndrome and hearing loss, especially in Chinese population. In the present study, a cross-sectional design using data from the Dongfeng-Tongji Cohort study was conducted to assess the association between metabolic syndrome and hearing loss. The metabolic syndrome, as well as its components central obesity, hyperglycemia, and low HDL-C levels were positively associated with hearing loss.

10 Article Cross-sectional epidemiology of hearing loss in Australian children aged 11-12 years old and 25-year secular trends. 2018

Wang, Jing / le Clercq, Carlijn M P / Sung, Valerie / Carew, Peter / Liu, Richard S / Mensah, Fiona K / Burt, Rachel A / Gold, Lisa / Wake, Melissa. ·Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Victoria, Australia. · Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia. · Department of Otolaryngology, Erasmus University Medical Center, Rotterdam, The Netherlands. · Department of Audiology and Speech Pathology, The University of Melbourne, Melbourne, Victoria, Australia. · The HEARing Cooperative Research Centre, The University of Melbourne, Melbourne, Victoria, Australia. · School of Health and Social Development, Deakin University, Geelong, Australia. · Department of Paediatrics, The Liggins Institute, The University of Auckland, Auckland, New Zealand. ·Arch Dis Child · Pubmed #29386180.

ABSTRACT: OBJECTIVE: In a national study of Australian children aged 11-12 years old, we examined the (1) prevalence and characteristics of hearing loss, (2) its demographic risk factors and (3) evidence for secular increases since 1990. METHODS: This is a cross-sectional CheckPoint wave within the Longitudinal Study of Australian Children. 1485 children (49.8% retention; 49.7% boys) underwent air-conduction audiometry. Aim 1: hearing loss (≥16 decibels hearing level (dB HL)) was defined in four ways to enable prior/future comparisons: high Fletcher Index (mean of 1, 2 and 4 kHz; primary outcome relevant to speech perception), four-frequency (1, 2, 4 and 8 kHz), lower frequency (1 and 2 kHz) and higher frequency (4 and 8 kHz); aim 2: logistic regression of hearing loss by age, gender and disadvantage index; and aim 3: P for trend examining CheckPoint and reported prevalence in studies arranged by date since 1990. RESULTS: For high Fletcher Index, the prevalence of bilateral and unilateral hearing loss ≥16 dB HL was 9.3% and 13.3%, respectively. Slight losses (16-25 dB HL) were more prevalent than mild or greater (≥26 dB HL) losses (bilateral 8.5% vs 0.8%; unilateral 12.5% vs 0.9%), and lower frequency more prevalent than higher frequency losses (bilateral 11.0% vs 6.9%; unilateral 15.4% vs 11.5%). Demographic characteristics did not convincingly predict hearing loss. Prevalence of bilateral/unilateral lower and higher frequency losses ≥16 dB HL has risen since 1990 (all P for trend <0.001). CONCLUSIONS AND RELEVANCE: Childhood hearing loss is prevalent and has risen since 1990. Future research should investigate the causes, course and impact of these changes.

11 Article The potential dysfunction of otolith organs in patients after mumps infection. 2017

Zhou, Yu-Juan / Yu, Jing / Wu, Yong-Zhen / Tian, Liang / Han, Zhao / Wang, Jing / Chi, Fang-Lu. ·Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, Shanghai, China. · Shanghai Auditory Medical Center, Shanghai, China. · Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. ·PLoS One · Pubmed #28746415.

ABSTRACT: OBJECTIVE: To investigate the relationship between mumps and the extent of hearing impairment and otolith organ damage. METHODS: A total of 27 patients with unilateral hearing impairment following mumps were enrolled. The degrees of hearing loss and otolith organ damage were confirmed by audiometric and vestibular evoked myogenic potential [VEMP] tests. All the results were compared and analyzed using Stata 13.0 software for Windows. RESULTS: The VEMP thresholds of the affected ears were significantly higher than those of the unaffected ears in both tests (cervical VEMP [cVEMP] test and ocular VEMP [oVEMP] test; p = 0.000 and 0.001, respectively). The mean cVEMP and oVEMP threshold values of the affected ears with hearing impairment for ≤10 years were significantly lower than those of affected ears with hearing impairment for >10 years [p = 0.009 and 0.004, respectively]. CONCLUSIONS: Deafness resulting from mumps is usually profound and permanent, which indicates severe damage to the cochlea due to the disease. The functions of otolith organs in the vestibular system are also impaired. Over time, the function of the otolith organs or their neural pathway may suffer secondary damage.

12 Article Contrasting results of tests of peripheral vestibular function in patients with bilateral large vestibular aqueduct syndrome. 2017

Zhou, Yu-Juan / Wu, Yong-Zhen / Cong, Ning / Yu, Jing / Gu, Jun / Wang, Jing / Chi, Fang-Lu. ·Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, Shanghai, China; Shanghai Auditory Medical Center, Shanghai, China; Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. · Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, Shanghai, China; Shanghai Auditory Medical Center, Shanghai, China; Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. Electronic address: jingwang6437@163.com. · Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, Shanghai, China; Shanghai Auditory Medical Center, Shanghai, China; Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. Electronic address: chifanglu@126.com. ·Clin Neurophysiol · Pubmed #28667933.

ABSTRACT: OBJECTIVE: To analyze and summarize the effect of bilateral large vestibular aqueducts in peripheral vestibular organ function. METHODS: Eighteen patients with bilateral large vestibular aqueduct syndrome (LVAS; Study Group) and 18 healthy volunteers (Control Group) were investigated using audiometry, caloric test, sensory organization test (SOT), and vestibular-evoked myogenic potential (VEMP) tests. RESULTS: All 18 patients (36 ears) exhibited sensorineural hearing loss. For cervical VEMP (cVEMP), the Study Group showed lower thresholds (Study Group vs. CONTROL GROUP: 71.4vs. 75.3dBnHL; p=0.006), N1 latencies (24.1vs. 25.2ms; p=0.026) and shorter P1 (15.3vs. 16.6ms; p=0.003), and higher amplitudes (400.7vs. 247.2µV; p<0.001) than the Control Group. For ocular VEMP (oVEMP), the Study Group had lower thresholds (79.3vs. 81.8dBnHL; p=0.046) and higher amplitudes (40.6vs. 14.4µV; p<0.001) than the Control Group. Fourteen of 16 patients (87.5%) who completed caloric tests had abnormal results, and 10 of 18 patients (55.6%) exhibited abnormal results in SOTs. CONCLUSIONS: The hyperfunction of vestibular test in otolithic organs and the hypofunction of vestibular test in semicircular canals, as well as the dysfunction in the balance test were demonstrated in patients with LVAS. SIGNIFICANCE: Our findings can help clinicians gain a better understanding of the characteristics of vestibular organ function in patients with LVAS, which can facilitate optimal targeted treatment.

13 Article Reversible p53 inhibition prevents cisplatin ototoxicity without blocking chemotherapeutic efficacy. 2017

Benkafadar, Nesrine / Menardo, Julien / Bourien, Jérôme / Nouvian, Régis / François, Florence / Decaudin, Didier / Maiorano, Domenico / Puel, Jean-Luc / Wang, Jing. ·INSERM - UMR 1051, Institut des Neurosciences de Montpellier, Montpellier, France. · Université de Montpellier, Montpellier, France. · Laboratoire d'Investigation Pré -Clinique/Service d'Hématologie Clinique, Institut Curie, Paris, France. · CNRS - UPR1142, Institut de Génétique Humaine, Montpellier, France. · INSERM - UMR 1051, Institut des Neurosciences de Montpellier, Montpellier, France jing.wang@inserm.fr. ·EMBO Mol Med · Pubmed #27794029.

ABSTRACT: Cisplatin is a widely used chemotherapy drug, despite its significant ototoxic side effects. To date, the mechanism of cisplatin-induced ototoxicity remains unclear, and hearing preservation during cisplatin-based chemotherapy in patients is lacking. We found activation of the ATM-Chk2-p53 pathway to be a major determinant of cisplatin ototoxicity. However, prevention of cisplatin-induced ototoxicity is hampered by opposite effects of ATM activation upon sensory hair cells: promoting both outer hair cell death and inner hair cell survival. Encouragingly, however, genetic or pharmacological ablation of p53 substantially attenuated cochlear cell apoptosis, thus preserving hearing. Importantly, systemic administration of a p53 inhibitor in mice bearing patient-derived triple-negative breast cancer protected auditory function, without compromising the anti-tumor efficacy of cisplatin. Altogether, these findings highlight a novel and effective strategy for hearing protection in cisplatin-based chemotherapy.

14 Article Association of the C47T polymorphism in superoxide dismutase gene 2 with noise-induced hearing loss: a meta-analysis. 2017

Wang, Jing / Li, Jun / Peng, Kang / Fu, Zi-Ying / Tang, Jia / Yang, Ming-Jian / Chen, Qi-Cai. ·Central China Normal University, School of Life Sciences and Hubei Key Lab of Genetic Regulation and Integrative Biology, Wuhan, China; Dali University, School of Basic Medicine, Dali, China. Electronic address: wangjing1807@foxmail.com. · Dali University, School of Basic Medicine, Dali, China; Dali University, School of Clinical Medicine, Dali, China. · Central China Normal University, School of Life Sciences and Hubei Key Lab of Genetic Regulation and Integrative Biology, Wuhan, China. · Central China Normal University, School of Life Sciences and Hubei Key Lab of Genetic Regulation and Integrative Biology, Wuhan, China. Electronic address: chenqc@mail.ccnu.edu.cn. ·Braz J Otorhinolaryngol · Pubmed #27161188.

ABSTRACT: INTRODUCTION: Currently, there is limited information about the relationship between manganese superoxide dismutase (sod2) c47t polymorphism and susceptibility to noise-induced hearing loss (NIHL). OBJECTIVE: The aim of this meta-analysis was to clarify the association between SOD2 C47T polymorphism and NIHL. METHODS: A search in PubMed and Web of Science was performed to collect data. All full-text, English-written studies containing sufficient and complete case-and-control data about the relationship between SOD2 C47T polymorphism and NIHL were included. Three eligible studies, comprising 1094 subjects, were identified. pooled odds ratios (ORs) and 95% confidence intervals (CI) were calculated to evaluate the strength of the association between SOD2 C47T polymorphism and NIHL. RESULTS: No significant association between C47T polymorphism and risk of NIHL was found with the following combinations: T vs. C (OR=0.83; 95% CI=0.63-1.09); TT vs. CC (OR=0.49; 95% CI=0.22-1.09); CT vs. CC (OR=0.54; 95% CI=0.25-1.17); TT vs. CC+CT (OR=0.82; 95% CI=0.50-1.32); CC vs. TT+TC (OR=0.49; 95% CI=0.23-1.04). However, in subgroup analysis, a significant association was found for TT vs. CC+CT (OR=0.77; 95% CI=0.42-1.41) in the Chinese population. CONCLUSION: The present meta-analysis suggests that SOD2 C47T polymorphism is significantly associated with increased risk of NIHL in the Chinese population. Further large and well-designed studies are needed to confirm this association.

15 Article The hidden dysfunction of otolithic organs in patients with profound sensorineural hearing loss. 2016

Xu, Xin-Da / Ding, Chen-Ru / Yu, Jing / Han, Zhao / Gu, Jun / Gao, Na / Jia, Xian-Hao / Luo, Xu / Wang, Jing / Chi, Fang-Lu. ·Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, 83 Fenyang Road, Shanghai 200031, China; Shanghai Auditory Medical Center, Shanghai, China; Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. · Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, 83 Fenyang Road, Shanghai 200031, China; Shanghai Auditory Medical Center, Shanghai, China; Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. Electronic address: jingwang615@126.com. · Department of Otology and Skull Base Surgery, Eye Ear Nose & Throat Hospital, Fudan University, 83 Fenyang Road, Shanghai 200031, China; Shanghai Auditory Medical Center, Shanghai, China; Key Laboratory of Hearing Science, Ministry of Health, Shanghai, China. Electronic address: chifanglu@126.com. ·Hear Res · Pubmed #26520583.

ABSTRACT: OBJECTIVE: To define the profiles of ocular and cervical vestibular-evoked myogenic potentials (oVEMP and cVEMP, respectively) in patients with profound sensorineural hearing loss (PSHL). METHODS: Twenty-nine patients with PSHL and 20 healthy volunteers were investigated. The patients' medical records were collected and analyzed. The ACS-evoked oVEMPs and cVEMPs and caloric test were tested and analyzed. RESULTS: The oVEMP and cVEMP response rates in the patients with PSHL were 38.9% and 44.4%, respectively, and these values were significantly less than those in the healthy volunteers (both were 100%). Regarding the oVEMPs, significantly higher threshold (p < 0.001) and smaller amplitude (p = 0.022) were observed in the patients. Regarding the cVEMPs, a significant elevation in the threshold (p < 0.001) and a decrease in the amplitude (p = 0.024) were observed, and marked reductions in the P1 (p = 0.002) and N1 latencies (p = 0.001) were observed in the patients. Regarding the caloric test, the ratio of semicircular canal dysfunction in patients with PSHL was significantly higher than that in healthy volunteers (p < 0.001). However, neither the patients nor the doctors noticed balance problems or the loss of otolithic function in the summaries of the medical records of all 29 of the patients. CONCLUSION: The utricular and saccular dysfunction that can be concealed in patients with PSHL can be observed in oVEMPs and cVEMPs. Otolithic function should receive attention in the diagnosis and treatment of PSHL. VEMPs have special value for the observation of hidden dysfunctions of the otolithic organs of patients with PSHL.

16 Article Comprehensive molecular diagnosis of 67 Chinese Usher syndrome probands: high rate of ethnicity specific mutations in Chinese USH patients. 2015

Jiang, Lichun / Liang, Xiaofang / Li, Yumei / Wang, Jing / Zaneveld, Jacques Eric / Wang, Hui / Xu, Shan / Wang, Keqing / Wang, Binbin / Chen, Rui / Sui, Ruifang. ·Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. jiang.lichun@foxmail.com. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. jiang.lichun@foxmail.com. · Department of Ophthalmology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, 1 Shuai Fu Yuan, Beijing, 10073, China. xiaofangliang@aliyun.com. · Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. yumeil@bcm.edu. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. yumeil@bcm.edu. · Department of Medical Genetics, School of Basic Medical Sciences, Capital Medical University, Beijing, China. jingwang@ccmu.edu.cn. · Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. Zaneveld@bcm.edu. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. Zaneveld@bcm.edu. · Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. hw11@bcm.edu. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. hw11@bcm.edu. · Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. shanx@bcm.edu. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. shanx@bcm.edu. · Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. keqingw@bcm.edu. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. keqingw@bcm.edu. · Graduate School of Peking Union Medical College, Beijing, China. wbbahu@163.com. · National Research Institute for Family Planning, Beijing, China. wbbahu@163.com. · Human Genome Sequencing Center, Baylor College of Medicine, Houston, TX, USA. ruichen@bcm.edu. · Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, 77030, USA. ruichen@bcm.edu. · Structural and Computational Biology and Molecular Biophysics Program, Baylor College of Medicine, Houston, TX, 77030, USA. ruichen@bcm.edu. · Program in Developmental Biology, Baylor College of Medicine, Houston, TX, 77030, USA. ruichen@bcm.edu. · Department of Ophthalmology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, 1 Shuai Fu Yuan, Beijing, 10073, China. hrfsui@163.com. ·Orphanet J Rare Dis · Pubmed #26338283.

ABSTRACT: BACKGROUND: Usher syndrome (USH) is the most common disease causing combined deafness and blindness. It is predominantly an autosomal recessive genetic disorder with occasionally digenic cases. Molecular diagnosis of USH patients is important for disease management. Few studies have tried to find the genetic cause of USH in Chinese patients. This study was designed to determine the mutation spectrum of Chinese USH patients. METHODS: We applied next generation sequencing to characterize the mutation spectrum in 67 independent Chinese families with at least one member diagnosed with USH. Blood was collected at Peking Union Medical College Hospital. This cohort is one of the largest USH cohorts reported. We utilized customized panel and whole exome sequencing, variant analysis, Sanger validation and segregation tests to find disease causing mutations in these families. RESULTS: We identified biallelic disease causing mutations in known USH genes in 70 % (49) of our patients. As has been previously reported, MYO7A is the most frequently mutated gene in our USH type I patients while USH2A is the most mutated gene in our USH type II patients. In addition, we identify mutations in CLRN1, DFNB31, GPR98 and PCDH15 for the first time in Chinese USH patients. Together, mutations in CLRN1, DNFB31, GPR98 and PCDH15 account for 11.4 % of disease in our cohort. Interestingly, although the spectrum of disease genes is quite similar between our Chinese patient cohort and other patient cohorts from different (and primarily Caucasian) ethnic backgrounds, the mutations themselves are dramatically different. In particular, 76 % (52/68) of alleles found in this study have never been previously reported. Interestingly, we observed a strong enrichment for severe protein truncating mutations expected to have severe functional consequence on the protein in USH II patients compared to the reported mutation spectrum in RP patients, who often carry partial protein truncating mutations. CONCLUSIONS: Our study provides the first comprehensive genetic characterization of a large collection of Chinese USH patients. Up to 90 % of USH patients have disease caused by mutations in known USH disease genes. By combining NGS-based molecular diagnosis and patient clinical information, a more accurate diagnosis, prognosis and personalized treatment of USH patients can be achieved.

17 Article Mutations in apoptosis-inducing factor cause X-linked recessive auditory neuropathy spectrum disorder. 2015

Zong, Liang / Guan, Jing / Ealy, Megan / Zhang, Qiujing / Wang, Dayong / Wang, Hongyang / Zhao, Yali / Shen, Zhirong / Campbell, Colleen A / Wang, Fengchao / Yang, Ju / Sun, Wei / Lan, Lan / Ding, Dalian / Xie, Linyi / Qi, Yue / Lou, Xin / Huang, Xusheng / Shi, Qiang / Chang, Suhua / Xiong, Wenping / Yin, Zifang / Yu, Ning / Zhao, Hui / Wang, Jun / Wang, Jing / Salvi, Richard J / Petit, Christine / Smith, Richard J H / Wang, Qiuju. ·Department of Otolaryngology-Head and Neck Surgery, Institute of Otolaryngology, PLA General Hospital, Beijing, China. · Molecular Otolaryngology and Renal Research Laboratories and the Department of Otolaryngology-Head and Neck Surgery, University of Iowa, Iowa City, Iowa, USA Department of Otolaryngology-Head & Neck Surgery, Stanford University School of Medicine, Stanford, California, USA. · Department of Otolaryngology-Head and Neck Surgery, Institute of Otolaryngology, PLA General Hospital, Beijing, China Beijing Institute of Otorhinolaryngology, Beijing Tongren Hospital, Capital Medical University, Beijing, China. · National Institute of Biological Sciences, Beijing, China. · Molecular Otolaryngology and Renal Research Laboratories and the Department of Otolaryngology-Head and Neck Surgery, University of Iowa, Iowa City, Iowa, USA. · Department of Communicative Disorders & Sciences, Center for Hearing and Deafness, University at Buffalo, Buffalo, New York, USA. · Department of Radiology, PLA General Hospital, Beijing, China. · Department of Neurology, PLA General Hospital, Beijing, China. · Key Laboratory of Mental Health, Institute of Psychology, Chinese Academy of Sciences, Beijing, China. · BGI-Shenzhen, Shenzhen, China. · Unité de Génétique et Physiologie de l'Audition, Institut Pasteur, Collège de France, Paris, France. ·J Med Genet · Pubmed #25986071.

ABSTRACT: BACKGROUND: Auditory neuropathy spectrum disorder (ANSD) is a form of hearing loss in which auditory signal transmission from the inner ear to the auditory nerve and brain stem is distorted, giving rise to speech perception difficulties beyond that expected for the observed degree of hearing loss. For many cases of ANSD, the underlying molecular pathology and the site of lesion remain unclear. The X-linked form of the condition, AUNX1, has been mapped to Xq23-q27.3, although the causative gene has yet to be identified. METHODS: We performed whole-exome sequencing on DNA samples from the AUNX1 family and another small phenotypically similar but unrelated ANSD family. RESULTS: We identified two missense mutations in AIFM1 in these families: c.1352G>A (p.R451Q) in the AUNX1 family and c.1030C>T (p.L344F) in the second ANSD family. Mutation screening in a large cohort of 3 additional unrelated families and 93 sporadic cases with ANSD identified 9 more missense mutations in AIFM1. Bioinformatics analysis and expression studies support this gene as being causative of ANSD. CONCLUSIONS: Variants in AIFM1 gene are a common cause of familial and sporadic ANSD and provide insight into the expanded spectrum of AIFM1-associated diseases. The finding of cochlear nerve hypoplasia in some patients was AIFM1-related ANSD implies that MRI may be of value in localising the site of lesion and suggests that cochlea implantation in these patients may have limited success.

18 Article Exome sequencing and linkage analysis identified tenascin-C (TNC) as a novel causative gene in nonsyndromic hearing loss. 2013

Zhao, Yali / Zhao, Feifan / Zong, Liang / Zhang, Peng / Guan, Liping / Zhang, Jianguo / Wang, Dayong / Wang, Jing / Chai, Wei / Lan, Lan / Li, Qian / Han, Bing / Yang, Ling / Jin, Xin / Yang, Weiyan / Hu, Xiaoxiang / Wang, Xiaoning / Li, Ning / Li, Yingrui / Petit, Christine / Wang, Jun / Wang, Huanming Yang Jian / Wang, Qiuju. ·Department of Otorhinolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, China. ·PLoS One · Pubmed #23936043.

ABSTRACT: In this study, a five-generation Chinese family (family F013) with progressive autosomal dominant hearing loss was mapped to a critical region spanning 28.54 Mb on chromosome 9q31.3-q34.3 by linkage analysis, which was a novel DFNA locus, assigned as DFNA56. In this interval, there were 398 annotated genes. Then, whole exome sequencing was applied in three patients and one normal individual from this family. Six single nucleotide variants and two indels were found co-segregated with the phenotypes. Then using mass spectrum (Sequenom, Inc.) to rank the eight sites, we found only the TNC gene be co-segregated with hearing loss in 53 subjects of F013. And this missense mutation (c.5317G>A, p.V1773M ) of TNC located exactly in the critical linked interval. Further screening to the coding region of this gene in 587 subjects with nonsyndromic hearing loss (NSHL) found a second missense mutation, c.5368A>T (p. T1796S), co-segregating with phenotype in the other family. These two mutations located in the conserved region of TNC and were absent in the 387 normal hearing individuals of matched geographical ancestry. Functional effects of the two mutations were predicted using SIFT and both mutations were deleterious. All these results supported that TNC may be the causal gene for the hearing loss inherited in these families. TNC encodes tenascin-C, a member of the extracellular matrix (ECM), is present in the basilar membrane (BM), and the osseous spiral lamina of the cochlea. It plays an important role in cochlear development. The up-regulated expression of TNC gene in tissue repair and neural regeneration was seen in human and zebrafish, and in sensory receptor recovery in the vestibular organ after ototoxic injury in birds. Then the absence of normal tenascin-C was supposed to cause irreversible injuries in cochlea and caused hearing loss.

19 Article Oxidative stress, inflammation, and autophagic stress as the key mechanisms of premature age-related hearing loss in SAMP8 mouse Cochlea. 2012

Menardo, Julien / Tang, Yong / Ladrech, Sabine / Lenoir, Marc / Casas, François / Michel, Christophe / Bourien, Jérôme / Ruel, Jérôme / Rebillard, Guy / Maurice, Tangui / Puel, Jean-Luc / Wang, Jing. ·Institut des Neurosciences de Montpellier, France. ·Antioxid Redox Signal · Pubmed #21923553.

ABSTRACT: AIMS: In our aging society, age-related hearing loss (ARHL) or presbycusis is increasingly important. Here, we study the mechanism of ARHL using the senescence-accelerated mouse prone 8 (SAMP8) which is a useful model to probe the effects of aging on biological processes. RESULTS: We found that the SAMP8 strain displays premature hearing loss and cochlear degeneration recapitulating the processes observed in human presbycusis (i.e., strial, sensory, and neural degeneration). The molecular mechanisms associated with premature ARHL in SAMP8 mice involve oxidative stress, altered levels of antioxidant enzymes, and decreased activity of Complexes I, II, and IV, which in turn lead to chronic inflammation and triggering of apoptotic cell death pathways. In addition, spiral ganglion neurons (SGNs) also undergo autophagic stress and accumulated lipofuscin. INNOVATION AND CONCLUSION: Our results provide evidence that targeting oxidative stress, chronic inflammation, or apoptotic pathways may have therapeutic potential. Modulation of autophagy may be another strategy. The fact that autophagic stress and protein aggregation occurred specifically in SGNs also offers promising perspectives for the prevention of neural presbycusis.