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Hearing Disorders: HELP
Articles by Yong Wang
Based on 11 articles published since 2010
(Why 11 articles?)
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Between 2010 and 2020, Yong Wang wrote the following 11 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Review Short-term plasticity and auditory processing in the ventral cochlear nucleus of normal and hearing-impaired animals. 2011

Wang, Yong / O'Donohue, Heather / Manis, Paul. ·Division of Otolaryngology and Neuroscience Program, 3C120 School of Medicine, 30 North, 1900 East, Salt Lake City, University of Utah, UT 84132, USA. yong.wang@hsc.utah.edu ·Hear Res · Pubmed #21586317.

ABSTRACT: The dynamics of synaptic transmission between neurons plays a major role in neural information processing. In the cochlear nucleus, auditory nerve synapses have a relatively high release probability and show pronounced synaptic depression that, in conjunction with the variability of interspike intervals, shapes the information transmitted to the postsynaptic cells. Cellular mechanisms have been best analyzed at the endbulb synapses, revealing that the recent history of presynaptic activity plays a complex, non-linear, role in regulating release. Emerging evidence suggests that the dynamics of synaptic function differs according to the target neuron within the cochlear nucleus. One consequence of hearing loss is changes in evoked release at surviving auditory nerve synapses, and in some situations spontaneous release is greatly enhanced. In contrast, even with cochlear ablation, postsynaptic excitability is less affected. The existing evidence suggests that different modes of hearing loss can result in different dynamic patterns of synaptic transmission between the auditory nerve and postsynaptic neurons. These changes in dynamics in turn will affect the efficacy with which different kinds of information about the acoustic environment can be processed by the parallel pathways in the cochlear nucleus.

2 Article Efficacy and Prognostic Factors of Combined Hyperbaric Oxygen Therapy in Patients With Idiopathic Sudden Sensorineural Hearing Loss. 2019

Wang, Yong / Gao, Yali / Wang, Boxuan / Chen, Liyan / Zhang, Xiaoxiao. ·Rehabilitation Medicine Center, Fuxing Hospital, Capital Medical University, Beijing, China. · Department of Otolaryngology Head and Neck Surgery, Fuxing Hospital, Capital Medical University, Beijing, China. ·Am J Audiol · Pubmed #30938564.

ABSTRACT: Purpose Idiopathic sudden sensorineural hearing loss is a clinical emergency with an increased incidence of occurrence in recent years. Hyperbaric oxygen has been widely used in the clinical treatment of sudden hearing loss. However, prognostic factors related to its curative effects are still not clear, which hinders its clinical application. The aim of this study was to investigate the efficacy and prognostic factors of hyperbaric oxygen therapy (HBOT) plus drug therapies in the treatment of sudden hearing loss. Method Patients with sudden hearing loss who were treated with hyperbaric oxygen from April to October 2017 were retrospectively included. Clinical information was collected, including pure-tone audiometry data before and after hyperbaric oxygen treatment, age, gender, hearing curve classification, concomitant symptoms, history of diseases (diabetes and hypertension), history of hormone therapy, start time of hyperbaric oxygen treatment, and total number of hyperbaric oxygen treatments. The prognostic factors were analyzed with univariate and multivariate analyses. Results Sixty-four patients with sudden hearing loss were enrolled, including 7 cases of low-frequency loss, 4 cases of high-frequency loss, 32 cases of flat loss, and 21 cases of complete hearing loss. After HBOT, there were 16 cases (25%) of complete recovery, 15 cases (23.43%) of partial recovery, 7 cases (10.93%) of slight improvement, and 26 cases (40.63%) of no improvement. The total effective rate was 59.37%. Twelve of 21 cases (57.14%) of complete hearing loss showed recovery. Multivariate logistic regression analysis showed that the start time of HBOT ≤ 7 days from disease onset was independently associated with hearing recovery ( OR = 27.763, 95% CI [4.209, 183.115], p = .001). Conclusion Combined HBOT can improve the hearing impairment of sudden hearing loss. The early HBOT showed the most promising therapeutic effects, especially among patients with complete hearing loss.

3 Article Mutations in KARS cause early-onset hearing loss and leukoencephalopathy: Potential pathogenic mechanism. 2017

Zhou, Xiao-Long / He, Long-Xia / Yu, Li-Jia / Wang, Yong / Wang, Xi-Jin / Wang, En-Duo / Yang, Tao. ·State Key Laboratory of Molecular Biology, CAS Center for Excellence in Molecular Cell Science, Shanghai Institute of Biochemistry and Cell Biology Chinese Academy of Sciences, University of Chinese Academy of Sciences, Shanghai, China. · Department of Otolaryngology-Head and Neck Surgery, Shanghai Ninth People's Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. · Department of Otorhinolaryngology-Head and Neck Surgery, Chengdu Integrated TCM & Western Medicine Hospital, Sichuan Province, China. · Department of Neurology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. · School of Life Science and Technology, Shanghai Tech University, Shanghai, China. ·Hum Mutat · Pubmed #28887846.

ABSTRACT: Leukoencephalopathies are a broad class of common neurologic deterioration for which the etiology remains unsolved in many cases. In a Chinese Han family segregated with sensorineural hearing loss and leukoencephalopathy, candidate pathogenic variants were identified by targeted next-generation sequencing of 144 genes associated with deafness and 108 genes with leukoencephalopathy. Novel compound heterozygous mutations p.R477H and p.P505S were identified in KARS, which encodes lysyl-tRNA synthetase (LysRS), as the only candidate causative variants. These two mutations were functionally characterized by enzymatic assays, immunofluorescence, circular dichroism analysis, and gel filtration chromatography. Despite no alteration in the dimer-tetramer oligomerization and cellular distribution by either mutation, the protein structure was notably influenced by the R477H mutation, which subsequently released the protein from the multiple-synthetase complex (MSC). Mutant LysRSs with the R477H and P505S mutations had decreased tRNA

4 Article Natural killer cells attenuate cytomegalovirus-induced hearing loss in mice. 2017

Almishaal, Ali A / Mathur, Pranav D / Hillas, Elaine / Chen, Liting / Zhang, Anne / Yang, Jun / Wang, Yong / Yokoyama, Wayne M / Firpo, Matthew A / Park, Albert H. ·Department of Communication Sciences and Disorders, University of Utah College of Health, Salt Lake City, Utah, United States of America. · Department of Ophthalmology and Visual Sciences, University of Utah School of Medicine, Salt Lake City, Utah, United States of America. · Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, United States of America. · Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah, United States of America. · Division of Otolaryngology, University of Utah School of Medicine, Salt Lake City, Utah, United States of America. · Division of Rheumatology, Washington University School of Medicine, St. Louis, Missouri, United States of America. · Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, Missouri, United States of America. ·PLoS Pathog · Pubmed #28859161.

ABSTRACT: Congenital cytomegalovirus (CMV) infection is the most common non-hereditary cause of sensorineural hearing loss (SNHL) yet the mechanisms of hearing loss remain obscure. Natural Killer (NK) cells play a critical role in regulating murine CMV infection via NK cell recognition of the Ly49H cell surface receptor of the viral-encoded m157 ligand expressed at the infected cell surface. This Ly49H NK receptor/m157 ligand interaction has been found to mediate host resistance to CMV in the spleen, and lung, but is much less effective in the liver, so it is not known if this interaction is important in the context of SNHL. Using a murine model for CMV-induced labyrinthitis, we have demonstrated that the Ly49H/m157 interaction mediates host resistance in the temporal bone. BALB/c mice, which lack functional Ly49H, inoculated with mCMV at post-natal day 3 developed profound hearing loss and significant outer hair cell loss by 28 days of life. In contrast, C57BL/6 mice, competent for the Ly49H/m157 interaction, had minimal hearing loss and attenuated outer hair cell loss with the same mCMV dose. Administration of Ly49H blocking antibody or inoculation with a mCMV viral strain deleted for the m157 gene rendered the previously resistant C57BL/6 mouse strain susceptible to hearing loss to a similar extent as the BALB/c mouse strain indicating a direct role of the Ly49H/m157 interaction in mCMV-dependent hearing loss. Additionally, NK cell recruitment to sites of infection was evident in the temporal bone of inoculated susceptible mouse strains. These results demonstrate participation of NK cells in protection from CMV-induced labyrinthitis and SNHL in mice.

5 Article A deafness-associated tRNAAsp mutation alters the m1G37 modification, aminoacylation and stability of tRNAAsp and mitochondrial function. 2016

Wang, Meng / Peng, Yanyan / Zheng, Jing / Zheng, Binjiao / Jin, Xiaofen / Liu, Hao / Wang, Yong / Tang, Xiaowen / Huang, Taosheng / Jiang, Pingping / Guan, Min-Xin. ·Division of Clinical Genetics and Genomics, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310058, China. · Institute of Genetics, Zhejiang University and Department of Genetics, Zhejiang University, School of Medicine, Hangzhou, Zhejiang 310058, China. · Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA. · Attardi Institute of Mitochondrial Biomedicine, Wenzhou Medical University, Wenzhou, Zhejiang 325600, China. · Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China. · Division of Clinical Genetics and Genomics, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310058, China ppjiang@zju.edu.cn. · Division of Clinical Genetics and Genomics, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310058, China gminxin88@zju.edu.cn. · Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, Zhejiang University, Hangzhou, Zhejiang 310058, China. ·Nucleic Acids Res · Pubmed #27536005.

ABSTRACT: In this report, we investigated the pathogenic mechanism underlying the deafness-associated mitochondrial(mt) tRNA

6 Article Individual USH2 proteins make distinct contributions to the ankle link complex during development of the mouse cochlear stereociliary bundle. 2015

Zou, Junhuang / Mathur, Pranav D / Zheng, Tihua / Wang, Yong / Almishaal, Ali / Park, Albert H / Yang, Jun. ·Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA. · Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA, Department of Neurobiology and Anatomy, University of Utah, 20 North 1900 East, Salt Lake City, UT 84132, USA. · Division of Otolaryngology, Department of Surgery, University of Utah, 30 North 1900 East, Salt Lake City, UT 84132, USA and. · Department of Communication Sciences and Disorders, University of Utah, 390 South 1530 East, Salt Lake City, UT 84112, USA. · Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA, Department of Neurobiology and Anatomy, University of Utah, 20 North 1900 East, Salt Lake City, UT 84132, USA, Division of Otolaryngology, Department of Surgery, University of Utah, 30 North 1900 East, Salt Lake City, UT 84132, USA and jun.yang@hsc.utah.edu. ·Hum Mol Genet · Pubmed #26401052.

ABSTRACT: Usher syndrome (USH) is the leading cause of inherited deaf-blindness, with type 2 (USH2) being the most common clinical form. Studies suggest that proteins encoded by USH2 causative genes assemble into the ankle link complex (ALC) at the hair cell stereociliary bundle; however, little is known about the in vivo assembly and function of this complex. Using various USH2 mutant mice, we showed by immunofluorescence that USH2 proteins play different roles in cochlear ALC assembly, with G protein-coupled receptor 98 being the most important protein. Complex assembly likely occurs at the stereociliary bundle but not along the protein transport route in the cell body. Stereociliary morphological defects in USH2 mutant mice suggest roles for the ALC in regulating inner hair cell stereociliary growth and differentiation as well as outer hair cell stereociliary rigidity and organization during development. These roles are unique from the bundle cohesion role of Usher syndrome type 1 protein complexes. Loss of individual USH2 gene expressions leads to variable morphological and functional consequences, correlating with the severity of ALC disruption. This finding suggests a potential genotype-phenotype correlation in USH2 patients. In summary, this study provides novel insights into the molecular mechanism underlying cochlear stereociliary bundle development and hearing loss pathogenesis of various USH2 subtypes. Our thorough phenotypical characterization of USH2 mouse models is essential for future use of these animal models in therapeutic development.

7 Article Distinct expression and function of whirlin isoforms in the inner ear and retina: an insight into pathogenesis of USH2D and DFNB31. 2015

Mathur, Pranav Dinesh / Zou, Junhuang / Zheng, Tihua / Almishaal, Ali / Wang, Yong / Chen, Qian / Wang, Le / Vashist, Deepti / Brown, Steve / Park, Albert / Yang, Jun. ·Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA, Department of Neurobiology and Anatomy, University of Utah, 20 North 1900 East, Salt Lake City, UT 84132, USA. · Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA. · Department of Communication Sciences and Disorders, University of Utah, 390 South 1530 East, Salt Lake City, UT 84112, USA. · Division of Otolaryngology, Department of Surgery, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, USA. · Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA, The First Affiliated Hospital, Jilin University, Changchun, Jilin 130061, China and. · Mammalian Genetics Unit, Medical Research Council, Harwell, Oxfordshire OX11 ORD, UK. · Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA, Department of Neurobiology and Anatomy, University of Utah, 20 North 1900 East, Salt Lake City, UT 84132, USA, Division of Otolaryngology, Department of Surgery, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, USA, jun.yang@hsc.utah.edu. ·Hum Mol Genet · Pubmed #26307081.

ABSTRACT: Usher syndrome (USH) is the most common inherited deaf-blindness with the majority of USH causative genes also involved in nonsyndromic recessive deafness (DFNB). The mechanism underlying this disease variation of USH genes is unclear. Here, we addressed this issue by investigating the DFNB31 gene, whose mutations cause USH2D or DFNB31 depending on their position. We found that the mouse DFNB31 ortholog (Dfnb31) expressed different mRNA variants and whirlin protein isoforms in the cochlea and retina, where these isoforms played different roles spatially and temporally. Full-length (FL-) whirlin in photoreceptors and hair cell stereociliary bases is important for the USH type 2 protein complex, while FL- and C-terminal (C-) whirlins in hair cell stereociliary tips participate in stereociliary elongation. Mutations in the whirlin N-terminal region disrupted FL-whirlin isoform in the inner ear and retina but not C-whirlin in the inner ear, and led to retinal degeneration as well as moderate to severe hearing loss. By contrast, a mutation in the whirlin C-terminal region eliminated all normal whirlin isoforms but generated a truncated N-terminal whirlin protein fragment, which was partially functional in the retina and thus prevented retinal degeneration. Mice with this mutation had profound hearing loss. In summary, disruption of distinct whirlin isoforms by Dfnb31 mutations leads to a variety of phenotype configurations and may explain the mechanism underlying the different disease manifestations of human DFNB31 mutations. Our findings have a potential to improve diagnosis and treatment of USH disease and quality of life in USH patients.

8 Article Deletion of PDZD7 disrupts the Usher syndrome type 2 protein complex in cochlear hair cells and causes hearing loss in mice. 2014

Zou, Junhuang / Zheng, Tihua / Ren, Chongyu / Askew, Charles / Liu, Xiao-Ping / Pan, Bifeng / Holt, Jeffrey R / Wang, Yong / Yang, Jun. ·Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of Utah, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA. ·Hum Mol Genet · Pubmed #24334608.

ABSTRACT: Usher syndrome type 2 (USH2) is the predominant form of USH, a leading genetic cause of combined deafness and blindness. PDZD7, a paralog of two USH causative genes, USH1C and USH2D (WHRN), was recently reported to be implicated in USH2 and non-syndromic deafness. It encodes a protein with multiple PDZ domains. To understand the biological function of PDZD7 and the pathogenic mechanism caused by PDZD7 mutations, we generated and thoroughly characterized a Pdzd7 knockout mouse model. The Pdzd7 knockout mice exhibit congenital profound deafness, as assessed by auditory brainstem response, distortion product otoacoustic emission and cochlear microphonics tests, and normal vestibular function, as assessed by their behaviors. Lack of PDZD7 leads to the disorganization of stereocilia bundles and a reduction in mechanotransduction currents and sensitivity in cochlear outer hair cells. At the molecular level, PDZD7 determines the localization of the USH2 protein complex, composed of USH2A, GPR98 and WHRN, to ankle links in developing cochlear hair cells, likely through its direct interactions with these three proteins. The localization of PDZD7 to the ankle links of cochlear hair bundles also relies on USH2 proteins. In photoreceptors of Pdzd7 knockout mice, the three USH2 proteins largely remain unchanged at the periciliary membrane complex. The electroretinogram responses of both rod and cone photoreceptors are normal in knockout mice at 1 month of age. Therefore, although the organization of the USH2 complex appears different in photoreceptors, it is clear that PDZD7 plays an essential role in organizing the USH2 complex at ankle links in developing cochlear hair cells. GenBank accession numbers: KF041446, KF041447, KF041448, KF041449, KF041450, KF041451.

9 Article A comparison of different murine models for cytomegalovirus-induced sensorineural hearing loss. 2013

Wang, Yong / Patel, Rusha / Ren, Chongyu / Taggart, Michael G / Firpo, Matthew A / Schleiss, Mark R / Park, Albert H. ·Division of Otolaryngology-Head and Neck Surgery, University of Utah, Salt Lake City, Utah. ·Laryngoscope · Pubmed #23616191.

ABSTRACT: OBJECTIVES/HYPOTHESIS: To compare three different inoculation techniques for the development of cytomegalovirus (CMV)-induced sensorineural hearing loss (SNHL) in a mouse model. STUDY DESIGN: A prospective experimental animal study. METHODS: BALB/c mice underwent inoculation using green fluorescent protein-expressing mouse cytomegalovirus (mCMV-GFP) via transtympanic (TT), intraperitoneal (IP), or intracranial (IC) routes. Control mice received an equal volume of saline. Hearing thresholds were measured using both distortion product otoacoustic emissions (DPOAE) and evoked auditory brainstem response studies (ABR). Cochleas were harvested for histological examination and cytocochleogram. RESULTS: No mice in the TT or IP groups showed significant hearing loss. All infected mice in the IC group showed significantly elevated ABR and DPOAE thresholds at 4 weeks of age. Ten mice (55%) had profound hearing loss (≥80 dB) at 4 weeks of age, while the other eight mice (45%) initially showed moderate hearing loss (≤20 dB), which progressed to profound hearing loss by 6 to 8 weeks. Asymmetric hearing loss was seen in 40% of the mice. Temporal bone histology showed diffuse loss of outer hair cells (OHC). Green fluorescent protein (GFP)-labeled virus was abundant in the spiral ganglion and adjacent to the scala tympani at the basal region of the cochlea at 7 days postinjection, and devoid of GFP labeling by 14 days postinfection. CONCLUSIONS: Intracerebral injection of mCMV preferentially causes mCMV-mediated hearing loss relative to IP or TT injections. These results are consistent with the hearing loss reported in human congenital infection and may have implications for understanding the pathophysiology of CMV-mediated labyrinthitis.

10 Article Effects of repeated "benign" noise exposures in young CBA mice: shedding light on age-related hearing loss. 2012

Wang, Yong / Ren, Chongyu. ·Division of Otolaryngology and Program in Neuroscience, University of Utah, 30 North, 1900 East, Salt Lake City, UT 84132-0002, USA. yong.wang@hsc.utah.edu ·J Assoc Res Otolaryngol · Pubmed #22532192.

ABSTRACT: Temporary hearing threshold shift (TTS) resulting from a "benign" noise exposure can cause irreversible auditory nerve afferent terminal damage and retraction. While hearing thresholds and acute tissue injury recover within 1-2 weeks after a noise overexposure, it is not clear if multiple TTS noise exposures would result in cumulative damage even though sufficient TTS recovery time is provided. Here, we tested whether repeated TTS noise exposures affected permanent hearing thresholds and examined how that related to inner ear histopathology. Despite a peak 35-40 dB TTS 24 hours after each noise exposure, a double dose (2 weeks apart) of 100 dB noise (8-16 kHz) exposures to young (4-week-old) CBA mice resulted in no permanent threshold shifts (PTS) and abnormal distortion product otoacoustic emissions (DPOAE). However, although auditory brainstem response (ABR) thresholds recovered fully in once- and twice-exposed animals, the growth function of ABR wave 1( p-p ) amplitude (synchronized spiral ganglion cell activity) was significantly reduced to a similar extent, suggesting that damage resulting from a second dose of the exposure was not proportional to that observed after the initial exposure. Estimate of surviving inner hair cell afferent terminals using immunostaining of presynaptic ribbons revealed ribbon loss of ∼ 40 % at the ∼ 23 kHz region after the first round of noise exposure, but no additional loss of ribbons after the second exposure. In contrast, a third dose of the same noise exposure resulted in not only TTS, but also PTS even in regions where DPOAEs were not affected. The pattern of PTS seen was not entirely tonotopically related to the noise band used. Instead, it resembled more to that of age-related hearing loss, i.e., high frequency hearing impairment towards the base of the cochlea. Interestingly, after a 3rd dose of the noise exposure, additional loss of ribbons (another ≈ 25 %) was observed, suggesting a cumulative detrimental effect from individual "benign" noise exposures, which should result in a significant deficit in central temporal processing.

11 Article [Diagnostic function of SLC26A4 hot spot mutations screening to enlarged vestibular aqueduct syndrome]. 2010

Li, Qi / Fang, Ruping / You, Yiwen / Wang, Yong / Dai, Pu. ·Department of Otolaryngology-Head and Neck Surgery, Nanjing Children's Hospital, Nanjing Medical University, Nanjing 210008, China. ·Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi · Pubmed #21174747.

ABSTRACT: OBJECTIVE: To investigate the frequencies of SLC26A4 hot spot mutations by genetic testing method in non-syndromic hearing loss children. The feasibility of genetic screening method in finding enlarged vestibular aqueduct syndrome was confirmed by temporal bone CT scan. METHOD: Ninety-two children with moderate-profound hearing loss were enrolled and DNA were extracted from peripheral blood. SLC26A4 IVS7-2A > G and H723R mutations were analyzed by direct sequencing. The individual with homozygous, compound heterozygous or heterozygous SLC26A4 mutations was given further temporal CT scan. RESULT: The sequencing results revealed 11 (12.0%) cases carrying SLC26A4 mutations, including 5 cases of bi-allelic mutation and 6 cases of single allelic mutation. CONCLUSION: The SLC26A4 mutations has a high carrying rate in non-syndromic hearing loss children. The screening for the SLC26A4 gene mutations is useful in the diagnosis of EVAS.