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Hearing Disorders: HELP
Articles from Chinese Academy of Sciences - Institute of Psychology
Based on 3 articles published since 2010
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These are the 3 published articles about Hearing Disorders that originated from Chinese Academy of Sciences - Institute of Psychology during 2010-2020.
 
+ Citations + Abstracts
1 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.

2 Article The social-neurophysiological model of tinnitus: theory and practice. 2015

Li, Zhicheng / Gu, Ruolei / Zeng, Xiangli. ·Hearing and Balance Rehabilitation Centre, Department of Otolaryngology Head and Neck Surgery, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China. · Key Laboratory of Behavioral Science, Institute of Psychology, Chinese Academy of Sciences, Beijing 100101, China. · Hearing and Balance Rehabilitation Centre, Department of Otolaryngology Head and Neck Surgery, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China. Electronic address: zengxl_2002@aliyun.com. ·J Formos Med Assoc · Pubmed #24083913.

ABSTRACT: -- No abstract --

3 Article Brain activations associated with sign production using word and picture inputs in deaf signers. 2011

Hu, Zhiguo / Wang, Wenjing / Liu, Hongyan / Peng, Danling / Yang, Yanhui / Li, Kuncheng / Zhang, John X / Ding, Guosheng. ·Key Laboratory of Mental Health, Institute of Psychology, Chinese Academy of Sciences, Beijing, China. ·Brain Lang · Pubmed #21215442.

ABSTRACT: Effective literacy education in deaf students calls for psycholinguistic research revealing the cognitive and neural mechanisms underlying their written language processing. When learning a written language, deaf students are often instructed to sign out printed text. The present fMRI study was intended to reveal the neural substrates associated with word signing by comparing it with picture signing. Native deaf signers were asked to overtly sign in Chinese Sign Language (CSL) common objects indicated with written words or presented as pictures. Except in left inferior frontal gyrus and inferior parietal lobule where word signing elicited greater activation than picture signing, the two tasks engaged a highly overlapping set of brain regions previously implicated in sign production. The results suggest that word signing in the deaf signers relies on meaning activation from printed visual forms, followed by similar production processes from meaning to signs as in picture signing. The present study also documents the basic brain activation pattern for sign production in CSL and supports the notion of a universal core neural network for sign production across different sign languages.