Results 131 to 140 of about 1,078,099 (366)

Gout with hearing loss [PDF]

Rheumatology and Immunology Research, 2021
Min Shen, Wenhan Huang, Wenhan Huang, Yi Wang   +3 more
openaire   +3 more sources

Single Administration of AAV‐mAtp6v1b2 Gene Therapy Rescues Hearing and Vestibular Disorders Caused by Atp6v1b2‐Induced Lysosomal Dysfunction in Hair Cells

Advanced Science, EarlyView.
Wei et al. establish a hair cell‐specific conditional knockout mouse model (Atp6v1b2fl/fl;Atoh1Cre/+), and demonstrate the importance of Atp6v1b2 for hair cell through maintaining the survival of lysosomes. A single administration of AAV‐ie‐Eh3‐mAtp6v1b2 through scala media at P0‐P2 realizes function compensation and restores hearing and balance ...
Gege Wei, Shiwei Qiu, Xue Gao, Lu Zheng, Yijin Chen, Ying Ma, Haifeng Feng, Jinyuan Yang, Guojie Dong, Huiyi Nie, Weihao Zhao, Xiaoge Li, Guangqin Wang, Wei Xiong, Pu Dai, Yongyi Yuan   +15 more
wiley   +1 more source

GDC: Integration of Multi‐Omic and Phenotypic Resources to Unravel the Genetic Pathogenesis of Hearing Loss

Advanced Science, EarlyView.
Overview of the Genetic Deafness Commons (GDC), integrating data from the Chinese Deafness Genetics Consortium (CDGC) and 51 public databases. The GDC provides tools for variant search, functional predictions, and gene‐disease visualization, offering insights into 201 hearing loss genes and facilitating novel gene discovery and clinical applications ...
Hui Cheng, Xuegang Wang, Mingjun Zhong, Jia Geng, Wenjian Li, Kanglu Pei, Jing Wang, Lanchen Wang, Yu Lu, Jing Cheng, Fengxiao Bu, Huijun Yuan   +11 more
wiley   +1 more source

Consequences and Mechanisms of Noise‐Induced Cochlear Synaptopathy and Hidden Hearing Loss, With Focuses on Signal Perception in Noise and Temporal Processing

Advanced Science, EarlyView.
Noise‐induced synaptopathy (NIS) is largely reversible due to self‐repair. NIS and noise‐induced hidden hearing loss are two concepts with similarities and differences. The major hearing deficits in NIHHL are temporal processing disorders. The translation of animal data in NIS studies to humans is hindered by many factors.
Hui Wang, Steven J Aiken, Jian Wang
wiley   +1 more source

New frontiers in tinnitus, hearing loss and hyperacusis [PDF]

, 2016
Although considerable progresses have been made in the recent years, there are still wide-open pathways for research in tinnitus, hearing loss and hyperacusis.
RALLI, MASSIMO
core   +1 more source

Long‐Lasting Auditory and Vestibular Recovery Following Gene Replacement Therapy in a Novel Usher Syndrome Type 1c Mouse Model

Advanced Science, EarlyView.
This study shows that gene replacement therapy using the AAV2/Anc80L65 virus can successfully restore hearing and balance in Ush1c knockout mice. The treatment leads to lasting improvements in both auditory and vestibular functions, highlighting its potential as a therapeutic approach for genetic hearing loss and vestibular disorders in humans ...
Weinan Du, Jun Huang, Aizhen Zhang, Fangfang Zhao, Tianwen Chen, Quinn M. McDermott, Tony Zheng, Haibo Wang, Rongli Zhang, Xiaolin Zhang, Jerome Allison, Hong Zhu, Wu Zhou, Qing Yin Zheng   +13 more
wiley   +1 more source

Deletion of the Ca2+-activated potassium (BK) alpha-subunit but not the BK-beta-1-subunit leads to progressive hearing loss [PDF]

, 2005
The large conductance voltage- and Ca2+-activated potassium (BK) channel has been suggested to play an important role in the signal transduction process of cochlear inner hair cells.
Arntz, Claudia, Braig, Claudia, Engel, Jutta, Hirt, Bernhard, Knipper, Marlies, Knirsch, Martina, Köpschall, Iris, Langer, Patricia, Müller, Marcus, Münkner, Stefan, Pfaff, Imke, Pfister, Markus, Rohbock, Karin, Ruth, Peter, Rüsch, Alfons, Rüttiger, Lukas, Sausbier, Matthias, Winter, Harald, Zimmermann, Ulrike   +18 more
core  

The Reconstruction of Peripheral Auditory Circuit: Recent Advances and Future Challenges

Advanced Science, EarlyView.
This paper summarizes the potential of biomaterials, stem cells, and gene editing technologies in the regeneration of inner ear hair cells, spiral ganglion neurons, and inner ear organoids. Challenges and potential developments are discussed and explored.
Zhe Li, Ying Zhang, Zhenyu Xiao, Jieyu Qi   +3 more
wiley   +1 more source

Clinical overview on RASopathies

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, Volume 190, Issue 4, Page 414-424, December 2022., 2022
Abstract RASopathies comprise a group of clinically overlapping developmental disorders caused by genetic variations affecting components or modulators of the RAS‐MAPK signaling cascade, which lead to dysregulation of signal flow through this pathway.
Martin Zenker
wiley   +1 more source

Roles of supporting cells in the maintenance and regeneration of the damaged inner ear: A literature review

Journal of Otology
The inner ear sensory epithelium consists of two major types of cells: hair cells (HCs) and supporting cells (SCs). Critical functions of HCs in the perception of mechanical stimulation and mechanosensory transduction have long been elucidated.
Jing-Ying Guo, Jun-Yi Xu, Shu-Sheng Gong, Guo-Peng Wang   +3 more
doaj