Results 61 to 70 of about 36,767 (171)

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

Modeling the Measurements of Cochlear Microcirculation and Hearing Function after Loud Noise [PDF]

, 2011
Objective: Recent findings support the crucial role of microcirculatory disturbance and ischemia for hearing impairment especially after noise-induced hearing loss (NIHL).
Attanasio G, Baker M, Bernhard Olzowy, Canis M, Fraenkel R, Fritz Ihler, Fujioka M, Klyscz T, Lamm K, Le Prell CG, Markus Suckfuell, Martin Canis, Nakai Y, Nuttall AL, Nuttall AL, Nuttall AL, Ohinata Y, Ohlemiller KK, Okamoto A, Okamoto A, Perlman HB, Prazma J, Quirk WS, Scheibe F, Scherer EQ, Scherer EQ, Sebastian Strieth, Seidman MD, Thorne PR, Wangemann P, Warangkana Arpornchayanon, Weille FL, Yamane H, Zeintl H   +33 more
core   +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

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

Presbycusis: Pathology, Signal Pathways, and Therapeutic Strategy

Advanced Science, EarlyView.
In ARHL, the stria vascularis, acting as a cochlear battery, gradually loses its ability to maintain the endocochlear potential, leading to impaired hair cell function and progressive hearing loss. Single‐cell sequencing reveals age‐related cellular changes in the cochlea, providing insights into the underlying mechanisms of aging and potential ...
Xiaoxu Zhao, Tian Shen, Shengda Cao, Ziyi Liu, Wendu Pang, Meixuan Li, Jingjing Liu, Wen Li, Yunhao Wu, Chengcheng Liu, Ming Xia, Xiaolong Fu, Cheng Cheng   +12 more
wiley   +1 more source

Mammalian cochlea as a physics guided evolution-optimized hearing sensor [PDF]

, 2014
Nonlinear physics plays an essential role in hearing, from sound signal generation to sound sensing to the processing of complex sound environments. We demonstrate that the evolution of the biological hearing sensors demonstrates a dramatic reduction in ...
Gomez, Florian, Lorimer, Tom, Stoop, Ruedi   +2 more
core   +2 more sources

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

The cochlea [PDF]

Current Biology, 2000
Ashmore, Jonathan, Gale, Jonathan
openaire   +2 more sources

Maturation of NaV and KV channel topographies in the auditory nerve spike initiator before and after developmental onset of hearing function [PDF]

, 2016
Auditory nerve excitation and thus hearing depend on spike-generating ion channels and their placement along the axons of auditory nerve fibers (ANFs).
Kim, Kyunghee X, Rutherford, Mark A
core   +2 more sources