Results 61 to 70 of about 20,481 (289)

AAVR Expression is Essential for AAV Vector Transduction in Sensory Hair Cells

Advanced Science, EarlyView.
Decreased sensitivity to AAV vector transduction in the outer hair cells (OHCs) of adult mice is primarily attributed to reduction of AAVR (Kiaa0319l; Au040320). Knockout of AAVR reduces AAV vector transduction efficiency in both inner hair cells (IHCs) and OHCs in neonatal mice.
Fan Wu, Guisheng Chen, Rui Hu, Peiwen Liu, Jintao Lou, Wenji Zhao, Zuhong He, Suhua Sha, Yiqing Zheng   +8 more
wiley   +1 more source

The braincase of Malawisaurus dixeyi (Sauropoda: Titanosauria): A 3D reconstruction of the brain endocast and inner ear.

PLoS ONE, 2019
A braincase of the Cretaceous titanosaurian sauropod Malawisaurus dixeyi, complete except for the olfactory region, was CT scanned and a 3D rendering of the endocast and inner ear was generated. Cranial nerves appear in the same configuration as in other
Kate A Andrzejewski, Michael J Polcyn, Dale A Winkler, Elizabeth Gomani Chindebvu, Louis L Jacobs   +4 more
doaj   +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

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

Mutation of Rubie, a novel long non-coding RNA located upstream of Bmp4, causes vestibular malformation in mice. [PDF]

PLoS ONE, 2012
The vestibular apparatus of the vertebrate inner ear uses three fluid-filled semicircular canals to sense angular acceleration of the head. Malformation of these canals disrupts the sense of balance and frequently causes circling behavior in mice.
Kristina A Roberts, Victoria E Abraira, Andrew F Tucker, Lisa V Goodrich, Nancy C Andrews   +4 more
doaj   +1 more source

Rational Design of Inner Ear Drug Delivery Systems

Advanced Science, EarlyView.
Hearing loss is a common disease affecting many people, and inner ear lesions are one of the most important causes. This review focuses on the treatment of inner ear hearing loss by drug delivery systems. It includes the current methods and technologies developed, and it predicts possible directions.
Xiayidan Maimaitikelimu, Zhiyan Xuan, Haoyu Ren, Keng Chen, Hui Zhang, Huan Wang   +5 more
wiley   +1 more source

A Biomechanical Model of the Inner Ear: Numerical Simulation of the Caloric Test

The Scientific World Journal, 2013
Whether two vertical semicircular canals can receive thermal stimuli remains controversial. This study examined the caloric response in the three semicircular canals to the clinical hot caloric test using the finite element method.
Shuang Shen, Yingxi Liu, Xiuzhen Sun, Wei Zhao, Yingfeng Su, Shen Yu, Wenlong Liu   +6 more
doaj   +1 more source

Semicircular canal plane head impulses detect absent function of individual semicircular canals [PDF]

Brain, 1998
We studied the human vestibulo-ocular reflex (VOR) in response to head 'impulses': brief, unpredictable, passive, high-acceleration (up to 4000 degrees/s2), low-amplitude (20-30 degrees) head rotations. We delivered the head impulses approximately in the plane of the semicircular canal (SCC) being tested.
Ian S. Curthoys, Michael J. Todd, Gabor Michael Halmagyi, Swee T. Aw, R. A. Black, I P Hannigan, Phillip D. Cremer, Leigh A. McGarvie   +7 more
openaire   +3 more sources

The State of High‐Resolution Imaging of the Human Inner Ear: A Look Into the Black Box

Advanced Science, EarlyView.
High‐resolution imaging of the cochlea is challenged by numerous unique aspects of this organ, where sensorineural cells, crucial for hearing, are too small for conventional modalities like magnetic resonance imaging and computed tomography. This article reviews current imaging techniques, emerging technologies, and innovations aimed at improving ...
Shelley Batts, Nancy Pham, Guillermo Tearney, Konstantina M. Stankovic   +3 more
wiley   +1 more source

Worth the Effort: Lessons for Discovery and Care From an Unusual Case of Gorlin Syndrome

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Gorlin‐Goltz Syndrome (GGS) is a rare autosomal dominant genetic disorder encompassing a diverse range of clinical manifestations, including congenital anomalies and predisposition to cancer. Pathogenic variants in PTCH1 and SUFU account for up to 79% and 6% of cases, respectively. Currently, an estimated 15%–27% of individuals with a clinical
V. Taliercio, J. Zhao, S. E. Boyden, R. Mao, P. Bayrak‐Toydemir, A. Pflaum, J. Palumbos, A. Andrews, E. E. Baldwin, C. Welt, Mackenzie Fait, Undiagnosed Diseases Network, L. D. Botto, D. Viskochil   +13 more
wiley   +1 more source