[HTML][HTML] Sensory processing at ribbon synapses in the retina and the cochlea

T Moser, CP Grabner, F Schmitz - Physiological reviews, 2019 - journals.physiology.org
In recent years, sensory neuroscientists have made major efforts to dissect the structure and
function of ribbon synapses which process sensory information in the eye and ear. This …

Toward cochlear therapies

J Wang, JL Puel - Physiological reviews, 2018 - journals.physiology.org
Sensorineural hearing impairment is the most common sensory disorder and a major health
and socio-economic issue in industrialized countries. It is primarily due to the degeneration …

Mapping the fine-scale organization and plasticity of the brain vasculature

C Kirst, S Skriabine, A Vieites-Prado, T Topilko… - Cell, 2020 - cell.com
The cerebral vasculature is a dense network of arteries, capillaries, and veins. Quantifying
variations of the vascular organization across individuals, brain regions, or disease models …

Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model

O Akil, F Dyka, C Calvet, A Emptoz… - Proceedings of the …, 2019 - National Acad Sciences
Autosomal recessive genetic forms (DFNB) account for most cases of profound congenital
deafness. Adeno-associated virus (AAV)-based gene therapy is a promising therapeutic …

Hearing of Otof-deficient mice restored by trans-splicing of N- and C-terminal otoferlin

H Tang, H Wang, S Wang, SW Hu, J Lv, M Xun, K Gao… - Human Genetics, 2023 - Springer
Mutations to the OTOF gene are among the most common reasons for auditory neuropathy.
Although cochlear implants are often effective in restoring sound transduction, there are …

Single-cell transcriptomic profiling of the mouse cochlea: An atlas for targeted therapies

P Jean, F Wong Jun Tai… - Proceedings of the …, 2023 - National Acad Sciences
Functional molecular characterization of the cochlea has mainly been driven by the
deciphering of the genetic architecture of sensorineural deafness. As a result, the search for …

Cochlear transcript diversity and its role in auditory functions implied by an otoferlin short isoform

H Liu, H Liu, L Wang, L Song, G Jiang, Q Lu… - Nature …, 2023 - nature.com
Isoforms of a gene may contribute to diverse biological functions. In the cochlea, the
repertoire of alternative isoforms remains unexplored. We integrated single-cell short-read …

The natural history, clinical outcomes, and genotype–phenotype relationship of otoferlin-related hearing loss: a systematic, quantitative literature review

CL Ford, WJ Riggs, T Quigley, OP Keifer Jr, JP Whitton… - Human Genetics, 2023 - Springer
Congenital hearing loss affects one in 500 newborns. Sequence variations in OTOF, which
encodes the calcium-binding protein otoferlin, are responsible for 1–8% of congenital …

Overloaded adeno-associated virus as a novel gene therapeutic tool for otoferlin-related deafness

V Rankovic, C Vogl, NM Dörje, I Bahader… - Frontiers in Molecular …, 2021 - frontiersin.org
Hearing impairment is the most common sensory disorder in humans. So far, rehabilitation
of profoundly deaf subjects relies on direct stimulation of the auditory nerve through cochlear …

Current advances in gene therapies of genetic auditory neuropathy spectrum disorder

AR Saidia, J Ruel, A Bahloul, B Chaix, F Venail… - Journal of Clinical …, 2023 - mdpi.com
Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments
characterized by an impaired transmission of sound from the cochlea to the brain. This …