Hearing loss in adults

LL Cunningham, DL Tucci - New England Journal of Medicine, 2017 - Mass Medical Soc
Hearing Loss in Adults | New England Journal of Medicine Skip to main content The New
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Deafness: from genetic architecture to gene therapy

C Petit, C Bonnet, S Safieddine - Nature Reviews Genetics, 2023 - nature.com
Progress in deciphering the genetic architecture of human sensorineural hearing
impairment (SNHI) or loss, and multidisciplinary studies of mouse models, have led to the …

Astrocyte-derived interleukin-33 promotes microglial synapse engulfment and neural circuit development

ID Vainchtein, G Chin, FS Cho, KW Kelley, JG Miller… - Science, 2018 - science.org
Neuronal synapse formation and remodeling are essential to central nervous system (CNS)
development and are dysfunctional in neurodevelopmental diseases. Innate immune …

Therapeutic AAV gene transfer to the nervous system: a clinical reality

E Hudry, LH Vandenberghe - Neuron, 2019 - cell.com
Gene transfer has long been a compelling yet elusive therapeutic modality. First mainly
considered for rare inherited disorders, gene therapy may open treatment opportunities for …

Delivery of gene therapy through a cerebrospinal fluid conduit to rescue hearing in adult mice

BK Mathiesen, LM Miyakoshi, CR Cederroth… - Science translational …, 2023 - science.org
Inner ear gene therapy has recently effectively restored hearing in neonatal mice, but it is
complicated in adulthood by the structural inaccessibility of the cochlea, which is embedded …

AAV1-hOTOF gene therapy for autosomal recessive deafness 9: a single-arm trial

J Lv, H Wang, X Cheng, Y Chen, D Wang, L Zhang… - The Lancet, 2024 - thelancet.com
Background Autosomal recessive deafness 9, caused by mutations of the OTOF gene, is
characterised by congenital or prelingual, severe-to-complete, bilateral hearing loss …

Advances in gene therapy hold promise for treating hereditary hearing loss

L Jiang, D Wang, Y He, Y Shu - Molecular Therapy, 2023 - cell.com
Gene therapy focuses on genetic modification to produce therapeutic effects or treat
diseases by repairing or reconstructing genetic material, thus being expected to be the most …

A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear

LD Landegger, B Pan, C Askew, SJ Wassmer… - Nature …, 2017 - nature.com
Efforts to develop gene therapies for hearing loss have been hampered by the lack of safe,
efficient, and clinically relevant delivery modalities,. Here we demonstrate the safety and …

Delivery of therapeutics to the inner ear: The challenge of the blood-labyrinth barrier

S Nyberg, NJ Abbott, X Shi, PS Steyger… - Science translational …, 2019 - science.org
Permanent hearing loss affects more than 5% of the world's population, yet there are no
nondevice therapies that can protect or restore hearing. Delivery of therapeutics to the …

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 …