Congenital hearing loss

AMH Korver, RJH Smith, G Van Camp… - Nature reviews Disease …, 2017 - nature.com
Congenital hearing loss (hearing loss that is present at birth) is one of the most prevalent
chronic conditions in children. In the majority of developed countries, neonatal hearing …

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 cell-type-specific atlas of the inner ear transcriptional response to acoustic trauma

B Milon, ED Shulman, KS So, CR Cederroth, EL Lipford… - Cell reports, 2021 - cell.com
Noise-induced hearing loss (NIHL) results from a complex interplay of damage to the
sensory cells of the inner ear, dysfunction of its lateral wall, axonal retraction of type 1C …

AAV-ie enables safe and efficient gene transfer to inner ear cells

F Tan, C Chu, J Qi, W Li, D You, K Li, X Chen… - Nature …, 2019 - nature.com
Hearing loss is the most common sensory disorder. While gene therapy has emerged as a
promising treatment of inherited diseases like hearing loss, it is dependent on the …

Single-cell RNA-Seq resolves cellular complexity in sensory organs from the neonatal inner ear

JC Burns, MC Kelly, M Hoa, RJ Morell… - Nature …, 2015 - nature.com
In the inner ear, cochlear and vestibular sensory epithelia utilize grossly similar cell types to
transduce different stimuli: sound and acceleration. Each individual sensory epithelium is …

DNA methylation in the mouse cochlea promotes maturation of supporting cells and contributes to the failure of hair cell regeneration

JD Nguyen, J Llamas, T Shi… - Proceedings of the …, 2023 - National Acad Sciences
Mammalian hair cells do not functionally regenerate in adulthood but can regenerate at
embryonic and neonatal stages in mice by direct transdifferentiation of neighboring …

Transient auditory nerve demyelination as a new mechanism for hidden hearing loss

G Wan, G Corfas - Nature communications, 2017 - nature.com
Hidden hearing loss (HHL) is a recently described auditory neuropathy believed to
contribute to speech discrimination and intelligibility deficits in people with normal …

Emerging gene therapies for genetic hearing loss

H Ahmed, O Shubina-Oleinik, JR Holt - … of the Association for Research in …, 2017 - Springer
Gene therapy, or the treatment of human disease using genetic material, for inner ear
dysfunction is coming of age. Recent progress in developing gene therapy treatments for …

Diversity of developing peripheral glia revealed by single-cell RNA sequencing

OE Tasdemir-Yilmaz, NR Druckenbrod, OO Olukoya… - Developmental cell, 2021 - cell.com
The peripheral nervous system responds to a wide variety of sensory stimuli, a process that
requires great neuronal diversity. These diverse neurons are closely associated with glial …

Engulfed by glia: glial pruning in development, function, and injury across species

S Raiders, T Han, N Scott-Hewitt… - Journal of …, 2021 - Soc Neuroscience
Phagocytic activity of glial cells is essential for proper nervous system sculpting,
maintenance of circuitry, and long-term brain health. Glial engulfment of apoptotic cells and …