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 …

[HTML][HTML] 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 …

Role of connexin 43 in different forms of intercellular communication–gap junctions, extracellular vesicles and tunnelling nanotubes

TM Ribeiro-Rodrigues… - Journal of cell …, 2017 - journals.biologists.com
Communication is important to ensure the correct and efficient flow of information, which is
required to sustain active social networks. A fine-tuned communication between cells is vital …

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 …

[HTML][HTML] Gap junction-mediated cell-to-cell communication in oral development and oral diseases: a concise review of research progress

W Liu, Y Cui, J Wei, J Sun, L Zheng, J Xie - International journal of oral …, 2020 - nature.com
Homoeostasis depends on the close connection and intimate molecular exchange between
extracellular, intracellular and intercellular networks. Intercellular communication is largely …

[HTML][HTML] Research progress in delineating the pathological mechanisms of GJB2-related hearing loss

Y Wang, Y Jin, Q Zhang, Y Xiong, X Gu… - Frontiers in Cellular …, 2023 - frontiersin.org
Hearing loss is the most common congenital sensory impairment. Mutations or deficiencies
of the GJB2 gene are the most common genetic cause of congenital non-syndromic …

Connexin-mediated signaling in nonsensory cells is crucial for the development of sensory inner hair cells in the mouse cochlea

SL Johnson, F Ceriani, O Houston… - Journal of …, 2017 - Soc Neuroscience
Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin
30 (Cx30) have been linked to syndromic and nonsyndromic hearing loss in mice and …

[HTML][HTML] Purinergic signaling in cochlear supporting cells reduces hair cell excitability by increasing the extracellular space

TA Babola, CJ Kersbergen, HC Wang, DE Bergles - Elife, 2020 - elifesciences.org
Neurons in developing sensory pathways exhibit spontaneous bursts of electrical activity
that are critical for survival, maturation and circuit refinement. In the auditory system …

[HTML][HTML] Supporting cells and their potential roles in cisplatin-induced ototoxicity

S Waissbluth, JC Maass, HA Sanchez… - Frontiers in …, 2022 - frontiersin.org
Cisplatin is a known ototoxic chemotherapy drug, causing irreversible hearing loss.
Evidence has shown that cisplatin causes inner ear damage as a result of adduct formation …

[HTML][HTML] GJB2 gene therapy and conditional deletion reveal developmental stage-dependent effects on inner ear structure and function

J Guo, X Ma, JM Skidmore, J Cimerman… - … Therapy-Methods & …, 2021 - cell.com
Pathogenic variants in GJB2, the gene encoding connexin 26, are the most common cause
of autosomal-recessive hereditary deafness. Despite this high prevalence, pathogenic …