Age-related hearing loss (ARHL) is one of the most common disorders affecting elderly individuals. There is an urgent need for effective preventive measures for ARHL because none are currently available. Cockayne syndrome (CS) is a premature aging disease that presents with progressive hearing loss at a young age, but is otherwise similar to ARHL. There are two human genetic complementation groups of CS, A and B. While the clinical phenotypes in patients are similar, the proteins have very diverse functions, and insight into their convergence is of great interest. Here, we use mouse models for CS ( and ) that recapitulate the hearing loss in human CS patients. We previously showed that NAD, a key metabolite with various essential functions, is reduced in CS and associated with multiple CS phenotypes. In this study, we report that NAD levels are reduced in the cochlea of mice and that short-term treatment (10 days) with the NAD precursor nicotinamide riboside (NR), prevents hearing loss, restores outer hair cell loss, and improves cochlear health in mice. Similar, but more modest effects were observed in mice. Remarkably, we observed a reduction in synaptic ribbon counts in the presynaptic zones of inner hair cells in both and mice, pointing to a converging mechanism for cochlear defects in CS. Ribbon synapses facilitate rapid and sustained synaptic transmission over long periods of time. Ribeye, a core protein of synaptic ribbons, possesses an NAD(H) binding pocket which regulates its activity. Intriguingly, NAD supplementation rescues reduced synaptic ribbon formation in both and mutant cochleae. These findings provide valuable insight into the mechanism of CS- and ARHL-associated hearing loss, and suggest a possible intervention.
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http://dx.doi.org/10.1038/s41514-019-0040-z | DOI Listing |
Front Biosci (Landmark Ed)
January 2025
Department of Otolaryngology, Head and Neck Surgery, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, 330006 Nanchang, Jiangxi, China.
Background: It has been reported the therapeutic effects of mesenchymal stem cells (MSCs) on hearing loss. This study explored the therapeutic effects of growth differentiation factor 6 (GDF6) overexpression-induced MSCs (MSCs-GDF6) on age-related hearing loss (ARHL) and its underlying mechanisms.
Methods: Reverse transcription-quantitative PCR and western blotting were used to evaluate gene expression.
Int J Speech Lang Pathol
January 2025
School of Allied Health, Exercise and Sports Sciences, Charles Sturt University, Albury, Australia.
Purpose: The parents of children who are deaf or hard-of-hearing may require a spoken language interpreter to access early-intervention services. This research sought to describe speech-language pathologists' perspectives regarding collaboration with interpreters in this space.
Method: Twenty-seven speech-language pathologists working in Australia completed a cross-sectional mixed-method online survey.
J Clin Med
January 2025
International Head and Neck Scientific Group, 35100 Padua, Italy.
Post/Long COVID (syndrome) is defined as a condition with symptoms persisting for more than 12 weeks after the onset of SARS-CoV-2 infection that cannot be explained otherwise. The prevalence of self-reported otorhinolaryngological Post/Long COVID symptoms is high. The aim of this review was to analyze the current literature regarding the actual prevalence, knowledge of the etiopathology, and evidence-based treatment recommendations of otorhinolaryngology-related Post/Long COVID symptoms.
View Article and Find Full Text PDFJ Clin Med
January 2025
Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Service d'Oto-Rhino-Laryngologie, 78 Rue du Général Leclerc, 94270 Le Kremlin-Bicêtre, France.
Hearing aids (HAs) have been used for standard high-frequency hearing loss and tinnitus, but their effects on speech intelligibility in noise (SIN) in people with normal hearing, including hidden hearing loss (HHL), have been little explored. We included in a prospective cohort study patients who experience poor SIN and have normal pure tone average in quiet conditions or slight HL. We used open-fit HAs.
View Article and Find Full Text PDFMolecules
January 2025
Department of Pharmacy, University of Pisa, Via Bonanno, 6, 56126 Pisa, Italy.
Hearing loss is one of the most common sensory disorders in humans, and a large number of cases are due to ear cell damage caused by ototoxic drugs including anticancer agents, such as cisplatin. The recent literature reported that hearing loss is promoted by an excessive generation of reactive oxygen species (ROS) in cochlea cells, which causes oxidative stress. Recently, polysaccharides from the cyanobacterium showed many biological activities, including antioxidant activity, suggesting their potential use to combat hearing loss.
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