Genetic hearing loss accounts for up to 50% of prelingual deafness worldwide, yet there are no biologic treatments currently available. To investigate gene therapy as a potential biologic strategy for restoration of auditory function in patients with genetic hearing loss, we tested a gene augmentation approach in mouse models of genetic deafness. We focused on DFNB7/11 and DFNA36, which are autosomal recessive and dominant deafnesses, respectively, caused by mutations in transmembrane channel-like 1 (TMC1). Mice that carry targeted deletion of Tmc1 or a dominant Tmc1 point mutation, known as Beethoven, are good models for human DFNB7/11 and DFNA36. We screened several adeno-associated viral (AAV) serotypes and promoters and identified AAV2/1 and the chicken β-actin (Cba) promoter as an efficient combination for driving the expression of exogenous Tmc1 in inner hair cells in vivo. Exogenous Tmc1 or its closely related ortholog, Tmc2, were capable of restoring sensory transduction, auditory brainstem responses, and acoustic startle reflexes in otherwise deaf mice, suggesting that gene augmentation with Tmc1 or Tmc2 is well suited for further development as a strategy for restoration of auditory function in deaf patients who carry TMC1 mutations.
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http://dx.doi.org/10.1126/scitranslmed.aab1996 | DOI Listing |
Adv Sci (Weinh)
January 2025
ENT Institute and Department of Otolaryngology, Eye & ENT Hospital of Fudan University, Shanghai, 200031, China.
Tinnitus, a widespread condition affecting numerous individuals worldwide, remains a significant challenge due to limited effective therapeutic interventions. Intriguingly, patients using cochlear implants (CIs) have reported significant relief from tinnitus symptoms, although the underlying mechanisms remain unclear and intracochlear implantation risks cochlear damage and hearing loss. This study demonstrates that targeted intracochlear electrical stimulation (ES) in guinea pigs with noise-induced hearing loss reversed tinnitus-related maladaptive plasticity in the cochlear nucleus (CN), characterized by reduced auditory innervation, increased somatosensory innervation, and diminished inhibitory neural networks.
View Article and Find Full Text PDFHeliyon
January 2025
Department of Laboratory Medicine, Fujian Medical University, Fuzhou, 350122, China.
Presbycusis, also referred to as age-related hearing loss, poses a substantial burden on both individuals and society. The hallmark of presbycusis is a progressive decrease in auditory sensitivity. Irreversible hearing loss occurs due to the limited regenerative capacity of spiral neurons and peripheral cochlear hair cells (HCs).
View Article and Find Full Text PDFJ Multidiscip Healthc
January 2025
USN Research Group for Older People's Health, University of South-Eastern Norway, Drammen, Norway.
Introduction: Aging is associated with the potential onset of vision and hearing problems, affecting the quality of life and functional independence of older adults. This study sought to investigate the prevalence of various vision and hearing problems in 76-year-old Faroese individuals and examine possible regional variations in these health issues.
Materials And Methods: A cross-sectional study design was used, surveying 175 participants, all 76-year-olds, from different regions in the Faroe Islands.
J Korean Med Sci
January 2025
Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Korea.
Background: Hearing level reference values based on the results of recent audiometry have not been established for the general population of South Korea. This study aimed to evaluate the mean hearing levels of each age group and to measure the annual progression of hearing loss.
Methods: We used the database of the eighth and ninth Korea National Health and Nutrition Examination Survey from 2020 to 2022, and included participants with normal tympanic membranes and without occupational noise exposure.
BMJ Open
December 2024
Department of Environmental and Occupational Health and Safety, College of Medicine and Health Science, Institute of Public Health, University of Gondar, Gondar, Ethiopia.
Objectives: This study was designed to assess occupational noise exposure levels, prevalence of temporary hearing loss and associated factors among textile industry workers in Amhara region, Ethiopia.
Design: An institution-based, cross-sectional study was conducted between June and July 2022. Participants were selected via a simple random sampling technique.
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