ArticleHearing research2012
Genetic background effects on age-related hearing loss associated with Cdh23 variants in mice.
Article in Hearing research, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 114 papers.
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Who cites it
114 citing papers in PubMed, 184 citations in OpenAlex.
- Multiplexed encoding of frequency-modulated sweep features in the inferior colliculus.Journal of neurophysiology · 2026Article
- Optogenetic circuit mapping reveals connectivity and synaptic physiology of T-stellate projections from the cochlear nucleus to the auditory midbrain.bioRxiv : the preprint server for biology · 2026Article
- Investigation of the Effects of Cadherin 23 and Oncomodulin on Early Progressive Hearing Loss Using a New Oncomodulin Mouse Model.International journal of molecular sciences · 2026Article
- Article
- Time-Series Transcriptome Analysis of the Older Adult Mouse Cochlea After Noise-Induced Hearing Loss Reveals an Acute Immune Response and Recovery-Associated Pathways.Molecular neurobiology · 2025Article
- Rapid rebalancing of co-tuned ensemble activity in the auditory cortex.bioRxiv : the preprint server for biology · 2025Article
- Combinatorial transcriptional regulation establishes subtype-appropriate synaptic properties in auditory neurons.Cell reports · 2025Article
- Foxg1 gene mutation impairs auditory cortex response and reduces sound tolerance.Cerebral cortex (New York, N.Y. : 1991) · 2025Article
- Middle-aged CBA/CaJ mice exhibit auditory dysfunction in background noise.Hearing research · 2025Article
- Cdh23 Gene Mutation-Induced Vestibular Dysfunction in Mice: Abnormal Stereocilia Bundle and Otolith Development and Activation of p53/FoxO Signaling Pathway.Journal of molecular neuroscience : MN · 2025Article
- Mutations in unfolded protein response regulator ATF6 cause hearing and vision loss syndrome.The Journal of clinical investigation · 2025Article
- Limiting Hearing Loss in Transgenic Mouse Models.eNeuro · 2025Article
- Review
- A new mutation of Sgms1 causes gradual hearing loss associated with a reduced endocochlear potential.Hearing research · 2024Article
- Piplartine attenuates aminoglycoside-induced TRPV1 activity and protects from hearing loss in mice.Science translational medicine · 2024Article
- Mixed Representations of Sound and Action in the Auditory Midbrain.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2024Article
- Sst- and Vip-Cre mouse lines without age-related hearing loss.bioRxiv : the preprint server for biology · 2024Article
- Deafness causing neuroplastin missense variants fail to promote plasma membrane CaThe Journal of biological chemistry · 2024Article
- CDH23-Associated Usher Syndrome: Clinical Features, Retinal Imaging, and Natural History.Investigative ophthalmology & visual science · 2024Article
- The ENCODE mouse postnatal developmental time course identifies regulatory programs of cell types and cell states.bioRxiv : the preprint server for biology · 2024Article
54 more citing papers are in PubMed but not listed here.
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
Inbred strain variants of the Cdh23 gene have been shown to influence the onset and progression of age-related hearing loss (AHL) in mice. In linkage backcrosses, the recessive Cdh23 allele (ahl) of the C57BL/6J strain, when homozygous, confers increased susceptibility to AHL, while the dominant allele (Ahl+) of the CBA/CaJ strain confers resistance. To determine the isolated effects of these alleles on different strain backgrounds, we produced the reciprocal congenic strains B6.CBACa-Cdh23(Ahl)(+) and CBACa.B6-Cdh23(ahl) and tested 15-30 mice from each for hearing loss progression. ABR thresholds for 8 kHz, 16 kHz, and 32 kHz pure-tone stimuli were measured at 3, 6, 9, 12, 15 and 18 months of age and compared with age-matched mice of the C57BL/6J and CBA/CaJ parental strains. Mice of the C57BL/6N strain, which is the source of embryonic stem cells for the large International Knockout Mouse Consortium, were also tested for comparisons with C57BL/6J mice. Mice of the C57BL/6J and C57BL/6N strains exhibited identical hearing loss profiles: their 32 kHz ABR thresholds were significantly higher than those of CBA/CaJ and congenic strain mice by 6 months of age, and their 16 kHz thresholds were significantly higher by 12 months. Thresholds of the CBA/CaJ, the B6.CBACa-Cdh23(Ahl)(+), and the CBACa.B6-Cdh23(ahl) strain mice differed little from one another and only slightly increased throughout the 18-month test period. Hearing loss, which corresponded well with cochlear hair cell loss, was most profound in the C57BL/6J and C57BL/6NJ strains. These results indicate that the CBA/CaJ-derived Cdh23(Ahl)(+) allele dramatically lessens hearing loss and hair cell death in an otherwise C57BL/6J genetic background, but that the C57BL/6J-derived Cdh23(ahl) allele has little effect on hearing loss in an otherwise CBA/CaJ background. We conclude that although Cdh23(ahl) homozygosity is necessary, it is not by itself sufficient to account for the accelerated hearing loss of C57BL/6J mice.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.