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Genetics of Age-related Hearing Loss

Description: Presbycusis or age-related hearing loss (AHL) is the most common sensory deficit in aging human populations and a major factor in reducing the quality of life of the elderly. Genetic studies in humans are difficult because of the late onset of the condition and because non-genetic factors such as noise trauma, disease, and ototoxic drugs confound interpretations. AHL is common in inbred mouse strains, and mice are more amenable to detailed genetic and pathological analyses, which could add significantly to our understanding of presbycusis in humans.
Funding type: research
Funder scheme: R01
Start date: 2003-05-05
End date: 2017-11-30
Investigator: KENNETH R JOHNSON
Institution: JACKSON LABORATORY
Subfield: Sensory Systems
Funded outputs count: 27
Funder award ID: R01DC005827
OpenAlex ID: G6429545595
Effects of Cdh23 single nucleotide substitutions on age-related hearing loss in C57BL/6 and 129S1/Sv mice and comparisons with congenic strains
2017 · Kenneth R. Johnson, Cong Tian, et al. · Scientific Reports · 77
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Application of Mouse Models to Research in Hearing and Balance
2016 · Kevin K. Ohlemiller, Sherri M. Jones, et al. · Journal of the Association for Research in Otolaryngology · 122
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N-acetyl-cysteine prevents age-related hearing loss and the progressive loss of inner hair cells in γ-glutamyl transferase 1 deficient mice
2016 · Dalian Ding, Haiyan Jiang, et al. · Aging · 66
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A QTL on Chr 5 modifies hearing loss associated with the fascin-2 variant of DBA/2J mice
2015 · Kenneth R. Johnson, Chantal M. Longo-Guess, et al. · Mammalian Genome · 11
β-Actin and Fascin-2 Cooperate to Maintain Stereocilia Length
2013 · Benjamin J. Perrin, Dana M. Strandjord, et al. · Journal of Neuroscience · 77
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Genetic background effects on age-related hearing loss associated with Cdh23 variants in mice
2011 · Kelly L. Kane, Chantal M. Longo-Guess, et al. · Hearing Research · 184
Association of a citrate synthase missense mutation with age-related hearing loss in A/J mice
2011 · Kenneth R. Johnson, Leona H. Gagnon, et al. · Neurobiology of Aging · 51
The R109H Variant of Fascin-2, a Developmentally Regulated Actin Crosslinker in Hair-Cell Stereocilia, Underlies Early-Onset Hearing Loss of DBA/2J Mice
2010 · J.-B. Shin, C. M. Longo-Guess, et al. · Journal of Neuroscience · 118
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Separate and combined effects of Sod1 and Cdh23 mutations on age-related hearing loss and cochlear pathology in C57BL/6J mice
2010 · Kenneth R. Johnson, Heping Yu, et al. · Hearing Research · 56
Inheritance patterns of progressive hearing loss in laboratory strains of mice
2009 · Konrad Noben-Trauth, Kenneth R. Johnson · Brain Research · 47
A locus on distal chromosome 11 (ahl8) and its interaction with Cdh23ahl underlie the early onset, age-related hearing loss of DBA/2J mice
2008 · Kenneth R. Johnson, Chantal Longo-Guess, et al. · Genomics · 72
A locus on distal chromosome 10 (ahl4) affecting age-related hearing loss in A/J mice
2008 · Qing Yin Zheng, Dalian Ding, et al. · Neurobiology of Aging · 70
Effects of antioxidants on the aging inner ear
2006 · Tima Le, Elizabeth M. Keithley · Hearing Research · 89
Strain background effects and genetic modifiers of hearing in mice
2006 · Kenneth R. Johnson, Qing Yin Zheng, et al. · Brain Research · 141
Cu/Zn superoxide dismutase and age-related hearing loss
2005 · Elizabeth M. Keithley, Cecilia Canto, et al. · Hearing Research · 137
Alms1-disrupted mice recapitulate human Alström syndrome
2005 · G.B. Collin, E. Cyr, et al. · Human Molecular Genetics · 181
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The Mass1frings mutation underlies early onset hearing impairment in BUB/BnJ mice, a model for the auditory pathology of Usher syndrome IIC
2005 · K.R. Johnson, Q.Y. Zheng, et al. · Genomics · 56
Expression of Type IV Pili by Moraxella catarrhalis Is Essential for Natural Competence and Is Affected by Iron Limitation
2004 · Nicole R. Luke, Amy J. Howlett, et al. · Infection and Immunity · 78
Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss
2003 · Konrad Noben-Trauth, Qing Yin Zheng, et al. · Nature Genetics · 479
Ahl2, a Second Locus Affecting Age-Related Hearing Loss in Mice
2002 · Kenneth R. Johnson, Qing Yin Zheng · Genomics · 92
Fierce: a new mouse deletion of Nr2e1; violent behaviour and ocular abnormalities are background-dependent
2002 · Kelly A Young, Melissa L Berry, et al. · Behavioural Brain Research · 122
Microtubule-associated protein 1A is a modifier of tubby hearing (moth1)
2002 · Akihiro Ikeda, Qing Yin Zheng, et al. · Nature Genetics · 100
Hearing loss associated with the modifier of deaf waddler (mdfw) locus corresponds with age-related hearing loss in 12 inbred strains of mice
2001 · Qing Yin Zheng, Kenneth R Johnson · Hearing Research · 59
A nuclear-mitochondrial DNA interaction affecting hearing impairment in mice
2001 · Kenneth R. Johnson, Qing Y Zheng, et al. · Nature Genetics · 157
Oxidative Stress in Aging in the C57B16/J Mouse Cochlea
2001 · Hinrich Staecker, Qing Yin Zheng, Thomas R. · Acta Oto-Laryngologica · 106
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