A major gene affecting age-related hearing loss in C57BL/6J mice

Johnson, Kenneth R.; Erway, Lawrence C.; Cook, Susan A.; Willott, James F.; Zheng, Qing Yin

doi:10.1016/s0378-5955(97)00155-x

Cited by 340 publications

(268 citation statements)

References 32 publications

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“…VsEP amplitudes (at +6 dB re: 1.0 g/ms) ranged from 1.99 to 3.31 µV for P1-N1 and 0.25 to 2.69 µV for P2-N2 (Jones et al, 2005). These normative ranges were originally obtained from screening results on young C57BL/6J mice (35 days old), at an age where presumably there are not yet any structural deficits in the vestibular end organs (the C57BL/6J strain harbors a genetic mutation that leads to structural deficits with age; Johnson et al, 1997Johnson et al, ,2000. However, the normative range defined above is also consistent with VsEP results from two other inbred strains (CBA/CaJ and C3HeB/FeJ) that have no known structural deficits nor any known genetic mutations affecting the inner ear (Jones et al, 2005).…”

Section: Resultsmentioning

confidence: 99%

“…Several of the strains studied here carry genes that have been shown to contribute to or modify aging of hearing function including C57BL/6J, A/J, BALB/cByJ, BUB/BnJ, DBA/ 2J, NOD/LtJ, and SKH2/J (Johnson and Zheng, 2002;Johnson et al, 1997Johnson et al, , 2000NobenTrauth et al, 2003). We are currently evaluating similar questions for vestibular function and further are making a comparison between the two inner ear sensory systems in terms of functional senescence.…”

Section: Discussionmentioning

confidence: 99%

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A comparison of vestibular and auditory phenotypes in inbred mouse strains

Jones

Johnson

et al. 2006

Brain Research

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The purposes of this research were to quantify gravity receptor function in inbred mouse strains and compare vestibular and auditory function for strain-and age-matched animals. Vestibular evoked potentials (VsEPs) were collected for 19 inbred strains at ages from 35 to 389 days old. On average, C57BL/6J (35 to 190 days), BALB/cByJ, C3H/HeSnJ, CBA/J, and young LP/J mice had VsEP thresholds comparable to normal. Elevated VsEP thresholds were found for elderly C57BL/ 6J, NOD.NONH2 kb , BUB/BnJ, A/J, DBA/2J, NOD/LtJ, A/WySnJ, MRL/MpJ, A/HeJ, CAST/Ei, SJL/J, elderly LP/J, and CE/J. These results suggest that otolithic function varies among inbred strains and several strains displayed gravity receptor deficits by 90 days old. Auditory brainstem response (ABR) thresholds were compared to VsEP thresholds for 14 age-matched strains. C57BL/6J mice (up to 190 days) showed normal VsEPs with normal to mildly elevated ABR thresholds. Four strains (BUB/BnJ, NOD/LtJ, A/J, elderly LP/J) had significant hearing loss and elevated VsEP thresholds. Four strains (DBA/2J, A/WySnJ, NOD. NONH2 kb , A/HeJ) had elevated VsEP thresholds (including absent VsEPs) with mild to moderate elevations in ABR thresholds. Three strains (MRL/MpJ, Ce/J, SJL/J) had significant vestibular loss with no concomitant hearing loss. These results suggest that functional change in one sensory system does not obligate change in the other. We hypothesize that genes responsible for early onset hearing loss may affect otolithic function, yet the time course of functional change may vary. In addition, some genetic mutations may produce primarily gravity receptor deficits. Potential genes responsible for selective gravity receptor impairment demonstrated herein remain to be identified.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A comparison of vestibular and auditory phenotypes in inbred mouse strains

Jones

Johnson

et al. 2006

Brain Research

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“…The ahl locus, responsible for age-related hearing loss and increased susceptibility to noise-induced hearing loss in C57BL/6 mice, has been mapped to chromosome 10 (Erway et al 1996;Johnson et al 1997Johnson et al , 2000Davis et al 2001). The position of this locus overlaps that of Cdh23 and it has been proposed that they may be allelic .…”

Section: Age-related Hearing Lossmentioning

confidence: 99%

Progressive Hearing Loss and Increased Susceptibility to Noise-Induced Hearing Loss in Mice Carrying a Cdh23 but not a Myo7a Mutation

Holme

Steel

2004

JARO - Journal of the Association for Research in Otolaryngolog

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Exposure to intense noise can damage the stereocilia of sensory hair cells in the inner ear. Since stereocilia play a vital role in the transduction of sound from a mechanical stimulus into an electrical one, this pathology is thought to contribute to noise-induced hearing loss. Mice homozygous for null mutations in either the myosin VIIa (Myo7a) or cadherin 23 (Cdh23) genes are deaf and have disorganized stereocilia bundles. We show that mice heterozygous for a presumed null allele of Cdh23 (Cdh23 v ) have low-and high-frequency hearing loss at 5-6 weeks of age, the high-frequency component of which worsens with increasing age. We also show that noise-induced hearing loss in 11-12-week-old Cdh23 v heterozygotes is two times greater than for wild-type littermates. Interestingly, these effects are dependent upon the genetic background on which the Cdh23 v mutation is carried. Noise-induced hearing loss in 11-12-week-old mice heterozygous for a null allele of Myo7a (Myo7a 4626SB ) is not significantly different from wildtype littermates. CDH23 is the first gene known to cause deafness in the human population to be linked with predisposition to noise-induced hearing loss.

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“…The CBA/J mouse was chosen because it carries the ahl-resistant gene [5] thus not rendering it prone to accelerated premature hearing loss as, for example, C57/BL6 or BALB strains. The ages selected for the study represent distinct stages in auditory development and deterioration of the mouse [24].…”

Section: Introductionmentioning

confidence: 99%

Oxidative imbalance in the aging inner ear

Jiang

Talaska

Schacht

et al. 2007

Neurobiology of Aging

178

122

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The mammalian inner ear loses its sensory cells with advancing age, accompanied by a functional decrease in balance and hearing. This study investigates oxidant stress in the cochlea of aging male CBA/J mice. Glutathione-conjugated proteins, markers of H 2 O 2 -mediated oxidation, began to increase at 12 months of age; 4-hydroxynonenal and 3-nitrotyrosine, products of hydroxyl radical and peroxynitrite action, respectively, were elevated by 18 months. Immunoreactivity to these markers was stronger in the supporting cells (Deiters and pillar cells) than the sensory cells. It appeared later (23 months) in the nerve fibers of the spiral ganglion and in the stria vascularis and spiral ligament. Conversely, antioxidant proteins (AIF) and enzymes (SOD2) decreased by 18 months in the organ of Corti (including the sensory cells) and nerve fibers but not in the stria vascularis. These results suggest the presence of different reactive oxygen species and differential time courses of oxidative changes in individual tissues of the aging cochlea. An imbalance of redox status may be a component of age-related hearing loss.

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A major gene affecting age-related hearing loss in C57BL/6J mice

Cited by 340 publications

References 32 publications

A comparison of vestibular and auditory phenotypes in inbred mouse strains

A comparison of vestibular and auditory phenotypes in inbred mouse strains

Progressive Hearing Loss and Increased Susceptibility to Noise-Induced Hearing Loss in Mice Carrying a Cdh23 but not a Myo7a Mutation

Oxidative imbalance in the aging inner ear

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