Analysis Tools
hearing/vestibular/ear
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• elevated threshold and reduced amplitudes
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Allele Symbol Allele Name Allele ID |
Myo6+ wild type MGI:2433285 |
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| Summary |
5 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• elevated threshold and reduced amplitudes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mutant mice lose the Preyer reflex between 4.5 and 10 months of age
• mutant and control mice show a robust reflex at 2.5 months
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• mice show an abnormal reaching response when suspended by tail in air at 2.5 months of age
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• significant deterioration at 4.5 months of age
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• headtossing begins at age 4-6 weeks
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• the mutant cochleas appear very immature
• a developmental delay is apparent in stereocilia
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• abnormal development of stereocilla in the cochlea is detectable as early as birth
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• reported degeneration of outer and inner hair cells
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• reported degeneration of vestibular hair cells
• the saccular and utricular otoliths are present and normal
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• the mutant cochleas appear very immature
• a developmental delay is apparent in stereocilia
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• abnormal development of stereocilla in the cochlea is detectable as early as birth
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• reported degeneration of outer and inner hair cells
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• reported degeneration of vestibular hair cells
• the saccular and utricular otoliths are present and normal
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice show mild behavioral anomalies around 5 months of age that deteriorate irreversibly to severe balance problems
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• most hair bundles anchored on remaining outer hair cells and inner hair cells retain normal morphology, but some are disorganized with fused stereocilia and rotated polarity
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• some hair bundles show rotated polarity
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• minor hair cell degeneration is seen on the basilar membrane at 3 weeks of age that progresses over time such that severe hair cell loss is seen at 9 weeks after birth
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• mice exhibit elevated ABR hearing thresholds at 3 weeks of age which progress irreversibly over time
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• most hair bundles anchored on remaining outer hair cells and inner hair cells retain normal morphology, but some are disorganized with fused stereocilia and rotated polarity
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• some hair bundles show rotated polarity
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• minor hair cell degeneration is seen on the basilar membrane at 3 weeks of age that progresses over time such that severe hair cell loss is seen at 9 weeks after birth
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal dominant nonsyndromic deafness 22 | DOID:0110552 |
OMIM:606346 |
J:288210 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• described as short ears
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• described as short ears
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• described as short ears
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• slightly increased risk of age related hearing loss
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 06/12/2024 MGI 6.13 |
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