Analysis Tools
hearing/vestibular/ear
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• reduction in number of outer hair cells
• however, mice show normally developed and patterned cochlea
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• outer hair cells show signs of degeneration with loss of outer hair cells by P90
• gradient of outer hair cell loss from apex to base with normal outer hair cell numbers in the apex and increasing hair cell loss from medial to basal turns of the cochlea at P30
• however, inner hair cell counts are normal at P15 and P30
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• missing or pyknotic supporting cells in the inner ears
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• general atrophy of the organ of Corti
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• degeneration of the spiral ligament
• however, organ of Corti develops with all cell types present
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• detached stria vascularis
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• intermediate cells in the stria vascularis are deficient
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• reduction in thickness and total strial area are seen at the base, mid-turn and apex of the cochlea
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• mice show a reduction in endocochlear potential at P60
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• mice show reduced distortion-product otoacoustic emissions (DPOAEs) at 8 weeks
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• mice show profound hearing loss at 4 weeks of age which is unchanged at 8 and 25 weeks
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nervous system
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• reduction in number of outer hair cells
• however, mice show normally developed and patterned cochlea
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• outer hair cells show signs of degeneration with loss of outer hair cells by P90
• gradient of outer hair cell loss from apex to base with normal outer hair cell numbers in the apex and increasing hair cell loss from medial to basal turns of the cochlea at P30
• however, inner hair cell counts are normal at P15 and P30
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pigmentation
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• intermediate cells in the stria vascularis are deficient
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skeleton
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• degeneration of the spiral ligament
• however, organ of Corti develops with all cell types present
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behavior/neurological
| N |
• no circling or head-bobbing and normal vestibular function
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive nonsyndromic deafness 39 | DOID:0110497 |
OMIM:608265 |
J:289982 | |
