Analysis Tools
hearing/vestibular/ear
|
• degeneration of most elements of the scala media complex is underway by three weeks of age
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|
• Dieters cell boundaries become indistinct and nuclei move to different levels by 10 days of age
|
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• by 9 months of age, the organ is a mass of undifferentiated cells, although the tunnel of Corti does not usually collapse
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• abnormalities can be detected at 10 days of age
|
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• the free end of the membrane curls up rather than resting on hair cells by 10 days
|
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• some degeneration is observed in most mice
• in severe cases, hair cells degenerate completely
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• in some mice, the otolith membrane degenerates completely and otoliths are lost
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behavior/neurological
head tossing
(
J:236
)
|
• tossing movement is observed in some mice although behavior is otherwise normal
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nervous system
| N |
• no abnormal central or peripheral nervous system phenotupes detected
|
|
• spiral ganglion degeneration begins at 50 days of age
• by 9 months, degeneration is nearly complete
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal dominant nonsyndromic deafness 36 | DOID:0110563 |
OMIM:606705 |
J:236 | |
| autosomal recessive nonsyndromic deafness 7 | DOID:0110520 |
OMIM:600974 |
J:236 | |
