Analysis Tools
mortality/aging
hearing/vestibular/ear
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• at 4 weeks of age, stereocilia of inner hair cells vary in length and compactness, by 8 weeks of age, many stereocilia appear to collapse against their nearest neighbors
• stereocilia are thicker at the base than in the middle or tips
• the few remaining inner hair cell stereocilia are arranged in a single row rather than 3 rows
• outer hair cell stereocilia show loss of structural integrity, particularly those in the outermost row
• distortion product otoacoustic emissions at 24 weeks of age show no difference in response at 12 kHz, indicating preservation of outer hair cell function
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• by 16 weeks, stereocilia are elongated and adhere to their neighbors, and at 24 weeks, stereocilia elongate even further and there are fewer stereocilia per inner hair cell
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• number of stereocilia per inner hair cell is reduced to approximately 5 compared to several dozen in wild-type mice at 24 weeks of age, with apparent fusion of the membrane surrounding them
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• inner hair cell ribbon loss is notable at 16 weeks and progress to 46% loss at 24 weeks
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• mice exhibit higher thresholds indicating that higher sound pressure levels are required to elicit a brainstem response
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• mild 20 dB hearing loss at 12 kHz at 4 and 8 weeks of age that progresses to 40 dB at 16 weeks and 60 dB at 24 weeks at both 12 and 24 Hz
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cardiovascular system
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• cardiac defects
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nervous system
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• at 4 weeks of age, stereocilia of inner hair cells vary in length and compactness, by 8 weeks of age, many stereocilia appear to collapse against their nearest neighbors
• stereocilia are thicker at the base than in the middle or tips
• the few remaining inner hair cell stereocilia are arranged in a single row rather than 3 rows
• outer hair cell stereocilia show loss of structural integrity, particularly those in the outermost row
• distortion product otoacoustic emissions at 24 weeks of age show no difference in response at 12 kHz, indicating preservation of outer hair cell function
|
|
• by 16 weeks, stereocilia are elongated and adhere to their neighbors, and at 24 weeks, stereocilia elongate even further and there are fewer stereocilia per inner hair cell
|
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• number of stereocilia per inner hair cell is reduced to approximately 5 compared to several dozen in wild-type mice at 24 weeks of age, with apparent fusion of the membrane surrounding them
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• inner hair cell ribbon loss is notable at 16 weeks and progress to 46% loss at 24 weeks
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behavior/neurological
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• mice do not show circling behavior
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal dominant auditory neuropathy 1 | DOID:0060690 |
OMIM:609129 |
J:193237 | |
