Analysis Tools
hearing/vestibular/ear
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• stereocilia are irregular at 3 weeks of age, are disrupted at 8 and 12 weeks of age, and are completely lost at 24 weeks of age
• stereocilia bundles are much less stiff at 8 and 12 weeks of age
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• stereocilia bundles are much shorter at 8 and 12 weeks of age
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• mice show progressive degeneration of hair cells in the cochlea, showing hair cell loss in the basal turns of the cochlea as early as 6 weeks of age, spreading to the middle and apical turns at 9 and 16 weeks of age, respectively, and showing more than 80% outer hair cell loss in the basal turns by 32 weeks of age and in the middle and apical turns at 40 weeks of age
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• DPOAE indicates early progressive functional impairment of outer hair cells
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• ABR thresholds increase with age from 3 to 24 weeks and reach a plateau of about 100 dB SPL at 32, 40, and 52 weeks of age at all stimulus frequencies
• ABR thresholds increase at stimulus frequencies of 16 and 32 kHz by 3 weeks of age, indicating early onset of hearing loss at high frequencies
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• distortion produce otoacoustic emission (DPOAE) amplitudes at 3 weeks of age are lower at f2 frequencies from 8844 to 35,444 Hz, especially at 17,672 Hz, and decline rapidly and become negative at 6 weeks of age at f2 frequency of 17,672
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• mice exhibit early onset of progressive hearing loss starting at 3 weeks of age and leading to near deafness at 24 weeks of age
• hearing impairment is more sensitive to pure tone stimuli at high frequencies than to those at low frequency
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vision/eye
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• the inner segments are thinner at 8, 16, and 24 weeks of age
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• the outer segments are thinner at 8, 16, and 24 weeks of age
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• the outer nuclear layer is thinner at 8, 16, and 24 weeks of age
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• mice exhibit thinner retinas at 8, 16, and 24 weeks of age
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• amplitude of dark-adapted a-waves in standard combined ERGs are lower or diminished at 4, 8, 12, and 24 weeks of age
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• amplitude of dark-adapted b-waves in standard combined ERGs are lower or diminished at 4, 8, 12, and 24 weeks of age
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• progressive vision impairment
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nervous system
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• stereocilia are irregular at 3 weeks of age, are disrupted at 8 and 12 weeks of age, and are completely lost at 24 weeks of age
• stereocilia bundles are much less stiff at 8 and 12 weeks of age
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• stereocilia bundles are much shorter at 8 and 12 weeks of age
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• mice show progressive degeneration of hair cells in the cochlea, showing hair cell loss in the basal turns of the cochlea as early as 6 weeks of age, spreading to the middle and apical turns at 9 and 16 weeks of age, respectively, and showing more than 80% outer hair cell loss in the basal turns by 32 weeks of age and in the middle and apical turns at 40 weeks of age
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• DPOAE indicates early progressive functional impairment of outer hair cells
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• the inner segments are thinner at 8, 16, and 24 weeks of age
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• the outer segments are thinner at 8, 16, and 24 weeks of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| auditory system disease | DOID:2742 | J:274170 | ||
| retinal degeneration | DOID:8466 | J:274170 | ||
