Analysis Tools
behavior/neurological
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• homozygous mutant male mice caged together exhibit heightened inter-male aggression, fighting with and inflicting wounds on each other
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• at 8 weeks of age, mutant mice exhibit no acoustic startle response to a 120 dB sound
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• mutant mice perform poorly in the lateral beam test
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head tossing
(
J:137493
)
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• this behavior is suppressed when the mice are sleeping or nursing
• mutant mice exhibit repetitive brief backward bending of the neck so that the head's long axis is vertical ("stargazing")
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• this behavior is suppressed when the mice are sleeping or nursing
• evident by three weeks of age
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• this behavior is suppressed when the mice are sleeping or nursing
• evident by three weeks of age
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hearing/vestibular/ear
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• revealed by scanning electron microscopy in four day old mice
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• progressive loss of inner hair cells (IHC)
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• progressive loss of outer hair cells
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• mutant mice exhibit progressive degeneration of the organ of Corti
• in five day old mice the inner ear appears histologically normal by light microscopy, but by 8 weeks severe degenerative changes are evident
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• 8 week old mutant mice exhibit no acoustic brainstem response (ABR) at 90 dB, vs. an ABR threshold of ~40 dB in wild-type control mice
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• the DPOAE of 8 week old mutant mice in response to stimuli at 0-70 dB and 6-28 kHz is within noise level
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nervous system
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• the cerebra of mutant mice appear histologically normal by light microscopy
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• revealed by scanning electron microscopy in four day old mice
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• progressive loss of inner hair cells (IHC)
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• progressive loss of outer hair cells
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• the density of spiral ganglion neurons appears normal in four day old mice, but has decreased by 8 weeks
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growth/size/body
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• homozygous mutant mice are leaner than their unaffected littermates; this may be due to their higher activity level
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive nonsyndromic deafness 63 | DOID:0110515 |
OMIM:611451 |
J:137493 | |
