Analysis Tools
behavior/neurological
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• at P30, homozygotes fail to exhibit a startle response in a click-box test, unlike wild-type controls
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head tossing
(
J:220419
)
hearing/vestibular/ear
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• homozygotes display structurally normal middle ear bones
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• SEM at ages P15-P90 revealed loss of outer hair cells (OHCs), evident from P30 onwards
• by P45, most OHCs have degenerated in the base and middle cochlear regions
• the innermost row of OHCs, closest to the inner hair cells (IHCs), appears to be affected first and most severely
• in contrast, IHCs remain morphologically normal through P90
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• H&E staining revealed that the stria vascularis is detached from the spiral ligament
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• at P0, paint-filled inner ears display shortening of the mutant cochlear duct by almost one turn, similar to a Mondini malformation of the bony labyrinth
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• homozygotes have an auditory threshold at a broadband sound stimulus above 90dB, whereas wild-type mice have a threshold of 30dB (+/-5dB)
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• homozygotes show severe vestibular dysfunction in a battery of vestibular tests, including contact righting, tail hang test, observation of circling behavior and swim test
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homeostasis/metabolism
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• high plasma potassium values (equal to or higher than 5.6 mmol/l) are, in most cases, associated with hearing impairment, head-tossing and occasional circling behavior
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nervous system
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• SEM at ages P15-P90 revealed loss of outer hair cells (OHCs), evident from P30 onwards
• by P45, most OHCs have degenerated in the base and middle cochlear regions
• the innermost row of OHCs, closest to the inner hair cells (IHCs), appears to be affected first and most severely
• in contrast, IHCs remain morphologically normal through P90
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