Analysis Tools
hearing/vestibular/ear
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• homozygotes show severe malformations of the inner ear semicircular canals
• however, young homozygotes (4-6 months) display no hearing deficits, as shown by a normal Preyer's reflex
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• at 3 weeks, homozygotes exhibit complete lack of the lateral semicircular canal
• the crista of the lateral semicircular duct is never formed
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• at 3 weeks, homozygotes exhibit posterior semicircular canal (PSC) defects ranging from complete absence to partial presence
• the degree of PSC dysgenesis is variable among individual mutants and between both ears within individuals
• no relationship could be established between phenotypic penetrance and genetic background
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• at 3 weeks, most homozygotes exhibit normal evagination of the anterior semicircular canal (ASC); however, the mutant ASC is significantly reduced in size
• the degree of ASC dysgenesis is variable among individual mutants and between both ears within individuals
• no relationship could be established between phenotypic penetrance and genetic background
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• homozygotes lack major parts of the vestibular apparatus
• in contrast, no apparent alterations in the cochlea or endolymphatic duct are observed
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behavior/neurological
hyperactivity
(
J:45576
)
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• at ~3 weeks after birth, homozygotes start to exhibit hyperactivity
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• at ~3 weeks after birth, homozygotes start to exhibit abnormal circling movements
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growth/size/body
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• homozygotes are viable, fertile and show no overt morphological anomalies, except for a slight but significant growth retardation
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