behavior/neurological
head shaking
(
J:67728
)
• subtle headweaving/shaking behavior
|
• described as poor negative geotaxis
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hearing/vestibular/ear
• supernumary or atypical outer hair cells sometimes found in inner hair cell rows
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• reduced numbers of outer hair cells
• some regions lacking entire rows while isolated hair cells missing in other regions
• decreased outer hair cell counts in both basal and apical turns
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• significantly increased inner hair cell counts in base of cochlea but not apical regions
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• either posterior or anterior semicircular canal truncated
• sometimes both anterior and posterior semicircular canals absent
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• absence of ampullae
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nervous system
• reduced numbers of outer hair cells
• some regions lacking entire rows while isolated hair cells missing in other regions
• decreased outer hair cell counts in both basal and apical turns
|
• significantly increased inner hair cell counts in base of cochlea but not apical regions
|