Analysis Tools
hearing/vestibular/ear
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• very few crossing medial olivocochlear (MOC) terminals are observed contacting outer hair cells in adult mice
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• at P8, no difference in shape or organization of outer hair cells (OHCs) is observed at the apical and basal turns relative to wild-type, but in adults (3 months), OHC rows are severely disorganized with loss of some hair cells in the apical turn of the cochlea; no inner hair cells (IHCs) are lost
• OHCs in the basal turns show only slight abnormalities in ciliar organization and orientation
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• at 3 months, threshold is elevated to 90 dB compared to the normal 40dB
• thresholds are elevated at all ages tested (from 1-12 months), increasing progressively to double the control level
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• mice raised in acoustic isolation display ABR threshold increases compared to controls, indicating that sensitivity to environmental sounds is not a significant factor
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nervous system
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• at P8, no difference in shape or organization of outer hair cells (OHCs) is observed at the apical and basal turns relative to wild-type, but in adults (3 months), OHC rows are severely disorganized with loss of some hair cells in the apical turn of the cochlea; no inner hair cells (IHCs) are lost
• OHCs in the basal turns show only slight abnormalities in ciliar organization and orientation
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• area of the ventral nucleus of the lateral lemniscus is severely reduced by about 90% compared to wild-type controls at P8
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• specification and innervation of olivocochlear neurons is abnormal, no medial olivocochlear (MOC) efferent neurons cross the midline at P8
• the cholinergic population of lateral olivocochlear (LOC) neurons is very small indicating defective specification
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• axon pathfinding defects are observed, with ectopic r4-derived projections crossing the midline and innervating the medial nucleus of the trapezoid body (MNTB)
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