hearing/vestibular/ear
• 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
• morphological defects of OHCs in adults are less severe than observed in constitutive Hoxb1-deficient (Hoxb1tm1.2Fmr) mice but statistically significant loss of OHCs and moderate OHC ciliar malformations are observed
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• at 3 months, threshold is pathologically elevated compared to the normal 40dB, but not as significantly as in Hoxb1tm1.2Fmr (null) mice
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nervous system
• 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
• morphological defects of OHCs in adults are less severe than observed in constitutive Hoxb1-deficient (Hoxb1tm1.2Fmr) mice but statistically significant loss of OHCs and moderate OHC ciliar malformations are observed
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• area of the ventral nucleus of the lateral lemniscus is reduced by about 40% compared to wild-type controls at P8
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• specification and innervation of olivocochlear neurons is abnormal, no medial olivocochlear neurons (MOC) 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 projections to the contralateral medial nucleus of the trapezoid body (cMNTB) from the posterior ventral cochlear nucleus (PVCN)
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