hearing/vestibular/ear
N |
• normal spiral ganglion neuron (SGN) density
• normal shape and arrangement of stereocilia and number of synaptic ribbons at ages 2, 7 and 13 months
• normal number of inner and outer hair cells (IHCs, OHCs) at age 3 months and OHCs age 7 months
• normal auditory brainstem response (ABR) at 16 kHZ at age 3 months
• normal distortion product otoacoustic emissions (DPOAEs) at 4-32 kHz at ages 3 and 6 months
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• at age 7 months
• normal at age 3 months
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• progressively narrower apical surface area of cochlear glia-like supporting cells (GLSs), specifically on organ of Corti inner border cells, from age 7 months
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• at 16 kHz at age 5 months
• reduced suprathreshold amplitude of wave I at 8 kHz at age 6 months
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• at 16 and 32 kHz at age 6 months
• at all frequencies from age 9 months
• normal at 8 kHz at age 6 months
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• from age 6 months
• 63% reduction in passive conductance of isolated cochlear glia-like supporting cells (GLSs)
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nervous system
• at age 7 months
• normal at age 3 months
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cardiovascular system
N |
• no arrhythmogenic right ventricular cardiomyopathy (ARVC)
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