Analysis Tools
hearing/vestibular/ear
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• at 14 weeks, homozygotes exhibit severe OHC degeneration in the basal and mid-basal cochlear turns
(J:92443)
• at 10 weeks, severe loss of OHCs occurs exclusively in high frequency (HF; midbasal) cochlear turns, whereas low frequency (LF; apical) OHCs remain histologically intact
(J:117764)
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• increased apoptosis in Deiters cells at 14 weeks of age
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• at >8 weeks, lack of distortion product otoacoustic emissions suggests OHC dysfunction
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• at 7 days after exposure to low frequency (LF) band-pass noise (4-8 kHz), 4-5 wk-old homozygotes, but not wild-type mice, exhibit a permanent hearing loss, as validated by click-ABR thresholds
• noise-exposed homozygotes develop a significant hearing loss between 8 and 16 kHz, as validated by frequency-specific ABR thresholds
• in homozygotes, the LF band-pass noise-induced threshold shift is accompanied by a specific loss of KCNQ4 exclusively in medial turns, whereas apical and midbasal/basal turns appear normal
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• at >8 weeks, homozygotes exhibit a slowly progressing high-frequency hearing loss
• no obvious hearing deficits are detected during the first 4 postnatal weeks
• hearing loss is linked to loss of the KCNQ4 potassium channel in OHC membranes in the basal and midbasal cochlear turns, precedes OHC degeneration, and resembles pharmacologic blockade of KCNQ4 channels
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nervous system
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• at 14 weeks, homozygotes exhibit severe OHC degeneration in the basal and mid-basal cochlear turns
(J:92443)
• at 10 weeks, severe loss of OHCs occurs exclusively in high frequency (HF; midbasal) cochlear turns, whereas low frequency (LF; apical) OHCs remain histologically intact
(J:117764)
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cellular
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• in Deiters cells at 14 weeks of age
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