hearing/vestibular/ear
N |
• homozygotes are viable with a normal Preyer's reflex and no gross deficits in balance or movement
(J:55406)
• no structural abnormalities in cochlear hair cells, supporting cells or spiral ganglion neurons are observed
(J:55406)
• in addition, development and position of the olivocochlear bundle remains unchanged
(J:55406)
• in vitro, mutant apical OHCs exhibit normal electromotility in response to a 100-Hz sinusoidal voltage command relative to wild-type OHCs
(J:100073)
|
• unlike wild-type mice, homozygotes appear to lack a sparse plexus of efferent terminals in close apposition to the IHC somata
• however, a dense plexus of terminals is still present below the IHCs
|
• most efferent terminals under mutant OHCs occur as large singlets rather than as clusters of roughly equal-size terminals
• most mutant OHCs are contacted by only one terminal (66.7%) and very few by >2 terminals, whereas 61.8% of wild-type OHCs are contacted by two terminals
|
• at 6-24 weeks, homozygotes fail to show suppression of compound action potential (CAP) amplitudes during efferent fiber activation by olivocochlear (OC) shocks
(J:55406)
• however, in the absence of crossing OC bundle electrical stimulation, homozygotes display normal CAP thresholds at 16 kHz relative to wild-type mice
(J:55406)
• also, at 4-9 weeks, homozygotes show normal mean CAP threshold curves over the entire range of mouse hearing (between 1.7 and 50 kHz) relative to 129S6/SvEvTac control mice
(J:100073)
|
• homozygotes fail to show suppression of distortion product otoacoustic emissions (DPOAEs) during efferent fiber activation by olivocochlear (OC) shocks
• however, in the absence of OC bundle electrical stimulation, homozygotes display normal DPOAEs relative to wild-type mice
|
nervous system
• at 6-24 weeks, homozygotes fail to show suppression of compound action potential (CAP) amplitudes during efferent fiber activation by olivocochlear (OC) shocks
(J:55406)
• however, in the absence of crossing OC bundle electrical stimulation, homozygotes display normal CAP thresholds at 16 kHz relative to wild-type mice
(J:55406)
• also, at 4-9 weeks, homozygotes show normal mean CAP threshold curves over the entire range of mouse hearing (between 1.7 and 50 kHz) relative to 129S6/SvEvTac control mice
(J:100073)
|
• at 6-24 weeks, homozygotes exhibit abnormal efferent innervation in both OHC and IHC regions of the cochlea
|
• unlike wild-type mice, homozygotes appear to lack a sparse plexus of efferent terminals in close apposition to the IHC somata
• however, a dense plexus of terminals is still present below the IHCs
|
• most efferent terminals under mutant OHCs occur as large singlets rather than as clusters of roughly equal-size terminals
• most mutant OHCs are contacted by only one terminal (66.7%) and very few by >2 terminals, whereas 61.8% of wild-type OHCs are contacted by two terminals
|