hearing/vestibular/ear
N |
• thresholds and amplitudes of distortion product otoacoustic emissions (DPOAEs)
• morphology of inner hair cells and their afferent synapses
|
• increased inactivation of voltage-gated type 1.3 Ca2+ channels in mature IHCs of cochlear apical coil in vitro at age 5 weeks, using long (500 ms) step depolarizations or trains of brief (10 ms with 5 ms interval) depolarizations
|
• reduced firing rates and delayed and more variable spike timing of spinal ganglion neurons (SGNs) at sound onset
|
• reduced amplitude of response to 80 dB click stimuli at 20 Hz rate at age P49-P63
|
• increased in 6-24 kHz frequency range at age P49-P63
|
nervous system
• increased inactivation of voltage-gated type 1.3 Ca2+ channels in mature IHCs of cochlear apical coil in vitro at age 5 weeks, using long (500 ms) step depolarizations or trains of brief (10 ms with 5 ms interval) depolarizations
|
mortality/aging
N |
• viable
|
reproductive system
N |
• fertile
|
vision/eye
N |
• intact retinal processing according to scotopic electroretinography (ERG) readings
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
autosomal recessive nonsyndromic deafness 93 | DOID:0110537 |
OMIM:614899 |
J:255028 |