behavior/neurological
• inability to swim
|
head shaking
(
J:98396
)
• shaking from side to side
|
hearing/vestibular/ear
• scanning electron microscopy analysis showed that inner and outer hair cells appear normal at birth
|
• by P8, inner hair cells appear disorganized with loss of typical cell morphology
• by P50, the inner hair cells are severely splayed
|
• by P8, outer hair cells appear disorganized with loss of typical cell morphology
• by P50, outer hair cells are absent, with empty patches appearing the basal portion of the cochlea
|
• degeneration was more pronounced in the basal region than in the apex
|
• ABR threshold testing showed that no mutant mouse tested exhibited a response to auditory stimuli up to 110 dB sound pressure level
|
nervous system
• scanning electron microscopy analysis showed that inner and outer hair cells appear normal at birth
|
• by P8, inner hair cells appear disorganized with loss of typical cell morphology
• by P50, the inner hair cells are severely splayed
|
• by P8, outer hair cells appear disorganized with loss of typical cell morphology
• by P50, outer hair cells are absent, with empty patches appearing the basal portion of the cochlea
|
• described as decreased spiral ganglion
|