behavior/neurological
head shaking
(
J:13121
)
retropulsion
(
J:13121
)
• while circling
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• animals breed poorly chiefly because the mothers do not nurse well
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hearing/vestibular/ear
• supporting cells in mice 11 days of age show irregularities
(J:7124)
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• at birth, some hair cells display protrusion and folding of cuticular plates
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• at birth, the stereocilia and kinocilia have lost stiffness and are merged together
• at P12, the individual stereocilia are dwarfed and have lost stiffness
• at P36, only remnants of stereocilia remain
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• at P12, hair cell degeneration is uniform along the entire organ of Corti
(J:1646)
• at P12, the cuticular plate is disintegrating and very few sensory hair rootlets remain
(J:1646)
• degeneration of hair cells is first noted at P12
(J:7124)
• by P15, progressive scattered loss of hair cells is seen, as well as extensive patchy areas of more rapid degeneration in the middle and upper part of the basal half turn
(J:7124)
• by P30, the apical turn hair cells are degenerating in a patchy pattern with scattered hair cell loss
(J:7124)
• by P100, few recognizable hair cells remain in the basal turn and only a few cells are left in the middle half-turn; the apical turn has a few outer and inner hair cells remaining
(J:7124)
|
• the outer hair cells degenerate sooner than the inner hair cells
|
• at P81 and P108, pillar cells are collapsing
(J:1646)
• by P30, few recognizable pillar cells remain in the basal and middle turns
(J:7124)
• by P100, only a few pillar cells are left in the middle half turn and some also remain in the apical turn
(J:7124)
|
• at P81 and P108, the organ of Corti consists of a mass of disorganized cells
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• histological abnormalities are notable at P17
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• slight abnormality of the membrane seen 4 days after birth; membrane proteins are normal
(J:7124)
• the membrane is abnormally shaped
(J:13046)
|
• the membrane is not in contact with the hair cells of the organ of Corti
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• the hair cells appear short and stumpy; however, they appear healthy
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• at birth, some hair cell abnormalities are apparent
• by 2 weeks of age, hair cells are clearly degenerating in the saccular macula
• by P70, most hair cells have withdrawn from the otolithic memebrane and the cell cytoplasm is reduced to a minimum
• by P240, most of the hair cells have disappeared
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• hair cells persist up to 50 days of age, but by 3 months of age, the cells are abnormally shaped and have migrated to the otolithic membrane
• by 1 year of age, the hair cells persist but the supporting cell layer is disrupted
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• by P452, no trace of hair cells or supporting cells is seen in the saccular macula
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• at P240, the otolithic membrane appears disorganized
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• the resting EP is significantly higher in homozygous mutant mice compared to heterozygotes up to 30 days of age
• during induced anoxia, the EP value falls at a slower rate than controls and only reaches a less negative value compared to controls
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• no detectable cochlear microphonics (CM) could be recorded from the round window at 12-20 days of age
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• no ABR can be elicited at any age
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• no detectable action potential could be recorded from the round window at 12-20 days of age
|
nervous system
• at birth, some hair cells display protrusion and folding of cuticular plates
|
• at birth, the stereocilia and kinocilia have lost stiffness and are merged together
• at P12, the individual stereocilia are dwarfed and have lost stiffness
• at P36, only remnants of stereocilia remain
|
• at P12, hair cell degeneration is uniform along the entire organ of Corti
(J:1646)
• at P12, the cuticular plate is disintegrating and very few sensory hair rootlets remain
(J:1646)
• degeneration of hair cells is first noted at P12
(J:7124)
• by P15, progressive scattered loss of hair cells is seen, as well as extensive patchy areas of more rapid degeneration in the middle and upper part of the basal half turn
(J:7124)
• by P30, the apical turn hair cells are degenerating in a patchy pattern with scattered hair cell loss
(J:7124)
• by P100, few recognizable hair cells remain in the basal turn and only a few cells are left in the middle half-turn; the apical turn has a few outer and inner hair cells remaining
(J:7124)
|
• the outer hair cells degenerate sooner than the inner hair cells
|
• at birth, some hair cell abnormalities are apparent
• by 2 weeks of age, hair cells are clearly degenerating in the saccular macula
• by P70, most hair cells have withdrawn from the otolithic memebrane and the cell cytoplasm is reduced to a minimum
• by P240, most of the hair cells have disappeared
|
• no detectable cochlear microphonics (CM) could be recorded from the round window at 12-20 days of age
|
• no detectable action potential could be recorded from the round window at 12-20 days of age
|
• by P8-9, the cytoplasm of the ganglion cells has shrunk and the cells have irregular shapes
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• by P50, the number of cells in the spiral ganglion is reduced; these numbers reduce further with age
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