behavior/neurological
head bobbing
(
J:99481
)
hyperactivity
(
J:99481
)
hearing/vestibular/ear
N |
• positive Preyer reflex to clickbox indicates heterozygotes are not profoundly deaf
|
• the round window is smaller or entirely absent
|
• the shape of the stapes varies greatly and is only occassionally normal
|
• additonal inner hair cells are found
|
• patches of an extra row of outer hair cells are frequent
|
• incomplete expressivity such that some inner ears lack semicircular canal whereas it is truncated in others
|
• majority of heterozygotes have truncated lateral semicircular canal rather than complete absence and ampulla is present
|
• thinning of the superior canal is occassionally seen but no truncation
|
nervous system
• additonal inner hair cells are found
|
• patches of an extra row of outer hair cells are frequent
|
skeleton
N |
• malleus and incus appear normal
|
• the round window is smaller or entirely absent
|
• the shape of the stapes varies greatly and is only occassionally normal
|
craniofacial
• the round window is smaller or entirely absent
|
• the shape of the stapes varies greatly and is only occassionally normal
|