Phenotypes Associated with This Genotype

Genotype
MGI:6160071

• Allelic Composition: Myo6^rsv/Myo6^rsv
• Genetic Background: involves: C57BL/6Slc

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

hearing/vestibular/ear

abnormal cochlear hair cell stereociliary bundle morphology ( J:261136 )

• stereocilia bundles become progressively more disorganized from P0 to P5

• practically all show a fused-like morphology by P10 and long and disrupted stereocilia are seen at P24

• at P27 there is excess growth and fusion of stereocilia resulting in large protrusions on top of hair cells, especially in inner hair cells

abnormal inner hair cell stereociliary bundle morphology ( J:261136 )

• have a swirled appearance at P0

fused inner hair cell stereocilia ( J:261136 )

• practically all show a fused-like morphology by P10

fused outer hair cell stereocilia ( J:261136 )

• practically all show a fused-like morphology by P10

cochlear hair cell degeneration ( J:261136 )

cochlear inner hair cell degeneration ( J:261136 )

• at P56

• degeneration is more pronounced in the base area than in the middle area at P0

cochlear outer hair cell degeneration ( J:261136 )

• at P56

• degeneration is more pronounced in the base area than in the middle area at P0

organ of Corti degeneration ( J:261136 )

• severe degeneration at P56

abnormal vestibular hair cell stereociliary bundle morphology ( J:261136 )

• stereocilia bundles on hair cells from utricles lack the normal staircase configuration

increased or absent threshold for auditory brainstem response ( J:261136 )

• absent at all intensities tested up to 90 dBpeSPL

behavior/neurological

absent pinna reflex ( J:261136 )

impaired balance ( J:261136 )

• at P10

head tossing ( J:261136 )

• at P20

bidirectional circling ( J:261136 )

• at P20

nervous system

abnormal cochlear hair cell stereociliary bundle morphology ( J:261136 )

• stereocilia bundles become progressively more disorganized from P0 to P5

• practically all show a fused-like morphology by P10 and long and disrupted stereocilia are seen at P24

• at P27 there is excess growth and fusion of stereocilia resulting in large protrusions on top of hair cells, especially in inner hair cells

abnormal inner hair cell stereociliary bundle morphology ( J:261136 )

• have a swirled appearance at P0

fused inner hair cell stereocilia ( J:261136 )

• practically all show a fused-like morphology by P10

fused outer hair cell stereocilia ( J:261136 )

• practically all show a fused-like morphology by P10

cochlear hair cell degeneration ( J:261136 )

cochlear inner hair cell degeneration ( J:261136 )

• at P56

• degeneration is more pronounced in the base area than in the middle area at P0

cochlear outer hair cell degeneration ( J:261136 )

• at P56

• degeneration is more pronounced in the base area than in the middle area at P0

abnormal vestibular hair cell stereociliary bundle morphology ( J:261136 )

• stereocilia bundles on hair cells from utricles lack the normal staircase configuration

cochlear ganglion degeneration ( J:261136 )

• at P56