Phenotypes Associated with This Genotype

Genotype
MGI:4436730

• Allelic Composition: Ush1c^dfcr/Ush1c^dfcr
• Genetic Background: involves: BALB/cBySmn * C57BL/6 * C57BL/Ka

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

hearing/vestibular/ear

abnormal cochlear hair bundle tip links morphology ( J:157359 )

• hair bundles in the cochlea lack upper tip-link densities but do contain tip links

organ of Corti degeneration ( J:157359 )

• degenerative changes in the organ of Corti are evident in mice over a few months in age

abnormal hair cell mechanoelectric transduction ( J:157359 )

• resting Po and adaptation time constants are significantly reduced and slow adaptation is significantly slowed compared to wild-type

• the mean Tfast in hair cells is nearly doubled

• activation of transducer currents at similar Po was significantly slowed

• activation time constants and resting Po is reduced

• both the kinetics of channel activation and adaptation are slowed

increased or absent threshold for auditory brainstem response ( J:157359 )

• by 4 weeks of age, auditory thresholds are greater than 90 dB

deafness ( J:157359 )

• by 4 weeks of age

nervous system

abnormal cochlear hair bundle tip links morphology ( J:157359 )

• hair bundles in the cochlea lack upper tip-link densities but do contain tip links

abnormal hair cell mechanoelectric transduction ( J:157359 )

• resting Po and adaptation time constants are significantly reduced and slow adaptation is significantly slowed compared to wild-type

• the mean Tfast in hair cells is nearly doubled

• activation of transducer currents at similar Po was significantly slowed

• activation time constants and resting Po is reduced

• both the kinetics of channel activation and adaptation are slowed

cochlear ganglion degeneration ( J:157359 )

• degenerative changes in the organ of Corti affecting the spiral ganglion neurons are evident in mice over a few months of age