Phenotypes Associated with This Genotype

Genotype
MGI:5552006

• Allelic Composition: Tg(Pax2-cre)1Akg/0; Vangl2^tm1.2Mdea/Vangl2^tm2.1Mdea
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * SJL

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

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:201321 )

N • cochlear length and number of turns are normal

abnormal orientation of inner hair cell stereociliary bundles ( J:201321 )

• misorientated

abnormal orientation of outer hair cell stereociliary bundles ( J:201321 )

• misorientated

abnormal Deiters cell morphology ( J:201321 )

• shifted out of the second and third outer hair cell rows and not centrally positioned between the hair cell lateral edges

abnormal pillar cell morphology ( J:201321 )

• at P10 and P12, more pronounced in the apical turn

• more compact and incorrectly positioned compared with control mice

abnormal auditory brainstem response ( J:201321 )

• reduced ABR amplitude

• however, the overall shape of the waveform is normal

increased or absent threshold for auditory brainstem response ( J:201321 )

• mild

abnormal distortion product otoacoustic emission ( J:201321 )

• decreased amplitude of the distortion product

nervous system

abnormal orientation of inner hair cell stereociliary bundles ( J:201321 )

• misorientated

abnormal orientation of outer hair cell stereociliary bundles ( J:201321 )

• misorientated