Phenotypes Associated with This Genotype

Genotype
MGI:5898367

• Allelic Composition: Cdh23^ahl/Cdh23^ahl; Mahl^C57BL/6J/Mahl^C57BL/6J
• Genetic Background: 129S1.B6-(rs3696307-rs257098870)/Kjn

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

hearing/vestibular/ear

cochlear hair cell degeneration ( J:241137 )

• progressive inner and outer hair cell loss occur as a result of the age related hearing loss mutation, but in the presence of the ameliorating C57BL/6J allele of Mahl the cochlear hair cell loss on this 129S1 congenic background is less severe at 9 months of age than it is at 6 months of age in mice homozygous for the 129S1/SvImJ allele of Mahl

increased or absent threshold for auditory brainstem response ( J:241137 )

• The age related hearing loss allele causes elevated ABR thresholds relative to the 129S1 host background, but this is partially ameliorated by the presence of the C57BL/6J allele of Mahl

nervous system

cochlear hair cell degeneration ( J:241137 )

• progressive inner and outer hair cell loss occur as a result of the age related hearing loss mutation, but in the presence of the ameliorating C57BL/6J allele of Mahl the cochlear hair cell loss on this 129S1 congenic background is less severe at 9 months of age than it is at 6 months of age in mice homozygous for the 129S1/SvImJ allele of Mahl