Phenotypes Associated with This Genotype

Genotype
MGI:3814540

• Allelic Composition: Coch^tm1Mrtn/Coch^tm1Mrtn
• Genetic Background: CBACa.129S4-Coch^tm1Mrtn

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:140594 , J:167669 )

• ABR thresholds are substantially higher than for wild-types at 21 months of age (J:140594)

• 6 of 8 mice at 21 months of age have no detectable ABR at the maximum stimulus levels of 32 and 41 kHz frequencies (J:140594)

• 4 of 8 mice have no detectable ABR at all frequencies tested (J:140594)

• increase in the ABR threshold can be detected at 19 months of age (J:167669)

abnormal distortion product otoacoustic emission ( J:140594 )

• mice at 19 months have slightly reduced DPOAE for the mid-frequencies (5-10 dB smaller)

• 21 months of age, DPOAEs are substantially smaller (up to 30 dB)

• 6 of 8 mice at 21 months of age have no detectable DPOAE at the maximum stimulus levels of 32 and 41 kHz frequencies

reduced linear vestibular evoked potential ( J:140594 , J:167669 )

• VsEP thresholds are elevated in mice spanning in age from 11 to 21 months (J:140594)

• these thresholds get higher with age (J:140594)

• at 19 and 21 months of age, 2 of 3 mice have no detectable VsEP (J:140594)

• increase in VsEP thresholds at 7 months of age but not at 5 months of age (J:167669)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal dominant nonsyndromic deafness 9		DOID:0110593	OMIM:601369	J:140594 , J:167669