All Phenotypes Cdh23<ahl> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Cdh23^ahl
age related hearing loss 1
MGI:3028349

Summary

13 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Cdh23^ahl/Cdh23^ahl	C57BL/6J	MGI:3849035
hm2	Cdh23^ahl/Cdh23^ahl	C57BL/6NJ	MGI:5898366
hm3	Cdh23^ahl/Cdh23^ahl	multiple strains	MGI:3028372
cx4	Cdh23^ahl/Cdh23^ahl Mahl^C57BL/6J/Mahl^C57BL/6J	129S1.B6-(rs3696307-rs257098870)/Kjn	MGI:5898367
cx5	Cdh23^ahl/Cdh23^tm1.1Kjn Mahl^C57BL/6J/Mahl^129S1/SvImJ	(129S1.B6-(rs3696307-rs257098870)/Kjn x 129S/Sv-Cdh23^tm1.1Kjn/Kjn)F1	MGI:5898375
cx6	Cdh23^ahl/Cdh23^ahl mt-Tr^m1	either: A/J X (A/J x CAST/Ei)F1 or A/J X (CAST/Ei x A/J)F1	MGI:3831893
cx7	Cdh23^ahl/Cdh23^ahl mt-Tr^m2	either: NOD/LtJ X (NOD/LtJ x CAST/Ei)F1 or SKH2/J X (SKH2/J x CAST/Ei)F1	MGI:4430404
cx8	Atp2b2^dfw-2J/Atp2b2⁺ Cdh23^ahl/Cdh23^ahl	involves: BALB/cByJ * MOLF	MGI:3695310
cx9	Adgrv1^frings/Adgrv1^frings Cdh23^ahl/Cdh23^ahl	involves: BUB/BnJ * CAST/EiJ	MGI:3581700
cx10	Adgrv1^frings/Adgrv1^frings Cdh23^ahl/Cdh23^ahl	involves: BUB/BnJ * MOLD/RkJ	MGI:3581701
cx11	Cdh23^ahl/Cdh23^ahl Adgrv1^frings/Adgrv1^frings	involves: BUB/BnJ * RB/1	MGI:3701599
cx12	Fscn2^ahl8/Fscn2^ahl8 Cdh23^ahl/Cdh23^ahl	involves: C57BL/6J * DBA/2J	MGI:3818004
cx13	Atp2b2^dfw-2J/Atp2b2⁺ Cdh23^ahl/Cdh23^ahl	involves: CAST/Ei * BALB/cByJ	MGI:3695309

Allelic Composition	Cdh23^ahl/Cdh23^ahl
Genetic Background	C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)

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

hearing/vestibular/ear

organ of Corti degeneration ( J:87783 )

• in 3 of 4 cochleae assesed at 12 months of age the organ of Corti had totally degenerated

nervous system

cochlear ganglion hypoplasia ( J:87783 )

• at 9 months of age there is a decrease in the ganglion cell density for each turn over time with the largest cell loss in the basal turn

Allelic Composition	Cdh23^ahl/Cdh23^ahl
Genetic Background	C57BL/6NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)

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

hearing/vestibular/ear

cochlear hair cell degeneration ( J:241137 )

• degeneration of outer hair cells is more extensive than that of inner hair cells and losses of both are more severe in the base of the cochlea than in the apex

cochlear inner hair cell degeneration ( J:241137 )

cochlear outer hair cell degeneration ( J:241137 )

nervous system

cochlear hair cell degeneration ( J:241137 )

• degeneration of outer hair cells is more extensive than that of inner hair cells and losses of both are more severe in the base of the cochlea than in the apex

cochlear inner hair cell degeneration ( J:241137 )

cochlear outer hair cell degeneration ( J:241137 )

Allelic Composition	Cdh23^ahl/Cdh23^ahl
Genetic Background	multiple strains

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)

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

hearing/vestibular/ear

increased susceptibility to age-related hearing loss ( J:86905 )

• mice carrying this mutation develop age related hearing loss

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)
Mahl^C57BL/6J mutation (1 available); any Mahl mutation (2 available)

♀	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 )

Allelic Composition	Cdh23^ahl/Cdh23^tm1.1Kjn Mahl^C57BL/6J/Mahl^129S1/SvImJ
Genetic Background	(129S1.B6-(rs3696307-rs257098870)/Kjn x 129S/Sv-Cdh23^tm1.1Kjn/Kjn)F1

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)
Cdh23^tm1.1Kjn mutation (1 available); any Cdh23 mutation (281 available)
Mahl^129S1/SvImJ mutation (1 available); any Mahl mutation (2 available)
Mahl^C57BL/6J mutation (1 available); any Mahl mutation (2 available)

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

hearing/vestibular/ear

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

• these mice, homozygous for the c.735A point mutation that causes age related hearing loss and heterozgyous for the ameliorating C57BL/6J-derived modifier, have a 16 kHz ABR threshold at 3 months of age that is less than mice homozygous for the 129S1/SvImJ-derived modifier, proving the C57BL/6J allele dominant

Allelic Composition	Cdh23^ahl/Cdh23^ahl mt-Tr^m1
Genetic Background	either: A/J X (A/J x CAST/Ei)F1 or A/J X (CAST/Ei x A/J)F1

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)
mt-Tr^m1 mutation (6 available); any mt-Tr mutation (10 available)

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:67312 )

• average ABR threshold is significantly increased by 3 months of age

increased susceptibility to age-related hearing loss ( J:67312 )

• the presence of this mitochondrial sequence polymorphism in mice homozygous for the ahl allele results in age related hearing loss by 3 months of age, which is absent when the CAST/Ei mitochondrial sequence is instead present

Allelic Composition	Cdh23^ahl/Cdh23^ahl mt-Tr^m2
Genetic Background	either: NOD/LtJ X (NOD/LtJ x CAST/Ei)F1 or SKH2/J X (SKH2/J x CAST/Ei)F1

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)
mt-Tr^m2 mutation (4 available); any mt-Tr mutation (10 available)

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:67312 )

• as in Cdh23^ahl homozygotes

increased susceptibility to age-related hearing loss ( J:67312 )

• as in Cdh23^ahl homozygotes

Allelic Composition	Atp2b2^dfw-2J/Atp2b2⁺ Cdh23^ahl/Cdh23^ahl
Genetic Background	involves: BALB/cByJ * MOLF

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp2b2^dfw-2J mutation (1 available); any Atp2b2 mutation (66 available)
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:38429 )

• at 4 to 6 weeks of age, significantly elevated sound pressure thresholds

deafness ( J:38429 )

• show age-dependent hearing loss by 12 weeks of age

Allelic Composition	Adgrv1^frings/Adgrv1^frings Cdh23^ahl/Cdh23^ahl
Genetic Background	involves: BUB/BnJ * CAST/EiJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adgrv1^frings mutation (1 available); any Adgrv1 mutation (318 available)
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)

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

hearing/vestibular/ear

deafness ( J:97534 )

• severe hearing loss by 5 months of age

Allelic Composition	Adgrv1^frings/Adgrv1^frings Cdh23^ahl/Cdh23^ahl
Genetic Background	involves: BUB/BnJ * MOLD/RkJ

Find Mice

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

hearing/vestibular/ear

deafness ( J:97534 )

• severe hearing loss by 5 months of age

Allelic Composition	Cdh23^ahl/Cdh23^ahl Adgrv1^frings/Adgrv1^frings
Genetic Background	involves: BUB/BnJ * RB/1

Find Mice

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

hearing/vestibular/ear

increased susceptibility to age-related hearing loss ( J:97534 )

• exhibit the highest ABR threshold compared to single homozygous mice Gpr98^frings/Gpr98^frings Cdh23^ahl/Cdh23⁺ and Gpr98^frings/Gpr98⁺ Cdh23^ahl/Cdh23^ahl

deafness ( J:97534 )

• already near deaf by 5 months of age

Allelic Composition	Fscn2^ahl8/Fscn2^ahl8 Cdh23^ahl/Cdh23^ahl
Genetic Background	involves: C57BL/6J * DBA/2J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23^ahl mutation (55 available); any Cdh23 mutation (281 available)
Fscn2^ahl8 mutation (3 available); any Fscn2 mutation (39 available)

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:139223 )

• 20 dB increase in 16 kHz ABR threshold at 6 weeks of age

• 50 dB increase in 16 kHz ABR threshold at 13 weeks of age

increased susceptibility to age-related hearing loss ( J:139223 )

Allelic Composition	Atp2b2^dfw-2J/Atp2b2⁺ Cdh23^ahl/Cdh23^ahl
Genetic Background	involves: CAST/Ei * BALB/cByJ

Find Mice

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:38429 )

• at 4 to 6 weeks of age, significantly elevated sound pressure thresholds

increased susceptibility to age-related hearing loss ( J:38429 )

deafness ( J:38429 )

• show age-dependent hearing loss by 12 weeks of age

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