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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Cdh23ahl
age related hearing loss 1
MGI:3028349
Summary 13 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Cdh23ahl/Cdh23ahl C57BL/6J MGI:3849035
hm2
Cdh23ahl/Cdh23ahl C57BL/6NJ MGI:5898366
hm3
Cdh23ahl/Cdh23ahl multiple strains MGI:3028372
cx4
Cdh23ahl/Cdh23ahl
MahlC57BL/6J/MahlC57BL/6J
129S1.B6-(rs3696307-rs257098870)/Kjn MGI:5898367
cx5
Cdh23ahl/Cdh23tm1.1Kjn
MahlC57BL/6J/Mahl129S1/SvImJ
(129S1.B6-(rs3696307-rs257098870)/Kjn x 129S/Sv-Cdh23tm1.1Kjn/Kjn)F1 MGI:5898375
cx6
Cdh23ahl/Cdh23ahl
mt-Trm1
either: A/J X (A/J x CAST/Ei)F1 or A/J X (CAST/Ei x A/J)F1 MGI:3831893
cx7
Cdh23ahl/Cdh23ahl
mt-Trm2
either: NOD/LtJ X (NOD/LtJ x CAST/Ei)F1 or SKH2/J X (SKH2/J x CAST/Ei)F1 MGI:4430404
cx8
Atp2b2dfw-2J/Atp2b2+
Cdh23ahl/Cdh23ahl
involves: BALB/cByJ * MOLF MGI:3695310
cx9
Adgrv1frings/Adgrv1frings
Cdh23ahl/Cdh23ahl
involves: BUB/BnJ * CAST/EiJ MGI:3581700
cx10
Adgrv1frings/Adgrv1frings
Cdh23ahl/Cdh23ahl
involves: BUB/BnJ * MOLD/RkJ MGI:3581701
cx11
Cdh23ahl/Cdh23ahl
Adgrv1frings/Adgrv1frings
involves: BUB/BnJ * RB/1 MGI:3701599
cx12
Fscn2ahl8/Fscn2ahl8
Cdh23ahl/Cdh23ahl
involves: C57BL/6J * DBA/2J MGI:3818004
cx13
Atp2b2dfw-2J/Atp2b2+
Cdh23ahl/Cdh23ahl
involves: CAST/Ei * BALB/cByJ MGI:3695309


Genotype
MGI:3849035
hm1
Allelic
Composition
Cdh23ahl/Cdh23ahl
Genetic
Background
C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• in 3 of 4 cochleae assesed at 12 months of age the organ of Corti had totally degenerated

nervous system
• 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




Genotype
MGI:5898366
hm2
Allelic
Composition
Cdh23ahl/Cdh23ahl
Genetic
Background
C57BL/6NJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• 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

nervous system
• 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




Genotype
MGI:3028372
hm3
Allelic
Composition
Cdh23ahl/Cdh23ahl
Genetic
Background
multiple strains
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• mice carrying this mutation develop age related hearing loss




Genotype
MGI:5898367
cx4
Allelic
Composition
Cdh23ahl/Cdh23ahl
MahlC57BL/6J/MahlC57BL/6J
Genetic
Background
129S1.B6-(rs3696307-rs257098870)/Kjn
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
MahlC57BL/6J mutation (1 available); any Mahl mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• 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
• 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
• 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




Genotype
MGI:5898375
cx5
Allelic
Composition
Cdh23ahl/Cdh23tm1.1Kjn
MahlC57BL/6J/Mahl129S1/SvImJ
Genetic
Background
(129S1.B6-(rs3696307-rs257098870)/Kjn x 129S/Sv-Cdh23tm1.1Kjn/Kjn)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
Cdh23tm1.1Kjn mutation (1 available); any Cdh23 mutation (196 available)
Mahl129S1/SvImJ mutation (1 available); any Mahl mutation (2 available)
MahlC57BL/6J mutation (1 available); any Mahl mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• 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




Genotype
MGI:3831893
cx6
Allelic
Composition
Cdh23ahl/Cdh23ahl
mt-Trm1
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:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
mt-Trm1 mutation (6 available); any mt-Tr mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• average ABR threshold is significantly increased by 3 months of age
• 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




Genotype
MGI:4430404
cx7
Allelic
Composition
Cdh23ahl/Cdh23ahl
mt-Trm2
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:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
mt-Trm2 mutation (4 available); any mt-Tr mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear




Genotype
MGI:3695310
cx8
Allelic
Composition
Atp2b2dfw-2J/Atp2b2+
Cdh23ahl/Cdh23ahl
Genetic
Background
involves: BALB/cByJ * MOLF
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp2b2dfw-2J mutation (1 available); any Atp2b2 mutation (54 available)
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• at 4 to 6 weeks of age, significantly elevated sound pressure thresholds
• show age-dependent hearing loss by 12 weeks of age




Genotype
MGI:3581700
cx9
Allelic
Composition
Adgrv1frings/Adgrv1frings
Cdh23ahl/Cdh23ahl
Genetic
Background
involves: BUB/BnJ * CAST/EiJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adgrv1frings mutation (1 available); any Adgrv1 mutation (188 available)
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• severe hearing loss by 5 months of age




Genotype
MGI:3581701
cx10
Allelic
Composition
Adgrv1frings/Adgrv1frings
Cdh23ahl/Cdh23ahl
Genetic
Background
involves: BUB/BnJ * MOLD/RkJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adgrv1frings mutation (1 available); any Adgrv1 mutation (188 available)
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• severe hearing loss by 5 months of age




Genotype
MGI:3701599
cx11
Allelic
Composition
Cdh23ahl/Cdh23ahl
Adgrv1frings/Adgrv1frings
Genetic
Background
involves: BUB/BnJ * RB/1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adgrv1frings mutation (1 available); any Adgrv1 mutation (188 available)
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• exhibit the highest ABR threshold compared to single homozygous mice Gpr98frings/Gpr98frings Cdh23ahl/Cdh23+ and Gpr98frings/Gpr98+ Cdh23ahl/Cdh23ahl
• already near deaf by 5 months of age




Genotype
MGI:3818004
cx12
Allelic
Composition
Fscn2ahl8/Fscn2ahl8
Cdh23ahl/Cdh23ahl
Genetic
Background
involves: C57BL/6J * DBA/2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
Fscn2ahl8 mutation (3 available); any Fscn2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• 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




Genotype
MGI:3695309
cx13
Allelic
Composition
Atp2b2dfw-2J/Atp2b2+
Cdh23ahl/Cdh23ahl
Genetic
Background
involves: CAST/Ei * BALB/cByJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp2b2dfw-2J mutation (1 available); any Atp2b2 mutation (54 available)
Cdh23ahl mutation (55 available); any Cdh23 mutation (196 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• at 4 to 6 weeks of age, significantly elevated sound pressure thresholds
• show age-dependent hearing loss by 12 weeks of age





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last database update
08/09/2022
MGI 6.21
The Jackson Laboratory