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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Fscn2ahl8
age related hearing loss 18
MGI:3807774
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
 		Fscn2ahl8/Fscn2ahl8 DBA/2J MGI:3807775
cx2
 		Fscn2ahl8/Fscn2ahl8
Cdh23ahl/Cdh23ahl 		involves: C57BL/6J * DBA/2J MGI:3818004


Genotype
MGI:3807775
hm1
Allelic
Composition		 Fscn2ahl8/Fscn2ahl8
Genetic
Background		 DBA/2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fscn2ahl8 mutation (3 available); any Fscn2 mutation (39 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
decreased inner hair cell stereocilia number ( J:162868 )
• hair bundles appear normal at 2 weeks of age but are progressively lost between 1 and 6 months of age
decreased outer hair cell stereocilia number ( J:162868 )
• degeneration occurs earliest here
abnormal auditory brainstem response ( J:139223 )
• although DBA/2J is homozygous for both Cdh23 and ahl8, through linkage analysis with CAST/Ei, which is wild-type at Cdh23, ahl8 is shown to contribute up to 37% of the 16 kHz ABR threshold variation in 13 week old homozygotes
increased susceptibility to age-related hearing loss ( J:139223 , J:162868 )
• hearing loss is progressive and shows a much earlier onset than in C57BL/6J, with average 16 kHz ABR thresholds approximately 50 dB higher at 10 weeks of age than in 30 week old C57BL/6J (J:139223)
• hearing appears normal at 2 weeks of age but is progressively lost, based upon measurement of auditory brainstem response between 1 and 6 months of age (J:162868)

nervous system
decreased inner hair cell stereocilia number ( J:162868 )
• hair bundles appear normal at 2 weeks of age but are progressively lost between 1 and 6 months of age
decreased outer hair cell stereocilia number ( J:162868 )
• degeneration occurs earliest here




Genotype
MGI:3818004
cx2
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 (281 available)
Fscn2ahl8 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




Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
11/12/2024
MGI 6.24 		The Jackson Laboratory