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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
hml
hypoplasia of the membranous labyrinth
MGI:3576616
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
hml/hml CByJ.Cg-hml/J MGI:5284727
ht2
hml/hml+ CByJ.Cg-hml/J MGI:5284858


Genotype
MGI:5284727
hm1
Allelic
Composition
hml/hml
Genetic
Background
CByJ.Cg-hml/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
hml mutation (2 available); any hml mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• Reissner membrane is significantly reduced in size
• the basilar membrane is significantly reduced in size
• the scala tympani is significantly reduced in size
• the scala vestibuli is greatly enlarged
• extensive hypoplasia of the membranous labyrinth
• the stria vaxcularis is significantly reduced in size
• rudimentary tectorial membrane
• the scala media is significantly reduced in size
• 21 of 24 tested at 19 days of age had no ABR response at maximal stimuli
• homozygotes fail to respond to tapping or clicking sounds
• 21 of 24 animals tested at 19 days of age were categorized as deaf by absence of an ABR response at maximum stimuli

behavior/neurological
• when positioned on their backs it takes up to a minute for them to right themselves
• homozygotes fail to orient in water
• unbalanced gait detectable by 10 days of age

growth/size/body
• smaller body size and failure to thrive behavior become can be detected as early as 10 days of age

mortality/aging
• there is significant loss of homozygotes through 2 months of age, after which they can live up to 18 months of age

reproductive system
• neither females nor males breed although the sperm shape and activity appear normal




Genotype
MGI:5284858
ht2
Allelic
Composition
hml/hml+
Genetic
Background
CByJ.Cg-hml/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
hml mutation (2 available); any hml mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
N
• at 4 months of age the inner ear structure is normal by light microscopic analysis





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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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last database update
01/12/2022
MGI 6.17
The Jackson Laboratory