Phenotypes associated with this allele

• Allele Symbol: Atoh1^trhl
• Allele Name: trembling with hearing loss
• Allele ID: MGI:2656758
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Atoh1^trhl/Atoh1^trhl	B6.Cg-Atoh1^trhl	MGI:5576735

Genotype
MGI:5576735

hm1

• Allelic Composition: Atoh1^trhl/Atoh1^trhl
• Genetic Background: B6.Cg-Atoh1^trhl

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

Cochlear hair cell loss and altered morphology in Atoh1^trhl/Atoh1^trhl mice

mortality/aging

mortality/aging ( J:209662 )

N • unlike other mutants for this gene, mice exhibit normal lifespan

hearing/vestibular/ear

decreased inner hair cell stereocilia number ( J:209662 )

decreased outer hair cell stereocilia number ( J:209662 )

cochlear inner hair cell degeneration ( J:209662 )

• at 3 weeks

• progressive loss

• greatest at the mid-basal turn at 3 and 7 weeks

cochlear outer hair cell degeneration ( J:209662 )

• at 3 weeks

• progressive loss

• greatest at the mid-basal turn at 3 and 7 weeks

increased or absent threshold for auditory brainstem response ( J:209662 )

• at P21

abnormal distortion product otoacoustic emission ( J:209662 )

• decreased

impaired hearing ( J:209662 )

• progressive starting at 3 weeks of age

• near-total deafness by 8 months

deafness ( J:209662 )

• starting at 10 months

nervous system

decreased inner hair cell stereocilia number ( J:209662 )

decreased outer hair cell stereocilia number ( J:209662 )

cochlear inner hair cell degeneration ( J:209662 )

• at 3 weeks

• progressive loss

• greatest at the mid-basal turn at 3 and 7 weeks

cochlear outer hair cell degeneration ( J:209662 )

• at 3 weeks

• progressive loss

• greatest at the mid-basal turn at 3 and 7 weeks

absent cerebellar foliation ( J:209662 )

abnormal cerebellum external granule cell layer morphology ( J:209662 )

• lack of robust external granular cell layer

• however, mice exhibit normal numbers of Purkinje cells

small cerebellum ( J:209662 )

behavior/neurological

abnormal pinna reflex ( J:209662 )

• reduced

abnormal gait ( J:209662 )

• trembling gait

• shaky gait at weaning