Phenotypes Associated with This Genotype

Genotype
MGI:5581451

• Allelic Composition: Alg10b^nse5/Alg10b^nse5
• Genetic Background: 129S6.B6-Alg10b^nse5

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

Outer hair cells of the cochlea are abnormal in Alg10b^nse5/Alg10b^nse5 mice

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:209766 )

N • endocochlear potential is normal

abnormal cochlear outer hair cell morphology ( J:209766 )

• severely; some with punctate pattern of prestin staining in cytosol

decreased cochlear outer hair cell number ( J:209766 )

cochlear outer hair cell degeneration ( J:209766 )

• however, degeneration is rescued by transgene expression of the gene

absent cochlear microphonics ( J:209766 )

increased or absent threshold for auditory brainstem response ( J:209766 )

• however, ABR threshold is partially rescued by transgene expression of the gene

absent distortion product otoacoustic emissions ( J:209766 )

• however, ABR threshold is partially rescued by transgene expression of the gene

deafness ( J:209766 )

• however, deafness is rescued by transgene expression of the gene

nervous system

abnormal cochlear outer hair cell morphology ( J:209766 )

• severely; some with punctate pattern of prestin staining in cytosol

decreased cochlear outer hair cell number ( J:209766 )

cochlear outer hair cell degeneration ( J:209766 )

• however, degeneration is rescued by transgene expression of the gene

absent cochlear microphonics ( J:209766 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
nonsyndromic deafness		DOID:0050563		J:209766