Symbol
Name
ID

Wfs1
wolframin ER transmembrane glycoprotein
MGI:1328355

Phenotype annotations related to hearing/vestibular/ear

Darker colors indicate more annotations

Human Phenotypes	Sensorineural hearing impairment	Low-frequency sensorineural hearing impairment	Progressive sensorineural hearing impairment	Hearing impairment	Progressive hearing impairment
Disease(s) Associated with WFS1	Sensorineural hearing impairment	Low-frequency sensorineural hearing impairment	Progressive sensorineural hearing impairment	Hearing impairment	Progressive hearing impairment
autosomal dominant nonsyndromic deafness 6
autosomal dominant Wolfram syndrome
optic atrophy
Wolfram syndrome
Wolfram syndrome 1

Mouse Phenotypes

abnormal inner hair cell stereociliary bundle morphology

fused inner hair cell stereocilia

absent outer hair cell stereocilia

cochlear outer hair cell degeneration

stria vascularis degeneration

fused vestibular hair cell stereocilia

vestibular hair cell degeneration

abnormal inner ear vestibule morphology

decreased endocochlear potential

increased or absent threshold for auditory brainstem response

absent distortion product otoacoustic emissions

impaired hearing

deafness

Availability

Mouse Genotype

Wfs1^tm1.1Bedel/Wfs1^tm1.1Bedel

Wfs1^tm1Koks/Wfs1^tm1Koks

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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