Mouse Genome Informatics
hm
    Ush1cdfcr-2J/Ush1cdfcr-2J
B6;129S4
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological

hearing/vestibular/ear
• at 3 weeks of age, before hair cell degeneration was apparent, examination of of the organ of Corti revealed that stereocilia of the inner hair cells displayed a milder disorganization compared to the outer hair cells
• at 3 weeks of age, before hair cell degeneration was apparent, examination of of the organ of Corti revealed disorganization of the normally rigid and structured outer hair cell stereocilia
• 3 month old mice displayed a progressive loss of vestibular hair cells
• complete absence of acoustic brainstem response at 3 weeks of age
• mice are congenitally deaf

nervous system
• at 3 weeks of age, before hair cell degeneration was apparent, examination of of the organ of Corti revealed that stereocilia of the inner hair cells displayed a milder disorganization compared to the outer hair cells
• at 3 weeks of age, before hair cell degeneration was apparent, examination of of the organ of Corti revealed disorganization of the normally rigid and structured outer hair cell stereocilia
• 3 month old mice displayed a progressive loss of vestibular hair cells

Mouse Models of Human Disease
OMIM IDRef(s)
Deafness, Autosomal Recessive 18a; DFNB18A 602092 J:85400
Usher Syndrome, Type Ic; USH1C 276904 J:85400