Mouse Genome Informatics
hm
    Tmc1stitch/Tmc1stitch
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
hearing/vestibular/ear
• at 4 weeks of age, the inner row of stereocilia of the OHC hair bundle is often irregular in length or missing
• loss of inner hair cells in the basal turn at 8 weeks of age
• loss of outer hair cells in the basal turn at 8 weeks of age
• at 4 weeks of age slight indications of hair cell degeneration are seen
• at 8 weeks, but not at 4 weeks, of age the basal region lacks sensory or supporting cells and the organ/tunnel of Corti has collapsed
• display a hearing loss ranging from 60 to 120 dB SPL at 4 weeks of age

behavior/neurological
N
• no circling or head tossing/tilting behaviors are seen (J:181985)

nervous system
• at 4 weeks of age, the inner row of stereocilia of the OHC hair bundle is often irregular in length or missing
• loss of inner hair cells in the basal turn at 8 weeks of age
• loss of outer hair cells in the basal turn at 8 weeks of age

Mouse Models of Human Disease
OMIM IDRef(s)
Deafness, Autosomal Recessive 7; DFNB7 600974 J:181985