Mouse Genome Informatics
hm
    Tmc1dn/Tmc1dn
STOCK Grhl3ct/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
hearing/vestibular/ear
• degeneration of most elements of the scala media complex is underway by three weeks of age
• Dieters cell boundaries become indistinct and nuclei move to different levels by 10 days of age
• by 9 months of age, the organ is a mass of undifferentiated cells, although the tunnel of Corti does not usually collapse
• abnormalities can be detected at 10 days of age
• the free end of the membrane curls up rather than resting on hair cells by 10 days
• some degeneration is observed in most mice
• in severe cases, hair cells degenerate completely
• in some mice, the otolith membrane degenerates completely and otoliths are lost
• mice do not respond to clicking

behavior/neurological
• tossing movement is observed in some mice although behavior is otherwise normal

nervous system
N
• no abnormal central or peripheral nervous system phenotupes detected (J:6806)
• spiral ganglion degeneration begins at 50 days of age
• by 9 months, degeneration is nearly complete

Mouse Models of Human Disease
OMIM IDRef(s)
Deafness, Autosomal Dominant 36; DFNA36 606705 J:236
Deafness, Autosomal Recessive 7; DFNB7 600974 J:236