Mouse Genome Informatics
hm
    Tmc1baringo/Tmc1baringo
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 (smallest) row of stereocilia is often missing or, if present, is of irregular height
• by 8 weeks of age remaining IHCs in the basal and mid region show severe degeneration and fusion of stereocilia
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height
• by 8 weeks of age lateral supporting cells show irregular morphological characteristics
• at 4 weeks of age extensive degeneration of hair and supporting cells, and the collapse of the tunnel of Corti, is evident in the basal to mid region
• severe to profound hearing loss at 4 weeks of age, ranging from 85 to 120 dB SPL

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

nervous system
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height
• by 8 weeks of age remaining IHCs in the basal and mid region show severe degeneration and fusion of stereocilia
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height

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