Mouse Genome Informatics
ht1
    Chd7Ome/Chd7+
involves: BALB/cByJ * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype

Ear abnormalities in Chd7Ome/Chd7+ mice

reproductive system
• slight (J:187200)

hearing/vestibular/ear
• mice exhibit decreased epithelial cilia density and increased goblet cell density in the middle ear compared with wild-type mice
• shifted towards the anterior crus and a smaller obturator foramen
• thinner and partially fused with surrounding bones
• greater Eustachian tube angle
• mean ABR thresholds in each age-group from P21 to P120 are significantly higher than those of wild-type mice at all the frequencies tested
• mutant mice have amplitudes below zero at every frequency tested from P21 to P120, substantially lower than those in wild-type mice
• as early as P11, most mice exhibit otitis media with effusion, thickened epithelia, a dilated periosteum and inflammatory cells, increased goblet cells, cilia loss in the middle ear and Eustachian without bacterial cause compared with wild-type mice

craniofacial
• larger skull height and length
• shifted towards the anterior crus and a smaller obturator foramen
• thinner and partially fused with surrounding bones
• larger nose bone length to skull length

growth/size
• larger nose bone length to skull length
• at weaning
• at weaning

behavior/neurological

immune system
• as early as P11, most mice exhibit otitis media with effusion, thickened epithelia, a dilated periosteum and inflammatory cells, increased goblet cells, cilia loss in the middle ear and Eustachian without bacterial cause compared with wild-type mice

nervous system

skeleton
• larger skull height and length
• shifted towards the anterior crus and a smaller obturator foramen
• thinner and partially fused with surrounding bones

Mouse Models of Human Disease
OMIM IDRef(s)
CHARGE Syndrome 214800 J:187200