Phenotypes Associated with This Genotype

Genotype
MGI:7496091

• Allelic Composition: Chd7^{Gt(S20-7E1)Sor}/Chd7⁺
• Genetic Background: involves: 129S1/SvImJ * 129S4/SvJae

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:332873 )

• at 4 and 16 kHz but not at 32 kHz

• however, Wave 1 peak amplitude and peak latency are similar to controls

nervous system

abnormal CNS glial cell morphology ( J:332873 )

• decrease in the number of satellite glial cells but neuron density in the adult spiral ganglion

abnormal vestibulocochlear ganglion morphology ( J:332873 )

• decrease in the number of satellite glial cells but neuron density in the adult spiral ganglion

hypermyelination ( J:332873 )

• increase in myelin thickness on the spiral ganglion neurons

• myelin thickness is increased by 10% in the apex and 15% in the base spiral ganglia

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
CHARGE syndrome		DOID:0050834	OMIM:214800	J:332873