CHARGE Syndrome/enzymology; DNA-Binding Proteins/metabolism; Ear, Inner/enzymology; Ear, Middle/enzymology; Hearing Loss, Conductive/enzymology; Hearing Loss, Sensorineural/enzymology; Acoustic Stimulation; Age Factors; Animals; Auditory Threshold; CHARGE Syndrome/genetics; CHARGE Syndrome/pathology; CHARGE Syndrome/physiopathology; DNA-Binding Proteins/genetics; Disease Models, Animal; Ear, Inner/abnormalities; Ear, Inner/physiopathology; Ear, Inner/ultrastructure; Ear, Middle/abnormalities; Ear, Middle/physiopathology; Ear, Middle/ultrastructure; Evoked Potentials, Auditory, Brain Stem; Female; Genes, Reporter; Hearing Loss, Conductive/genetics; Hearing Loss, Conductive/pathology; Hearing Loss, Conductive/physiopathology; Hearing Loss, Sensorineural/genetics; Hearing Loss, Sensorineural/pathology; Hearing Loss, Sensorineural/physiopathology; Immunohistochemistry; Male; Mice; Mice, 129 Strain; Mice, Transgenic; Microscopy, Electron, Scanning; Molecular Motor Proteins/metabolism; Mutation; Noise; Otoacoustic Emissions, Spontaneous; Promoter Regions, Genetic; beta-Galactosidase/genetics; beta-Galactosidase/metabolism