Loss of SRRM4 function due to a deletion mutation in the Bronx waltzer mouse line (Srrm4bv/bv) is associated with balance defects, deafness, and perinatal degeneration of nearly all VHCs as well as ∼75% of inner HCs (IHCs) (Nakano et al, 2012). This evidence concerns the gene SRRM4 and deafness.