The GOF mutations, A419P (Va) and A419/P/I362T (VaJ), cause the Varitint-waddler phenotype characterized by circling behavior, deafness, and coat color dilution.1070., 1071., 1072., 1073., 1074.,1083,1084 Introducing A419P or A419P/I362T mutations into WT human TRPML3 likewise result in GOF effects.1070. This evidence concerns the gene MCOLN3 and deafness.