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Homozygosity for the del10 mutation results in a failure of neural crest-derived melanocytes to incorporate into the SV during development, leading to a reduced intermediate cell layer and consequently, compromised endocochlear potential (EP), deafness and subsequent hair cell loss

Indeed, DNA methylation was later linked to heritable inactivation of X chromosomes and genomic imprinting (Wolffe and Matzke, 1999)