In melanocytes, SOX10 manipulates proliferation, survival, and differentiation by activating downstream target genes, like MITF. Mutations in SOX10 may result in a developmental defect in melanocyte-derived cell lineage of the inner ear, called intermediate cells of the stria vascularis, necessary for inner ear homeostasis, and eventually cause HL. The gene discussed is SOX10; the disease is Hodgkins lymphoma.