Autoimmunity has been proposed as a mechanism in patients with bilateral SNHL and MD [2] and although preliminary candidate gene studies found that bilateral MD was associated with allelic variants of genes HLA-DRB1[31], PTPN22[32], these findings have not been formally replicated in the current study including 168 patients with bilateral MD, the largest collection so far. Here, HLA-DRB1 is linked to Menkes disease.