NFKB1 and Menkes disease: The role of genetic component in inflammatory response has been documented through discovery of allelic variations in the TLR10 gene that may influence bilateral affectation and clinical course in MD and variants in the NFKB1 gene that influence the hearing outcome in patients with unilateral MD through suspected mediation of the interaction of NFKB1 with other transcription factors conditioning the inflammatory response in the inner ear (Requena et al., 2013; Cabrera et al., 2014).