Our study suggests MD as a model of cochlear neurodegenerative disease for the identification of potent inducers of the Nrf2-vitagene pathway, able to reduce the deleterious consequences associated with neurodegenerative damage, probably by indirectly acting on a-synuclein expression and on inflammatory processes NF-kB-mediated. This evidence concerns the gene NFKB1 and Menkes disease.