Although clinical symptoms of systemic autoimmune disorder were not present in patients who were suspected to have autoimmune pathology-associated MD, studies have identified in these patients an excessive immune response due to an attack of the specific inner ear structures by cochlear innate immune cells that recognize self-antigens, which leads to the release of inflammatory cytokines, including TNF-α, IL-1β, interferon-γ (IFN-γ) and IL-17 (23–25). Here, IL1B is linked to Menkes disease.