In the present study, the elevated levels of TNF-α and IL-6 in the luminal fluid of the ES, and a positive expression of TNF-α and IL-6 in the epithelial cells lining the sac from patients with MD compared with those of the controls, may represent a prolonged immune response and excessive inflammation with epithelial immunological injury and remodeling in the ES, where the epithelial cells lining parts of the endolymphatic spaces are crucially important for Na+ transport capacity (33). This evidence concerns the gene TNF and Menkes disease.