The immune response genes that control the immune response and recognition of ‘self’ from ‘nonself’ are closely linked with the human leukocyte antigen (HLA) complex. Sixty-six HLA antigens were typed in 42 Caucasian patients with inner ear diseases of unknown etiology. All of these patients had positive serum antibodies against the inner ear structures as detected by an indirect immunofluorescence assay. Results revealed that none of the 66 HLA antigens was associated with the inner ear diseases. Reviewing results from other studies on HLA association in inner ear diseases shows that the findings are inconsistent with each other. The conclusion that HLA associations occur with inner ear diseases cannot be reached.