Autoimmune sensorineural hearing loss has been increasingly recognized as a clinical entity since its description by McCabe in 1979. Recognition and proper management of this condition is important, as it is one of the very few forms of sensorineural hearing loss that can be successfully treated by medical therapy. Recent studies have provided experimental evidence to suggest that immune processes can cause sensorineural hearing loss in animals and humans. However, antigenic targets within the inner ear are diverse and as a result conclusive evidence for specific autoimmune damage to the inner ear has been elusive. This review focuses on the recent progress in understanding of the aetio-pathogenesis of autoimmune hearing loss along with a description of the various clinical conditions in which they occur. Recent advances in the laboratory diagnosis and management of this interesting condition are also described.