Secondary autoimmune sensorineural hearing loss (sASNHL, secondary autoimmune inner ear disease, sAIED) is hearing impairment that might occur in case of systemic or organ-specific autoimmune disorders before or after the main disease manifestation. The onset of sensorineural hearing loss before the specific clinical picture of the underlying disease with a difference of several months and years can lead to serious diagnostic errors. The relevance of the sASNHL issue is due to the risk of developing profound hearing loss that is poorly corrected using traditional methods and may finally require cochlear implantation. All the diagnostic mistakes do happen because of lack of data about sASNHL pathogenesis and clinical features that delay its diagnostics and thus treatment. The significance of sASNHL problem is determined by increase of number of autoimmune pathology in recent decades and their adverse outcomes. The article presents up-to-date information on etiopathogenesis, pathomorphology, clinical features, laboratory, genetic and radiation diagnostic methods, and criteria for the verification of sASNHL. Therapy options using both traditional means of immunosuppression (corticosteroids, cytostatics) and modern biological drugs, as well as progressive and promising semi-surgical methods are described in detail. Gene therapy opportunities are also shown.