Since 1979 when McCabe first described the category of autoimmune sensorineural hearing loss, there have been several reports of sensorineural hearing loss observed in many autoimmune diseases. Autoimmune sensorineural hearing loss has the characteristics of bilateral or symmetric, slowly progressive sensorineural hearing loss with a period of progression over weeks or months. Recently, studies about the immunology of the inner ear has been proceeded and a category of sensorineural hearing loss was defined which is not treated by conventional therapy but can be effectively managed by immunosuppressive therapy. And a high index of suspicion is likely to be required by the otolaryngologists about this category of hearing loss because it is a potentially treatable cause of sensorineural hearing loss. We observed sensorineural hearing losses in two partients who have autoimmune diseases, a 27 year old female with systemic lupus erythematosus and a 60 year old male with ankylosing spondylitis and uveitis and experienced some improvement of hearing with the administration of immunosuppressive therapy.