Autoimmune inner ear disease (AIED) is a rare disease, accounting for < 1% of all cases of hearing impairment or dizziness. It is characterized by sensorineural hearing loss (SNHL) or vestibular dysfunction that results from an immunemediated process. Clinical features of AIED is SNHL that progresses over weeks to month with fluctuating hearing symptoms. Because there are no diagnostic laboratory and clinical feature, response to immunosuppressive therapy were important for diagnosis of AIED. Many diseases such as sudden SNHL and Meniere disease may also mimic AIED, a broad differential must be maintained in patients suspected of having AIED. We report a case of a 46-year-old female who presented with sudden hearing loss and vertigo. We could diagnose her as AIED with systemic lupus erythematous. The symptoms were improved treated with steroids.