Learning Objectives: To highlight Autoimmune Inner Ear Disease as a condition that may mimic Meniere's Disease.

Introduction: Autoimmune inner ear disease (AIED) is a rare cause of sensorineural hearing loss, accounting for less than 1% of all cases. However, it is also one of the few forms of sensorineural deafness that can potentially be treated. The diagnosis of AIED may be missed for several years as it often mimics the symptoms of other inner ear pathologies such as Menière's disease (MD), with up to 50% of patients meeting the criteria for MD.

Method & Results: We present a 52-year-old man, previously diagnosed with MD, manifesting the classical symptoms – sensorineural deafness, tinnitus, aural fullness and episodic vertigo. 4 years after the onset of MD symptoms, he was discovered to have autoimmune-associated conditions, namely psoriatis, joint pains and anterior uveitis. Given the patient's autoimmune-related diseases, we suspected the diagnosis of AIED and started him on a therapeutic trial of steroids. He responded favorably to the therapy, and was subsequently switched to a steroid-sparing immunomodulator treatment. His vestibular symptoms were abolished and there was also significant sustained improvement in his hearing tests, demonstrating an autoimmune cause for his audiovestibular symptoms.

Conclusion: Our case report illustrates the difficulty in differentiating the idiopathic MD from AIED. As the history was typical of MD, it was easy to have concurred with the initial diagnosis. However, this patient had features of autoimmune diseases that raised our suspicion of AIED. The response to immunosuppressant therapy confirmed an autoimmune etiology for his symptoms.

With no diagnostic tests to confirm AIED available, clinicians must maintain a high index of suspicion when treating patients with symptoms of MD who have one or more autoimmune conditions, bilateral symptoms, or a rapid progression of disease. Starting the patient on a trial of treatment with steroids and monitoring his response closely can often be a simple way of confirming the diagnosis.