Autoimmune inner ear disease (AIED) refers to a condition in which the body’s immune system mistakenly attacks the inner ear. Sometimes, this occurs in patients who have other autoimmune diseases, but often it appears to occur spontaneously. It manifests with bilateral hearing loss that progresses quite rapidly (over weeks to months), and can be accompanied by other ear symptoms (aural fullness, tinnitus) and sometimes vertigo. One of the features of this disease is that the hearing loss often improves (though may not normalize) when treated with oral steroids. This feature, sometimes called “steroid-responsive hearing loss,” is one of the cardinal features of the diagnosis. If a patient’s hearing improves with steroids, then they may consider longer-term treatment (sometimes called “maintenance therapy”) with an immunomodulatory agent (such as adalimumab or etanercept), as such medications tend to be better tolerated than steroids.