Autoimmune Inner Ear Disease (AIED)
Autoimmune Inner Ear Disease (AIED) is caused by one’s own immune system attacking the inner ears. People with AIED experience progressive, bilateral, typically step-wise sensorineural hearing loss as well as ringing in the ears called tinnitus that worsens over a period of weeks to a couple of months. At least half of such patients also describe balance problems. AIED typically begins in one ear but progresses to involve the second ear quickly. This differentiates this disease from idiopathic Sudden Sensorineural Hearing Loss.
The diagnosis of AIED requires (1) a hearing test confirming bilateral, new-onset, progressive sensorineural hearing loss, (2) the appropriate symptom time-course as determined by patient history, and (3) an MRI Brain with Internal Auditory Canal protocol that finds no evidence of retrocochlear mass lesions. Laboratory tests such as Lyme Titers, FTA-abs (syphilis test), and HIV tests could be obtained to rule out treatable causes of inner ear dysfunction, and laboratory markers of an activated immune response such as erythrocyte sedimentation rate (ESR) and C-reactive Protein (CRP) may be ordered.
In many cases, AIED is steroid responsive and early intervention is recommended. Oral steroids at high dose are typically prescribed for at least one month followed by a slow taper. Those who relapse after termination of oral steroids are referred to Rheumatology for a discussion regarding long-term oral steroid use. Intra-tympanic steroids (injected directly into the middle ear space) may be an adjunct or alternative treatment, depending on the particular circumstance. Unfortunately, not all patients are improved or stabilized by use of steroids and some progress to bilateral severe to profound sensorineural hearing loss. Many such patients are excellent candidates for cochlear implantation.