Autoimmune Inner Ear Disorder (AIED)

Autoimmune inner ear disease (AIED) is an inflammatory condition of the inner ear. It occurs when the body’s immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occurring in less than one percent of the 28 million Americans with a hearing loss.

The symptoms of AIED are sudden hearing loss in one ear progressing rapidly to the second ear. The hearing loss can progress over weeks or months, and can fluctuate within one or both ears. Patients may feel fullness in the ear and experience vertigo. In addition, a ringing, hissing, or roaring sound in the ear may be experienced.

Diagnosis of AIED is difficult and is often delayed. One diagnostic blood test that can be helpful in determining the diagnosis is the Western blot immunoassay.

Most patients with AIED respond to the initial treatment of steroids, either oral prednisone or intratympanic injection of steroids, or both. Patients whose hearing improves on steroid treatment are usually switched to a different type of medication for long-term management.

Often, patients with persistent hearing loss will benefit from the use of hearing aids. If patients are unresponsive to drug therapy and remain with severe to profound hearing loss, cochlear implantation may be considered.

(Adapted from

Dr. Chandrasekhar is at the forefront of clinical and laboratory research and patient management in the field of intratympanic injection of steroids for hearing loss. You can read more about her work in the medical literature in the articles and book chapters cited below.

  1. Chandrasekhar SS: Intratympanic Dexamethasone for Sudden Sensorineural Hearing Loss: Clinical and Laboratory Evaluation. Otol & Neurotol 2001;22:18-23.
  2. Chandrasekhar SS, Rubinstein RY, et al: Dexamethasone Pharmacokinetics in the Inner Ear. Otolaryngol Head Neck Surg 2000 Apr; 122:521-8.
  3. Chandrasekhar SS: Sudden Sensorineural Hearing Loss. In Essentials of Otology and Neuro-Otology, A De La Cruz & MA Arriaga, editors.
  4. Chandrasekhar SS: Intratympanic Therapy for Hearing Loss. In: Ear and Temporal Bone Surgery: Minimizing Risks and Complications, R Wiet, editor, 2006.