Autoimmune inner ear disease (AIED) is characterized by rapidly progressive bilateral SNHL. Which immunological marker is MOST specific for AIED, and which treatment is first-line?

• A Anti-dsDNA antibodies; first-line = hydroxychloroquine
• B Anti-thyroid peroxidase; first-line = thyroid replacement
• C Anti-cardiolipin antibodies; first-line = anticoagulation
• D Anti-68 kDa protein (HSP-70) antibodies detected by Western blot; first-line = systemic corticosteroids (prednisone 1 mg/kg/day) ✓

Explanation

AIED is associated with antibodies against a 68 kDa inner ear antigen (thought to be HSP-70) detectable by Western blot assay, though sensitivity is limited. High-dose systemic corticosteroids (prednisone) are first-line treatment and serve both as therapy and as a diagnostic trial — a positive response within 4 weeks supports the diagnosis. Methotrexate has been used as steroid-sparing therapy. Intratympanic steroids may be used for steroid-resistant cases or as adjuncts.

Reference: Dhingra Diseases of Ear, Nose and Throat, 7th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.