Autoimmune Inner Ear Disease — RACP Adult Medicine MCQ

Instant feedback + full explanation. One question, done properly.

HardGeneral Internal MedicineAutoimmune Inner Ear DiseaseRACP Adult Medicine

A 35-year-old woman presents with progressive bilateral hearing loss, tinnitus, and episodic vertigo. Audiometry shows bilateral low-frequency sensorineural hearing loss. She has interstitial keratitis on slit lamp examination. Anti-Hsp70 antibodies are positive. What is the most likely diagnosis?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: C — Autoimmune inner ear disease (AIED)

Bilateral progressive sensorineural hearing loss with vestibular symptoms and associated autoimmune features (interstitial keratitis suggesting Cogan syndrome spectrum) with positive anti-heat shock protein 70 antibodies is consistent with autoimmune inner ear disease (AIED). AIED can be primary (organ-specific) or secondary (associated with systemic autoimmune diseases – SLE, RA, vasculitis, Cogan syndrome). Treatment with high-dose corticosteroids (prednisolone 1 mg/kg) can halt and sometimes reverse hearing loss if commenced early. Steroid-sparing agents (methotrexate, rituximab) are used for relapsing disease.

Reference: eTG – 2025 – Rheumatology