A 46-year-old has rapidly progressive, fluctuating bilateral sensorineural hearing loss over 10 weeks with episodic imbalance. MRI excludes retrocochlear disease; infection and ototoxicity have been excluded. ESR is raised, but a commercial inner-ear antibody panel is negative. What is the best next step?
Rapid bilateral fluctuating SNHL is compatible with AIED, but there is no pathognomonic blood test; serial objective hearing data and a supervised treatment response are central.
A. Exclude autoimmune inner-ear disease because antibody testing is negative: No serological test has sufficient sensitivity or specificity to rule the diagnosis in or out.
B. Proceed directly to bilateral labyrinthectomy: This would destroy residual hearing and vestibular function without treating the suspected immune process.
C. Diagnose Ménière disease solely from fluctuation: The rapid bilateral progressive pattern and systemic inflammatory signal demand a broader exclusion-based assessment.
D. Wait six months for spontaneous stabilisation: Potentially reversible cochlear loss makes delay hazardous.
E. Coordinate otology-rheumatology assessment, document serial audiometry and discuss a monitored corticosteroid trial: AIED is a clinical diagnosis of exclusion; objective response and toxicity monitoring guide further intratympanic or steroid-sparing treatment.
If audiometry instead showed stable unilateral loss with asymmetric speech discrimination, retrocochlear investigation would remain the priority.
Independent Clinora educational preparation material; not official JCIE content, an accredited programme, or a substitute for local policy and specialist clinical judgement.
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