Biomarkers for Autoimmune Inner Ear Disease (AIED) Improve Differential Diagnosis and Guide Therapy

Autoimmune inner ear disease (AIED) is a rare but potentially reversible cause of sensorineural hearing loss, resulting from immune-mediated damage to cochlear and vestibular structures. Differentiating AIED from other inner ear conditions like Meniere's disease (MD) or chronic otitis media (COM) is challenging, often leading to misdiagnosis and delayed treatment. Unlike autoinflammation, which involves innate immune system dysregulation and inflammasome hyperactivation, AIED is driven by adaptive immune responses and autoantibodies. Current diagnostic methods lack specificity, creating a critical need for reliable biomarkers to enable timely and targeted interventions.

This comprehensive review synthesized recent advancements in biomarkers for the differential diagnosis of Autoimmune inner ear disease (AIED). The authors focused on distinguishing AIED from conditions such as Meniere's disease (MD) and chronic otitis media (COM) by evaluating the utility of various serological, immunological, and radiological markers. The review integrated insights from studies on autoantibodies, cytokines, and magnetic resonance imaging (MRI) findings. The aim was to propose diagnostic algorithms that could facilitate early detection and guide personalized therapeutic strategies, including the use of corticosteroids and biologics, based on biomarker profiles.

The review identified several key biomarkers with significant roles in the diagnostic sensitivity and specificity of AIED, as well as correlations with treatment responsiveness. These include specific autoantibodies such as anti-heat shock protein 70 (HSP70), antiphospholipid antibodies, anti-type II collagen, and anti-cochlin antibodies. Cytokines, particularly interleukin-1β (IL-1β), tumor necrosis factor-α (TNF-α), and interleukin-6 (IL-6), were also highlighted for their diagnostic value. Additionally, selective immunoglobulin A deficiency (sIgAD) was noted as a relevant marker. The utility of MRI in identifying inner ear enhancements was discussed as a complementary diagnostic tool. The review underscored that integrating these serological, immunological, and radiological insights can significantly improve diagnostic accuracy. > The synthesis of evidence strongly supports the use of anti-HSP70, IL-1β, TNF-α, and IL-6 as crucial indicators for distinguishing AIED and guiding therapeutic decisions.