Autoimmune Inner Ear Disease from a Rheumatologic Perspective

Area of Science:

• Otolaryngology
• Rheumatology
• Immunology

Background:

• Autoimmune inner ear disease (AIED) causes sensorineural hearing loss, often fluctuating and asymmetric.
• AIED can be primary or secondary to systemic autoimmune diseases.
• High-dose corticosteroids are the standard first-line treatment for AIED.

Purpose of the Study:

• To describe the demographics, treatment, and outcomes of a primary AIED cohort.
• To analyze treatment responses to corticosteroids and other immunosuppressants.
• To evaluate the effectiveness of initial and subsequent therapies for AIED.

Main Methods:

• Retrospective chart review of patients diagnosed with AIED at Mayo Clinic.
• Comparison of patient cohort characteristics by sex.
• Analysis of treatment interventions and symptom responses.

Main Results:

• Thirty-one patients with primary AIED were included; mean age 48.5 years, 17 males.
• Corticosteroid treatment improved hearing and vestibular symptoms within the first month.
• Methotrexate showed symptom improvement in 11 of 17 patients; other agents had varied responses.

Conclusions:

• Initial high-dose corticosteroid therapy is effective for AIED symptom management.
• Corticosteroid-sparing agents should be considered for sustained treatment.
• Prompt management is essential to prevent cochlear damage in AIED.