Immune-Mediated Inner Ear Disease Associated with Type 1 Autoimmune Hepatitis: A Challenging Coexistence.

Area of Science:

• Hepatology
• Immunology
• Otolaryngology

Background:

• Autoimmune hepatitis (AIH) is a chronic liver disease with diverse extrahepatic manifestations.
• Immune-mediated inner ear disease (IMIED) can cause sensorineural hearing loss and is often associated with systemic autoimmune conditions.

Observation:

• A 60-year-old male patient presented with rapidly progressive, bilateral, asymmetrical sensorineural hearing loss.
• This hearing impairment emerged as a late complication six months after the diagnosis of type 1 AIH.

Findings:

• The patient received corticosteroids and azathioprine, achieving biochemical remission of AIH within two months.
• Treatment resulted in partial hearing improvement in the right ear, but significant hearing loss persisted in the left ear.

Implications:

• This is the first reported case of concurrent type 1 AIH and IMIED.
• Highlights the importance of considering IMIED in AIH patients with unexplained hearing loss.
• Suggests potential benefits of early immunosuppressive therapy for both conditions.