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Mediterr J Rheumatol 2022;33(3):349-60
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Immune-Mediated Inner Ear Disease Associated with Type 1 Autoimmune Hepatitis: A Challenging Coexistence
Athina Zarachi1, Eleftherios Pelechas2, Alkistis Tsikou1, Georgios N. Kalambokis3, Aikaterini Zioga4, Katerina E. Panteli3, Iro Rapti3, Sempastian Filippas-Ntekouan3, Ioannis Kastanioudakis1, Fotini B. Karassa2
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1Department of Otorhinolaryngology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece

2Department of Rheumatology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece

 31st Department of Internal Medicine, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece

4Department of Pathology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece

A Zarachi, E Pelechas, I Rapti, S Filippas-Ntekouan, I Kastanioudakis, FB Karassa

Abstract

Autoimmune hepatitis (ΑΙΗ) is characterized by elevated serum transaminase, increased immunoglobulin G levels, presence of autoantibodies, and hepatocellular damage. Coexistence with other autoimmune diseases has been reported in almost half of patients with AIH. Here, we report a 60-year-old man who developed rapidly progressive, bilateral, asymmetrical, and asynchronous sensorineural hearing loss that was consistent with immune-mediated inner ear disease (IMIED). This devastating presentation evolved as a late manifestation in the context of a six-month systemic illness that had previously resulted in type 1 AIH. A biochemical remission with normalization of aminotransferases achieved within two months after the initiation of corticosteroids with azathioprine. Further, an acceptable response has also been achieved at the patient regarding the right ear-hearing impairment; though, treatment could not reverse the substantial decrement in hearing capability of the left ear. To our knowledge, this is the first case report of the concurrent development of type 1 AIH and IMIED.

Cite this article as: Zarachi A, Pelechas E, Tsikou A, Kalambokis GN, Zioga A, Panteli KE, Rapti I, Filippas-Ntekouan S, Kastanioudakis I, Karassa FB. Immune-Mediated Inner Ear Disease Associated with Type 1 Autoimmune Hepatitis: A Challenging Coexistence. Mediterr J Rheumatol 2022;33(3):349-60.

Article Submitted: 27 Aug 2021; Revised Form: 19 Jul 2022; Article Accepted: 30 Jul 2022; Available Online: 30 Sep 2022

https://doi.org/10.31138/mjr.33.3.349

This work is licensed under a Creative Commons Attribution 4.0 International License.

©Zarachi A, Pelechas E, Tsikou A, Kalambokis GN, Zioga A, Panteli KE, Rapti I, Filippas-Ntekouan S, Kastanioudakis I, Karassa FB.

Keywords: immune-mediated inner ear disease, type 1 autoimmune hepatitis