Autoimmune inner ear disease in a young patient – an unsolvable challenge?

Abstract

We report on a 21-year-old male patient, suffering from bilateral acute hearing loss and vertigo. The article presents the clinical course of autoimmune inner ear disease, including diagnostic approaches and challenges, as well as therapeutic strategies and clinical follow-up. Response to corticosteroids often decreases during the course of the disease, requiring a more targeted treatment, such as the use of biological agents to block cytokines like interleukin 1 (IL-1) and tumor necrosis factor alpha (TNFα). In our case, hearing thresholds improved considerably under immune-modulating therapy, while vestibular function remained reduced. In case of autoimmune inner ear disease, therapies targeting IL-1 and TNFα for consecutive reduction of cytokine activity represent an effective remedy for the measurable restoration of sensory function.

Keywords:

• Sudden sensorineural hearing loss
• asymmetric bilateral hearing loss
• autoimmune inner ear disease
• interleukin
• TNF alpha

Acknowledgements

None.

Ethical approval

This retrospective review of patient data did not require ethical approval in accordance with local/national guidelines. Written informed consent was obtained from the patient for publication of the details of his medical case and any accompanying images.

AIED is a rare and hardly understood disease, which is why there is a lack of reliable controlled clinical trials. Immune modulating agents like anakinra and adalimumab are officially approved for the treatment of common rheumatologic diseases e. g. rheumatoid arthritis. However, the use in AIED can only be recommended based on previous clinical reports, all in all with a small number of patients. In our case the use of these both substances were clearly off-label.

Treatment options were discussed and reviewed by the Department of Otorhinolaryngology and the Department of Rheumatology. Generally, all performed procedures were decided after critical risk-benefit analysis and always in consensus with the patient and attending physicians.

Informed consent

Authors confirm that consent was obtained from the patients for this study.

Disclosure statement

The authors declare that they have no conflict of interest or competing interests.

Additional information

Funding

This manuscript did not receive any funding.