Can environmental or occupational hazards alter the sex ratio at birth? A systematic review.

INTRODUCTION

Cogan's syndrome is a rare autoimmune disease typically affecting the ocular and audiovestibular system. Inner ear involvement is associated with a poor prognosis, as despite therapy, over 50% of patients develop profound deafness. The standard treatment remains the use of high dose glucocorticosteroids and immunosuppressants. Studies from recent years have reported significantly higher rates of improvement and recovery among patients treated with biologic disease modifying antirheumatic drugs (bDMARDs), particularly infliximab.

CASE DESCRIPTION: We report the case of a 39-year-old Caucasian female who experienced bilateral interstitial keratitis, sudden hearing loss, labyrinthine symptoms and arthralgia. Based on the clinical picture, Cogan's syndrome was diagnosed. Initial treatment included high doses of glucocorticosteroids which did not result in a satisfactory clinical response. Due to the high risk of permanent deafness, it was decided to administer infliximab. Simultaneously, subcutaneous methotrexate was introduced, and the patient was referred for hyperbaric oxygen therapy. Follow-up hearing tests conducted four weeks later revealed a significant improvement. As a result of the treatment, hearing normalized and labyrinthine and ophthalmological as well as systemic symptoms disappeared.

CONCLUSION

Considering the unfavourable prognosis in Cogan's syndrome with vestibulocochlear involvement, it seems reasonable to quickly escalate treatment if there is no sufficient response to glucocorticosteroids within two weeks. Infliximab has the most documented effectiveness among DMARDs. Combining pharmacotherapy with HBOT can significantly improves treatment outcomes.

LEARNING POINTS: Improved prognosis This case highlights a potential therapeutic strategy that may improve outcomes in patients with Cogan's syndrome and reduce the risk of disability.Disease reminder Knowledge of this rare disease is essential so that the internist, in cooperation with the otolaryngologist and ophthalmologist, can quickly make the appropriate diagnosis and implement intensive treatment.