Sensorineural Hearing Loss In A Patient With HLA-B27 Sclero-Uveitis

Abstract

Background: Audiovestibular disease exists in conjunction with several autoimmune disorders and while the exact inter-relation is not fully understood, their overlap warrants further investigation. We present a patient with HLA-B27-associated sclerouveitis and sensorineural hearing loss, and postulate a possible association between atypical Cogan's syndrome and HLA-B27 autoimmunity.

Brief Report: A 52 year-old HLA-B27 positive Caucasian woman with a history of intermittent sclerouveitis, presented with a two-month history of redness and discomfort in both eyes associated with left sided hearing deficit and tinnitus. Review of systems revealed a prodrome of fever, chills, myalgia, night sweats and arthralgic symptoms with pain involving her lower back, upper limbs and knee joints. An audiogram performed had demonstrated a left-sided sensorineural deficit. The patient's auditory symptoms had responded to oral corticosteroids which she taking, at the time of presentation.Upon intial work-up and exam, Cogan's syndrome was suspected and since the patient had responded to systemic corticosteroids,immunomodulatory therapy was considered.The patient was started on oral methotrexate and her ocular symptoms resolved.

Conclusion: While the association between HLA-B27 and Cogan's syndrome has not been previously described, it may have profound clinical implications. Untreated autoimmune disease can result in significant morbidity and mortality. While the course and prognosis of HLA-B27 associated diseases are well delineated, their possible association with Cogan's syndrome must alert physicians to the possibility of significant small vessel autoimmune disease and require prompt systemic immunomodulation