Cogan’s syndrome is an uncommon disorder marked by an inflammatory
process that affects the eyes, inner ears, and systemic vasculitis. The
complications of the disease can be severe, in extreme cases leading to loss of
vision or hearing. The disease may probably have an immunological basis, but
the exact cause remains unidentified. The clinical appearance includes ocular,
audiovestibular, and general symptoms. Detection could be demanding and
delayed due to the rare occurrence of this condition and the analogy of
symptoms of other diseases. Therefore, clinicians should exclude different
abnormalities before making the final diagnosis. The treatment focuses on
limiting the inflammatory process and preventing complications. However,
glucocorticoids remain the first-line choice. The prognosis varies and depends on
the detection time and appropriate therapy implementation. Some patients may
achieve remission, but the other may develop chronic symptoms. Because Cogan
syndrome is a sparse condition, knowledge about it may be limited. This article
aims to change this state. In this paper, we provide an overview of the
pathophysiology, clinical manifestations, and diagnostic options. Moreover, we
highlight the differences between typical and atypical forms, analyze the utility
of therapeutic strategies, and indicate possible research directions.
Keywords: Cogan’s syndrome, vasculitis, etiology, diagnosis, treatment