Cogan’s Syndrome

, David G. I. Scott² and Chetan Mukhtyar²

(1)

Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK

(2)

Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK

19.1 Introduction

Cogan’s syndrome (CS) is a rare inflammatory disease of unknown etiology, characterized by the involvement of the eye and inner ear.

19.2 Definition and Classification

There are no validated diagnostic criteria or classification criteria.

19.3 Epidemiology

The incidence and prevalence of CS are unknown, but it occurs mainly in young adults with a peak incidence in those aged 20–30 years. There is no gender predominance.

19.4 Etiology

The etiology is unknown, but like many autoimmune conditions, is generally believed to result from an environmental trigger interacting with a genetically predisposed host. Infection has long been suspected as a trigger, but not proven. The eye and the ear are both capable of mounting a vigorous response to infection, trauma, or toxins. In an experimental model of autoimmune keratitis in rats, transfer of corneal specific T cells produces a severe keratitis. Similarly, transfer of activated T cells can induce vestibular inflammation in animal models.

19.5 Clinical Features

The combination of ocular and vestibular symptoms in a young adult should raise a suspicion of CS [1–3].

19.5.1 Systemic

The initial symptoms may be nonspecific with fever, weight loss and fatigue.

19.5.2 Ocular

The predominant complaints are of ocular pain, redness and photophobia. Blurring of vision, tearing, diplopia, a sensation of foreign body or visual field defects occur frequently. Slit lamp examination is necessary to observe interstitial keratitis, which occurs in 70 % of patients. The earliest findings are faint corneal infiltrates which are 0.5–1 mm in diameter. Other frequent manifestations are conjunctivitis (35 %), iridocyclitis (30 %) and episcleritis/scleritis (30 %). Less common features include posterior uveitis, and papillitis.

19.5.3 Vestibular and Auditory

The typical features are sudden onset of vertigo, nausea, vomiting, tinnitus and hearing loss. Attacks are similar to Meniere’s disease. Hearing loss occurs in 95 %, ataxia (45 %), nystagmus (30 %), and oscillopsia (15 %).

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