First Author: J.Phee UK
Co Author(s): A. Kennedy N. Saunders E. Hughes
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Cogan’s syndrome is a rare chronic inflammatory disease that can lead to blindness, deafness and even death. It is described as “a syndrome of non-syphilitic interstitial keratitis (IK) and Ménière-like audiovestibular dysfunction”. The cause is still unknown and the age of onset can range from five to sixty-five. The diagnosis is usually made on clinical grounds, as there is no specific investigation for the disease. Here we present a case of Cogan’s syndrome with a rare ocular manifestation.
A 32-year-old man presented to the emergency department in the Sussex Eye Hospital, UK with a 10-day history of blurred vision and pain in his right eye. Prior to his ocular symptoms, he had 2-week history of deteriorating balance for which he had been prescribed prochlorperazine by his GP.
On examination, he had best corrected visual acuity of 6/6 in the left eye and 6/60 in the right eye. There was no relative afferent pupillary defect and no red desaturation. His intraocular pressures were 10mmHg in the left eye and 18mmHg in his right eye. His right eye had keratic precipitates, anterior chamber and vitreous inflammatory cells and, on dilated funduscopy, multiple white-centreed blot and flame shaped haemorrhages, which resembled Roth spots. He was prescribed topical steroid drops and admitted for further investigation to rule out endocarditis. However, he was apyrexial, had no bruits or murmurs on cardiac auscultation and had negative blood cultures. His echocardiogram was normal. There was no evidence of infection around his dialysis fistula. He had no oral or genital ulcers. His blood pressure was 134/78. His full blood count showed only a mild chronic anaemia with a haemoglobin level of 97 g/L but his platelet and white cell count were normal and his C-reactive protein was 5.2 mg/L. A random glucose level, ANA and ANCA screens were also normal along with Brucella, Fabry’s and Q Fever. His total CSF protein was 490 mg/L and glucose was 2.2 mmol/L, with a negative culture.
The otorhinolaryngology (ENT) team saw him a week later as he had developed bilateral moderate sensorineural hearing loss, tinnitus and true vertigo. His hearing loss, presumed idiopathic at the time, was treated with high dose oral prednisolone for 1 week. By this stage, the patient no longer had any anterior chamber activity and his vitreous cells had completely settled in response to topical steroids. His Roth spots had also faded. This combination of signs and symptoms led to a diagnosis of Cogan’s syndrome and he was weaned off oral steroids and started on cyclophosphamide. After two weeks of treatment, the patient’s visual acuity in the right eye had improved to 6/18. The ENT team reported that his hearing in the left ear was now normal in the lower mid frequencies with a residual high frequency drop off. However, in the right ear, his hearing was still significantly impaired and his balance had remained poor. His immunosuppressive therapy is ongoing.
Cogan’s Syndrome is a rare debilitating condition, which is diagnosed on clinical grounds, as there is no specific investigation available. The underlying mechanism responsible for the condition is still unknown. This is a case of Roth spots caused by Cogan’s syndrome, an association that has not previously been described.