Cogan’s syndrome: eye and inner ear inflammation explained

Cogan’s syndrome is a rare condition in which the immune system appears to cause inflammation in two places: the front of the eye and the inner ear. Vasculitis UK notes that commonly there is also inflammation in other organs, particularly the heart and large blood vessels, the nervous system and the bowels. The Vasculitis Foundation, a US patient organisation, classes it as a form of vasculitis, meaning inflammation of blood vessels, and says it primarily affects the large vessels, especially the aorta.

Because it is so rare, many doctors will never see a case. Both eye and ear symptoms have to be put together, so diagnosis often needs input from ophthalmology, ENT and rheumatology.

Vasculitis UK lists the most common symptoms as a red, painful, light-sensitive or blurred eye, hearing loss that may become profound and permanent, vertigo (dizziness), poor balance, nausea and vomiting, and fever, fatigue and weight loss. The Vasculitis Foundation adds excess tear production, tinnitus or a sense of pressure in the ear, and says the condition typically starts with either eye inflammation or inner ear inflammation but often progresses to include both. Symptoms can come and go.

• Eye redness, pain, light sensitivity and blurred or decreased vision.

• Hearing loss, sometimes with tinnitus or a feeling of pressure in the ear.

• Vertigo and poor balance, with nausea and vomiting.

• Fever, tiredness and weight loss.

• Joint or muscle pain, headache and heart symptoms where blood vessels are involved.

Sudden hearing loss in one or both ears, and a painful red eye with reduced vision, both need prompt assessment, ideally the same day. Contact NHS 111 or your GP urgently, or attend A&E or an eye casualty department, if these occur. Call 999 for sudden severe chest or back pain, collapse or stroke-like symptoms.

The Vasculitis Foundation emphasises that early diagnosis and treatment are important to lower the risk of permanent hearing or vision loss. If you already have a diagnosis, report returning or new symptoms as soon as possible.

Vasculitis UK states that there are no specific diagnostic tests, and the diagnosis is made on clinical examination and history where a combination of eye and inner ear problems is described. Other infections and diseases, including granulomatosis with polyangiitis and rheumatoid arthritis, need to be excluded. The Vasculitis Foundation lists blood tests and urine tests, detailed examination by an eye doctor and an ear, nose and throat specialist, echocardiography, magnetic resonance angiography of blood vessels and, when indicated, a tissue biopsy. It says doctors will attempt to rule out conditions such as infections, Crohn’s disease, other vasculitis syndromes, lupus, certain cancers and multiple sclerosis.

In the UK, the typical route is a GP referral to ophthalmology or ENT, then rheumatology or a vasculitis centre if the picture suggests Cogan’s syndrome. Because many conditions can cause a red eye or hearing loss, it can take time and several appointments to reach the diagnosis.

Vasculitis UK says there are no clinical trials of treatment in Cogan’s syndrome. Most people need moderately high doses of prednisolone or other steroids. A few people with very mild eye disease may be treated with steroid or non-steroidal anti-inflammatory eye drops. Many also need other immunosuppressive drugs, which it lists as methotrexate, ciclosporin, azathioprine, tacrolimus or cyclophosphamide.

The Vasculitis Foundation notes that if hearing is impaired and does not respond to medicines, cochlear implants may help; that corneal transplant may be an option when the cornea is severely damaged; and that heart problems may need surgical procedures such as aortic valve replacement. Treatment decisions depend on severity and on whether blood vessels are involved.

These medicines have significant side effects, including a lower ability to fight infection and potential bone loss. The Vasculitis Foundation therefore stresses regular check-ups, infection prevention and discussing vaccinations with your doctor.

Vasculitis UK says the course varies significantly between patients. Some have an initial flare lasting weeks to months followed by a slowly progressive course, while others have complete remission with intermittent episodes of activity. Blindness is uncommon, at less than 5%, whereas deafness is a frequent and debilitating outcome, reported in up to 54% of patients. The Vasculitis Foundation adds that vertigo tends to improve with time although poor balance can persist.

It is a chronic condition with periods of relapse and remission, so ongoing medical care is needed. Hearing support, such as hearing aids, communication tactics and support from audiology, is often an important part of living with it. Some people choose private ENT or ophthalmology appointments to speed up assessment, but long-term immunosuppression is usually managed alongside an NHS rheumatology team.