Cogan Syndrome (Non-syphilitic Keratitis And Vestibular Auditory Syndrome)

Cogan Syndrome
Body Parts: Head Whole Body
Medical Subjects: Nervous System

What Is Cogan Syndrome?

Cogan syndrome is a chronic inflammatory disease of unknown etiology.

It mainly presents with specific lesions in the eyes (redness, pain, photophobia and blurred vision of the eyes), and the inner ear (vertigo, uncoordinated movements, nausea, vomiting, tinnitus and hearing loss, etc.). It can also present as systemic vasculitis or other nonspecific systemic symptoms.

Most patients can be controlled by the use of glucocorticoids and immunosuppressive therapy, and some patients may require surgery. Isolation is not required during the illness, but the quality of life of most patients will be affected to a certain extent.

Is Cogan syndrome common?

Although no exact statistics are available, Cogan syndrome is not common clinically.


What Is The Cause Of Cogan Syndrome?

The cause of Cogan syndrome is still unclear. There are many hypotheses, and most of them believe that it may be caused by an autoimmune response to an unknown common autoantigen in the cornea and inner ear.

Who is Cogan syndrome common to?

Cogan syndrome is common in young people, and the peak age of onset is in the 20s. The current study did not find differences in gender and race.


What Are The Symptoms Of Cogan Syndrome?

The most specific manifestations of Cogan syndrome are in the eyes and inner ear:

  • Ocular lesions: The main manifestation is corneal stromitis (IK). Patients may have eye redness, eye pain, photophobia and blurred vision, and even cause visual impairment, and a few may even cause permanent visual loss.
  • Inner ear lesions: Mainly manifested as auditory-vestibular dysfunction, patients can experience dizziness, ataxia (incoordination of movements), nausea, vomiting, tinnitus and hearing loss, vibration hallucinations, etc., similar to the clinical manifestations of Meniere's disease.

The first symptom of most patients is one of the two major lesions mentioned above, and the probability of occurrence is almost equal. However, as the disease progresses, about 5 months later, 75% of patients will have symptoms of the ocular and vestibular auditory system at the same time.

At the same time, a small number of patients will show systemic vasculitis, which will show corresponding manifestations according to whether it involves large, medium and small blood vessels or aorta. For example, aortitis can cause severe consequences such as aortic valve insufficiency, thoracic and abdominal aortic aneurysm, severe chest pain, limb pain and other symptoms, even threatening life.

In addition, Cogan syndrome can also show some nonspecific systemic symptoms, such as fever, fatigue, weight loss, enlarged lymph nodes, hepatomegaly, splenomegaly, pulmonary nodules, pericarditis, abdominal pain, joint pain, arthritis, myalgia and urticaria, etc.

What parts does Cogan syndrome usually occur?

As mentioned above, Cogan syndrome most often occurs in the eye and inner ear. There are also a few manifestations of systemic vasculitis, involving large, medium and small blood vessels and even aorta.

How does Cogan syndrome develop?

Most patients with Cogan syndrome start from either the eye or the inner ear. As the disease progresses, they will also have both lesions at the same time.

10% to 30% of cases are also accompanied by severe systemic vasculitis, which may occur within weeks to years of the onset of the disease.

What are the serious consequences of Cogan syndrome?

Cogan syndrome may cause permanent vision, hearing loss, vasculitis-related aortic arch occlusion, proximal aortic dilation, aortic insufficiency, thoracic-abdominal aortic aneurysm, etc. depending on the site involved. In severe cases, it may be life-threatening. 


How Is Cogan Syndrome Diagnosed?

The diagnosis of Cogan syndrome mainly relies on clinical examination to determine whether there are specific eye and inner ear lesions. At the same time, some related serological examinations may need to be perfected to rule out other diseases (such as syphilis, Lyme disease, Epstein-Barr virus infection, etc.).

For patients with suspected Cogan syndrome, based on their first symptoms:

  • An ophthalmic examination can be performed to determine the presence of corneal stromitis (IK), scleritis, and superficial scleritis, and to rule out other diseases and ocular lesions.
  • Nervous system and otological examinations can be done to determine whether there is abnormal auditory vestibular function.
  • If the systemic symptoms are onset, a rheumatological examination can be done to look for evidence of systemic vasculitis.

Cogan syndrome is easy to confuse with which diseases? How to distinguish?

Cogan syndrome is a disease with diverse symptoms and is easily confused with various diseases that can cause similar eye and inner ear specific clinical manifestations. Including the following diseases:

  • Congenital syphilis, tuberculosis, chlamydia infection and viral infection.
  • Behcet syndrome, Crohn's disease, sarcoidosis and Lyme disease.
  • Polyarteritis nodosa, granulomatous polyangiitis, rheumatoid arthritis and so on.

The distinction method has been mentioned in the previous article. According to the specific location and clinical manifestation, the corresponding serological and immunological blood test, vascular, cardiac ultrasound, vascular CTA and other imaging examinations, and even tissue biopsy are used for differential diagnosis.


Can Cogan Syndrome Be Prevented?

Unfortunately, because the exact pathogenesis of Cogan syndrome is still unclear, it cannot be prevented for the time being.

How can patients with Cogan syndrome prevent complications?

Seeing the corresponding specialist as soon as possible when the symptoms appear, confirming the diagnosis and receiving the correct treatment as soon as possible, can effectively prevent the occurrence of complications. Especially during the period of immunosuppressive treatment, it is necessary to strictly follow the doctor's instructions to take the medicine, and regular follow-up visits to monitor possible adverse reactions.


How Is Cogan Syndrome Treated?

The treatment of Cogan syndrome is mainly drug therapy, and the first choice is glucocorticoids and immunosuppressive agents.

For ocular lesions

Patients with inflammation of the anterior segment of the eye, such as corneal stromal inflammation and anterior uveitis, generally use glucocorticoids and mydriatics. The role of mydriatics is to reduce eye inflammation, prevent adhesions, and relieve eye discomfort.

Patients with inflammation of the posterior segment of the eye generally also need to use glucocorticoids, such as prednisone. If the effect is not good, other immunosuppressive agents, such as methotrexate, cyclophosphamide, etc. must be used.

For inn ear lesions and systemic vasculitis

The treatment is the same as for patients with inflammation of the posterior segment of the eye.

The dosage, course of treatment and reduction methods of hormones and other immunosuppressants are highly individualized. At the same time, it is necessary to closely monitor whether there are related adverse drug reactions in the process of medication, and strictly follow the guidance of the attending doctor. Never add or stop medication by yourself!

For acute vestibular dysfunction

The main treatment is bed rest. Antihistamines or benzodiazepines can be used, such as meclizine hydrochloride and diazepam.

For complications

  • People with severe hearing loss who do not respond to medication may require a cochlear implant.
  • Patients with progressive corneal opacities may require corneal transplantation.
  • If cataracts occur, cataract extraction may be necessary to correct the loss of vision.
  • Some patients with vasculitis involving heart vessels and valves may require surgical bypass or aortic valve replacement.

Can Cogan syndrome heal on its own?

can not. Cogan syndrome is a group of chronic inflammatory diseases. Without intervention, the disease usually progresses and worsens gradually, which may lead to permanent vision and hearing damage, and even life-threatening systemic macrovasculitis, especially aortitis.

Does Cogan syndrome need to be hospitalized?

Simple eye or inner ear disease usually does not need to be hospitalized. After the outpatient visit, you can take medicine at home and check it regularly.

If there are systemic manifestations or systemic vasculitis, depending on the specific blood vessels, organs and the severity of the disease involved, hospitalization or even surgery may be required.

What are the common side effects of drugs used to treat Cogan syndrome?

The most important treatment for Cogan syndrome is immunosuppressive therapy, among which:

  • Long-term use of glucocorticoids may cause side effects such as weight gain, inducing or aggravating infections, peptic ulcers, increased blood sugar, increased blood pressure, femoral head necrosis, and osteoporosis.
  • The use of immunosuppressive agents such as cyclophosphamide and methotrexate may cause gastrointestinal reactions such as nausea, vomiting, and loss of appetite. As well as hair loss, skin rash, anemia, liver and kidney damage, bone marrow suppression and other adverse reactions.

Use the medicine rationally under the guidance of the doctor, and check the blood routine, liver and kidney function during the medicine. If you can follow the doctor's advice, hormones and immunosuppressants are still safe and effective treatments.

Can Cogan syndrome be cured?

Local eye or inner ear disease can usually be cured after regular treatment, but it still needs regular outpatient review in the later period.

For diseases involving the whole body, especially large vasculitis, in addition to long-term medication control, further surgical treatment may be required to solve the problem.


What Impact Does Cogan Syndrome Have On Life?

What should the patient pay attention to in diet?

Patients with Cogan syndrome have nothing to pay special attention to in their diet. In general, eat more fruits and vegetables. When cooking, pay attention to putting less salt, using less salty seasonings, eating less pickled products, and not drinking as much as possible.

For patients with long-term hormone therapy, it is best to follow the diabetic diet, try to avoid foods that cause high blood sugar, and pay attention to your own blood sugar.

Do patients with Cogan syndrome need to be reviewed?

Need to review.

Patients with Cogan syndrome, regardless of the manifestations of the eyes, inner ears, or systemic vasculitis, need to go to the hospital for regular medication, and if necessary, undergo surgical treatment.

In the process of diagnosis and treatment, you should go to the hospital for regular review, especially for patients who have long-term systemic use of glucocorticoids and immunosuppressants, and need to monitor blood routine, liver and kidney function, bone mineral density and other related indicators.

Does Cogan syndrome affect fertility?

Cogan syndrome itself does not affect fertility, but if it is in the active stage of disease, especially when hormones and other immunosuppressants are used systemically, it is suggested to postpone pregnancy, because these drugs have potential embryotoxicity and teratogenicity and may cause adverse consequences.

Can people with Cogan syndrome fly?

For patients with Cogan syndrome who only show ocular lesions, flying is not affected. However, patients with acute vestibular dysfunction, i.e., dizziness, ataxia, vibration hallucinations, etc., are advised to stay in bed instead of flying.

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