Cogan I Syndrome

The Cogan I syndrome is a combination of inflammation of the cornea of the eyes (keratitis) and a disturbance of the sense of balance due to irritation of the 8th cranial nerve. Cogan I syndrome, often simply referred to as Cogan syndrome, is a rare clinical picture.

What is Cogan I Syndrome?

The Cogan I syndrome was first described in 1945 by the American ophthalmologist Cogan. The clinical picture was already known before this time, but was mistakenly taken for an atypical course of Meniere’s disease. The symptoms of both diseases are similar, but Meniere’s disease has completely different causes.

The Cogan I syndrome is also known as oculovestibulo-auditory syndrome in medical parlance. The eyes and ears are mainly affected by Cogan I syndrome. What often complicates the diagnosis in medical practice is the fact that many other organs and organ systems can also be affected and, as a result, cause many different symptoms.

The Cogan-I syndrome is further subdivided into a so-called typical and atypical Cogan-I syndrome. In addition, there must be a therapeutic differentiation from the Cogan II syndrome, which has no parallels to the Cogan I syndrome. In the Cogan II syndrome, the ability to coordinate actions and movements of the eyes is disturbed. The Cogan II syndrome is therefore a congenital form of oculomotor apraxia.

Causes

The cause of Cogan I syndrome is still unknown today. Since this is a rare clinical picture, the cause research for the Cogan I syndrome is not carried out in the way it would be necessary to unambiguously identify the cause due to a lack of financial means. However, this represents a general problem in research into the causes of rare and very rare diseases. It is assumed that the occurrence of Cogan I syndrome is based on inflammatory processes.

It can be assumed that the actual cause can be traced back to an autoimmune process, in the course of which fine connective tissue structures become chronically inflamed and then trigger the symptoms of the disease. In the case of the Cogan I syndrome, in the narrower sense, it is likely to be a question of so-called vascular and interstitial inflammatory processes.

On the one hand, the inner walls of the vessels are changed by the chronic inflammatory process, and on the other hand, the intercellular tissue as well. Depending on the severity of the inflammatory changes, the Cogan I syndrome can occur in phases, with almost symptom-free intervals. However, after a certain degree of cell damage, the symptoms persist.

Symptoms, ailments & signs

The 4 main symptoms of Cogan I syndrome are keratitis, inner ear hearing loss, tinnitus and dizziness. These complaints usually occur in both forms, i.e. in the typical and atypical Cogan I syndrome, to varying degrees, individually or together. The course of the disease and the appearance of the symptoms can occur acutely or over a longer period of time in the form of relapses.

All patients with a diagnosed Cogan I syndrome suffer a more or less pronounced loss of hearing during the course of the disease. After all, the increasing hearing loss leads to complete deafness in about half of the patients. Excruciating dizziness, vertigo, and ringing in the ears, tinnitus, are common accompanying symptoms in most patients. The most common symptom of the eyes is keratitis, while scleritis or panuveitis have also been described.

The pathological changes in the eyes always occur in both eyes in Cogan I syndrome and can vary considerably in terms of duration, treatability and severity. A so-called visual acuity reduction usually does not occur despite the severe inflammatory eye changes. Patients do not lose sight of their eyes either, as the retina is typically not affected by Cogan I syndrome.

In addition to the ears and eyes, the entire rest of the body can also be affected by the vasculitides. If the heart, aorta, central nervous system or kidneys are affected, one speaks of generalized vasculitis, as a result of which around 10 percent of patients with Cogan I syndrome die. Generalized lymph node swelling, joint inflammation and fever can also occur in the chronic course of the disease.

Diagnosis & course

The diagnosis of Cogan I syndrome is based solely on symptoms and findings. Due to the inflammatory changes in the blood, acute phase proteins play a central role in the diagnosis. If there are significant ocular signs, the diagnosis does not cause the specialist any problems; if these are missing, the disease is still not recognized correctly for years. In addition, if there are no eye findings, it is more difficult to differentiate precisely from Meniere’s disease. The Cogan I syndrome is characterized by a chronic, relapsing and intermittent course.

Complications

In Cogan I syndrome, the patient’s ears are most affected. In most cases, people will develop severe hearing loss and tinnitus. Both symptoms limit the patient’s everyday life extremely and can also lead to psychological problems and depression.

The beeping in the ear often causes sleep disorders and headaches. The symptoms greatly reduce the quality of life. It also leads to dizziness and thus to coordination disorders. The symptoms do not have to be permanent, but can also go away on their own in the course of a lifetime and reappear suddenly. In the worst case, Cogan I syndrome can lead to complete hearing loss.

In severe cases, other organs are also affected, such as the kidneys and heart. If Cogan I syndrome is not treated, it usually leads to death. Those affected often complain of swelling and inflammation in the joints and a high fever.

Targeted treatment and healing of the symptom is not possible. Because of this, the Cogan I Syndrome loading is aimed at minimizing the symptoms. Immunosupressants are administered. There are usually no further complications, but the life of the person affected is very limited by the numbness.

When should you go to the doctor?

As a rule, a doctor should always be consulted with Cogan I syndrome if the person concerned suffers from ear discomfort. This could be a pain in the ear or an inner ear hearing loss. Tinnitus can also occur in Cogan I syndrome and significantly reduce the quality of life of the person affected. If the tinnitus persists and does not go away on its own, an ENT doctor should be consulted. Medical treatment is also very sensible and advisable if these symptoms occur in a relapsing manner.

In the worst case, it can lead to numbness. In many cases, the Cogan I syndrome also leads to psychological complaints or depression. If these symptoms occur together with a fever or swelling of the lymph nodes, an examination is also advisable. A psychologist should be consulted in the event of mental health problems. The diagnosis of this disease can be made by the general practitioner or the ENT doctor. However, further treatment is carried out by the respective specialist and depends largely on the severity of the symptoms.

Treatment & Therapy

A cure for Cogan I syndrome is still not possible due to the unknown causes. However, with anti-inflammatory and immunosuppressive therapy, the symptoms can be greatly alleviated or temporarily made to disappear completely. It is not uncommon for those affected to have lifelong therapy.

If the symptoms only affect the eyes and ears, the general life expectancy is not reduced. If the vasculitis occurs in other organs and organ system, however, the mortality increases significantly. In addition to the use of high-dose immunosuppressants such as cyclophosphamide or cortisone, hearing aids or so-called cochlear implants are used to compensate for the hearing loss in the inner ear. If the disease progresses, bilateral deafness cannot be prevented despite therapy. The severe side effects of the drugs used are also a problem.

Outlook & forecast

There is no complete cure in Cogan I syndrome. The syndrome can only be treated symptomatically, as causal therapy is not possible. In some cases, however, it is possible to limit the symptoms completely, although these limitations often only occur for a short time. The success of the treatment depends very much on the exact form of the Cogan I syndrome, so that a generally valid prediction is not possible here.

Patients are often dependent on lifelong therapy in order to improve their quality of life. Since the symptoms usually only affect the ears and eyes, the life expectancy of the patient is not negatively affected. In other regions of the body, however, the symptoms can reduce the quality of life. The hearing loss can be limited with the help of hearing aids. Furthermore, those affected are also dependent on taking strong medication, which is associated with many side effects.

If no treatment is given for Cogan I syndrome, the result is a significantly reduced quality of life and severe restrictions in everyday life. Often the symptoms continue to worsen as a result. This may also lead to premature death of the person concerned.

Prevention

Cogan I syndrome occurs in all age groups and in both sexes equally. Since prophylaxis is not possible, the prognosis depends mainly on the earliest possible diagnosis, which in most patients is made in early adulthood. Only an immediate bilateral supply of cochlear implants can prevent the otherwise progressive deafness.

Aftercare

Cogan I syndrome is a multi-system disease. Because of the various affected areas in the organism, this requires careful follow-up care. The problem with all diseases that occur so rarely is that medicine has so far not known enough about them. The research deficit also affects treatment and follow-up measures.

Both typical and atypical Cogan I syndrome have various sequelae. These require symptomatic treatment. For this, and for aftercare, different doctors or interdisciplinary approaches can be considered.

Consequences of illness such as keratitis, inner ear hearing loss or tinnitus as well as dizziness must be treated. If the numbness increases, a cochlear implant may be necessary. The increasing eye problems with Cogan I syndrome also require careful follow-up care.

What is difficult is that generalized vasculitis can occur as a result of inflammatory processes. About ten percent of all affected patients with chronic Cogan I syndrome die from it. Even good aftercare cannot prevent this. Another problem is that many doctors fail to recognize this multi-system disease. If the Cogan I syndrome is correctly identified, only symptomatic treatment and follow-up care can be provided in the absence of meaningful therapeutic concepts.

You can do that yourself

Cogan I syndrome is a very rare disease, the causes of which are still unknown. Therefore, the patient cannot take any self-help measures that have a causal effect. But there are some measures that can help you cope better with everyday life.

Severe dizziness, tinnitus and a disorder of the sense of balance are typical of the disease. The latter in particular can severely restrict the mobility of those affected and enormously increase the risk of accidents. Patients should not be afraid to use walking aids and, if necessary, a wheelchair to help prevent falls and improve their mobility.

In many of those affected, hearing also deteriorates. Hearing aids or implants usually help here. However, despite these aids, complete hearing loss cannot always be prevented. If complete deafness becomes apparent, those affected should learn sign language as early as possible in order to be able to communicate even after the hearing loss. Lessons are easier as long as those affected still understand spoken instructions.

Although Cogan I syndrome is a very rare disease, a self-help group has now been set up in German-speaking countries that is also active on the Internet. Patients and their relatives should take the opportunity to exchange ideas with other affected persons.

Cogan I Syndrome