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Cogan syndrome

AI overview

Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus (ringing in the ears) and hearing loss.

Key points
  • It is a rare, rheumatic disease characterized by inflammation of the ears and eyes.
    source quote
    Cogan syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes.
  • It can lead to deafness or blindness if untreated.
    source quote
    It can lead to deafness or blindness if untreated.
  • It may be associated with blood-vessel inflammation (vasculitis) that can cause major organ damage in 15% of those affected or even death in a small number of cases.
    source quote
    The condition may also be associated with blood-vessel inflammation (called vasculitis ) in other areas of the body that can cause major organ damage in 15% of those affected or, in a small number of cases, even death.
  • It most commonly occurs in a person's 20s or 30s.
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    It most commonly occurs in a person's 20s or 30s.
  • It is currently thought to be an autoimmune disease.
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    It is currently thought that Cogan syndrome is an autoimmune disease .
  • Infection with Chlamydia pneumoniae has been demonstrated in some patients prior to the development of Cogan syndrome.
    source quote
    Infection with the bacteria Chlamydia pneumoniae has been demonstrated in some patients prior to the development of Cogan syndrome
Symptoms
  • Recurrent inflammation of the front of the eye (the cornea).
    source quote
    recurrent inflammation of the front of the eye (the cornea )
  • Fever, fatigue, and weight loss.
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    often fever, fatigue, and weight loss
  • Episodes of vertigo (dizziness).
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    episodes of vertigo (dizziness)
  • Tinnitus (ringing in the ears).
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    tinnitus (ringing in the ears)
  • Hearing loss.
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    and hearing loss
  • Vision difficulty.
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    Cogan syndrome can lead to vision difficulty
Diagnosis
  • White blood cell count, erythrocyte sedimentation rate, and C-reactive protein tests may be abnormal but none is a reliable indicator.
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    none of these findings is a reliable indicator of the disease
  • There may be abnormally high levels of platelets or too few red blood cells.
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    there may be abnormally high levels of platelets in the blood or too few red blood cells in the blood
  • A slit-lamp examination is essential.
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    A slit-lamp examination is essential.
  • High-resolution MRI and antibodies to inner ear antigens may be helpful.
    source quote
    high-resolution MRI and antibodies to inner ear antigens may be helpful
  • It can occur in children and is particularly difficult to recognize in that situation.
    source quote
    Cogan syndrome can occur in children, and is particularly difficult to recognize in that situation.
Treatment
  • For more severe disease, oral corticosteroids may be necessary to reduce the inflammatory response.
    source quote
    For more severe disease, oral corticosteroids may be necessary to reduce the inflammatory response.
  • Other immunosuppressive medications are recommended when large amounts of steroids are required or if the disease is not responding to steroid therapy.
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    When large amounts of steroids are required or if the disease is severe and is not responding to steroid therapy, other immunosuppressive medications often are recommended.
  • Immunosuppressive drugs include methotrexate, cyclophosphamide, cyclosporine or azathioprine.
    source quote
    These immunosuppressive drugs include methotrexate , cyclophosphamide , cyclosporine or azathioprine .
  • In some cases, combinations of these medicines are prescribed.
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    In some cases, combinations of these medicines are prescribed.
  • Cochlear implantation may be used to restore some sense of hearing if the disease has damaged blood vessels in the ear.
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    if the disease has damaged blood vessels in the ear, cochlear implantation may be used to restore some sense of hearing
  • Cinnarizine is used to treat nausea and vomiting associated with Cogan syndrome and has shown significant improvement in hearing loss in some patients.
    source quote
    Studies have shown it to produce significant improvement in hearing loss in some patients.
Red flags
  • Can lead to deafness or blindness if untreated.
    source quote
    It can lead to deafness or blindness if untreated.
  • Blood-vessel inflammation (vasculitis) can cause major organ damage in 15% of those affected.
    source quote
    can cause major organ damage in 15% of those affected
  • In a small number of cases, it can cause death.
    source quote
    in a small number of cases, even death
Prognosis

Can lead to deafness or blindness if untreated; vasculitis can cause major organ damage in 15% of those affected or even death in a small number of cases.

AI-synthesized from the Wikipedia article “Cogan syndrome”. Not medical advice. Verify source →
Cogan syndrome
Other namesCogan's syndrome
SpecialtyRheumatology 

Cogan syndrome (also Cogan's syndrome) is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus (ringing in the ears) and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D. G. Cogan in 1945.

Cogan syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes. Cogan syndrome can lead to vision difficulty, hearing loss and dizziness. The condition may also be associated with blood-vessel inflammation (called vasculitis) in other areas of the body that can cause major organ damage in 15% of those affected or, in a small number of cases, even death. It most commonly occurs in a person's 20s or 30s. The cause is not known. However, one theory is that it is an autoimmune disorder in which the body's immune system mistakenly attacks tissue in the eye and ear.[1]

It is currently thought that Cogan syndrome is an autoimmune disease. The inflammation in the eye and ear are due to the patient's own immune system producing antibodies that attack the inner ear and eye tissue. Autoantibodies can be demonstrated in the blood of some patients, and these antibodies have been shown to attack inner ear tissue in laboratory studies. Infection with the bacteria Chlamydia pneumoniae has been demonstrated in some patients prior to the development of Cogan syndrome, leading some researchers to hypothesize that the autoimmune disease may be initiated by the infection. C. pneumoniae is a common cause of mild pneumonia, and the vast majority of patients who are infected with the bacteria do not develop Cogan syndrome.[2]

While the white blood cell count, erythrocyte sedimentation rate, and C-reactive protein tests may be abnormal and there may be abnormally high levels of platelets in the blood or too few red blood cells in the blood, none of these findings is a reliable indicator of the disease. A slit-lamp examination is essential. Recent work has suggested that high-resolution MRI and antibodies to inner ear antigens may be helpful. Cogan syndrome can occur in children, and is particularly difficult to recognize in that situation.[3]

For more severe disease, oral corticosteroids may be necessary to reduce the inflammatory response. When large amounts of steroids are required or if the disease is severe and is not responding to steroid therapy, other immunosuppressive medications often are recommended. These immunosuppressive drugs include methotrexate, cyclophosphamide, cyclosporine or azathioprine. In some cases, combinations of these medicines are prescribed. Occasionally, if the disease has damaged blood vessels in the ear, cochlear implantation may be used to restore some sense of hearing.[4]

Cinnarizine is mainly used to treat nausea and vomiting associated with motion sickness, vertigo, Ménière's disease, or Cogan syndrome.[5] Studies have shown it to produce significant improvement in hearing loss in some patients.[6]

In 1945, the ophthalmologist David Glendenning Cogan (1908–1993) first described the "nonsyphilitic interstitial keratitis and vestibuloauditory symptoms" that would later bear his name.[7] In 1963, the atypical form of Cogan syndrome, also known as "Logan Syndrome" was first described.[8]

  1. ↑ "Cogan's Syndrome". Archived from the original on 2008-10-14. Retrieved 2008-11-06.
  2. ↑ Timothy C. Hain. "Cogan's Syndrome". Archived from the original on May 17, 2008. Retrieved 2008-11-06.
  3. ↑ Timothy C. Hain. "Cogan's syndrome". Retrieved 2008-11-06.
  4. ↑ Chertok, Barbara Liss (27 October 2020). "Hearing Is Believing". Hearing Health Foundation. Retrieved 2 December 2020.
  5. ↑ "Cogan's Syndrome – Treatment". Retrieved 2008-11-06.{{cite web}}: CS1 maint: deprecated archival service (link)
  6. ↑ Timothy C. Hain: Cogan's Syndrome Archived 2008-05-17 at the Wayback Machine at the American Hearing Research Foundation, Chicago, Illinois 2008.
  7. ↑ Cogan, David G. (1945). "Syndrome of Nonsyphilitic Interstitial Keratitis and Vestibuloauditory Symptoms". Archives of Ophthalmology. 33 (2): 144–9. doi:10.1001/archopht.1945.00890140064007.
  8. ↑ Bennett FM (April 1963). "Bilateral recurrent episcleritis associated with posterior corneal changes, vestibulo-auditory symptoms and rheumatoid arthritis". American Journal of Ophthalmology. 55: 815–8. doi:10.1016/0002-9394(63)92451-6. PMID 13967466.