Cogan I Syndrome

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The Cogan-I syndrome , often also called oculovestibulo-auditory syndrome or simply Cogan's syndrome , is a rare, audio-vestibular disease of the inner ear with involvement of the eyes , the cause of which is suspected to be an autoimmune reaction. Its name goes back to the American ophthalmologist David Glendering Cogan (1908-1993), who in 1945 first described the complete clinical picture with inflammation of the cornea , a so-called interstitial keratitis , deafness and dizziness .

root cause

The exact cause of the disease is not yet known. Vascular inflammatory processes ( vasculitis ) are suspected .

Symptoms

Classification according to ICD-10
H16.3 Interstitial and deep keratitis
H90.3 Inner ear hearing loss
H93.1 Tinnitus
H81.3 peripheral vestibular dizziness
ICD-10 online (WHO version 2019)

The occurrence and course of the disease are very different. The symptoms can appear simultaneously or consecutively. The distribution of the ear and eye symptoms as an initial finding are about the same frequency. The changes occur acutely or in bursts over the years.

Ears

Almost all patients are affected by hearing loss, which can lead to deafness in 25–50% of cases . Tinnitus and dizziness are also common.

eyes

In addition to keratitis, the ocular symptoms often consist of episcleritis , scleritis and panuveitis . As a rule, the pathological changes occur on both sides and vary in severity, treatability and duration. A sustained decrease in visual acuity , however, usually does not occur, with the exception of scleritis and panuveitis.

General symptoms

General symptoms are fever , arthritis , myalgia , lymph node swellings , polyneuropathy , and localized and generalized vasculitis in the area of, for example, the aorta , myocardium , CNS , and kidney in about 10-20% of patients . These symptoms are responsible for the lethality of the Cogan I syndrome of about 10%.

Diagnosis

The diagnosis must predominantly be made on the basis of clinical findings. If there are no significant ocular signs, the diagnosis of Cogan I syndrome is difficult: the typical ear findings "often intermittent hearing loss, dizziness, tinnitus" are difficult to differentiate from Menière's disease , especially if there are no eye findings .

therapy

High-dose cortisone is used therapeutically in acute illnesses. If the symptoms are worsened with general symptoms, immunosuppressants such as cyclophosphamide , azathioprine etc. a. Use. A plasmapheresis has brought considerable improvement in individual cases. There are indications that, especially in children, only early treatment (up to 2 weeks after occurrence) can have a positive effect on the course of the disease. Otherwise, the hearing loss is compensated conventionally with a hearing aid or a cochlear implant .

Demarcation

The Cogan I syndrome is often referred to as Cogan syndrome for short . Its namesake described another clinical picture in 1952, oculomotor apraxia , which is sometimes also called Cogan II syndrome . Another independent eye disease is listed as Cogan-Reese syndrome , also known as iris nevus syndrome , part of iridocorneal endothelial syndrome (ICE). However, all three diseases are different and independent clinical pictures.

Individual evidence

  1. ↑ David C. Cogan: Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. In: Archives of Ophthalmology. Vol. 33, No. 2, 1945, ISSN  0093-0326 , pp. 144-149, doi : 10.1001 / archopht.1945.00890140064007 .
  2. ^ Commission "Guidelines of the German Society for Neurology": Guidelines for diagnostics and therapy in neurology. 4th revised edition. Georg Thieme, Stuttgart et al. 2008, ISBN 978-3-13-132414-6 , pp. 654 ff., Online .
  3. ↑ Rudolf Sachsenweger (Ed.): Neuroophthalmology . 3rd, revised edition. Thieme, Stuttgart et al. 1982, ISBN 3-13-531003-5 , p. 514 .
  4. ↑ a b Randall S. Vollertsen, Thomas J. MacDonald, Brian R. Younge, Peter M. Banks, Anthony W. Stanson, Duane M. Ilstrup: Cogan's syndrom: 18 cases and a review of literature . In: Mayo Clinic Proceedings . tape 61 , no. 5 , 1986, ISSN  0025-6196 , pp. 344-361 , doi : 10.1016 / S0025-6196 (12) 61951-X , PMID 3486332 .
  5. ^ G. Migliori, E. Battisti, M. Pari, N. Vitelli, C. Cingolani: A shifty diagnosis: Cogan's syndrome. A case report and review of the literature. In: Acta Otorhinolaryngologica Italica. Vol. 29, No. 2, 2009, ISSN  0392-100X , pp. 108-113, PMID = 20111622, online (PDF; 2.3 MB) .
  6. ↑ Michael B. Gluth, Keith H. Baratz, Eric L. Matteson, Colin LW Driscoll: Cogan syndrome: a retrospective review of 60 patients throughout a half century. In: Mayo Clinic Proceedings. Vol. 81, No. 4, 2006, ISSN  0025-6196 , pp. 483-488, doi : 10.4065 / 81.4.483 , PMID 16610568 .
  7. ↑ M. Zierhut, T. Schlote, T. Daikeler, N. Stübiger, I. Kötter, D. Bless: Ocular manifestation in the Cogan I syndrome. Abstracts for: 96th annual meeting of the DOG. 1998.
  8. ↑ Manfred Zierhut, Torsten Schlote, Nicole Stübiger, Thomas Daikeler, Ina Kötter, Dominik Bless, Assen Koitschev: Cogan-I syndrome: Clinic, therapy and prognosis . In: The ophthalmologist . tape 97 , no. 3 , 2000, ISSN  0941-293X , p. 197-202 , doi : 10.1007 / s003470050514 .
  9. ↑ a b Alexandra Dorn, Ali Baghai, Gerhard Dockter, Klaus Wilhelm Ruprecht, Friedrich Carl Sitzmann: The Cogan I Syndrome . In: Monthly Pediatrics . tape 143 , 1995, ISSN  0026-9298 , pp. 328-333 .
  10. ↑ O. Selivanova, BR Haxel, WJ Mann: The Cogan Syndrome . In: ENT . tape 54 , no. 8 , 2006, ISSN  0017-6192 , p. 619–623 , doi : 10.1007 / s00106-005-1316-0 .
  11. ^ Nancy B. Allen, C. Christine Cox, Mary R. Jacobs, Rex M. McCallum, Barton F. Haynes, Joseph Kisslo, Michael Cobo: Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan's syndrome . In: The American Journal of Medicine . tape 88 , no. 3 , 1990, ISSN  1548-2766 , pp. 296-301 , doi : 10.1016 / 0002-9343 (90) 90157-9 , PMID 2309745 .