Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2009 Apr;29(2):108-13.

A shifty diagnosis: Cogan's syndrome. A case report and review of the literature

G Migliori  1 , E Battisti, M Pari, N Vitelli, C Cingolani
Affiliations
Review

A shifty diagnosis: Cogan's syndrome. A case report and review of the literature

G Migliori et al. Acta Otorhinolaryngol Ital. 2009 Apr.

Abstract
in English, Italian

Cogan's syndrome is defined as a chronic inflammatory disease of unknown origin, an autoimmune disease, characterized by bilateral sensorineural hearing loss, vestibular symptoms, inflammatory ocular manifestations with variable risk of developing into a systemic disease. The onset of disease is variable but is often characterized by isolated ocular symptoms or acute ear and/or vestibular manifestations, variably associated. The diagnosis of Cogan's syndrome can be a challenge as is evident in the case described here since it is based only on the association between bilateral ocular and vestibuloauditory symptoms with no specific diagnostic tests available.

La sindrome di Cogan viene descritta come una patologia infiammatoria cronica di incerta origine, ma più propriamente è una patologia autoimmune, caratterizzata da ipoacusia neurosensoriale bilaterale, sintomatologia vestibolare e manifestazioni infiammatorie oculari con un rischio variabile di sviluppare una malattia sistemica. La malattia può insorgere soltanto con sintomi oculari o con sintomi acuti uditivi e/o vestibolari, variamente associati. Porre diagnosi di sindrome di Cogan può essere una sfida come risulta evidente nel caso descritto perché si basa soltanto sull’associazione di sintomi oculari e audio-vestibolari, in mancanza di tests diagnostici specifici.

Keywords: Autoimmunity; Cogan’s syndrome; Hearing loss; Interstitial keratitis.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Normal hearing function.
Fig. 2
Fig. 2
Mild left sensorineural hearing loss.
Fig. 3
Fig. 3
Caloric testing showing left vestibular paresis.
Fig. 4
Fig. 4
HR-MRI showing abnormal MRI findings in the vestibule, semicircular canals, cochlea, vestibular nerve and first part of facial nerve on left side of head.
Fig. 5
Fig. 5
Moderate to severe left sensorineural hearing loss at high frequencies.
Fig. 6
Fig. 6
Severe to profound left hearing loss and mild right sensorineural hearing loss.
Fig. 7
Fig. 7
Caloric testing showing bilateral vestibular paresis.
Fig. 8
Fig. 8
Mild to severe right sensorineural hearing loss at high frequencies, severe to profound left hearing loss.
Fig. 9
Fig. 9
Mild right sensorineural hearing loss limited to high frequencies, severe to profound left hearing loss.
Fig. 10
Fig. 10
Mild right sensorineural hearing loss limited to 8KHz, severe to profound left hearing loss.
See this image and copyright information in PMC

References

    1. Cundiff J, Kansal S, Kumar A, Goldstein DA, Tessler HH. Cogan’s syndrome: a cause of progressive hearing deafness. Am J Otolaryngol 2006;27:68-70. - PubMed
    1. Cogan DG. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 1945;33:144-9. - PubMed
    1. Morgan RF, Baumgartner CJ. Ménière’s disease complicated by recurrent interstitial keratitis: excellent result following cervical ganglionectomy. West J Surg 1934;42:628-31.
    1. Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore) 1980;59:426-41. - PubMed
    1. McDonald TJ, Vollertsen RS, Younge BR. Cogan’s syndrome: audiovestibular involvement and prognosis in 18 patients. Laryngoscope 1985;95:650-4. - PubMed

LinkOut - more resources