Cogan syndrome is a rare vasculitis of children and young adults which primarily characterised by 1,4,6:
- inflammatory eye disease (ocular keratitis, uveitis, scleritis, optic neuritis) 6
- audiovestibular symptoms (similar to Meniere disease) 6
However, it can potentially affect a multitude of other organs (classically aortitis) 4.
The exact aetiology is not well known although an autoimmune aetiology has been postulated.
Treatment and prognosis
The onset of the disease is usually rapid with ocular findings preceding tinnitus and vertigo. If untreated the majority of patients will become deaf and 5% will be blind. Aortitis and aortic insufficiency develop in 10-15% of patients. Prognosis is good in patients without symptoms of aortitis and early steroid treatment may prevent deafness 6.
History and etymology
It was initially described by David Glendenning Cogan, American ophthalmologist (1908-1993) in 1945 5.
- 1. Albayram MS, Wityk R, Yousem DM et-al. The cerebral angiographic findings in Cogan syndrome. AJNR Am J Neuroradiol. 2001;22 (4): 751-4. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Casselman JW, Majoor MH, Albers FW. MR of the inner ear in patients with Cogan syndrome. AJNR Am J Neuroradiol. 1994;15 (1): 131-8. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 3. Gluth MB, Baratz KH, Matteson EL et-al. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin. Proc. 2006;81 (4): 483-8. doi:10.4065/81.4.483 - Pubmed citation
- 4. Grasland A, Pouchot J, Hachulla E et-al. Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology (Oxford). 2004;43 (8): 1007-15. doi:10.1093/rheumatology/keh228 - Pubmed citation
- 5. Norton EW, Cogan DG. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms; a long-term follow-up. AMA Arch Ophthalmol. 1959;61 (5): 695-7. - Pubmed citation
- 6. CT and MR angiography. Lippincott Williams & Wilkins Publishers. ISBN:078174525X. Read it at Google Books - Find it at Amazon