Skip to main content

Advertisement

Log in

Cogan’s syndrome: present and future directions

  • Review
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Cogan’s syndrome, typified by the combination of interstitial keratitis and immune-mediated sensorineural hearing loss, is a rare condition, and commonly associated with a diagnostic delay. Using a standard search protocol, we review the literature to date, focusing on a number of key areas pertaining to diagnosis, presentation and treatment. Using a case illustration of atypical disease which led to fulminant aortic regurgitation, we highlight the need for continued and collaborative research in order to identify negative prognostic factors and thus tailor therapeutic regimens. Atypical Cogan’s syndrome is more commonly associated with systemic manifestations than typical disease, and may be refractory to immunosuppressive treatment. We discuss the application of laboratory (e.g antibodies targeting inner ear antigens) and radiological (PET-CT) aids to disease confirmation and detection of sub-clinical vascular inflammation. As illustrated by the included case description, some patients remain refractory to intense immunosuppression and delineation of adverse prognostic factors which may direct treatment, perhaps including the use of PET-CT, will contribute in the future to improving patient outcomes

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Cogan DG (1945) Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 33:144–149

    Google Scholar 

  2. Haynes BF, Kaiser-Kupfer MI, Mason P et al (1980) Cogan syndrome: studies in thirteen patients, long-term follow-up and a review of the literature. Medicine 59:426–441. doi:10.1097/00005792-198011000-00003

    Article  PubMed  CAS  Google Scholar 

  3. Morgan RF, Baumgarten CF (1934) Meniere’s disease complicated by recurrent interstitial keratitis: excellent results following cervical ganglionectomy. West J Surg Obstet Gynaecol 42:628–631

    Google Scholar 

  4. Grasland A, Pouchot J, Hachulla E et al (2004) Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology 43:1007–1015. doi:10.1093/rheumatology/keh228

    Article  PubMed  CAS  Google Scholar 

  5. Gluth MB, Baratz KH, Matteson EL et al (2006) Cogans syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 81(4):483–488

    Article  PubMed  Google Scholar 

  6. McDonald TJ, Volertsen RS, Younge BR (1985) Cogan’s syndrome: audiovestibular involvement and prognosis in 18 patients. Laryngoscope 95:650–654. doi:10.1288/00005537-198506000-00003

    Article  PubMed  CAS  Google Scholar 

  7. Gloddek B, Gloddek J, Arnold W (1999) A rat T-cell line that mediates autoimmune disease of the inner ear in the Lewis rat. ORL J Otorhinolaryngol Relat Spec 61:181–187. doi:10.1159/000027668

    PubMed  CAS  Google Scholar 

  8. Garcia Berrocal JR, Vargas JA, Vaquero M, Ramon y Cajal S, Ramirez-Camacho RA (1999) Cogan’s syndrome: an ocular-audiovestibular disease. Postgrad Med J 75(883):262–264

    PubMed  CAS  Google Scholar 

  9. Merle H, Trode M, Smadje D (1995) Interstitial keratitis and Cogan syndrome. J Fr Ophthamlol 18(1):50–54

    CAS  Google Scholar 

  10. St Clair EW, McCallum RM (1999) Cogan’s syndrome. Curr Opin Rheumatol 11(1):47–52. doi:10.1097/00002281-199901000-00008

    Article  PubMed  CAS  Google Scholar 

  11. Shah P, Luqmani PA, Murray PI, Honan WP, Corrigan PG, Emery O (1994) Posterior scleritis—an unusual manifestation of Cogan’s syndrome. Br J Rheumatol 33(8):774–775. doi:10.1093/rheumatology/33.8.774

    Article  PubMed  CAS  Google Scholar 

  12. Covelli M, Lapadula G, Pipitone V (1999) Cogan’s syndrome: unsuccessful outcome with early combination therapy. Clin Exp Rheumatol 17(4):479–483

    PubMed  CAS  Google Scholar 

  13. Bachynski B, Wise J (1984) Cogan’s syndrome: a treatable cause of neurosensory deafness. Can J Ophthalmol 19(3):145–147

    PubMed  CAS  Google Scholar 

  14. Helmchen C, Arbusow V, Jager L, Strupp M, Stocker W, Schulz P (1999) Cogan’s syndrome: clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol 119(5):528–536. doi:10.1080/00016489950180748

    Article  PubMed  CAS  Google Scholar 

  15. Gelfand ML, Kantor T, Gorstein F (1972) Cogan’s syndrome with cardiovascular involvement: aortic insufficiency. Bull N Y Acad Med 48(4):647–660

    PubMed  CAS  Google Scholar 

  16. Cremer J, Laas J, Heublein B (1993) A case of coronary stenosis developing after successful aortic valve repair in Cogan’s syndrome. Thorac Cardiovasc Surg 41(4):255–257. doi:10.1055/s-2007-1013866

    Article  PubMed  CAS  Google Scholar 

  17. Lunardi C, Basson C, Leandri M, Navone R, Lestani M, Millo E et al (2002) Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet 360(9337):915–921. doi:10.1016/S0140-6736(02)11028-2

    Article  PubMed  CAS  Google Scholar 

  18. Loveman D, De Comarmond C, Cepero R, Baldwin D (2004) Autoimmune sensorineural hearing loss. Semin Arthr Rheum 34(2):538–543. doi:10.1016/j.semarthrit.2003.10.002

    Article  CAS  Google Scholar 

  19. Naumann A, Hempel JM, Schorn K (2001) Detection of humoral immune response to inner ear proteins in patients with sensorineural hearing loss. Laryngorhinootologie 80(5):237–244. doi:10.1055/s-2001-13883

    Article  PubMed  CAS  Google Scholar 

  20. Moscicki RA, San Martin JE, Quintero CH, Rauch SD, Nadol JB, Bloch KJ (1994) Serum antibodies to inner ear proteins in patients with progressive hearing loss. Correlation with disease activity and response to corticosteroid therapy. JAMA 272(8):211–216. doi:10.1001/jama.272.8.611

    Article  Google Scholar 

  21. Balink H, Bruyn JA (2007) The role of PET/CT in Cogan’s syndrome. Clin Rheumatol 26(12):2177–2179. doi:10.1007/s10067-007-0663-5

    Article  PubMed  CAS  Google Scholar 

  22. Mazlumzadeh M, Lowe VJ, Mullan BP, Fabry DA, McDonald TJ, Matteson EL (2003) The utility of positron emission tomography in the evaluation of autoimmune hearing loss. Otol Neurotol 24:201–204. doi:10.1097/00129492-200303000-00012

    Article  PubMed  Google Scholar 

  23. Cinamon U, Kronenberg J, Hildeshimer M, Taitelbaum R (1997) Cochlear implantation in patients suffering from Cogan’s syndrome. J Laryngol Otol 111(10):928–930. doi:10.1017/S002221510013899X

    Article  PubMed  CAS  Google Scholar 

  24. Dl Matteson, Fabry DA, Facer GW et al (2001) Open trial of methotrexate as treatment of autoimmune hearing loss. Arth Rheum 45(2):146–150. doi:10.1002/1529-0131(200104)45:2<146::AID-ANR167>3.0.CO;2-I

    Article  Google Scholar 

  25. Watanabe K, Nishimaki T, Yoshida M et al (2000) Atypical Cogan’s syndrome successfully treated with corticosteroids and pulse cyclophosphamide therapy. Fukushima J Med Sci 46(1–2):49–54

    PubMed  CAS  Google Scholar 

  26. Matteson EL, Choi HK, Poe DS et al (2005) Etanercept therapy for immune-mediated cochleo-vestibular disorders—a multi-centre open-label pilot study. Arthritis Rheum 53(3):337–342. doi:10.1002/art.21179

    Article  PubMed  CAS  Google Scholar 

  27. Fricker M, Baumann A, Wermelinger F, Villiger PM, Helbling A (2007) A novel therapeutic option in Cogan diseases? TNF-alpha blockers. Rheumatol Int 27(5):493–495. doi:10.1007/s00296-006-0252-y

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interest statement

The authors declare no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Grainne Murphy.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Murphy, G., Sullivan, M.O., Shanahan, F. et al. Cogan’s syndrome: present and future directions. Rheumatol Int 29, 1117–1121 (2009). https://doi.org/10.1007/s00296-009-0945-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-009-0945-0

Keywords

Navigation