Neuro-Ophthalmology & Visual Neuroscience
ISSN (Print): 2572-7257 ISSN (Online): 2572-7281 Website: http://www.sciepub.com/journal/novn Editor-in-chief: Carlo Aleci
Open Access
Journal Browser
Go
Neuro-Ophthalmology & Visual Neuroscience. 2015, 1(1), 8-12
DOI: 10.12691/novn-1-1-2
Open AccessArticle

Typical and Atypical Cogan’s Syndrome: 7 Cases and Review of the Literature

Sara Belghmidi1, Btissaam. Belhoucha2, , Ibtissam Hajji1, Youssef. Rochdi2, Ennasiri wiam1, Hassan. Nouri2, Baha ali1, lahcen. Aderdour2, Abdelaziz. Raji2 and A Moutaouakil1

1Department of ohtalmology, CHU Mohammed VI, Marrakech, Maroc

2Department of ENT, CHU Med VI, Marrakech 2360, Morocco

Pub. Date: September 16, 2014

Cite this paper:
Sara Belghmidi, Btissaam. Belhoucha, Ibtissam Hajji, Youssef. Rochdi, Ennasiri wiam, Hassan. Nouri, Baha ali, lahcen. Aderdour, Abdelaziz. Raji and A Moutaouakil. Typical and Atypical Cogan’s Syndrome: 7 Cases and Review of the Literature. Neuro-Ophthalmology & Visual Neuroscience. 2015; 1(1):8-12. doi: 10.12691/novn-1-1-2

Abstract

Cogan's syndrome (CS) is a rare presumed autoimmune disorder characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms similar to those of Meniere's syndrome. Material and methods: We reviewed the records of a prospective study carried in CHU Mohammed VI of Marrakech, from September 2012 to June 2014, including 7 patients with a Cogan’s syndrome. Clinical data regarding age, sex, ethnic origin, presenting manifestations, ocular, audiovestibular and systemic manifestations, treatment and outcome were collected. Results: 7 patients were identified, 3 with typical Cogan’s syndrome and the remaining 4 with atypical Cogan’s syndrome. The age ranged from 12 to 65 years, with median age of 31 years, The presenting manifestations were ocular in four patients and audio vestibular in three. Examination revealed a bilateral interstitial keratitis (stromal scarring with ghost vessels) in six patients. Which was isolated in three cases and associated in 1 case with conjunctivitis, 1 with subconjunctival hemorrhage and one with corneal neovascularization.Audiovestibular manifestations in the two groups were typical, with vestibular symptoms, followed by progressive hearing loss of variable severity. Five of our patients presented systemic manifestations. All patients initially received local and systemic corticosteroids (CTC) given the occuular symptom but Clinical follow-up showed recurrence of audiovestibular symptoms after dose reduction of prednisone in 4 patients. In these cases, immunosuppressive therapy was able to improve hearing in 3 cases and visual function in 2 cases. Overall, 5 patients maintained good visual; 2 patients did not respond to medical treatment and thus were candidates for corneal transplant, 1 patient presented a total right deafness 3 years after the onset. The treatment strategies are not clearly defined; therefore, early assessment and treatment for systemic inflammation are needed to prevent life threatening complications.

Keywords:
cogan’s syndrome interstitial keratitis deafness

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References:

[1]  Greco A, et al, Cogan's syndrome: An autoimmune inner ear disease, Autoimmun Rev (2012).
 
[2]  Rarey et al.1ntralabyrinthine osteogenesis in cogan's syndrome. Am J Otolaryngol 4'.387-390, 1986.
 
[3]  Cody DTR, Williams HL. Cogan's syndrome. Laryngoscope 1960; 70: 447-75.
 
[4]  Vollertsen RS, McDonald TJ, Younge BR, Banks PM, Stanson AW, Ilstrup DM. Cogan's syndrome: 18 cases and a review of the literature. Mayo Clin Proc 1986; 61: 344-61.
 
[5]  Ho AC, Roat MI, Venbrux A, Hellmann DB. Cogan's syndrome with refractory aortitis and mesenteric vasculitis. J Rheumatol 1999; 26: 1404-7.
 
[6]  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 1980; 59: 426-41.
 
[7]  Gluth MB, Baratz KH, Matteson EL, Driscoll CL. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 2006; 81: 483-8.
 
[8]  Casselman JW, Majoor MH, Albers FW. MR of the inner ear in patients with Cogan syndrome. AJNR Am J Neuroradiol 1994; 15: 131-8.
 
[9]  Majoor MHJM, Albers FWJ, Van Der Gaag R, Gmelig-Meyling F, Huizing EH. Corneal autoimmunity in Cogan's syndrome? Report of two cases. Ann Otol Rhinol Laryngol 1992; 101: 679-84.
 
[10]  Froehlich F, Fried M, Gonvers JJ, Saraga E, Thorens J, Pecoud A. Association of Crohn's disease and Cogan's syndrome. Dig Dis Sci 1994; 39: 1134-7.
 
[11]  Schuknecht HF. Ear pathology in autoimmune diseases. Adv Otorhinolaryngol 1991; 46: 50-70.
 
[12]  Van Doornum S, McColl G, Walter M, Jennens I, Bhathal P, Wicks IP. Prolonged prodrome, systemic vasculitis, and deafness in Cogan's syndrome. Ann Rheum Dis 2001; 60: 69-71
 
[13]  Fischer ER, Hellstrom HR: Cogan's syndrome and systemicsystemic vascular disease: analysis of pathologic features with reference to its relationship to thromboangiitis obliterans (Buerger). Arch Pathal 72: 572-592, 1961 6.