[Atypical Cogan's syndrome: report of two cases and revision of literature].
Cogan's syndrome is a rare autoimmune disease with systemic involvement. It appears in young people and has two presentations: the typical form with keratitis, sudden deafness with or without vestibular syndrome, and the atypical form with different non keratitic ocular diseases and a great variety of systemic symptoms in relation with the autoimmune etiology of the process. Cogan's syndrome has a bad prognosis and deafness appears in 25% of the cases with the right treatment and in 60% of patients without treatment. The best treatment is systemic and ocular corticotherapy. The second treatment of choice is cyclophosphamide or cyclosporine A. We present two cases of atypical Cogan's syndrome with unilateral deafness in both.
Sanz
J J
JJ
Servicio de Otorrinolaringología, Hospital Clínic, Barcelona.
Martínez
P
P
Escobar
J J
JJ
Menéndez
L M
LM
D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D016572:Cyclosporine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D003639:Hearing Loss, Sudden / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007634:Keratitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D015837:Vestibular Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy
D007166:Immunosuppressive Agents; D016572:Cyclosporine