Cogan syndrome in children: early diagnosis and treatment is critical to prognosis

Am J Ophthalmol. 2004 Apr;137(4):757-8. Cogan syndrome in children: early diagnosis and treatment is critical to prognosis. Orsoni JG, Zavota L, Vincenti V, Pellistri I, Rama P. SourceOphthalmology Department, University of Parma, Parma, Italy. jorsoni@unipr.it Abstract PURPOSE: To present two cases of pediatric Cogan Syndrome and to highlight the differences between the adult and pediatric forms of the disease, as well as the importance of early diagnosis and treatment. DESIGN: Interventional case report. METHODS: Institutional setting. RESULTS: Corneal lesions were much more diffuse than those observed in adult Cogan syndrome. Immunosuppressive drug combination therapy successfully resolved systemic and ocular inflammation, but the involvement of the pupillary area caused permanent low vision in one case and amblyopia in the other. CONCLUSION: When chronic ocular inflammation is observed in association with sensory neural hearing loss and any systemic signs of autoimmune inflammation, a diagnosis of Cogan syndrome should be suspected. If immunosuppressive treatment is not initiated as soon as possible, permanent low vision and deafness can result.