61 research outputs found
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. [email protected]
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
Cogan syndrome
To lead ophthalmologists to consider Cogan syndrome
when managing a patient presenting with keratitis or other ocular
inflammation accompanied by sensorineural hearing loss. Methods.
Seven patients affected by Cogan syndrome were studied: two
males and five females, ranging from 27 to 65 years of age (mean
age: 41 years). Subjects were evaluated for a period ranging from
22 to 46 months (mean follow up time: 29.2 months). All patients
were treated with immunosuppressive drug combination therapy
(IDCT). Results. Three patients were affected by classic Cogan
syndrome (i.e., vestibuloauditory symptoms and later sensorineural
hearing loss and interstitial keratitis). Four patients presented
atypical Cogan syndrome (i.e., sensorineural hearing loss and
chronic ocular inflammation such as uveitis, scleritis, conjunctivitis,
retinal vasculitis, etc.). Four of these patients had a late diagnosis.
Two of them were diagnosed when they already had a
cochlear implant, one with bilateral deafness underwent cochlear
implantation 1 year after the beginning of IDCT, one had severe
bilateral hearing loss that improved during the first year of IDCT,
and then rapidly worsened to total deafness in 1 month following
an episode of severe systemic hypotension. Three patients who had
an early diagnosis of Cogan syndrome had no worsening of vestibuloauditory
dysfunction during the follow up period. Conclusion.
Diagnosis of Cogan syndrome should not be overlooked by
ophthalmologists in all patients with recurrent ocular inflammatory
disease associated with vestibuloauditory symptoms. Early diagnosis
is essential to commence the appropriate immunosuppressive
therapy that may prevent permanent hearing loss and ocular
dysfunctionPurpose. To lead ophthalmologists to consider Cogan syndrome when managing a patient presenting with keratitis or other ocular inflammation accompanied by sensorineural hearing loss. Methods. Seven patients affected by Cogan syndrome were studied: two males and live females, ranging from 27 to 65 years of age (mean age: 41 years). Subjects were evaluated for a period ranging from 22 to 46 months (mean follow up time: 29.2 months). All patients were treated with immunosuppressive drug combination therapy (IDCT). Results. Three patients Were affected by classic Cogan syndrome (i.e., vestibuloauditory symptoms and later sensorineural hearing loss and interstitial keratitis). Four patients presented atypical Cogan syndrome (i.e., sensorineural hearing loss and chronic ocular inflammation such as uveitis, scleritis, conjunctivitis, retinal vasculitis, etc.). Four of these patients had a late diagnosis. Two of them were diagnosed when they already had a cochlear implant, one with bilateral deafness underwent cochlear implantation I year after the beginning of IDCT, one had severe bilateral hearing loss that improved during the first year of IDCT, and then rapidly worsened to total deafness in 1 month following an episode of severe systemic hypotension. Three patients who had an early diagnosis of Cogan syndrome had no worsening of vestibuloauditory dysfunction during the follow up period. Conclusion. Diagnosis of Cogan syndrome should riot be overlooked by ophthalmologists in all patients with recurrent ocular inflammatory disease associated with vestibuloauditory symptoms. Early diagnosis is essential to commence the appropriate immunosuppressive therapy that may prevent permanent hearing loss and ocular dysfunction
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