Original ArticleClinical Features and Outcome of Cogan Syndrome
Section snippets
Methods
Since 1995, we have followed 3 patients with Cogan syndrome who had at least 1 year of follow-up. Two of these patients have been seen at the Pediatric Rheumatology Unit of Padua, and the other has been treated at the Pediatric Rheumatology Unit of the Meyer Children’s Hospital in Florence. To investigate the clinical course and prognosis of the Cogan syndrome presenting in childhood, we conducted a MEDLINE search of all articles published in the English language during the last 30 years. The
Results
This study included 23 children with Cogan syndrome, 20 from previously published reports and 3 from our own series (Table; available at www.jpeds.com). The cohort comprised 15 males and 8 females, with a median age at disease onset of 11 years, 4 months, (range, 4-18 years), a median age at diagnosis of 12 years (range, 4-21 years), and a median follow-up period after diagnosis of 2 years (range, 1-16 years).
Eleven patients (47.8%) demonstrated systemic features at disease onset (Figure),
Discussion
Since its first description published in 1945,28 few cases of Cogan syndrome with pediatric onset have been reported.4, 8, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 29, 30, 31 Cogan syndrome is a very rare disorder, particularly in children. Its diagnosis is essentially clinical and based mainly on ocular and ENT symptoms often associated with systemic manifestations, including fever, musculoskeletal complaints, headache, and weight loss. These systemic symptoms were related to a good
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2016, Taylor and Hoyt's Pediatric Ophthalmology and Strabismus, Fifth EditionOtologic disorders causing dizziness, including surgery for vestibular disorders
2016, Handbook of Clinical NeurologyCitation Excerpt :We will not discuss diseases for which vestibular symptoms have been rarely described, may be purely coincidental, or are predominantly due to central nervous system involvement. Cogan syndrome predominantly affects young adults between the ages of 20 and 40 years, but can occur in a pediatric population (Cogan, 1945; Pagnini et al., 2012). Typical Cogan syndrome is defined by nonsyphilitic interstitial keratitis associated with audiovestibular symptoms (Cogan, 1945).
The authors declare no conflicts of interest.
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Contributed equally to this work.