Complete restoration of auditory impairment in a pediatric case of Cogan's syndrome: Report of a rare case with long-term follow-up and literature review

Atypical Cogan's syndrome: A case report and summary of current treatment options

“…Nevertheless, their relevance remains to be confirmed and they seem to be of no significance in the diagnosis of the atypical form of CS. Thus, the diagnosis of CS is still clinical, based on the combination of audiovestibular and ocular symptomatology [2,3,7]. Audiovestibular symptoms usually start with an isolated vestibular syndrome.…”

“…But unlike Menier's disease, audiovestibular symptoms are usually bilateral, and in most cases the vestibular syndrome regresses when the auditory deficit appears. Hearing loss in CS is usually sensorineural, bilateral and progressive [2,3,6]. If untreated or not treated in a timely fashion, the result in most patients is complete and irreversible deafness in a matter of weeks or years [2].…”

“…CS is a rare autoimmune vasculitis characterized by non-syphylitic interstitial keratitis and bilateral cochlear and vestibular dysfunction [1][2][3][4]. In 1980, Haynes et al defined the atypical form of Cogan's syndrome as including audiovestibular symptoms and accompanying intraocular lesions, such as episcleritis, scleritis, iritis, conjunctivities, vitritis, retinitis, choroiditis and optical neuritis [3].…”

“…CS is a rare condition and its incidence remains unknown. It is extremely rare in children, with only a few cases of the atypical form described in the literature [2]. Without treatment, profound hearing loss develops in a matter of weeks or months in most patients [1,3].…”

“…Without treatment, profound hearing loss develops in a matter of weeks or months in most patients [1,3]. In children, slightly better outcomes have been observed [2].…”