Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

Abstract

ABSTRACT. Cogan's syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan's syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the longterm, the disease was controlled in 2 cases but continued to progress in the other. Cogan's syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits. Pediatrics 2002;109(2). URL: http://www. pediatrics.org/cgi/content/full/109/2/e38; children, Cogan's syndrome, sudden hearing loss, imbalance, vertigo, conjunctivitis.ABBREVIATIONS. EVAR, earth vertical axis rotation; OVAR, off vertical axis rotation test; VOR, vestibuloocular responses; SNHL, sensorineural hearing loss; NSAID, nonsteroidal antiinflammatory drugs; MRI, magnetic resonance imaging; EBV, Epstein-Barr virus. C ogan's syndrome is a relatively rare inflammatory disease characterized by nonsyphilitic ocular interstitial keratitis associated with sudden hearing loss and vestibular impairment. However, its early diagnosis and treatment with steroids can prevent loss of inner-ear sensorial function and limit ophthalmologic aftereffects. In 1934, Morgan and Baumgartner 1 published the first case of the syndrome. In 1945, Cogan 2 published the full description of the syndrome as an inner-ear disorder in its typical form. Several authors later reported atypical forms 3,4 that differed from the typical presentation by the type of ocular lesions, which can include superficial keratitis, scleritis, episcleritis, iritis, or choroiditis, as well as variable hearing impairment and often systemic inflammation. 5 Cogan's syndrome is generally considered an autoimmune disease despite the frequent lack of direct immunologic...

Mycophenolate mofetil as a treatment of steroid dependent Cogan's syndrome in childhood

“…When hearing loss occurs, early steroid systemic treatment, (typically 1-2 mg/kg per day) reduces the risk of development of profound hearing loss [6,13] and leads to an improvement in 50% of cases as early as the first week [3]-which was the case for our patient. One major problem for patients with Cogan's syndrome is that despite a rapid and sometimes spectacular initial response to treatment, relapses are frequent when steroids are stopped or during the decrease of dosing.…”

“…In addition to the inflammatory lesions of the eye and ear, systemic symptoms are present in 50-70% of cases [5]. Early steroid treatment improves long term prognosis [6]. Standard management is oral prednisone at 1-2 mg/kg per day.…”

“…However, there is considerable risk for steroid associated complications in particular if high doses are required to maintain remission. Some authors have proposed alternative, steroid sparing immunosuppressive agents [6][7][8][9][10]. We report a pediatric case of Cogan's syndrome requiring long term high dose of oral steroids, which was successfully managed with mycophenolate mofetil (MMF).…”

Audiological Findings in Autoimmune Hepatitis: Hearing Loss at High Frequencies

“…It may be due to vasculitis, ossicular involvement, neuritis or ototoxicity of the drugs used for the treatment of rheumatologic diseases (18). Thus, hearing loss may be seen in conductive, sensorineural or in mixed pattern in secondary AIED (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(19)(20). Özcan et al suggested middle ear involvement for the reason of hearing loss in RA (18).…”

“…Bununla birlikte, otoakustik emisyon (OAE) gibi diğer testleri de içeren ve daha büyük örneklem büyüklüğüne sahip başka çalışmalara da ihtiyaç vardır. (5)(6)(7)(8)(9)(10)(11)(12)(13)(14). The inner ear pathologies caused by these disorders are commonly mild and slowly progressive whereas primary AIED is characterized by sudden onset, commonly bilateral, fluctuant and progressive sensorineural hearing loss (SNHL) with accompanying vertigo in some occasions.…”