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4 results

  • 46 - RHEUMATOID ARTHRITIS AND CEREBROVASCULAR DISEASE

  • from PART V: - SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
  • Edited by Louis R. Caplan, Julien Bogousslavsky
  • Book:
    Uncommon Causes of Stroke
    Published online:
    06 January 2010
    Print publication:
    09 October 2008, pp 343-346

  • Summary

    Cogan's syndrome is characterized by nonsyphilitic interstitial keratitis, vestibulo-auditory Menière-like symptoms, and, occasionally, systemic manifestations of vasculitis. Although neurologic manifestations are rare, several patients with stroke in the setting of Cogan's syndrome have been reported. The most common and classic ocular manifestation of Cogan's syndrome is bilateral interstitial keratitis. The diagnosis is classically suggested by the association of interstitial keratitis with acute-onset sensorineuronal hearing loss in a patient who has a negative laboratory evaluation for syphilis. Computed tomography (CT) scans may occasionally show intralabyrinthine calcifications, whereas magnetic resonance imaging (MRIs) often show soft tissue obliteration of the membranous labyrinth and may also show multiple lesions of the white matter consistent with cerebral vasculitis. The treatment of Cogan's syndrome varies based on the severity of the clinical manifestations. Due to the presumed autoimmune mechanism with vasculitis, most treatments have included steroids and immunosuppressants.

  • Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell anaemia

  • A D Mace, M S Ferguson, M Offer, K Ghufoor, M J Wareing
  • Journal:
    The Journal of Laryngology & Otology / Volume 123 / Issue 7 / July 2009
    Published online by Cambridge University Press:
    17 September 2008, pp. 811-816
    Print publication:
    July 2009
  • Objective:

    To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.

    Method:

    Case report and review of world literature.

    Case report:

    The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.

    Conclusion:

    Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.

  • Side selection for cochlear implantation in a case of Cogan's syndrome

  • G J Im, H H Jung
  • Journal:
    The Journal of Laryngology & Otology / Volume 122 / Issue 3 / March 2008
    Published online by Cambridge University Press:
    25 May 2007, pp. 310-313
    Print publication:
    March 2008

  • Cogan's syndrome is a rare clinical entity that is characterised by non-syphilitic interstitial keratitis and audiovestibular symptoms. The cause of Cogan's syndrome is considered to be autoimmune disease, which is supported by the resolution of hearing loss after steroid treatment, and the association with other autoimmune diseases. The sensorineural hearing loss of Cogan's syndrome is progressive over a few months, and sudden, bilateral deafness often occurs, which may be an indication for cochlear implantation. This paper presents the case of a young woman suffering from Cogan's syndrome and sudden, bilateral deafness. With reference to this case, we describe problems regarding cochlear implantation for Cogan's syndrome and radiological findings aiding selection of cochlear implantation side.

  • Cochlear implantation in patients suffering from Cogan's syndrome

  • Udi Cinamon, Jona Kronenberg, Minka Hildesheimer, Riki Taitelbaum
  • Journal:
    The Journal of Laryngology & Otology / Volume 111 / Issue 10 / October 1997
    Published online by Cambridge University Press:
    29 June 2007, pp. 928-930
    Print publication:
    October 1997

  • Among patients who receive cochlear implants, those with Cogan's syndrome make a unique group. On one hand they are part of the post-lingual patients and good results can be anticipated. On the other hand, their basic illness is thought to have an autoimmune aetiology and for that reason more susceptible to complications, especially flap problems. In a series of 60 patients who were implantedat the Sheba Medical Center, three had Cogan's syndrome. No post-operative complications, including flap problems, were observed (followed-up for at least 18 months). Subjective and objective hearing results were very good.