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Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

  • A C Hall (a1), A C Leong (a1), D Jiang (a1) and A Fitzgerald-O'Connor (a1)

Abstract

Background:

Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.

Method:

To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.

Results:

The patient underwent a cochlear implantation with a modest outcome.

Conclusion:

Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

Copyright

Corresponding author

Address for correspondence: Miss A C Leong, Department of ENT, Guy's Hospital, London SE1 9RT, UK Fax: 0207 188 2206 E-mail: ackleong@gmail.com

Footnotes

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Presented orally at the 141st Semon Club meeting, 2 June 2011, London, UK.

Footnotes

References

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1Muckle, TJ, Wells, SM.Urticaria, deafness and amyloidosis: a new heredo-familial syndrome. QJM 1962;31:235–48
2Kummerle-Deschner, J, Tyrrell, P, Reess, F, Kotter, I, Lohse, P, Girschick, H et al. Risk factors for severe Muckle-Wells syndrome. Arthritis Rheum 2010;62:3783–91
3Biswas, D, Stafford, N. Otolaryngological manifestations of “Muckle-Wells syndrome”. Int J Pediatr Otorhinolaryngol 2010;74:553–5
4Cuisset, L, Drenth, JP, Berthelot, JM, Meyrier, A, Vaudour, G, Watts, RA et al. Genetic linkage of the Muckle-Wells syndrome to chromosome 1q44. Am J Hum Genet 1999;65:1054–9
5Kastbom, A, Verma, D, Eriksson, P, Skogh, T, Wingren, G, Soderkvist, P.Genetic variation in proteins of the cryopyrin inflammasome influences susceptibility and severity of rheumatoid arthritis (the Swedish TIRA project). Rheumatology (Oxford) 2008;47:415–17
6Hawkins, PN, Lachmann, HJ, Aganna, E, McDermott, MF.Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra. Arthritis Rheum 2004;50:trun 607–12
7Aksentijevich, ID, Putnam, C, Remmers, EF, Mueller, JL, Le, J, Kolodner, RJ et al. The clinical continuum of cryopyrinopathies: novel CIAS1 mutations in North American patients and a new cryopyrin model. Arthritis Rheum 2007;56:1273–85
8Hawkins, PN, Lachmann, HJ, McDermott, MF.Interleukin-1-receptor antagonist in the Muckle-Wells syndrome. N Engl J Med 2003;348:2583–4
9Rynne, M, Maclean, C, Bybee, A, McDermott, MF, Emery, P.Hearing improvement in a patient with variant Muckle-Wells syndrome in response to interleukin 1 receptor antagonism. Ann Rheum Dis 2006;65:533–4

Keywords

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

  • A C Hall (a1), A C Leong (a1), D Jiang (a1) and A Fitzgerald-O'Connor (a1)

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