Hostname: page-component-cd9895bd7-jkksz Total loading time: 0 Render date: 2024-12-27T10:33:43.453Z Has data issue: false hasContentIssue false
  • English
  • Français

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

Published online by Cambridge University Press:  20 May 2013

A C Hall ,
A C Leong ,
D Jiang  and
A Fitzgerald-O'Connor
A C Hall
Affiliation:
Auditory Implant Centre, Guy's and St Thomas' Hospital, London, UK
A C Leong*
Affiliation:
Auditory Implant Centre, Guy's and St Thomas' Hospital, London, UK
D Jiang
Affiliation:
Auditory Implant Centre, Guy's and St Thomas' Hospital, London, UK
A Fitzgerald-O'Connor
Affiliation:
Auditory Implant Centre, Guy's and St Thomas' Hospital, London, UK
*
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
Get access Rights & Permissions [Opens in a new window]

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.

Keywords

Sensorineural Hearing LossUrticariaVasculitisMuckle–Wells SyndromeCochlear ImplantationInterleukin
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 127 , Issue 7 , July 2013 , pp. 708 - 711
Copyright
Copyright © JLO (1984) Limited 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

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

References

1Muckle, TJ, Wells, SM.Urticaria, deafness and amyloidosis: a new heredo-familial syndrome. QJM 1962;31:235–48Google ScholarPubMed
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;62:3783–91CrossRefGoogle ScholarPubMed
3Biswas, D, Stafford, N. Otolaryngological manifestations of “Muckle-Wells syndrome”. Int J Pediatr Otorhinolaryngol 2010;74:553–5CrossRefGoogle ScholarPubMed
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;65:1054–9CrossRefGoogle ScholarPubMed
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;47:415–17CrossRefGoogle ScholarPubMed
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 607–12CrossRefGoogle ScholarPubMed
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;56:1273–85CrossRefGoogle Scholar
8Hawkins, PN, Lachmann, HJ, McDermott, MF.Interleukin-1-receptor antagonist in the Muckle-Wells syndrome. N Engl J Med 2003;348:2583–4CrossRefGoogle ScholarPubMed
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;65:533–4CrossRefGoogle Scholar