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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
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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 

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Footnotes

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

References

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