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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)



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.


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.


The patient underwent a cochlear implantation with a modest outcome.


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.


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:


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



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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)


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