a1 Auditory Implant Centre, Guy's and St Thomas' Hospital, London, UK
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.
(Accepted September 07 2012)
(Online publication May 20 2013)
Presented orally at the 141st Semon Club meeting, 2 June 2011, London, UK.
Miss A C Leong takes responsibility for the integrity of the content of the paper
Competing interests: None declared