The Journal of Laryngology & Otology

Clinical Records

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

A C Halla1, A C Leonga1 c1, D Jianga1 and A Fitzgerald-O'Connora1

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)

Key words

  • Sensorineural Hearing Loss;
  • Urticaria;
  • Vasculitis;
  • Muckle–Wells Syndrome;
  • Cochlear Implantation;
  • Interleukin


c1 Address for correspondence: Miss A C Leong, Department of ENT, Guy's Hospital, London SE1 9RT, UK Fax: 0207 188 2206 E-mail:


  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