title: Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Auditory Implant Centre, Guy's and St Thomas' Hospital, London, UK.
authors.forename: A C
authors.initials: AC
authors.lastname: Hall
authors.forename: A C
authors.initials: AC
authors.lastname: Leong
authors.forename: D
authors.initials: D
authors.lastname: Jiang
authors.forename: A
authors.initials: A
authors.lastname: Fitzgerald-O'Connor
mesh_terms: D001301:Audiometry, Pure-Tone; D019929:Cochlear Implantation; D056587:Cryopyrin-Associated Periodic Syndromes / Q000503:physiopathology*; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D003639:Hearing Loss, Sudden / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D014581:Urticaria / Q000209:etiology