title: Reversible cochlear function with ANCA-associated vasculitis initially diagnosed by otologic symptoms.
abstract: OBJECTIVE
  To present 8 cases with hearing loss as an initial symptom of antineural cytoplasmic antibody (ANCA)-associated vasculitis (AAV) involving granulomatosis with polyangiitis (GPA) and to discuss the treatment and mechanisms of hearing outcomes after immunosuppressive therapy.
  
  
  METHODS
  Retrospective case review.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  Eight patients were referred to our university hospital between 2004 and 2012 for intractable otitis media with acute progressive mixed (conductive and sensorineural) hearing loss and facial palsy.
  
  
  METHODS
  Diagnostics and treatment.
  
  
  METHODS
  Otologic symptoms as initial manifestations of otitis media with AAV and cochlear function after treatment.
  
  
  RESULTS
  Eight cases (6 female and 2 male subjects; aged 54-73 yr; 6 MPO [myeloperoxidase]-ANCA-positive and 2 PR3 [proteinase 3]-ANCA-positive cases) were included. Progressive hearing loss was present in all patients, and facial palsy was present in 5 of 8 patients total. Patients with hearing levels better than 95 dB improved with good speech discrimination after immunosuppressive therapy, but the completely deaf could not be recovered. All patients have been successfully controlled for 1 to 8 years without any systemic disorders.
  
  
  CONCLUSIONS
  This study showed the difficulty of diagnosing localized AAV and the effectiveness of immunosuppressive therapy for hearing loss in the early stage. Based on these results, early-stage AAV would influence the stria vascularis in the cochlea. Otitis media with ANCA-associated vasculitis is a new entity among the causes of intractable otitis media and progressive hearing loss.
authors: Department of Otolaryngology, Jichi Medical University Saitama Medical Center, Saitama, Japan.
authors: Naohiro
authors: N
authors: Yoshida
authors: Mariko
authors: M
authors: Hara
authors: Masayo
authors: M
authors: Hasegawa
authors: Shingo
authors: S
authors: Matsuzawa
authors: Akihiro
authors: A
authors: Shinnabe
authors: Hiromi
authors: H
authors: Kanazawa
authors: Yukiko
authors: Y
authors: Iino
mesh_terms: D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D003051:Cochlea / Q000503:physiopathology*; D005158:Facial Paralysis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D005260:Female; D034381:Hearing Loss / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents