title: Otologic and Rhinologic Manifestations of Eosinophilic Granulomatosis with Polyangiitis.
abstract: BACKGROUND
  Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic autoimmune disease that manifests as asthma, recurrent sinusitis and peripheral eosinophilia. In this study, we investigated the clinical features of the ear and nasal manifestations of EGPA in comparison with those of granulomatosis with polyangiitis (GPA).
  
  
  METHODS
  Twenty-one patients diagnosed with EGPA were studied. The frequency of otologic manifestations, the degree of hearing loss and the frequency of nasal symptoms were assessed. The onset of ear symptoms, sinusitis and asthma in patients with EGPA were also examined.
  
  
  RESULTS
  Eleven patients (52.4%) with EGPA demonstrated otologic symptoms. The EGPA patients commonly presented mild-to-moderate mixed or sensorineural hearing loss. The pattern of hearing loss was mainly flat, and all but 1 patient achieved complete remission from their hearing impairments. Eighteen patients (85.7%) with EGPA demonstrated nasal symptoms. Patients with EGPA showed a significantly higher incidence of nasal polyps than did those with GPA. The median Lund and Mackey scoring system score was 13.7 for patients with EGPA, and ethmoid sinus shadows were more severe than those of the maxillary sinus. Most ear symptoms associated with EGPA were observed after definitive diagnosis, although sinusitis and asthma tended to manifest themselves before diagnosis. There were significant differences between the onset of ear symptoms and those of asthma and sinusitis.
  
  
  CONCLUSIONS
  As over 80% of patients with EGPA had nasal symptoms and over half had ear symptoms, otolaryngologists should be aware of this disease. Recognition of the characteristic ear and nasal symptoms are thought to be particularly important to obtain an early diagnosis of EGPA.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Nakamaru
authors.forename: Dai
authors.initials: D
authors.lastname: Takagi
authors.forename: Masanobu
authors.initials: M
authors.lastname: Suzuki
authors.forename: Aya
authors.initials: A
authors.lastname: Homma
authors.forename: Shinya
authors.initials: S
authors.lastname: Morita
authors.forename: Akihiro
authors.initials: A
authors.lastname: Homma
authors.forename: Satoshi
authors.initials: S
authors.lastname: Fukuda
mesh_terms: D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D016022:Case-Control Studies; D015267:Churg-Strauss Syndrome / Q000188:drug therapy / Q000453:epidemiology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000453:epidemiology; D046089:Hearing Loss, Mixed Conductive-Sensorineural / Q000453:epidemiology*; D006319:Hearing Loss, Sensorineural / Q000453:epidemiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009298:Nasal Polyps / Q000453:epidemiology*; D012852:Sinusitis / Q000000981:diagnostic imaging / Q000453:epidemiology*; D014057:Tomography, X-Ray Computed
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide