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). Materials and 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. Conclusion: 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.

1.
Bacciu A, Bacciu S, Mercante G, Ingegnoli F, Grasselli C, Vaglio A, Pasanisi E, Vincenti V, Garini G, Ronda N, Ferri T, Corradi D, Buzio C: Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 2006;126:503-509.
2.
Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V, Mercante G, Grasselli C, Bacciu S: Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 2008;118:325-329.
3.
Churg J, Strauss L: Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.
4.
Dhong HJ, Kim HY, Chung YJ, Kim TW, Kim JH, Chung SK, Kim MG: Computed tomographic assessment of chronic rhinosinusitis with asthma. Am J Rhinol 2006;20:450-452.
5.
Franssen C, Gans R, Kallenberg C, Hageluken C, Hoorntje S: Disease spectrum of patients with antineutrophil cytoplasmic autoantibodies of defined specificity: distinct differences between patients with anti-proteinase 3 and anti-myeloperoxidase autoantibodies. J Intern Med 1998;244:209-216.
6.
Ishiyama A, Canalis RF: Otological manifestations of Churg-Strauss syndrome. Laryngoscope 2001;111:1619-1624.
7.
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, et al: Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-192.
8.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-11.
9.
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al: The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-1107.
10.
Lund VJ, Mackay IS: Staging in rhinosinusitus. Rhinology 1993;31:183-184.
11.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, et al: The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-1100.
12.
Mazzoli M, Camp GV, Newton V, Giarbini N, Declau F, Parving A: Recommendations for the description of genetic and audiological data for families with nonsyndromic hereditary hearing impairment. Audiol Med 2003;1:148-150.
13.
Mehta V, Campeau NG, Kita H, Hagan JB: Blood and sputum eosinophil levels in asthma and their relationship to sinus computed tomographic findings. Mayo Clin Proc 2008;83:671-678.
14.
Meltzer EO, Hamilos DL, Hadley JA, Lanza DC, Marple BF, Nicklas RA, Bachert C, Baraniuk J, Baroody FM, Benninger MS, Brook I, Chowdhury BA, Druce HM, Durham S, Ferguson B, Gwaltney JM Jr, Kaliner M, Kennedy DW, Lund V, Naclerio R, Pawankar R, Piccirillo JF, Rohane P, Simon R, Slavin RG, Togias A, Wald ER, Zinreich SJ; American Academy of Allergy, Asthma and Immunology, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Allergy, Asthma and Immunology, American Rhinologic Society: Rhinosinusitis: establishing definitions for clinical research and patient care. Otolaryngol Head Neck Surg 2004;131:S1-S62.
15.
Noth I, Strek ME, Leff AR: Churg-Strauss syndrome. Lancet 2003;361:587-594.
16.
Rosenfeld RM, Andes D, Bhattacharyya N, Cheung D, Eisenberg S, Ganiats TG, Gelzer A, Hamilos D, Haydon RC 3rd, Hudgins PA, Jones S, Krouse HJ, Lee LH, Mahoney MC, Marple BF, Mitchell CJ, Nathan R, Shiffman RN, Smith TL, Witsell DL: Clinical practice guideline: adult sinusitis. Otolaryngol Head Neck Surg 2007;137:S1-S31.
17.
Srouji I, Lund V, Andrews P, Edwards C: Rhinologic symptoms and quality-of-life in patients with Churg-Strauss syndrome vasculitis. Am J Rhinol 2008;22:406-409.
18.
Stachler RJ, Chandrasekhar SS, Archer SM, Rosenfeld RM, Schwartz SR, Barrs DM, Brown SR, Fife TD, Ford P, Ganiats TG, Hollingsworth DB, Lewandowski CA, Montano JJ, Saunders JE, Tucci DL, Valente M, Warren BE, Yaremchuk KL, Robertson PJ, American Academy of Otolaryngology-Head and Neck Surgery: Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg 2012;146:S1-S35.
19.
Vaglio A, Buzio C, Zwerina J: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy 2013;68:261-273.
20.
Vaglio A, Casazza I, Grasselli C, Corradi D, Sinico RA, Buzio C: Churg-Strauss syndrome. Kidney Int 2009;76:1006-1011.
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