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Year : 2014  |  Volume : 20  |  Issue : 4  |  Page : 222

Wegener's granulomatosis: A diagnostic dilemma for otologists

Department of Otorhinolaryngology, Head and Neck Suregry, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication13-Dec-2014

Correspondence Address:
Kapil Sikka
Department of ENT and Head and neck surgery, Room Number 4057, 4th Floor Teaching Block, All India Institute of Medical Sciences, New Delhi 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-7749.146945

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How to cite this article:
Gupta MP, Sikka K, Thakar A. Wegener's granulomatosis: A diagnostic dilemma for otologists. Indian J Otol 2014;20:222

How to cite this URL:
Gupta MP, Sikka K, Thakar A. Wegener's granulomatosis: A diagnostic dilemma for otologists. Indian J Otol [serial online] 2014 [cited 2021 May 13];20:222. Available from: https://www.indianjotol.org/text.asp?2014/20/4/222/146945


A 26-year-old female, software engineer by profession, presented with 1-month history of right earache and persistent headache with fever. The otological examination was suggestive of right middle ear effusion,and audiological examination showed right sided severe mixed hearing loss. Patient had also developed progressive involvement of left ear with impairment of hearing. Nasal endoscopy was normal. Routine blood investigations and serum biochemical evaluation were normal. Patient underwent regular charting of erythrocyte sedimentation rate (ESR), hemogram, and liver and renal function test. All blood investigations were normal except ESR, which was persistently high. Comprehensive autoimmunological workup was performed including antineutrophilic cytoplasmic antibodies (ANCA), RH factor, antinuclear antibody, anti-ds-DNA, C3, immunoglobulin -G, A, M, cryoglobulin, which was normal. The myringotomy and ventilation tube insertion was tried but had failed to halt the disease. Right mastoid exploration was hence performed considering the diagnosis of intractable otitis media. Granulation tissue was found in middle ear cleft, which on histopathology revealed inflammatory granulation tissue and was not helpful in attaining an accurate diagnosis. In view of high suspicion of an autoimmune disorder, further workup was planned. The final diagnosis of this case was clinched by high-resolution computed tomography (CT) chest finding [Figure 1], suggestive of nodule in the right lung and confirmed by CT guided fine-needle aspiration cytology to be a case of Wegeners granulomatosis (WG). The patient was initiated on cyclophosohamide therapy along with steroids. Patient at 2½ year of follow-up is asymptomatic with very significant improvement of hearing loss allowing her to lead a normal professional and social life.
Figure 1: Multiple round soft tissue density pulmonary nodules of varying sizes without any evidence of cavitation in superior segments of bilateral lower lobes

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Pagnoux et al. [1] on assessment of 16 patients found that WG strictly and persistently localized to one organ is rare. On review of 112 patients with WG, McCaffrey et al. [2] found only 21 cases (19%) with ear involvement and out of them only nine patients (8%) had sensorineural hearing loss, and most of them had more than one organ involvement and serological ANCA positive. Verfaillie et al. [3] described two cases with acute otitis media with hearing loss but both of them were ANCA positive. Pulmonary involvement is one of the cardinal features of WG. It occurs in 45% of patients at presentation and 87% during the course of the disease. [4] Our case of young female with intractable otitis media with effusion and atypical presentation of ANCA negative WG is being reported to emphasize the need for a thorough search for systemic lesions in unresolving serous otitis.

  References Top

Pagnoux C, Stubbe M, Lifermann F, Decaux O, Pavic M, Bérezné A, et al. Wegener's granulomatosis strictly and persistently localized to one organ is rare: Assessment of 16 patients from the French Vasculitis Study Group database. J Rheumatol 2011;38:475-8.  Back to cited text no. 1
McCaffrey TV, McDonald TJ, Facer GW, DeRemee RA. Otologic manifestations of Wegener's granulomatosis. Otolaryngol Head Neck Surg 1980;88:586-93.  Back to cited text no. 2
Verfaillie J, Clarysse J, Loncke R, Tanghe W. Otologic manifestations of Wegener's disease. Acta Otorhinolaryngol Belg 1992;46:369-73.  Back to cited text no. 3
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992;116:488-98.  Back to cited text no. 4


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