Wegener's Granulomatosis Presenting as Bilateral Otalgia with Facial Palsy: A Case Report
A 59-year old woman visited our hospital for otalgia, hearing loss of both ears beginning a month ago. On examination, otoendoscopic examination revealed thickened and hyperemic tympanic membrane with granulation of the both ear (). In initial blood tests, serum white blood cells was within normal limit (6470), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated (33 mm/hr, 1.10 mg/dL)(). The rest of the routine hematological, biochemical tests and chest posteroanterior (PA) were normal.\nThe pure tone audiometry showed bilateral mixed hearing loss with bone conduction threshold 30-35 dB. Computed tomography scanning of the temporal bone showed abnormal soft tissue densities in both mastoid, middle ear, epitympanum with left side worse. The canal of facial nerve was intact throughout its course.\nIntravenous antibiotics treatment was started with impression of bilateral acute middle ear infection. On 6th day in hospital, we did myringotomy in left tympanic membrane and found yellowish granulation and scanty mucopus from middle ear. On 7th day, she expressed more otalgia and left facial nerve palsy (House-Brackmann grade III) was noticed. After considering aggravation of lest facial palsy (H-B grade V), hearing deterioration, and continuous elevation of ESR and CRP, we decided to perform left mastoidectomy on 11th day (). Intraoperative finding showed loose granulation tissues filling entire mastoid and middle ear, which were easily sucked out with suction tip. Tissues were collected for bacterial, fungus, tuberculosis culture and pathologic evaluation. After left mastoidectomy, her left hearing seemed to improve slightly because of reduced air-bone gap. But otalgia, facial expression, ESR and CRP didn't show any improvement. Eight days after left ear surgery, right otalgia was dramatic aggravated with development of right facial palsy (House-Brackmann grade V). Wide myringotomy was done. At this time point, pathologic report showed necrotizing granulomatosis with negative Acid Fast Bacillus, tuberculosis-polymerase chain reaction results (). We started high dose steroid treatment with suspicion of WG and checked serologic test including pancytoplastmic granular staining pattern anti-neutrophil cytoplasmic antibodies (c-ANCA) and rechecked chest PA. Follow-up chest PA showed enlarged both hilar mass lesions, which were not showed on initial chest PA (). Subsequent chest CT showed multiple consolidations in both lungs with narrowing of left bronchus (). Ophthalmological evaluation of her left eye revealed conjunctivitis and marginal keratitis ().\nSerology test showed elevated c-ANCA, antineutrophil antibodies, RA factor. Left facial expression started to recover. We transferred her to rheumatology department for further treatment. Her facial expression came back to normal function during follow-up, but both tympanic membrane perforation and intermittent ear discharge and conductive hearing loss have remained until last follow-up (up to 1 year from first visit).