Wegener’s Granulomatosis Presenting as Facial Palsy Giuseppe Magliulo, MD, Serena Varacalli, MD, and Carmen Sepe, MD (Editorial Comment: This is indeed a unique presenting symptom of Wegner’s granulomatosis and reiterates to the otolaryngologist that one needs to consider various granulomatosis diseases in one’s differential of chronic otitis media, especially with such mitigating circumstances as facial nerve paraly- sis and sensorineural hearing loss.) Wegener’s granulomatosis (WG) is a necros- ing vasculitis that develops a granulomatous lesion affecting both arterioles and venules. The classical triad is an acute infection of the superior and inferior respiratory tracts with renal involvement. However, any organ can be the site of granulomatous disease or vasculitis or b0th.l Thirty-eight percent of the cases have ear involvement. Occasionally, the ear is the only site of the disease.2 Facial nerve palsy is rarely seen as the initial clinical findings3 The purpose of this study is to report a patient that was affected by facial nerve palsy that occurred as the first symptom of WG. The importance of early diagnosis and adequate medical therapy are highlighted and dis- cussed. CASE REPORT A 25-year-old man was referred to our Ear, Nose, and Throat department because he was suffering from left facial nerve palsy (grade VI, House- Brackmann classification). The initial symptomatol- ogy also included an intermittent otalgia and a continuous tinnitus of moderate intensity that started immediately after scuba diving 1 month before. No previous history for trauma or upper respira- tory diseases (ie otorrhea) was reported, and no spontaneous or induced nystagmus was detected at vestibular tests. An initial treatment with nonste- From the 2nd Ear, Nose, and Throat Clinic, Universita’ Deali Studidi Roma. La Sahenza. Rome. Italv. iddress reprint ;eque&s to Giuseppe Migliulo, MD, via Gregorio VII 80,00165 Rome, Italy. Copyright 0 1999 by W.B. Saunders Company 0196-0709/99/2001-0009$10.00/O roid anti-inflammatory drugs was a complete fail- ure. Further therapy with antibiotics and steroids did not achieve significant improvement. On otos- copy, the tympanic membrane was intact with a disappearance of the Politzer’s triangle and a hyper- emia of the posterior quadrant. Audiometry showed an ipsilateral sensorineural hearing loss. Contralat- era1 hearing thresholds were in the normal range. Erythrocyte sedimentation rate was markedly in- creased (30 mm/h). Electromyography responses confirmed a severe peripheric dysfunction of the left facial nerve. Computed tomographic scans docu- mented a chronic inflammatory reaction that was complicated by cholesteatoma of the tympanic cav- ity and of the epitympanum (Figs 1 and 2). Mag- netic resonance imaging confirmed the diagnosis of chronic otitis media with cholesteatoma. Tympanoplasty with mastoidectomy was planned to remove the inflammatory process. Surgery did not confirm the presence of cholesteatoma. Frozen section and histologic examination of the specimen disclosed granulomatous inflammatory tissue that was interpreted as an otic polyp (Fig 3). The facial nerve was decompressed at the level of the labyrin- thine, tympanic, and mastoid segments. The postoperative course was uneventful, and the patient was discharged 7 days later. During the following 2 months, the patient was in good health and did not complain of local or general symptom- atology. The sudden development of signs and symptoms of bowel obstruction obliged the patient to undergo intestinal resection. The specimen was examined histologically, confirming the diagnosis of WG (Fig 4). After a l-year follow-up, the inflammatory pro- cess of the mastoid completely subsided (Fig 4). WG was found to be in the quiescent phase, even in other locations. DISCUSSION An otologic involvement of WG is a quite common clinical finding. Nicklasson and Stangeland2 reported that 38% of the cases in their study exhibited such a condition. McCaffrey et al4 observed that the middle ear is the most frequently involved site of disease. They reported unilateral serous otitis media in 90% of the cases with bilateral otitis in 33% of the cases. Chronic otitis media with American Journal of Otolaryngology, Vol20, No 1 (January-February), 1999: pp 43-45 43