1
Case report
In October 2006, a 53-year-old obese woman with type 2 diabetes and hypothyroidism was admitted to our otolaryngology department with a recurrent left facial nerve palsy lasting for 7 months; she also referred concomitant malaise. The palsy responded to administration of oral prednisone 50 mg a day, but it recurred each time a few days after stopping therapy. Otoscopy showed retrotympanic phlogosis, so suspecting a complicated otitis media, she was also prescribed antibiotics, without success. Audiometric evaluation showed a left mixed hearing loss on medium-low frequencies and normal hearing on the right side. Routine blood testing, chest radiographs, and electrocardiogram were normal. Erythrocyte sedimentation rate was raised (54 mm/h), whereas antinuclear antibody, perinuclear antineutrophil cytoplasmic antibody (ANCA), and circulating ANCA testing were negative. Temporal bone computed tomography (CT) scan documented isodense tissue involving the left middle ear and mastoid. Head and brain magnetic resonance imaging (MRI) did not add any further information.
In November 2006, because of the anomalous clinical picture, she underwent a left explorative tympanotomy: middle ear mucosa and facial nerve appeared slightly swelled, but histology from biopsies of the middle ear did not reveal anything except for nonspecific phlogosis.
In December 2006, she was again admitted to our hospital for a sudden visual loss in the left eye (finger count at 20 cm). She also referred cough and dyspnea, so a chest radiograph and then a confirmative chest CT scan were performed: a right hilar mass (4.4 × 2.5 cm) in a subcarinal position was noticed, compressing the superior cava vein, intermediate bronchus, and superior lobar bronchus.
A few days later, a marked pain in the whole left face was referred, and subsequently, a palsy of the third, fourth, sixth, ninth, and tenth left cranial nerves, amaurosis (80/200) in the right eye, and diabetes insipidus appeared. To exclude a paraneoplastic polyneuritis from a lung neoplasm, we then performed a bronchoscopy with multiple biopsies of bronchial mucosa: tissue withdrawn was compatible with a granulomatous inflammatory pathology, without any sign of malignancy. This time, head and brain MRI documented pathologic tissue uptaking gadolinium in the left rhinopharynx, parapharyngeal space, middle cranial fossa floor, cavernous sinus, and parasellar region ( Fig. 1 ). Renal function and renal ultrasound were normal. Again, the ANCA profile was repeated and was still negative: a hypothesis of tuberculous meningitis with diffuse skull base involvement was posed, but Ziehl-Neelsen staining did not reveal mycobacteria in the histologic samples.
Some days later, a necrotizing rhinosinusitis appeared, so a biopsy of nasal mucosa was performed, and this time, histologic findings ( Fig. 2 ) were strongly consistent with Wegener’s granulomatosis (WG). The patient was placed on a regimen of 400 mg/d of methylprednisolone for 3 days, 2 administrations of 5 mg/kg of infliximab (anti–tumor necrosis factor α antibodies) with a 2-week interval, and then cyclophosphamide 150 mg/d. Her symptoms markedly improved during the following 3 weeks, and she recovered her visual acuity (200/200) on the right side and third, fourth, sixth, ninth, and tenth left cranial nerve function, although she completely lost her vision in the left eye. She was discharged with 25 mg/d of oral prednisone, desmopressin acetate 0.1 mg BID, and cyclophosphamide 150 mg/d.
