TABLE I. function.

Mild to moderate cyclophosphamide-induced leu-

Otologic Findings in 15 Patients With Wegener’s Granulomatosis.
Finding No. of Patients (%)
Chronic otitis media 9 (60)
Unilateral* 5 (33)
Bilateral 4 (27 )
Otitis media with effusion 4 (27 )
Sensorineural hearing loss† 2 (13)
*One patient developed unilateral facial nerve palsy after mastoidec-
tomy.
†In one case there was associated vertigo.
Table II. Fourteen (93%) of the 15 patients had the disease
affecting the nose or paranasal sinuses.
We further analyzed 7 patients (case nos. 1–7) whose
otologic symptoms were caused by WG. We excluded 4
patients with serous otitis media secondary to the nasal
involvement and 4 patients whose otologic symptoms
could not be followed up. There were 6 patients with
chronic otitis media and 1 with sensorineural hearing loss.
Five of the patients had mixed hearing loss. The other 2
patients showed conductive hearing loss and sensorineu-
ral hearing loss, respectively. The time from onset of hear-
ing loss to initiation of the treatment ranged from 2 weeks
to 8 months. Positive c-ANCA was confirmed in all 7
patients at some time during their illnesses. Only 3 of 7
patients were histologically diagnosed with WG, and their
specimens were taken from the paranasal sinuses.
All 7 patients were treated with glucocorticoids and oral
immunosuppressive drugs. Two patients were given meth-
ylprednisolone pulse therapy (1 g per d) for 3 days followed
by intravenous prednisolone. Six were started with oral cy-
clophosphamide (100 mg per d) and 1 patient (a 20-year-old
woman) was treated with azathioprine to prevent ovary dys-
kopenia occurred in 4 patients, forcing the discontinuation of
the medication in 2 of them. Two patients experienced severe
pulmonary infection and were treated with intravenous an-
tibiotics. One woman stopped having menses after treat-
ment with cyclophosphamide. Mild cyclophosphamide-
induced hair loss was shown in 1 patient. Drug-induced
cystitis and bladder cancer or other malignancies were not
observed in the 6 cases treated with cyclophosphamide. Two
patients who had undergone methylprednisolone pulse ther-
apy achieved complete improvement in hearing, and each
had started treatment within 1 month of symptom onset.
One patient achieved partial remission, 2 showed no
improvement, and 2 died with pulmonary failure, which
was attributed to treatment toxicity (Table III).
CASE REPORTS
Case No. 1
A 20-year-old woman presented with a 2-week history of
left-sided otalgia and ear fullness. Examination revealed redness
and swelling of the left tympanic membrane. The diagnosis of
acute otitis media was made and a myringotomy was performed.
Twenty-four hours later she developed severe left-sided postau-
ricular pain, otorrhea, and hearing loss. She was admitted to the
hospital and intravenous antibiotics were administered. On ad-
mission, the leukocyte count was 7880/mm3 with 71.1% neutro-
phils and 3% eosinophils. The erythrocyte sedimentation rate was
47 mm/hour, and the level of c-reactive protein (CRP) was slightly
elevated to 0.8 mg/dL. The otorrhea was cultured for tuberculosis
with negative results. A purified protein derivative test with 10
UI purified tuberculin was positive after 48 hours. The symptoms
and signs failed to respond to treatment, and 30 mg per day oral
prednisolone was initiated with no improvement.
A pure-tone audiogram revealed bilateral conductive hear-
ing loss with an air– bone gap of 50 dB on the left ear and 35 dB
on the right (Fig. 1). A computed tomography (CT) scan of the
temporal bone showed a thickening of mucosa in the mastoid

TABLE II.
Organ Involvement of Patients With Otological Manifestations.
Case No. Age (y)/Sex Ear Findings Nose Larynx Lung Kidney Eye Muscle Skin

1 20/F COM*
2 31/F COM ! ! !
3 41/M COM ! ! !
4 62/M COM ! ! ! ! ! !
5 7 1/M SNHL† ! ! !
6 38/F COM ! ! !
7 60/F COM ! ! !
8 37 /M OME‡ ! !
9 64/F OME ! ! ! !
10 37 /F COM ! ! !
11 63/F COM ! ! ! !
12 55/F COM !
13 48/M OME ! ! !
14 29/F OME ! ! !
15 56/M SNHL ! ! ! !
*Chronic otitis media.
†Sensorineural hearing loss.
‡Otitis media with effusion.

Laryngoscope 112: September 2002 Takagi et al.: Otologic Manifestations of WG

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