TABLE III.

Cases With Chronic Otitis Media and Sensorineural Hearing Loss in WG Our series
Treatment

Case No. Age (y)/Sex Findings Pulse* PSL† CPA‡ AZP§ Duration¶ Outcome

1 20/F Chronic otitis media E E E 1M Recovered

2 31/F Chronic otitis media E E 8M No change
3 41/M Chronic otitis media E E E 2W Recovered
4 62/M Chronic otitis media E E 3M No change
5 71/M Sensorineural hearing loss E E 2W Recovered
6 38/F Chronic otitis media E E 3M Dead (complication)
Facial nerve palsy
7 60/F Chronic otitis media E E 4M Dead (complication)
*Methylprednisolone pulse therapy.
†Prednisolone.
‡Cyclophosphamide.
§Azathioprine
¶Duration between onset of hearing loss and initiation of the treatment.

cavities, but the internal auditory structures were normal (Fig.
2). Serology using enzyme-linked immunosorbent assay (ELISA)
for c-ANCA tested positive with a titer of 20 U. The diagnosis of
WG was made based on these findings. A CT scan of the parana-
sal sinuses and lungs was normal. Fiberscopic bronchoscopy
showed no abnormalities in the bronchial tree. Renal function
was normal as well.
Methylprednisolone pulse therapy (1 g per day) was initi-
ated and her symptoms improved thereafter. Two weeks after the
start of the treatment, when the prednisolone was tapered to 40
mg per day, she again experienced bilateral profound hearing
loss. An audiogram demonstrated bilateral 65 dB conductive
hearing loss. The immunosuppressive therapy was started with
oral azathioprine (100 mg per day) combined with methylpred-
nisolone pulse therapy. One week later there was marked im-
provement in her hearing and she became asymptomatic. On
review at 6 months, she was doing well with a normal sedimen-
tation rate and CRP and hearing (Fig. 3).
At no time during the course of the disease was there any
evidence of involvement of the nose, lung, or kidney.
Case No. 2
A 31-year-old woman presented with a 1-week history of
left-sided otalgia and ear fullness. Examination revealed redness
of the left tympanic membrane, and a diagnosis of left-sided acute
otitis media was made. She failed to respond to treatment with
oral antibiotics and myringotomy. Two months later she devel-
oped bilateral hearing loss and right-sided otalgia accompanied
by nasal obstruction. A pure-tone audiogram demonstrated bilat-
eral mixed hearing loss (Fig. 4) and crusting in the bilateral nasal
cavity was discovered. A biopsy specimen was taken from the
nasal mucosa, which showed no evidence of WG.
Two months later, her hearing impairment began to
progress gradually. A CT scan of the temporal bone showed
thickening of the mucosal walls of the bilateral mastoid cavity.

Fig. 1. Case no. 1: pretreatment audiogram showing bilateral conductive hearing loss.

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

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