Fig. 5. Case no. 3: pretreatment audiogram showing left-sided mixed hearing loss (A).
Three months after treatment, there is marked
improvement, except at 4 kHz (B).
sive drugs are not always needed in patients who lack
kidney involvement and recommended stage-adapted
treatment. Furthermore, in young patients Gross sug-
gested that cyclophosphamide should be switched to aza-
thioprine in the maintenance phase.
Thus, in case no. 1 in this study, we substituted
azathioprine for cyclophosphamide. However, Fauci13 re-
ported that azathioprine is not nearly as effective as it is
for inducing remission of active WG. Thus, careful
follow-up is required to detect recurrence of the lesion as
early as possible.
The majority of patients with serous otitis media
resulting from eustachian tube dysfunction by WG could
be helped by tympanostomy tube placement.4 Chronic oti-
tis media and sensorineural hearing loss occur from pri-
mary involvement of the ear by WG and they fail to re-
spond to conventional treatment such as antibiotics.
However, early treatment with glucocorticoids and cyclo-
phosphamide can resolve these symptoms.4,17,24 Because
it was reported that glucocorticoid treatment alone cannot
achieve complete remission of an otologic manifestation in
patients with WG,22 we recommended combined use of
immunosuppressive drugs when there is middle ear and
inner ear involvement.
In the present study, we performed methylpred-
nisolone pulse therapy for 2 patients who presented with
acute otitis media as an improved therapy, which resulted
in marked improvement. Furthermore, steroid pulse ther-
apy combined with cyclophosphamide should be consid-
ered in any case in which there is acute onset.
Laryngoscope 112: September 2002
CONCLUSION
We reviewed cases of WG that presented with oto-
logic manifestations. The most frequent finding was
chronic otitis media. Occasionally, otologic manifestations
presented as the first sign of the disease, which made
diagnosis more difficult. Therefore, WG should be in-
cluded in the differential diagnosis in cases of atypical
inflammatory states of the ear. The biopsy specimens are
often small and histologic diagnosis from the middle ear is
usually difficult. c-ANCA is helpful in making a diagnosis
in these localized cases. Early diagnosis and appropriate
treatment is important to prevent the progression of this
disease to an irreversible phase.
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