WG: 1) granulomatous lesions of the upper respiratory tract,
2) necrotizing vasculitis, and 3) glomerulonephritis. Subse-
Fig. 2. Axial CT scan of the temporal bone showing thickening of
the mucosa of the mastoid cavity and middle ear without bone
destruction.
The leukocyte count was 5400/mm3 and CRP was elevated to 2.6
mg/dL. A c-ANCA test was positive and a diagnosis of WG was
made. A transcutaneous renal biopsy was performed with non-
specific inflammatory changes. Treatment with oral cyclophosph-
amide (100 mg per day) and oral prednisolone (60 mg per day)
was initiated. Her hearing had improved to 25 dB in the left ear
with no improvement in the right ear, and the cyclophosphamide
and prednisolone were then tapered. However, 3 months later she
again experienced left-sided hearing loss accompanied by fever.
Her general condition improved after immunosuppressive treat-
ment but the hearing loss persisted.
Case No. 3
A 41-year-old man had a 2-month history of nasal obstruc-
tion, headache, and low-grade fever. He was treated for chronic
sinusitis without improvement. He developed proptosis of the
right eye, and a CT scan of the orbita and paranasal sinus showed
thickening of the mucosal wall of the right ethmoid sinus and
orbital cellulitis. The leukocyte count was 12,900/mm3 and ESR
was elevated to 84 mm/hr. He was admitted and treated with
intravenous antibiotics and prednisolone. Five days later, he
developed left-sided otalgia and hearing loss with a severe head-
ache, and a left myringotomy was therefore performed.
The patient failed to respond to the treatment and was
therefore treated using intranasal ethmoidectomy, also without
improvement. Histology of the ethmoid mucosa showed nonspe-
cific inflammatory changes. An audiogram showed mixed hearing
loss on the left with 65 dB (Fig. 5A). He was referred to Hokkaido
University Graduate School of Medicine for further investigation.
Serology using immunofluorescence for ANCA was positive with
a titer of 1/64. The diagnosis was a localized form of WG involving
the ear, eye, nose, and paranasal sinuses. Treatment with oral
cyclophosphamide (100 mg per day) and methylprednisone pulse
therapy (500 mg per day) was initiated. Two months after the
start of the treatment, his hearing had reverted to normal (Fig.
5B) and the titer of c-ANCA was negative. Two months later, the
cyclophosphamide was discontinued because of pancytopenia and
the patient was maintained on prednisolone alone. Nine years
after the initiation of the therapy, he is alive and doing well and
was therefore taken off all medications.
DISCUSSION
Wegener’s granulomatosis is a relatively rare disease.
Godman and Churg9 established the diagnostic criteria of
Laryngoscope 112: September 2002
quently, “limited forms” of the disease were described,1 and
the majority of cases present with involvement of the head
and neck region. The nasal and paranasal sinus is involved
in 85% of cases at some time over the course of the disease,10
and otologic involvement may occasionally be the first and
only sign of the disease.11,12
Fauci et al.13 reported that 25% of patients with WG
presented with serous otitis media and 6% of patients
presented with hearing loss as the initial sign of the dis-
ease. Kempf14 reported that approximately half of the
patients with WG developed otologic manifestations in the
early stage of the disease. One case of WG limited entirely
to the ear has also been reported.5
Otologic involvement was divided into the following
basic types: 1) serous otitis media resulting from eusta-
chian tube obstruction and nasopharyngeal involve-
ment11; otologic involvement appears most often as serous
otitis media4,15; 2) chronic otitis media, which is caused by
primary involvement of the middle ear and mastoid cav-
ity; 3) sensorineural hearing loss: the etiology is unknown
but is considered to involve vasculitis of the cochlear ves-
sels and deposition of the immune complex in the co-
chlea15; 4) vertigo involving several etiologic theories: a)
immune complex deposition in the vestibular portion, and
b) manifestation of central nervous system involvement
caused by a polyneuritis; and 5) facial nerve palsy, which
is seen in 8% to 10% of cases, usually associated with otitis
media.3 In the majority of cases, the facial paralysis im-
proves with cytotoxic therapy.
If untreated, the disease usually runs a rapidly fatal
course and 82% of patients die within 1 year. Thus, accu-
rate and early diagnosis has become of paramount impor-
tance to improve the prognosis.
Biopsy specimens from the head and neck region are
often small and it is usually difficult to make a definite
histologic diagnosis,8,13,16,17 particularly when it is taken
from the middle ear.18,19 Kempf14 reported that the ex-
pected typical histologic picture of WG was not found in
the middle ear biopsy. Devaney et al.20 reported that only
one of three mastoid biopsy specimens showed evidence of
WG, as did none of four middle ear specimens. In the head
and neck region, a biopsy from the paranasal sinuses
showed a higher positive rate. Therefore, it is recom-
mended to take biopsy specimens from the paranasal si-
nus or nose.21,22
As was the case in the present study, there are
several case reports of patients whose symptoms be-
came worse after myringotomy or who developed facial
nerve paralysis after mastoidectomy.12 It is uncertain
whether this resulted from the surgical procedure or
from the progress of the disease. However, the decision
concerning the surgical procedure to the ear should be
made carefully, particularly in the active phase of the
disease.
It has been reported that c-ANCA is highly specific
for active WG and that the c-ANCA titer is directly related
to the disease activity.7,8 At Hokkaido University Gradu-
ate School of Medicine, we start treatment when the titer
of c-ANCA is positive and when the clinical features of WG
Takagi et al.: Otologic Manifestations of WG
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