The Laryngoscope
Lippincott Williams & Wilkins, Inc., Philadelphia
© 2002 The American Laryngological,
Rhinological and Otological Society, Inc.
Otologic Manifestations of Wegener’s
Granulomatosis
Dai Takagi, MD; Yuji Nakamaru, MD; Shiroh Maguchi, MD; Yasushi Furuta, MD; Satoshi Fukuda, MD
Objective/Hypothesis: To evaluate the clinical fea-
tures, treatment, and outcomes of otologic manifesta-
tions in Wegener’s granulomatosis (WG) treated at
Hokkaido University Graduate School of Medicine,
Sapporo, Japan. Study Design: We retrospectively re-
viewed 15 cases of WG with ear involvement. Methods:
Twenty-five patients with WG were treated at Hok-
kaido University Graduate School of Medicine be-
tween 1992 and 2001. Fifteen of these patients had
otologic symptoms. We evaluated the clinical course,
method of therapy, and outcomes in all cases. Diagno-
sis of WG was made when the patients had clinical
findings and a positive titer of cytoplasmic pattern
antineutrophil cytoplasmic antibodies (c-ANCA), or
when there were clear histologic findings. We also
present three case reports. Results: In 15 cases, the
most frequent finding was chronic otitis media. Sen-
sorineural hearing loss was present in 2 patients. In 7
patients whose otologic manifestations were the
primary involvement of WG, all were confirmed
positive for c-ANCA and were treated with glu-
cocorticoids and immunosuppressive drugs. Three
patients who could be treated within 1 month of
symptom onset showed marked improvement. Con-
clusions: In localized cases, biopsy specimens are
often small, and it is frequently difficult to make a
histologic diagnosis. The prognosis for hearing was
poor when appropriate treatment was not given in
the early stages of the disease. Therefore, WG
should be included in the differential diagnosis in
cases of atypical inflammatory states of the ear.
Early diagnosis and appropriate treatment are im-
portant to prevent irreversible changes in the middle
ear and inner ear. Key Words: Wegener’s granuloma-
tosis, otologic manifestations, c-ANCA, prednisolone,
immunosuppressive drugs.
Laryngoscope, 112:1684 –1690, 2002
INTRODUCTION
Wegener’s granulomatosis (WG) is a systemic vascu-
litic disease characterized by necrotizing granulomas and
From the Department of Otolaryngology and Head & Neck Surgery,
Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Editor’s Note: This Manuscript was accepted for publication March
25, 2002.
Send Correspondence to Dai Takagi, MD, Department of Otolaryn-
gology and Head & Neck Surgery Hokkaido University Graduate School of
Medicine, West 7 North 15 Sapporo, 060-8638, Japan. E-mail:
daita@med.hokudai.ac.jp
Laryngoscope 112: September 2002
1684
vasculitis of arterioles and venules. There is also a local-
ized form of WG limited to the upper and lower respiratory
tract.1 The majority of patients who present with WG have
problems with the nasal or paranasal sinuses.2 The prev-
alence of ear involvement varies from 19% to 61% of all
cases.3 Occasionally the otologic manifestation may be the
first and only sign of the disease.4,5 In these localized
cases, biopsy specimens are often small, and it is fre-
quently difficult to make a definite histologic diagnosis on
this alone.6 Cytoplasmic pattern antineutrophil cytoplas-
mic antibodies (c-ANCA), first reported in 1985 by Van der
Woude et al.,7 are highly specific for WG, especially in the
active phase. Thus, the presence of c-ANCA in WG is a
great aid to diagnosis. The difficulty of diagnosis often
delays the initiation of treatment, and it occasionally
progresses to the irreversible phase.
The purpose of this article is to present 3 cases of WG
that presented with acute otitis media and to better un-
derstand WG with ear involvement, especially in patients
primarily having ear involvement.
Clinical Material
Twenty-five patients were diagnosed with WG at
Hokkaido University between 1992 and 2001. There were
12 men and 13 women in the study group. Fifteen patients
(60%) had developed an otologic manifestation. Their ages
at the time of disease onset ranged from 20 to 76 years.
Diagnosis of WG in the present study was based on the
histopathologic identification of granulomatous inflamma-
tion, multinucleated giant cell, necrosis, and vasculitis in
biopsy specimens; or based on a positive titer of c-ANCA.8
RESULTS
Table I presents the otologic manifestations of WG in
the present study. The most frequent finding among these
was chronic otitis media. Serous otitis media was present
in 4 patients. Sensorineural hearing loss was present in
only 2, and 1 of these developed severe vertigo at the onset
of the disease. One patient had chronic otitis media and
developed unilateral facial nerve palsy following mastoid-
ectomy. The otologic manifestations were the first sign of
WG in 4 patients with chronic otitis media and in 2 pa-
tients with serous otitis media. The other organ involve-
ment in 15 patients with ear disease are presented in
Takagi et al.: Otologic Manifestations of WG
 TABLE I.
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-
function. Mild to moderate cyclophosphamide-induced leu-
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 71/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.

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1685
 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

1686

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
WG: 1) granulomatous lesions of the upper respiratory tract,
2) necrotizing vasculitis, and 3) glomerulonephritis. Subse-
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
1687
Fig. 3. Case no. 1: audiogram after treatment. There is improvement in bilateral conductive hearing loss, except in the low tone of the right
ear.

are present, even if a histologic diagnosis cannot be
made.8 Gross et al.23 recommended starting treatment
before the clinical diagnosis had been confirmed histolog-
ically in fulminant cases of WG. Repeated invasive proce-
dures such as kidney biopsies can delay both diagnosis
and initiation of the therapy until the onset of systemic
involvement of the disease. Macias et al.24 reported that in
such cases the c-ANCA serologic test is useful for early
diagnosis. As was the case in the present study, delays in
diagnosis and initiation of therapy negatively affect the
prognosis for hearing. Therefore, it is important to start
treatment before irreversible change occurs in the middle
ear and inner ear. On the other hand, there are differing
opinions on whether the diagnosis should be confirmed
histologically.25 In any case, only a high index of suspicion
will ensure an early diagnosis when otologic manifestation
is the first sign of the disease.
In 1983, Fauci13 reported the efficacy of a treatment
using a combination of glucocorticoids and cyclophospha-
mide, and this treatment has become the standard ther-
apy for WG. However, there are reports of complications of
cyclophosphamide, including cystitis, myelodysplasia, in-
fections, and infertility.16 In some cases, this treatment
has caused death. Gross23 reported that immunosuppres-

Fig. 4. Case no. 2: pretreatment audiogram showing bilateral mixed hearing loss.

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1688
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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