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