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Labyrinthine calcification in ears with otitis media and antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV): A report of two cases

Yoshida, Tadao Kobayashi, Masumi Sugimoto, Satofumi Naganawa, Shinji Sone, Michihiko 名古屋大学

2023.04

概要

The etiology of cochlear calcification includes otosclerosis, chronic otitis media,
temporal bone fractures, idiopathic meningitis, and Cogan’s syndrome. Calcification of
the cochlea caused by otosclerosis is the most common form reported, and exclusive
round window involvement of calcification has been seen in more than half of the cases
reported [1]. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
is a group of autoimmune diseases characterized by necrotizing vasculitis. Otitis media
is one of the intractable symptoms of AAV. Otitis media with AAV (OMAAV) was
defined as being caused by granulomatosis with polyangiitis (GPA), microscopic
polyangiitis, and eosinophilic granulomatosis with polyangiitis, but did not meet the
ordinary diagnostic criteria for systemic AAV. Calcification of the cochlea in cases of
OMAAV has not been reported to date. The diagnostic criteria of OMAAV proposed by
the Japan Otological Society are met if the following three criteria (A, B, and C) are
fulfilled. (A) At least one of the following two features: (1) presence of intractable otitis
media with effusion or granulation resistant to antibiotics and insertion of a
tympanostomy tube, or (2) progressive deterioration of bone conduction hearing levels.
(B) At least one of the following four features: (1) a previous diagnosis of AAV based
on the involvement of other organs, (2) positivity for serum myeloperoxidase (MPO)- or
proteinase 3 (PR3)-linked ANCA, (3) histopathology consistent with AAV, or (4) at least
one accompanying sign/symptom of AAV-related involvement other than the ear. (C)
Exclusion of the other types of intractable otitis media [2]. ...

参考文献

[1]

Vashishth A, Fulcheri A, Prasad SC, Bassi M, Rossi G, Caruso A, et al. Cochlear

implantation in cochlear ossification: retrospective review of etiologies, surgical

considerations, and auditory outcomes. Otol Neurotol 2018;39:17–28.

[2]

Harabuchi Y, Kishibe K, Tateyama K, Morita Y, Yoshida N, Okada M, et al.

Clinical characteristics, the diagnostic criteria and management recommendation

of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated

vasculitis (OMAAV) proposed by Japan Otological Society. Auris Nasus Larynx

2021;48:2–14.

[3]

Harabuchi Y, Kishibe K, Tateyama K, Morita Y, Yoshida N, Kunimoto Y, et al.

Clinical features and treatment outcomes of otitis media with antineutrophil

cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV): A retrospective

analysis of 235 patients from a nationwide survey in Japan. Modern

Rheumatology 2017;27:87–94.

[4]

Watanabe T, Yoshida H, Kishibe K, Morita Y, Yoshida N, Takahashi H, et al.

Cochlear implantation in patients with bilateral deafness caused by otitis media

with ANCA-associated vasculitis (OMAAV): A report of four cases. Auris Nasus

Larynx 2018;45:922–8.

[5]

Abou-Elhmd KA, Hawthorne MR, Flood LM. Cochlear implantation in a case

of Wegener’s granulomatosis. J Laryngol Otol 1996;110:958–61.

[6]

Ohtani I, Baba Y, Suzuki C, Sakuma H, Kano M. Temporal bone pathology in

Wegener’s granulomatosis. Fukushima J Med Sci 2000;46:31–9.

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[7]

Kato K, Sone M, Teranishi M, Yoshida T, Otake H, Nakashima T, et al. [Inner

ear 3D-FLAIR magnetic resonance image evaluation of MPO-ANCA-related

angitis patients]. Nihon Jibiinkoka Gakkai Kaiho 2013;116:1192–9. [in

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[8]

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[9]

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granulomatosis with polyangiitis. J Clin Imaging Sci 2019;9:13.

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2016;91:19–22.

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Figure legends

FIG. 1. Pure-tone audiometry of Case 1 at the first visit to our department.

FIG. 2. Axial temporal bone computed tomography (CT) centered on the mid-to-apical

turn of the cochlea of Case 1. A and B are axial CT images immediately after the onset

of the disease. There are shadows in the middle ear cavity and mastoid bone (arrow) but

no calcification in the cochlea. C shows calcification of the mid-to-apical turn of the

right ear (arrow); D shows no calcification of the cochlea. Reproduced in part from

reference [7].

FIG. 3. Axial temporal bone CT centered on the basal turn of the cochlea of Case 1. A

and B are axial CT images immediately after the onset of the disease. There are

shadows in the middle ear cavity and mastoid bone but no calcification in the cochlea. C

and D also show no calcification of the cochlea. Reproduced in part from reference [7].

FIG. 4. Three-dimensional fluid-attenuated inversion recovery magnetic resonance

imaging (MRI) showed high signals in the cochlea and vestibule (arrow). Reproduced in

part from reference [7].

FIG. 5. Pure-tone audiometry of Case 2 at the first visit to our department.

FIG. 6. Axial temporal bone CT scan and magnified images of Case 2. Panels A and B

show axial CT images obtained immediately after the onset of the disease. There are

shadows in the middle ear cavity and mastoid bone (arrow) but no calcification in the

14

cochlea. Panel C shows calcification of the scala tympani in the basal turn of the right

cochlea (arrow) and thickening of the ossicular laminae. Panel D shows calcification of

the scala tympani near the round window of the left cochlea (arrow).

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