Case Reports| Volume 50, ISSUE 2, P299-304, April 2023

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


      Otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) has been proposed as a new type of otitis media. The hearing loss caused by OMAAV can be expected to improve with early detection and intervention, but if it continues to worsen and leads to deafness, it is challenging to recover the patient's hearing. When bilateral deafness occurs, cochlear implant (CI) surgery is the only way to improve hearing. Here, Case 1 showed unilateral cochlear calcification, and Case 2 showed bilateral cochlear calcification. In Case 1, CI surgery was performed on the ear lacking calcification, and in Case 2 it was performed on the ear with milder calcification. In Case 2, granulation was present from the tympanic space to the mastoid, the round window was closed, and the basal turn of the cochlea was narrowed. Such calcification of the cochlea caused by OMAAV has not been reported so far. It is essential to detect these changes by computed tomography scans at an early stage and to perform CI surgery at an appropriate time, because hearing improvements are not expected in patients who become deaf because of OMAAV.


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        • 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
        • 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
        • 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 Rheumatol. 2017; 27: 87-94
        • 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: 922-928
        • Abou-Elhmd KA
        • Hawthorne MR
        • Flood LM.
        Cochlear implantation in a case of Wegener's granulomatosis.
        J Laryngol Otol. 1996; 110: 958-961
        • Ohtani I
        • Baba Y
        • Suzuki C
        • Sakuma H
        • Kano M.
        Temporal bone pathology in Wegener's granulomatosis.
        Fukushima J Med Sci. 2000; 46: 31-39
        • 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 ([in Japanese]): 1192-1199
        • Yoshida N
        • Iino Y.
        Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis.
        Allergol Int. 2014; 63: 523-532
        • Livingston D
        • Rajan C
        • McKinney JM
        • Bhatt S.
        Tram-line” calcifications in granulomatosis with polyangiitis.
        J Clin Imaging Sci. 2019; 9: 13
        • Ayub B
        • Young NM.
        Cochlear implantation of solid organ transplant patients receiving immunosuppressive therapy.
        Int J Pediatr Otorhinolaryngol. 2016; 91: 19-22