Combined Electric acoustic stimulation in a patient with otitis media with antineutrophil cytoplasmic antibody-associated vasculitis


      There are few reports of the treatment for severe hearing loss due to otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) achieved by cochlear implantation (CI). Here, we have reported the case of a patient with severe bilateral sensorineural hearing loss with low-frequency residual hearing by OMAAV. CI was performed in her right ear based on the results of contrast-enhanced magnetic resonance imaging (CE-MRI) and promontory stimulation test (PST). The residual hearing in her right ear was preserved after CI and utilized for combined electric acoustic stimulation (EAS). The combined EAS was used for 3 years until the residual hearing became stabilized. However, the usable hearing in low frequency worsened gradually, and the fitting strategy of cochlear implant was changed from combined EAS to CI alone 4 years after CI. Even when the speech discrimination score with CI no longer exceeds 50 %, the patient continued using CI because of its advantages in maintaining the quality of life of the patient. The combined EAS was found to be a feasible option even in an OMAAV patient with residual hearing. CE-MRI and PST may thus be helpful in deciding the side of CI surgery in a patient with OMAAV.


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        • 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 ontological society.
        Auris Nasus Larynx. 2020; 8: 54
        • 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.
        Mod Rheumatol. Jan 2017; 27: 87-94
        • Yoshida N.
        • Hara M.
        • Hasegawa M.
        • Matsuzawa S.
        • Shinnabe A.
        • Kanazawa H.
        • et al.
        Reversible cochlear function with ANCA-associated vasculitis initially diagnosed by otologic symptoms.
        Otol Neurotol. 2014; 35: 114-120
        • 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
        • Tagboto S.K.
        • Venkatesh AG.
        Progressive dyspnoea following the treatment of mycobacterium abscessus infection in an individual with relapsing granulamatosis with polyangiitis (Wegener's), complicated by hearing loss requiring cochlear implantation.
        BMC Pulm Med. 2012; 12: 47-51
        • Abou-Elhmd K.A.
        • Hawthorne M.R.
        • Flood LM.
        Cochlear implantation in a case of wegener's granulomatosis.
        J Laryngol Otol. 1996; 110: 958-961
        • House W.F.
        • Brackmann DE.
        Electrical promontory testing in differential diagnosis of sensorineural hearing impairment.
        Laryngoscope. 1974; 84: 2163-2171
        • Alfelasi M.
        • Piron J.P.
        • Mathiolon C.
        • Lenel N.
        • Mondain M.
        • Uziel A.
        • et al.
        The transtympanic promontory stimulation test in patients with auditory deprivation: correlations with electrical dynamics of cochlear implant and speech perception.
        Eur Arch Otorhinolaryngol. 2013; 270: 1809-1815
        • Kastenbauer S.
        • Klein M.
        • Koedel U.
        • Pfister HW.
        Reactive nitrogen species contribute to blood-labyrinth barrier disruption in suppurative labyrinthitis complicating experimental pneumococcal meningitis in the rat.
        Brain Res. 2001; 904: 208-217
        • Sugiura M.
        • Naganawa S.
        • Teranishi M.
        • Sato E.
        • Kojima S.
        • Nakashima T.
        Inner ear hemorrhage in systemic lupus erythematosus.
        Laryngoscope. 2006; 116: 826-828
        • von Ilberg C.
        • Kiefer J.
        • Tillein J.
        • Pfenningdorff T.
        • Hartmann R.
        • Sturzebecher E.
        • et al.
        Electric-acoustic stimulation of the auditory system. new technology for severe hearing loss.
        ORL J Otorhinolaryngol Relat Spec. 1999; : 61334-61340
        • Helbig S.
        • Helbig M.
        • Rader T.
        • Mack M.
        • Baumann U.
        Cochlear reimplantation after surgery for electric-acoustic stimulation.
        ORL J Otorhinolaryngol Relat Spec. 2009; 71: 172-178
        • Heba I.
        • Liu A.S.
        • Dunn C.
        • Gantz B.J.
        • McMurray B.
        Patient specific factors that influence hearing preservation following cochlear implantation.
        in: Proceedings of the 42nd Annual MidWinter Meeting of the Association for Research in Otolaryngology (ARO). University of Iowa Hospitals and Clinics, Department of Otolaryngology H and NS, 2019
        • Buchner A.
        • Illg A.
        • Majdani O.
        • Lenarz T.
        Investigation of the effect of cochlear implant electrode length on speech comprehension in quiet and noise compared with the results with users of electro-acousticstimulation, a retrospective analysis.
        PLoS One. 2017; 12 (e0174900 pone.0174900 PMID: 28505158