Objective: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature.
Case presentation and management: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation.
Results: The surgery was without complications and postoperative course was uneventful. Two years after surgery, pure-tone and speech soundfield audiometry with left cochlear implant switched on showed a good improvement in pure-tone threshold and a word recognition score of 50% at 60 dB nHL. Literature review does not report any previous case of AIED EGPA-related.
Conclusions: Cochlear implantation in AIED EGPA-related have been shown to be a viable treatment option in a stabilized phase of disease.
Keywords: Churg-Strauss syndrome; audiology; autoimmune hearing loss; cochlear implantation; sudden hearing loss.