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Cochlear implantation (CI) has been regarded as a safe and effective treatment for patients with severe to profound sensorineural hearing loss. The increase of experience in CI thus has had the effect of leading more children with inner ear anomaly to be considered as cochlear implant candidates. For patients with inner ear anomaly, auditory rehabilitation has been reported to be improved after CI; however, there are several factors such as cerebrospinal fluid leakage, facial nerve injury and abnormal positioning of electrode during surgery and uncertainty of auditory rehabilitation and possibility of the recurrent meningitis after CI that should be considered. Several studies proved that the incidence of otogenic meningitis is higher in patients with inner ear anomaly than in patients with normal inner ear anatomy. We experienced a case of recurrent meningitis due to an unusual cause after cochlear implantation in a patient with inner ear anomaly.