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However, the benefits of the CI declined about three and a half years postoperation. Though the right ear had been reimplanted, the outcomes were still worse than before. A novel frame shift variant c.400_401insACTC (p.Q136LfsX58) in the gene was identified in a Chinese family with X-linked inheritance hearing loss. A patient with this mutation and IP-III malformation could get good benefits from CI. However, the outcomes of the cochlear implantation might decline as the patient grows old. A novel frame shift variant c.400_401insACTC (p.Q

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