Cochlear implantation for hearing loss due to an A8296G mitochondrial DNA mutation

Abstract Objective To characterize the clinical findings in a patient with hearing loss harboring an A8296G mitochondrial DNA mutation and the outcome of cochlear implantation. Patients A case report of a patient with hearing loss caused by an A8296G mitochondrial DNA mutation. Intervention The patient underwent cochlear implantation (CI). Main outcome and results: Bilateral moderate to severe hearing loss was found at high school age and progressed to severe hearing loss bilaterally at the age of 22. The patientu0027s low-tone hearing was relatively well preserved compared to high frequency, although it eventually declined. Speech perception in silence and at S/N10 improved to 100% and 92% for sentences, respectively, 3 years after CI. Conclusions We detailed the case of a patient with hearing loss due to an A8296G mitochondrial DNA mutation. Bilateral progressive hearing loss starting from high frequency was observed. Speech discrimination after CI was very good, indicating that a patient with this mutation is a good candidate for CI.