RESUMO
OBJECTIVES: To evaluate the outcome and to confirm the validity of cochlear implantation for syndromic deafness in patients with mitochondrial disease. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: We reviewed medical charts of 367 cochlear implantation cases at Kyoto University Hospital between 1987 and 2012. We identified five patients with syndromic mitochondrial disease who underwent cochlear implantation surgery. The mean age of the patients (four women and one man) when they underwent surgeries was 44.4 years (range, 30-64 yr; median, 41 yr). INTERVENTIONS: Therapeutic and rehabilitative. MAIN OUTCOME MEASURE: In four of five patients, speech perception performance was measured using Japanese vowels, consonant-vowel syllables, and short sentences. RESULTS: Only 1.4% (5 of 367) of cochlear implantation cases at Kyoto University Hospital underwent cochlear implantation surgery because of syndromic mitochondrial diseases. Four of those patients showed significantly improved speech perception outcomes, and the beneficial effects of the intervention continued long after surgery. One patient could not perform speech perception test presumably because of poor cognitive function. CONCLUSION: Mitochondrial disease patients who underwent cochlear implantation surgery sustained gains in hearing performance even long after surgery. A single patient showed poor postoperative speech perception associated with cognitive problems. Cochlear implantation for mitochondrial disease patients seems to be a viable treatment option in the absence of significant cognitive impairment.