Cochlear implantation in a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes syndrome.

OBJECTIVE

The aim of this study was to investigate the usefulness of a Nucleus C124M cochlear implant in a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome.

MATERIAL AND

METHODS

A 29-year-old woman with MELAS syndrome presented with sensorineural hearing loss and diabetes mellitus and underwent multichannel cochlear implantation. The follow-up period was 10 months. The case history, assessment of mitochondrial disease, indications for the cochlear implant and the benefits of cochlear implantation were evaluated.

RESULTS

Nine months after the surgery the patient could use the telephone and was quite satisfied with the improvement in communication due to the cochlear implant.

CONCLUSION

Cochlear implantation can be recommended for patients with MELAS syndrome if they have residual retrocochlear function. Single photon emission computerized tomography was found to be very useful for evaluating retrocochlear function.