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.


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.


CONCLUSIONS
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.
Yasumura
Satsuki
S
Department of Otolaryngology, Toyama Medical and Pharmaceutical University, Sugitani, Toyama, Japan. satsuki@ms.toyama-mpu.ac.jp
Aso
Shin
S
Fujisaka
Michiro
M
Watanabe
Yukio
Y
D000328:Adult; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold / Q000502:physiology; D001933:Brain Stem / Q000000981:diagnostic imaging; D003054:Cochlear Implants; D003638:Deafness / Q000000981:diagnostic imaging / Q000534:rehabilitation*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000000981:diagnostic imaging / Q000534:rehabilitation*; D009778:Occipital Lobe / Q000000981:diagnostic imaging; D011474:Prosthesis Design; D013069:Speech Reception Threshold Test; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome