Successful cochlear implantation in a patient with MELAS syndrome.
OBJECTIVE
To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration.


METHODS
A patient with a degenerative CNS disease (MELAS syndrome) undergoing evaluation for cochlear implantation is described.


METHODS
This study took place at a tertiary care center.


METHODS
A patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) who had cortical blindness and profound sensorineural hearing loss was evaluated and rehabilitated with cochlear implantation.


METHODS
Pure-tone audiogram, behavioral responses to promontory stimulation electrical auditory brainstem response, and electrically evoked middle-latency responses (MLRs) were used to assess eighth nerve, auditory brainstem, and cortical auditory pathways. Cochlear implantation with Cochlear Corporation mini 22 implant was performed.


RESULTS
Repeatable electrically evoked MLRs and behavioral responses to promontory stimulation documented the presence of auditory cortical responses. Successful implantation resulted in open set speech recognition and communication using the auditory/oral mode.


CONCLUSIONS
This report describes successful implantation in a patient with MELAS syndrome and demonstrates the ability to preoperatively confirm the integrity of brainstem and cortical auditory pathways despite significant CNS degeneration.
Rosenthal
E L
EL
Department of Otolaryngology-Head and Neck Surgery, The University of Michigan, Ann Arbor, USA.
Kileny
P R
PR
Boerst
A
A
Telian
S A
SA
D000328:Adult; D001301:Audiometry, Pure-Tone; D001306:Auditory Pathways / Q000502:physiology; D019929:Cochlear Implantation; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications*; D008279:Magnetic Resonance Imaging; D008297:Male; D009778:Occipital Lobe / Q000473:pathology; D011182:Postoperative Care; D016896:Treatment Outcome; D000159:Vestibulocochlear Nerve / Q000502:physiology