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Successful cochlear implantation in a patient with MELAS syndrome
E L Rosenthal1, P R Kileny, A Boerst
1Department of Otolaryngology-Head and Neck Surgery, The University of Michigan, Ann Arbor, USA.
Objective:
To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration.
Study Design:
A patient with a degenerative CNS disease (MELAS syndrome) undergoing evaluation for cochlear implantation is described.
Setting:
This study took place at a tertiary care center.
Patient:
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.
Interventions:
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.
Conclusion:
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.