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  • Title: Successful cochlear implantation in a patient with MELAS syndrome.
    Author: Rosenthal EL, Kileny PR, Boerst A, Telian SA.
    Journal: Am J Otol; 1999 Mar; 20(2):187-90; discussion 190-1. PubMed ID: 10100521.
    Abstract:
    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.
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