Successful cochlear implantation in a patient with MELAS syndrome.
Am J Otol
; 20(2): 187-90; discussion 190-1, 1999 Mar.
Article
en En
| MEDLINE
| ID: mdl-10100521
ABSTRACT
OBJECTIVE:
To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration. STUDYDESIGN:
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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Banco de datos:
MEDLINE
Asunto principal:
Síndrome MELAS
/
Implantación Coclear
/
Pérdida Auditiva Sensorineural
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Am J Otol
Año:
1999
Tipo del documento:
Article
País de afiliación:
Estados Unidos