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Cochlear implantation in a patient with profound hearing loss with the A1555G mitochondrial DNA mutation and no history of aminoglycoside exposure

Published online by Cambridge University Press:  25 November 2005

S Arif Ulubil ,
Alexis D Furze  and
Simon I Angeli
S Arif Ulubil
Affiliation:
Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida, USA
Alexis D Furze
Affiliation:
Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida, USA
Simon I Angeli
Affiliation:
Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida, USA
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Abstract

The A1555G mitochondrial deoxyribonucleic acid (mtDNA) point mutation has classically been associated with sensorineural hearing loss in patients following aminoglycoside exposure. More recently, the mutation has been implicated in sensorineural hearing loss in patients without previous aminoglycoside use. In addition, cochlear implantation has been shown to be effective in the group of patients with prior aminoglycoside exposure but, to date, no case of cochlear implantation in a patient with the A1555G mutation and no prior exposure to aminoglycosides has been explicitly described in the literature.

We report the case of an 80-year-old woman with the A1555G mtDNA mutation, a 35-year history of bilateral progressive hearing loss and no history of aminoglycoside exposure who underwent successful implantation of a Nucleus 24 Contour device at our institution. Post-operatively, the patient exhibited marked improvement in tests of auditory performance.

We conclude that cochlear implantation can be an effective method to restore some sense of hearing in patients with the A1555G mtDNA mutation and sensorineural hearing loss.

Keywords

Hearing Loss SensorineuralDNA MitochondrialMutationCochlear Implants
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 120 , Issue 3 , March 2006 , pp. 230 - 232
Copyright
© 2005 JLO (1984) Limited

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