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Cochlear implantation of a deaf blind patient with mitochondrial cytopathy

  • Helen E. Cullington (a1)


Genetic defects of the mitochondrial DNA often cause sensorineural hearing impairment, accompaniment by disorders of organs within the body. This case report describes cochlear implantation of a 33-year-old deaf blind female with mitochondrial cytopathy. The outcome was very successful, and vastly improved quality of life for this patient. Many cases of mitochondrial cytopathy cause progressive deafness; it is, therefore, likely that other patients with this unusual disorder will present for cochlear implant assessment.


Corresponding author

Address for correspondence: Helen E. Cullington, Hearing and Balance Centre, Institute of Sound and Vibration Research, University of Southampton, Highfield, Southampton SO17 1BJ.


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Gold, M., Rapin, I. (1994) Non-Mendelian mitochondrial inheritance as a cause of progressive genetic sensorineural hearing loss. International Journal of Pediatric Otorhinolaryngology 30: 91104.
Jacobs, H. T., Shah, Z. H., Migliosi, V., Lehtinen, S. K., Rovis, A., O'Dell, K. (1998) Nuclear candidate genes for ‘mitochondrial deafness’. In Developments in Genetic Hearing Impairment (Stephens, D., Read, A., Martini, A., eds.) Whurr, London, pp 104115.
Schapira, A. H. (1993) Mitochondrial cytopathies. Current Opinions in Neurobiology 3(5): 760767.



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