Mitochondrial Sensorineural Hearing Loss: A Retrospective Study and a Description of Cochlear Implantation in a MELAS Patient.

Genetics Research International
Mauro ScarpelliGiuliano Tomelleri

Abstract

Hearing impairment is common in patients with mitochondrial disorders, affecting over half of all cases at some time in the course of the disease. In some patients, deafness is only part of a multisystem disorder. By contrast, there are also a number of "pure" mitochondrial deafness disorders, the most common probably being maternally inherited. We retrospectively analyzed the last 60 genetically confirmed mitochondrial disorders diagnosed in our Department: 28 had bilateral sensorineural hearing loss, whereas 32 didn't present ear's abnormalities, without difference about sex and age of onset between each single group of diseases. We reported also a case of MELAS patient with sensorineural hearing loss, in which cochlear implantation greatly contributed to the patient's quality of life. Our study suggests that sensorineural hearing loss is an important feature in mitochondrial disorders and indicated that cochlear implantation can be recommended for patients with MELAS syndrome and others mitochondrial disorders.

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Citations

Apr 12, 2016·International Journal of Cardiology·Josef Finsterer, Sinda Zarrouk-Mahjoub
Jun 23, 2015·Molecular Genetics and Metabolism·Ayman W El-HattabFernando Scaglia
Apr 15, 2017·Journal of Cellular and Molecular Medicine·Ying WangSiegfried Hekimi

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Methods Mentioned

BETA
biopsy
PCR
Assay
cochlear implant

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