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Correspondence
Cochlear implantation in a profoundly deaf patient with MELAS syndrome
  1. A R Sinnathuray1,
  2. V Raut1,
  3. J G Toner1,
  4. A Magee2
  1. 1Department of Otolaryngology, Belfast City Hospital, Belfast, UK
  2. 2Department of Medical Genetics, Queen's University, Belfast at the Belfast City Hospital
  1. Correspondence to:
 Mr J G Toner, Director, Regional Cochlear Implant Centre, Belfast City Hospital, Lisburn Road, Belfast BT9 7AB, Northern Ireland, UK;
 jgtoner{at}ntlworld.com

https://doi.org/10.1136/jnnp.73.1.97

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    In response to the article “Cochlear implantation in a profoundly deaf patient with MELAS syndrome” (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes),1 we feel concerned that this patient may have a different diagnosis. This woman who received a cochlear implant is described as having the MELAS syndrome, in both the title and the text, when perhaps she has the less severe maternally inherited diabetes mellitus with deafness (MIDD) syndrome.

    She has the A3243G point mitochondrial DNA (mtDNA) mutation associated with insulin dependent diabetes mellitus, congenital cataracts, short stature, leg weakness, fatigue, and sensorineural hearing loss (SNHL), with no encephalopathy or strokes. The age of onset of SNHL was 22 years, with a slow deterioration to right profound SNHL at the age of 29 years, and bilateral profound SNHL and tinnitus at the age of …

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