Cead Melas Bsno

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Cead Melas Bsno

  1. 1. MELAS Syndrome: the role of the otologist Petros Karkos, AFRCSI Murray Waldron, FRCS Ian Johnson, FRCS
  2. 2. MELAS <ul><li>M itochondrial cytopathy </li></ul><ul><li>E ncephalopathy </li></ul><ul><li>LA ctic acidosis </li></ul><ul><li>S troke-like episodes </li></ul><ul><li>AND… </li></ul><ul><li>SNHL </li></ul>
  3. 3. WHY? <ul><li>Early diagnosis </li></ul><ul><li>Succesful Rehabilitation </li></ul>
  4. 4. PATHOPHYSIOLOGY <ul><li>A POINT MUTATION of mt-DNA </li></ul><ul><li>> 16 in 100,000 </li></ul><ul><li>ATROPHY OF THE STRIA VASCULARIS </li></ul>
  5. 6. CLINICAL FEATURES <ul><li>CARDINAL </li></ul><ul><li>FREQUENT( SNHL) </li></ul><ul><li>OTHER </li></ul><ul><li>SNHL is frequent (44-75%) </li></ul><ul><li>( Hirano et al, Chinnery et al) </li></ul>
  6. 7. DIAGNOSIS <ul><li>HIGH INDEX OF SUSPICION </li></ul><ul><li>COMBINATION OF FEATURES: Myopathy& CNS involvement </li></ul><ul><li>GENETIC TESTS: A3243G mutation </li></ul><ul><li>MUSCLE Bx: ragged-red fibres </li></ul>
  7. 8. TREATMENT <ul><li>MULTIDISCIPLINARY Tx: </li></ul><ul><li>1)GUIDANCE ABOUT THE FUTURE </li></ul><ul><li>2)REGULAR F.Ups </li></ul><ul><li>3)INTERVENTION </li></ul><ul><li>ANAESTHETIC CONSIDERATIONS </li></ul>
  8. 9. HEARING LOSS
  9. 10. CONCLUSION <ul><li>ALL MELAS MUST BE SCREENED </li></ul><ul><li>ALL MELAS MUST BE FOLLOWED UP </li></ul><ul><li>OTOLOGIST NEEDS TO BE PART OF THE MELAS TEAM </li></ul>

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