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Audiology in Norrie Disease: Research and Hearing Aids

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Dr. Chris Halpin discusses findings in audiology with respect to Norrie Disease. He concludes with a discussion of hearing aids for people with Norrie Disease. (NDA International Conference, 2009)

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Audiology in Norrie Disease: Research and Hearing Aids

  1. 1. Chris Halpin, Ph.D. Massachusetts Eye and Ear Infirmary Harvard Medical School August 14, 2009 First International Norrie Disease Association Conference 2009: AUDIOLOGY IN NORRIE DISEASE: RESEARCH AND HEARING AIDS
  2. 2. Word Recognition: Right: 86% Left: 80% AUDIOLOGY TESTING:
  3. 3. Word Recognition: Right: 86% Left: 80% AUDIOLOGY TESTING: AUDIOGRAM FREQUENCY LOUDNESS
  4. 4. AUDIOLOGY TESTING:
  5. 5. AUDIOLOGY TESTING: PURE TONE: 250 Hz
  6. 6. AUDIOLOGY TESTING: PURE TONE: 250 Hz LEVEL: 70 dBHL:
  7. 7. AUDIOLOGY TESTING: PURE TONE: 250 Hz LEVEL: 70 dBHL: symbol
  8. 8. AUDIOLOGY TESTING: PURE TONE: 250 Hz LEVEL: 70 dBHL: NORMAL RANGE flat
  9. 9. Word Recognition: Right: 86% Left: 80% AUDIOLOGY TESTING: “You will say …DOG” Percent Correct
  10. 10. Word Recognition: Right: 86% Left: 80% AUDIOLOGY TESTING: NORRIE DISEASE Parving.gene.60%tb
  11. 11. 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base Apex Base FREQUENCY 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K FREQUENCY FREQUENCY NEURONS OUTER HAIR CELLS STRIA VASCULARIS 88% 44% 80% A B C @Temporal.are.moredata
  12. 12. 60
  13. 13. Age 14 Age 40 Age 58 %Correct Speech Level dBHL %Correct Speech Level dBHL %Correct Speech Level dBHL Case A Case B Case C You.sp.box
  14. 14. Age 14 Age 40 Age 58 %Correct Speech Level dBHL %Correct Speech Level dBHL %Correct Speech Level dBHL Case A Case B Case C EARLY MIDDLE LATE end
  15. 15. 2000 Hz THRESHOLDS BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years dBHL U.S. Costa Rica WORD RECOGNITION BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years PercentCorrect Axes.
  16. 16. 2000 Hz THRESHOLDS BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years dBHL U.S. Costa Rica WORD RECOGNITION BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years PercentCorrect axes.2.asy.outly.sp
  17. 17. AGE 7 %Correct Speech Level dBHL Speech Level dBHL %Correct%Correct Speech Level dBHL AGE 14 AGE 20 20yr
  18. 18. 0 10 20 30 40 50 60 70 80 90 100 110 120 0 5 10 15 20 25 30 Left 2KHz Right 2KHz 0 10 20 30 40 50 60 70 80 90 100 0 5 10 15 20 25 30 Left Word % Right Word % Threshold at 2000 Hz. Word Recognition Age In Years Age In Years dBHLPercentCorrect Thanks.19
  19. 19. 0 10 20 30 40 50 60 70 80 90 100 110 120 0 5 10 15 20 25 30 Left 2KHz Right 2KHz 0 10 20 30 40 50 60 70 80 90 100 0 5 10 15 20 25 30 Left Word % Right Word % Threshold at 2000 Hz. Word Recognition Age In Years Age In Years dBHLPercentCorrect
  20. 20. 2000 Hz THRESHOLDS BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years dBHL U.S. Costa Rica WORD RECOGNITION BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years PercentCorrect
  21. 21. 2000 Hz THRESHOLDS BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years dBHL U.S. Costa Rica WORD RECOGNITION BY AGE 0 10 20 30 40 50 60 70 80 90 100 0 10 20 30 40 50 60 70 Age in Years PercentCorrect Import.yr
  22. 22. 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base Apex Base FREQUENCY 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K FREQUENCY FREQUENCY NEURONS OUTER HAIR CELLS STRIA VASCULARIS 88% 44% 80% A B C Read.aud
  23. 23. Early Stria Vascularis Pathology in the ND Cochlea Norrie Disease Mouse (3 months) Control Mouse (3 months)
  24. 24. 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base Apex Base FREQUENCY 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K FREQUENCY FREQUENCY NEURONS OUTER HAIR CELLS STRIA VASCULARIS 88% 44% 80% A B C
  25. 25. 5656 Formula.SII
  26. 26. 5656 @90 dBHL pred.=94% (.6 AI), act.=84% (.4 AI); loss (.2) / info (.6) = 33% @70 dBHL pred.=67% (.3 AI), act.=40% (.2 AI); loss (.1) / info (.3) = 33%
  27. 27. STRIA OUTER HAIR CELLS apply ha.@
  28. 28. STRIA OUTER HAIR CELLS apply ha
  29. 29. In 2008, about 1.4 Million People Bought 2.4 Million Hearing Aids They Are Now $1500 - $3500 each; 5B About 31 Million Americans “Have Hearing Loss” And 7 Million Have Bought Hearing Aids About Half Are “Satisfied” Many Do Not Wear Hearing Aids Have a Bad Reputation Which Is Addressed With Anecdotes of Success, Promises of New Technology HEARING AIDS
  30. 30. In 2007, about 1.4 Million People Bought 2.4 Million Hearing Aids They Are Now $1500 - $3500 each; 5B About 31 Million Americans “Have Hearing Loss” And 7 Million Have Bought Hearing Aids About Half Are “Satisfied” Many Do Not Wear Hearing Aids Have a Bad Reputation Which Is Addressed With Anecdotes of Success, Promises of New Technology HEARING AIDS
  31. 31. Making Sound Loud: Whisper Talk Yell Words Benefit: Improved Word Recognition axes
  32. 32. Making Sound Loud: Whisper Talk Yell Words Benefit: Improved Word Recognition axes
  33. 33. Making Sound Loud: Whisper Talk Yell Words Benefit: Improved Word Recognition
  34. 34. Benefit: Improvement With Level Making Sound Loud: Whisper Talk Yell Cochlea Match means
  35. 35. Making Sound Loud: Whisper Talk Yell Cochlea Loud.axis
  36. 36. Making Sound Loud: Whisper Talk Yell Cochlea Action of Aid
  37. 37. Making Sound Loud: Whisper Talk Yell Cochlea Action of Aid Benefit @another
  38. 38. Making Sound Loud: Whisper Talk Yell Cochlea Why.drawer.diff.fail
  39. 39. Making Sound Loud: Whisper Talk Yell Cochlea
  40. 40. Making Sound Loud: Whisper Talk Yell Cochlea
  41. 41. Making Sound Loud: Whisper Talk Yell Cochlea ? Hasloss.@notNor
  42. 42. Making Sound Loud: Whisper Talk Yell Cochlea Action of Aid Benefit Nor
  43. 43. Making Sound Loud: Whisper Talk Yell Cochlea Benefit Nor
  44. 44. Word Improvement Limits: Limit.apply.todig
  45. 45. O DIGITAL HEARING AIDS... kates
  46. 46. O DIGITAL HEARING AIDS... Same.limits
  47. 47. O DIGITAL HEARING AIDS... Are Not Louder... Are Not Smaller... Are Not Clearer... They Are More Flexible (More Features)
  48. 48. Receiver (Speaker)
  49. 49. Receiver In The Canal (RITE) SlimTube (Open Fit) Slim-Tube v. Receiver In The Canal Why.tiny
  50. 50. Receiver (Speaker) upgrade
  51. 51. Receiver (Speaker)
  52. 52. “So, Can you make it too loud with no whistle?” advice
  53. 53. “So, Can you make it too loud with no whistle?” Mine doesn’t have a volume control...
  54. 54. “So, Can you make it too loud with no whistle?” “It’s Whistling... That’s how I know it’s too loud.” (WRONG) That’s how we know It’s too limited.
  55. 55. “So, Can you make it too loud with no whistle?” “It’s Whistling... That’s how I know it’s too loud.” (WRONG) That’s how we know It’s too limited.
  56. 56. “So, Can you make it too loud with no whistle?” People’s voices are too loud (Jackpot!)
  57. 57. “So, Can you make it too loud with no whistle?” “I don’t like it, but it’s easier to hear people” “I turn it down a little so it doesn’t drive me crazy” (OK) Hearing aids should be loud… to reduce the workload of communicating. People’s voices are too loud (Jackpot!)
  58. 58. 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons 0 10 20 30 40 50 60 70 80 90 100 110 IHC O H C Stria Vasc Neur- ons Apex Base Apex Base FREQUENCY 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K 62.5 125 250 500 1K 2K 4K 8K 16K FREQUENCY FREQUENCY NEURONS OUTER HAIR CELLS STRIA VASCULARIS 88% 44% 80% A B C
  59. 59. National Temporal Bone Donor Program • Enlists people with otologic disorders to become temporal bone donors • Registry arranges the pledge, obtains medical records, and updates records periodically
  60. 60. Thank You...
  61. 61. Carrier,Age 68 Age 44 Age 38 %Correct Speech Level dBHL %Correct Speech Level dBHL Speech Level dBHL %Correct Case E Case D Case F
  62. 62. Anecdotes: at start
  63. 63. Anecdotes: “I tried the new Zipzip 440 and they loved it” “This thing is no good. You ripped me off...” “I baked you these cookies...” dif is coch calc
  64. 64. “So, Can you make it too loud with no whistle?” Is It On? I guess so... Is It Too Loud? No... unclear
  65. 65. Left 88% Right 72% Age 14 Left 84% Right 72% Speaker 100% Age 7 Age 25
  66. 66. Distortion Product Otoacoustic Emissions: Age 14 -20 -10 0 10 20 1000 2000 3000 4000 5000 6000 Geometric Mean of F1 and F2 dBSPL Noise L DP L Noise R DP R
  67. 67. Left 24% Right 40% Similar Genes, Age 48

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