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. Word Recognition:
Right: 86%
Left: 80%
AUDIOLOGY TESTING:

3. Word Recognition:
Right: 86%
Left: 80%
AUDIOLOGY TESTING: AUDIOGRAM
FREQUENCY
LOUDNESS

4. AUDIOLOGY TESTING:

5. AUDIOLOGY TESTING:
PURE TONE: 250 Hz

6. AUDIOLOGY TESTING:
PURE TONE: 250 Hz
LEVEL: 70 dBHL:

7. AUDIOLOGY TESTING:
PURE TONE: 250 Hz
LEVEL: 70 dBHL:
symbol

8. AUDIOLOGY TESTING:
PURE TONE: 250 Hz
LEVEL: 70 dBHL:
NORMAL RANGE
flat

9. Word Recognition:
Right: 86%
Left: 80%
AUDIOLOGY TESTING:
“You will say
…DOG”
Percent
Correct

10. Word Recognition:
Right: 86%
Left: 80%
AUDIOLOGY TESTING: NORRIE DISEASE
Parving.gene.60%tb

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. 60

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. 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. 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. 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. AGE
7
%Correct
Speech Level dBHL
Speech Level dBHL
%Correct%Correct
Speech Level dBHL
AGE
14
AGE
20
20yr

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. 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. 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. 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. 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. Early Stria Vascularis Pathology in the ND Cochlea
Norrie
Disease
Mouse
(3 months)
Control
Mouse
(3 months)

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. 5656
Formula.SII

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. STRIA OUTER HAIR CELLS
apply ha.@

28. STRIA OUTER HAIR CELLS
apply ha

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. 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. Making Sound Loud:
Whisper Talk Yell
Words
Benefit: Improved Word Recognition
axes

32. Making Sound Loud:
Whisper Talk Yell
Words
Benefit: Improved Word Recognition
axes

33. Making Sound Loud:
Whisper Talk Yell
Words
Benefit: Improved Word Recognition

34. Benefit: Improvement
With Level
Making Sound Loud:
Whisper Talk Yell
Cochlea
Match means

35. Making Sound Loud:
Whisper Talk Yell
Cochlea
Loud.axis

36. Making Sound Loud:
Whisper Talk Yell
Cochlea
Action of Aid

37. Making Sound Loud:
Whisper Talk Yell
Cochlea
Action of Aid
Benefit
@another

38. Making Sound Loud:
Whisper Talk Yell
Cochlea
Why.drawer.diff.fail

39. Making Sound Loud:
Whisper Talk Yell
Cochlea

40. Making Sound Loud:
Whisper Talk Yell
Cochlea

41. Making Sound Loud:
Whisper Talk Yell
Cochlea
?
Hasloss.@notNor

42. Making Sound Loud:
Whisper Talk Yell
Cochlea
Action of Aid
Benefit
Nor

43. Making Sound Loud:
Whisper Talk Yell
Cochlea
Benefit
Nor

44. Word Improvement Limits:
Limit.apply.todig

45. O
DIGITAL HEARING AIDS...
kates

46. O
DIGITAL HEARING AIDS...
Same.limits

47. O
DIGITAL HEARING AIDS...
Are Not Louder...
Are Not Smaller...
Are Not Clearer...
They Are More Flexible
(More Features)

48. Receiver
(Speaker)

49. Receiver In The Canal
(RITE)
SlimTube
(Open Fit)
Slim-Tube v. Receiver In The Canal
Why.tiny

50. Receiver
(Speaker)
upgrade

51. Receiver
(Speaker)

52. “So, Can you make it too loud with no whistle?”
advice

53. “So, Can you make it too loud with no whistle?”
Mine doesn’t have a volume control...

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. “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. “So, Can you make it too loud with no whistle?”
People’s voices are too loud (Jackpot!)

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. 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. 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. Thank You...

62. 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

63. Anecdotes:
at start

64. 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

65. “So, Can you make it too loud with no whistle?”
Is It On? I guess so...
Is It Too Loud? No...
unclear

66. Left 88%
Right 72%
Age 14
Left 84%
Right 72%
Speaker
100%
Age 7
Age 25

67. 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

68. Left 24%
Right 40%
Similar Genes, Age 48