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Audiology in Norrie Disease: Research and Hearing Aids
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)
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)
Audiology in Norrie Disease: Research and Hearing Aids
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
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
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
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
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.
“So, Can you make it too loud with no whistle?”
Is It On? I guess so...
Is It Too Loud? No...
unclear
65.
Left 88%
Right 72%
Age 14
Left 84%
Right 72%
Speaker
100%
Age 7
Age 25
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