audiology in norrie disease: research and hearing aids
DESCRIPTION
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)TRANSCRIPT
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Chris Halpin, Ph.D.
MassachusettsEye and EarInfirmary
Harvard MedicalSchool
August 14, 2009
First International
Norrie Disease Association Conference 2009:
AUDIOLOGY IN NORRIE DISEASE:RESEARCH
AND HEARING AIDS
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Word Recognition:Right: 86% Left: 80%
AUDIOLOGY TESTING:
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Word Recognition:Right: 86% Left: 80%
AUDIOLOGY TESTING: AUDIOGRAM
FREQUENCY
LO
UD
NE
SS
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AUDIOLOGY TESTING:
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AUDIOLOGY TESTING:
PURE TONE: 250 Hz
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AUDIOLOGY TESTING:
PURE TONE: 250 Hz
LEVEL: 70 dBHL:
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AUDIOLOGY TESTING:
PURE TONE: 250 Hz
LEVEL: 70 dBHL:
symbol
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AUDIOLOGY TESTING:
PURE TONE: 250 Hz
LEVEL: 70 dBHL:
NORMAL RANGE
f lat
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Word Recognition:Right: 86% Left: 80%
AUDIOLOGY TESTING:
“You will say…DOG”
Percent Correct
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Word Recognition:Right: 86% Left: 80%
AUDIOLOGY TESTING: NORRIE DISEASE
Parving.gene.60%tb
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FREQUENCY62.5 125 250 500 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
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60
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Age14
Age40
Age58
%C
orre
ct
Speech Level dBHL
%C
orre
ct
Speech Level dBHL
%C
orre
ct
Speech Level dBHL
Case A
Case B
Case C
You.sp.box
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Age14
Age40
Age58
%C
orre
ct
Speech Level dBHL
%C
orre
ct
Speech Level dBHL
%C
orre
ct
Speech Level dBHL
Case A
Case B
Case C
EARLY
MIDDLE
LATE
end
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2000 Hz THRESHOLDS BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
dB
HL
U.S. Costa Rica
WORD RECOGNITION BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
Per
cen
t C
orr
ect
Axes.
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2000 Hz THRESHOLDS BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
dB
HL
U.S. Costa Rica
WORD RECOGNITION BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
Per
cen
t C
orr
ect
axes.2.asy.outly.sp
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AGE 7
%C
orre
ct
Speech Level dBHL
Speech Level dBHL
%C
orre
ct%
Cor
rect
Speech Level dBHL
AGE 14
AGE 20
20yr
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010
20304050
60708090
100110120
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
dB
HL
Per
cen
t C
orr
ect
Thanks.19
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010
20304050
60708090
100110120
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
dB
HL
Per
cen
t C
orr
ect
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2000 Hz THRESHOLDS BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
dB
HL
U.S. Costa Rica
WORD RECOGNITION BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
Per
cen
t C
orr
ect
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2000 Hz THRESHOLDS BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
dB
HL
U.S. Costa Rica
WORD RECOGNITION BY AGE
0102030405060708090
100
0 10 20 30 40 50 60 70
Age in Years
Per
cen
t C
orr
ect
Import.yr
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Apex Base
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Neur-ons
Apex Base Apex Base
FREQUENCY62.5 125 250 500 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
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Early Stria Vascularis Pathology in the ND Cochlea
NorrieDiseaseMouse
(3 months)
ControlMouse
(3 months)
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0
1020
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80
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100
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IHC
OHC
StriaVasc
Neur-ons
Apex Base
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StriaVasc
Neur-ons
0
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IHC
OHC
StriaVasc
Neur-ons
Apex Base Apex Base
FREQUENCY62.5 125 250 500 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
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5656
Formula.SII
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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%
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STRIA OUTER HAIR CELLS
apply ha.@
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STRIA OUTER HAIR CELLS
apply ha
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In 2008, about 1.4 Million PeopleBought 2.4 Million Hearing AidsThey 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 ReputationWhich Is Addressed With Anecdotes of Success, Promises of New Technology
HEARING AIDS
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In 2007, about 1.4 Million PeopleBought 2.4 Million Hearing AidsThey 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 ReputationWhich Is Addressed With Anecdotes of Success, Promises of New Technology
HEARING AIDS
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Making Sound Loud:
Whisper Talk Yell
Words
Benefit: Improved Word Recognition
axes
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Making Sound Loud:
Whisper Talk Yell
Words
Benefit: Improved Word Recognition
axes
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Making Sound Loud:
Whisper Talk Yell
Words
Benefit: Improved Word Recognition
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Benefit: Improvement With Level
Making Sound Loud:
Whisper Talk Yell
Cochlea
Match means
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Making Sound Loud:
Whisper Talk Yell
Cochlea
Loud.axis
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Making Sound Loud:
Whisper Talk Yell
Cochlea
Action of Aid
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Making Sound Loud:
Whisper Talk Yell
Cochlea
Action of Aid
Ben
efit
@another
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Making Sound Loud:
Whisper Talk Yell
Cochlea
Why.drawer.diff.fail
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Making Sound Loud:
Whisper Talk Yell
Cochlea
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Making Sound Loud:
Whisper Talk Yell
Cochlea
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Making Sound Loud:
Whisper Talk Yell
Cochlea
?
Hasloss.@notNor
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Making Sound Loud:
Whisper Talk Yell
Cochlea
Action of Aid
Ben
efit
Nor
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Making Sound Loud:
Whisper Talk Yell
Cochlea
Ben
efit
Nor
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Word Improvement Limits:
Limit.apply.todig
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O
DIGITAL HEARING AIDS...
kates
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O
DIGITAL HEARING AIDS...
Same.limits
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O
DIGITAL HEARING AIDS...
Are Not Louder...
Are Not Smaller...
Are Not Clearer...
They Are More Flexible(More Features)
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Receiver(Speaker)
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Receiver In The Canal(RITE)
SlimTube(Open Fit)
Slim-Tube v. Receiver In The Canal
Why.tiny
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Receiver(Speaker)
upgrade
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Receiver(Speaker)
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“So, Can you make it too loud with no whistle?”
advice
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“So, Can you make it too loud with no whistle?”
Mine doesn’t have a volume control...
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“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 knowIt’s too limited.
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“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 knowIt’s too limited.
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“So, Can you make it too loud with no whistle?”
People’s voices are too loud (Jackpot!)
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“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!)
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Apex Base
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StriaVasc
Neur-ons
0
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50
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IHC
OHC
StriaVasc
Neur-ons
Apex Base Apex Base
FREQUENCY62.5 125 250 500 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
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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
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Thank You...
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Carrier,Age 68
Age44
Age38
%C
orre
ct
Speech Level dBHL
%C
orre
ct
Speech Level dBHL
Speech Level dBHL
%C
orre
ctCase E
Case D
Case F
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Anecdotes:
at start
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Anecdotes:
“I tried the new Zipzip 440and they loved it”
“This thing is no good. You ripped me off...”
“I baked you these cookies...”
dif is coch calc
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“So, Can you make it too loud with no whistle?”
Is It On? I guess so...
Is It Too Loud? No...
unclear
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Left 88%Right 72%
Age 14
Left 84%Right 72%
Speaker 100%
Age 7
Age 25
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Distortion Product Otoacoustic Emissions: Age 14
-20
-10
0
10
20
1000 2000 3000 4000 5000 6000
Geometric Mean of F1 and F2
dB S
PL
Noise L
DP L
Noise R
DP R
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Left 24%Right 40%
Similar Genes, Age 48