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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

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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Page 1: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 2: Audiology in Norrie Disease:  Research and Hearing Aids

Word Recognition:Right: 86% Left: 80%

AUDIOLOGY TESTING:

Page 3: Audiology in Norrie Disease:  Research and Hearing Aids

Word Recognition:Right: 86% Left: 80%

AUDIOLOGY TESTING: AUDIOGRAM

FREQUENCY

LO

UD

NE

SS

Page 4: Audiology in Norrie Disease:  Research and Hearing Aids

AUDIOLOGY TESTING:

Page 5: Audiology in Norrie Disease:  Research and Hearing Aids

AUDIOLOGY TESTING:

PURE TONE: 250 Hz

Page 6: Audiology in Norrie Disease:  Research and Hearing Aids

AUDIOLOGY TESTING:

PURE TONE: 250 Hz

LEVEL: 70 dBHL:

Page 7: Audiology in Norrie Disease:  Research and Hearing Aids

AUDIOLOGY TESTING:

PURE TONE: 250 Hz

LEVEL: 70 dBHL:

symbol

Page 8: Audiology in Norrie Disease:  Research and Hearing Aids

AUDIOLOGY TESTING:

PURE TONE: 250 Hz

LEVEL: 70 dBHL:

NORMAL RANGE

f lat

Page 9: Audiology in Norrie Disease:  Research and Hearing Aids

Word Recognition:Right: 86% Left: 80%

AUDIOLOGY TESTING:

“You will say…DOG”

Percent Correct

Page 10: Audiology in Norrie Disease:  Research and Hearing Aids

Word Recognition:Right: 86% Left: 80%

AUDIOLOGY TESTING: NORRIE DISEASE

Parving.gene.60%tb

Page 11: Audiology in Norrie Disease:  Research and Hearing Aids

0

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IHC

OHC

StriaVasc

Neur-ons

Apex Base

0

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StriaVasc

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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

@Temporal.are.moredata

Page 12: Audiology in Norrie Disease:  Research and Hearing Aids

60

Page 13: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 14: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 15: Audiology in Norrie Disease:  Research and Hearing Aids

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.

Page 16: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 17: Audiology in Norrie Disease:  Research and Hearing Aids

AGE 7

%C

orre

ct

Speech Level dBHL

Speech Level dBHL

%C

orre

ct%

Cor

rect

Speech Level dBHL

AGE 14

AGE 20

20yr

Page 18: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 19: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 20: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 21: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 22: Audiology in Norrie Disease:  Research and Hearing Aids

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Apex Base

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100

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IHC

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StriaVasc

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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

Page 23: Audiology in Norrie Disease:  Research and Hearing Aids

Early Stria Vascularis Pathology in the ND Cochlea

NorrieDiseaseMouse

(3 months)

ControlMouse

(3 months)

Page 24: Audiology in Norrie Disease:  Research and Hearing Aids

0

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OHC

StriaVasc

Neur-ons

Apex Base

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80

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100

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StriaVasc

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100

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StriaVasc

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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

Page 25: Audiology in Norrie Disease:  Research and Hearing Aids

5656

Formula.SII

Page 26: Audiology in Norrie Disease:  Research and Hearing Aids

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%

Page 27: Audiology in Norrie Disease:  Research and Hearing Aids

STRIA OUTER HAIR CELLS

apply ha.@

Page 28: Audiology in Norrie Disease:  Research and Hearing Aids

STRIA OUTER HAIR CELLS

apply ha

Page 29: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 30: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 31: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Words

Benefit: Improved Word Recognition

axes

Page 32: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Words

Benefit: Improved Word Recognition

axes

Page 33: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Words

Benefit: Improved Word Recognition

Page 34: Audiology in Norrie Disease:  Research and Hearing Aids

Benefit: Improvement With Level

Making Sound Loud:

Whisper Talk Yell

Cochlea

Match means

Page 35: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Loud.axis

Page 36: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Action of Aid

Page 37: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Action of Aid

Ben

efit

@another

Page 38: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Why.drawer.diff.fail

Page 39: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Page 40: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Page 41: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

?

Hasloss.@notNor

Page 42: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Action of Aid

Ben

efit

Nor

Page 43: Audiology in Norrie Disease:  Research and Hearing Aids

Making Sound Loud:

Whisper Talk Yell

Cochlea

Ben

efit

Nor

Page 44: Audiology in Norrie Disease:  Research and Hearing Aids

Word Improvement Limits:

Limit.apply.todig

Page 45: Audiology in Norrie Disease:  Research and Hearing Aids

O

DIGITAL HEARING AIDS...

kates

Page 46: Audiology in Norrie Disease:  Research and Hearing Aids

O

DIGITAL HEARING AIDS...

Same.limits

Page 47: Audiology in Norrie Disease:  Research and Hearing Aids

O

DIGITAL HEARING AIDS...

Are Not Louder...

Are Not Smaller...

Are Not Clearer...

They Are More Flexible(More Features)

Page 48: Audiology in Norrie Disease:  Research and Hearing Aids

Receiver(Speaker)

Page 49: Audiology in Norrie Disease:  Research and Hearing Aids

Receiver In The Canal(RITE)

SlimTube(Open Fit)

Slim-Tube v. Receiver In The Canal

Why.tiny

Page 50: Audiology in Norrie Disease:  Research and Hearing Aids

Receiver(Speaker)

upgrade

Page 51: Audiology in Norrie Disease:  Research and Hearing Aids

Receiver(Speaker)

Page 52: Audiology in Norrie Disease:  Research and Hearing Aids

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

advice

Page 53: Audiology in Norrie Disease:  Research and Hearing Aids

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

Mine doesn’t have a volume control...

Page 54: Audiology in Norrie Disease:  Research and Hearing Aids

“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.

Page 55: Audiology in Norrie Disease:  Research and Hearing Aids

“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.

Page 56: Audiology in Norrie Disease:  Research and Hearing Aids

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

People’s voices are too loud (Jackpot!)

Page 57: Audiology in Norrie Disease:  Research and Hearing Aids

“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!)

Page 58: Audiology in Norrie Disease:  Research and Hearing Aids

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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

Page 59: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 60: Audiology in Norrie Disease:  Research and Hearing Aids

Thank You...

Page 61: Audiology in Norrie Disease:  Research and Hearing Aids
Page 62: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 63: Audiology in Norrie Disease:  Research and Hearing Aids

Anecdotes:

at start

Page 64: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 65: Audiology in Norrie Disease:  Research and Hearing Aids

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

Is It On? I guess so...

Is It Too Loud? No...

unclear

Page 66: Audiology in Norrie Disease:  Research and Hearing Aids

Left 88%Right 72%

Age 14

Left 84%Right 72%

Speaker 100%

Age 7

Age 25

Page 67: Audiology in Norrie Disease:  Research and Hearing Aids

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

Page 68: Audiology in Norrie Disease:  Research and Hearing Aids

Left 24%Right 40%

Similar Genes, Age 48