Audiology in Norrie Disease: Research and Hearing Aids

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

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

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

AUDIOLOGY TESTING:

AUDIOLOGY TESTING: PURE TONE: 250 Hz

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

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

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

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

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

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

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

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

axes.2.asy.outly.sp

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

Threshold at 2000 Hz. Word Recognition Age In Years Age In Years dB HL Percent Correct Thanks.19

Threshold at 2000 Hz. Word Recognition Age In Years Age In Years dB HL Percent Correct

Import.yr

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

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

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

56 Formula.SII

56 @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%

STRIA OUTER HAIR CELLS apply ha.@

STRIA OUTER HAIR CELLS apply ha

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

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

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

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

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

Making Sound Loud: Whisper Talk Yell Cochlea Loud.axis

Making Sound Loud: Whisper Talk Yell Cochlea Action of Aid

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

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

Making Sound Loud: Whisper Talk Yell Cochlea

Making Sound Loud: Whisper Talk Yell Cochlea ? [email_address]

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

Making Sound Loud: Whisper Talk Yell Cochlea Benefit Nor

Word Improvement Limits: Limit.apply.todig

O DIGITAL HEARING AIDS... kates

O DIGITAL HEARING AIDS... Same.limits

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

Receiver (Speaker)

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

Receiver (Speaker) upgrade

Receiver (Speaker)

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

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

“ 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.

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

“ 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!)

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

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

Thank You...

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

Anecdotes: at start

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

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

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

Distortion Product Otoacoustic Emissions: Age 14 -20 -10 0 10 20 1000 2000 3000 4000 5000 6000 Geometric Mean of F1 and F2 dB SPL Noise L DP L Noise R DP R

Left 24% Right 40% Similar Genes, Age 48

Audiology in Norrie Disease: Research and Hearing Aids

by Norrie Disease Association on Sep 09, 2009

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Audiology in Norrie Disease: Research and Hearing Aids — Webinar Transcript

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