MASTER
SLIDES
NORWAY
The Diagnosis and Management of
Auditory Neuropathy/Dyssynchrony
:
Auditory Neuropathy Spectrum Disorders
•
Recently re-named at the Lake Como Consensus Conference
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Charles I. Berlin, PhD, Professor
Benjamin A. Russell, Esteemed Colleague
University of South Florida Tampa FL. USA
cberlin@cas.usf.edu
barussell@gmail.com
Also: Fifty-three Years of
Mistakes I have Made, and how
to avoid them.
Charles I. Berlin, PhD
Formerly Director of the Kresge Hearing Research
Laboratory
LSU Health Sciences Center, Dept Otolaryngology,
Head and Neck Surgery
Post-Katrina: Research Professor of CSD and
Otolaryngology Head and Neck Surgery
University of South Florida
Tampa, FL
Sampling audience agendas
• The prepared materials are subject to
improvisational change in response to
your special interests and needs.
• Let’s review those first.
• The handouts will retain their utility as we
cross various streams.
Stream 1
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The basic rules of speech and hearing and
their interactions.
F.I.T.
S===H
Demonstrations
Speech is in the ear of the listener not the
mouth of the speaker. Examples.
Rules and principles as they apply to
audiological assessment, hearing aid fitting
and educational management.
Additional principles and rules
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Articulation Index
Context vs. Intelligibility
Language vs. Speech
Luria effects.
Other principles based on Speech
Acoustics.
If you have no
loss of outer hair
cells…this is
what clear
speech sounds
like to you. The
dot system
assumes normal
effort speech at
roughly 50 dB
Hearing Level at
6 feet distance.
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
have a
sharply
sloping
high
frequency
loss and
hear
mostly
voice but
do not
understan
d
speech…
this is
what the
same
passage
sounds
Shortcut to 250
If your loss
begins at 500 Hz
you will hear
more of the
speech signal but
still have difficulty
understanding
without lip
reading
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
If your loss
begins at
1000 Hz
you will
hear much
more of
speech but
still be
handicappe
d especially
in noisy
situations
A hearing loss
above 4000 Hz is
often (incorrectly)
considered
inconsequential.
Some people may
still have trouble in
noise and in
multiple
conversations and
locating sound
sources.
.
Copy of 4k HIGH PASS.wma
Many people
mistakenly believe that
only high frequency
losses cause
listening problems;
listen to this severe
LOW frequency loss
and see
if you agree!?
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
Now the low
frequency
loss allows
you to hear
sounds
down to
2000 Hz.
Notice the
sudden
improvemen
t!
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
Now even
more of the
important
frequencies
become
audible but
the speech
still sounds
“tinny”.
Now things
are slightly
more natural
but still “low
fidelity”.
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
Now even
more natural
but still lacking
full fidelity
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
And finally
back to full
fidelity and a
“normal
audiogram”.
From C.Berlin, Hair
Cells and Hearing
Aids, 1996 Singular
Publ. Group
Real Time Demonstration of
Speech Acoustics and Analysis
Sub question: Are Consonants
really “high frequency” and vowels
really “low frequency”?
But first an exercise…
•Draw a sine wave.
•You have probably
drawn a picture in
Time-by-amplitude
as follows:
Time-by-amplitude sine wave
Continuing with the exercise
•Draw the same
sine wave in a
frequency-byamplitude plot.
Frequency-by-amplitude (insert)
compared to Time-by-amplitude
(background)
Now a time-by-frequency plot or
spectrogram.
These exercises will help us better
apply and understand the appearance
and…
• …the nature of frequency response curves in
speakers, earphones, and hearing aids.
• …the nature of otoacoustic emissions displays.
• …the differences between DPOAE and TEOAE
recordings.
• …cochlear microphonics, summating potentials,
and other electrical events recordable from the
ear.
• How to display and analyze speech of the deaf
and hard-of-hearing.
Stream 2
Examining our assumptions
The overall goals of this
presentation
• To review 53 years of mistakes I personally have
made in audiological management and how I
would prevent them today.
• To review faulty assumptions I have made and
both taught and been taught.
• To share techniques to both prevent errors,
increase accuracy and validity, speed data
collection, and predict success of various
management strategies from hearing aids , to
Audiory Verbal Therapy, to more visual
strategies, etc.
To remind us that we are here to
serve patients and meet their
needs
• And to do that we have to listen to
them, with respect and dignity,
and care…deeply…very
deeply…about what happens to
them and their family-members.
The Value of “Knowing the
Results Before You Get the
Results” (Credit Edward de
Bono)
Read this word:
MOCK
Now Read It Again:
MOCKBA
Why is the Sky Dark at
Night?
A question designed to
awaken the process of rethinking a question to which
you THINK you know the
answer
How about these? In your
latitude…
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Where does the sun rise? Always?
Where does it set? Always?
Where does the moon rise?
Where does it set?
Is it always visible?
Similar questions:
• Why do we have middle ear muscle
reflexes?
• How intense is your voice at your mouth
when you say /a/?
• How intense it your voice at 6 feet?
• The vowels, the consonants?
• Are the vowels low frequency and the
consonants high frequency?
STREAM 3
• KNOWING WHEN A HEARING AID WONT WORK... Without asking
your patient to “get used to it”. Research shows that you don’t need
six weeks as an experienced adult to accommodate to a hearing aid.
If it works well, the response is almost immediate. It is the patient’s
attitude toward the aid from the beginning that should be
considered. But in this stream we will discuss PHYSIOLOGIC
predictors, not psychosocial ones.
•
•
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Reasons for referral and attitudes toward hearing aids: do they affect outcome?
Wilson C, Stephens D.
Clin Otolaryngol. 2003 Apr;28(2):81-4.
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Population study of the ability to benefit from amplification and the provision of a hearing aid in 55-74-year-old first-time
hearing aid users.
Davis A.
Int J Audiol. 2003 Jul;42 Suppl 2:2S39-52.
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Technology, expectations, and adjustment to hearing loss: predictors of hearing aid outcome.
Jerram JC, Purdy SC.
J Am Acad Audiol. 2001 Feb;12(2):64-79.
Think some more…
• Is a voluntary pure tone audiogram the gold
standard for a hearing test?
• It must be, because we used to fit a hearing aid
to it and design educational startegies around it.
• If not, why do we fit hearing aids and design
educational strategies based on pure tone
audiograms alone?
• What do I really need to know about a patient’s
auditory system to habilitate him?
The secret lies in
•The Inner Hair Cell
and
•Its connections to
and action with the
primary nerve fibers.
What Does This Have to Do With
Audiological Management and knowing
the validity of your Audiogram?
• About 10% of hearing impaired people have
poor audiograms that look manageable but
they have normal outer hair cells as
evidenced by normal otoacoustic emissions
• Normal outer hair cells, which are nature’s
low level preamplifiers, mitigate AGAINST
hearing aid success…there is no need for
amplifying faint sounds if the outer hair cells
are already doing that job.
• The INNER HAIRCELLS AND NERVE
FIBERS ARE COMPROMISED.
More…
• Putting hearing aids on someone with normal
OAEs can destroy their emissions but still
“improve their audiogram sensitivity”. It has
never been shown to help them learn language
auditorally.
• Tympanometry, reflexes and emissions should
be done on all new patients to rule out
AN/AD…if the reflexes are absent and the
emissions are present, AN/AD is highly likely
Treat the Physiology, Not the
Audiogram.
• Four cases of identical audiograms which
require differing management strategies
• The proper differentiation is acquired through
Triage with these tests. We recommend they
be done BEFORE pure tone audiometry to
speed data collection and reduce the need for
re-checking and re-testing.
• They also reduce the intrusion of technical
errors during pure tone audiometry.
The Tests for AUDIOLOGIC
TRIAGE (classifying a PATIENT’S
CONDITION and predicting its
outcome) . These are essential for
planning an educational strategy.
• 1. Tympanometry
• 2. Middle Ear Muscle reflexes
• 3. Otoacoustic Emissions
Using multiple tests is not original
with us…
• Probably the single most cited article on related
principles was published by Jerger and Hayes.
The cross-check principle in pediatric audiometry.
Jerger JF, Hayes D.
Arch Otolaryngol. 1976 Oct;102(10):614-20.
• Many other colleagues (Jay Hall, Frank Musiek,
Michael Gorga, Mead Killion, Maureen
Hannley,have made similar observations). E.g.
•
Screening for and assessment of infant hearing impairment.
Hall JW 3rd J Perinatol. 2000 Dec;20(8 Pt 2):S113-21.
Relationships among auditory brain stem responses, masking level differences and the acoustic reflex in multiple sclerosis.
Hannley M, Jerger JF, Rivera VM.Audiology. 1983;22(1):20-33.
View The Value of Triage
(Sorting by Likely Outcomes)
From Book Chapter “The
Physiological Basis of
Audiologic Practice”
Berlin, CI, Ricci and Hood (Eds) Hair Cells Micomechanics and Otoacoustic Emissions DelmarThompson 2002.
Review of underlying
principles behind the tests:
1. Middle Ear Muscle Reflexes
2. Otoacoustic Emissions
Middle Ear Muscle Reflexes
• Requires three pre-existing conditions to be
seen:
– Normal middle ear mechanics
– A “loud sound” …75-95 dB HL
– An intact reflex arc and GOOD NEURAL
SYNCHRONY
– Note that when one ear is stimulated
sufficiently, the reflex contraction of the
stapedius muscle is obtained in BOTH ears
– MOVIE OF THE REFLEX IN ACTION.
Otoacoustic Emissions
• Noises which come from the outer hair cells
of the cochlea
• To see them you must have:
– 1. Normal Middle ears (measured by
tympanometry)
– 2. Normal Cochlear Battery (Endocochlear
Potential but reflected in CM)
– 3. Normal Outer Hair Cells (reflected in
CM)
– FILM of the moving hair cells.
Basic Review of Physiologic
Principles Behind Middle Ear
Muscle Reflexes and Emissions
• The Four Electrical Events in the Cochlea
– The endocochlear potential
– The cochlear hair cell potential or cochlear
microphonic
– The compound action potential or Wave I of
the ABR
– The summating potential
• Oto-Acoustic Emissions
Charles I. Berlin, Ph.D.
Staff of the Kresge Lab
at LSUMC Dept of ORL
AN/AD audiogram samples…all patients have NO ABR,
no reflexes and normal emissions (lowest row right),.
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Reflexes?:
80
90
Emissions?:
100
ABR?:
110
Right
Left
Brothers A and B
• Tymps Normal
• Reflexes absent
• Emissions not available at the time,
absent when tested
• ABRs absent……
• Management…..
Siblings C and D
• Tympanometry normal
• Reflexes absent
• Emissions Present
• ABR…
• Management…
What does a normal temporal bone
in a premature baby look like:
• Figure 1. Normal organ of Corti from the
upper middle turn of a cochlea showing
excellent preservation. From patient 15, a
full-term baby who passed the auditory
brainstem response screening on day 6
and died on day 8. Note stereocilia of the
inner hair cell (short arrow) and outer hair
cells (long arrows) (hematoxylin-eosin,
original magnification ×250).
Normal Cochlea in a Premature
Temporal Bone of Premature, Courtesy
of Mass. Eye and Ear Published in
June 2001 Arch Otolaryngology
Temporal Bone of Premature, Courtesy
of Mass. Eye and Ear Published in
June 2001 Arch Otolaryngology
Normal nerve fiber count inside
the habenula perforata,
Temporal Bone of Premature, Courtesy
of Mass. Eye and Ear Published in
June 2001 Arch Otolaryngology
Note missing Inner Hair cell,
Normal nerve fiber count inside
the habenula perforata,
Temporal Bone of Premature, Courtesy
of Mass. Eye and Ear Published in
June 2001 Arch Otolaryngology
Note missing Inner Hair cell,
Normal nerve fiber count inside
the habenula perforata,
and normal outer hair cells which would lead
to normal emissions and NO ABR
Alternatively, Starr et al. show this
type of pathophysiology
Comparing a normal cochlea (right)
to one with Auditory Neuropathy
ABR from two of Starr’s MPZ Gene
subjects
From Starr et al. MPZ Gene paper
More from Starr paper
Patient E
• History of normal hearing until she was beaten
unconscious by an abusive boyfriend
• Claimed to be totally deaf to speech and pure tones
• Tympanometry normal
• Emissions normal
• Initially diagnosed from emissions alone as
“hysterical” or “malingering”
• Reflexes ABSENT
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30
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Reflexes?:
80
90
Emissions?:
100
ABR?:
110
Right
Left
Right Ear with 100,90
and 80 dB, opposite polarity
Clicks…all waves are CM
Cond
100 dB
Rare
Cond
90 dB
Rare
C & R 80 dB
Patient F
• Normal hearing until after dental anesthesia,
she awoke totally deaf
• She claimed to be unresponsive to speech
and pure tones and her voluntary audiograms
and speech responses confirmed her
symptoms
• Tympanometry normal
• Emissions normal
• Reflexes present
• ABR and management...
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Reflexes?:
80
90
Emissions?:
100
ABR?:
110
Right
Left
The Take-Home Message: Testing in This
Order Will Save Time Minimize the Need for
Re-Test and Help Direct the Diagnostic Tree
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Tympanometry
Middle ear muscle reflexes
Otoacoustic emissions then if adult
Speech audiometry…then either
Pure tone based tests including Stenger and
MLD if appropriate…or
• ABR related physiological tests
The Triage (Division of Possible
Outcomes) Prevents Mis-diagnosis of
Ordinary Sensorineural Loss When
the Patient Might Really Have
Auditory Neuropathy/Dys-synchrony
or You Have Some Audiological
Errors
More Clinical Examples
• Consider how you would manage these
patients if you ONLY had air-bone and
speech, the usual request from our
surgical colleagues and the maximal
allowable by hearing instrument
specialists.
• Look at these patients from a
management point of view again:
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40% 60%
Reflexes?:
Emissions?:
90
100
110
Right
Left
bone
How the Triage Works:
• If emissions were normal and
• If emissions were absent,
reflexes were absent, while
and reflexes were present
tymps are normal you would
you would have expected
have expected…Auditory
hearing aids to help and
Neuropathy, and requested an
communication to
ABR. If positive for AN, Hearing
improve; spending time
aids would not be likely to help
and money on the best
teach language by ear to a
possible aids and devices,
child, or improve
doing real ear measures,
communication in an adult.
etc., would be worthwhile.
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Disc 80% 10%
70
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Reflexes?:
90
Emissions?:
100
110
Right
Left
How the Triage Works:
• If emissions were normal
and reflexes were absent
in BOTH ears, while
tymps are normal you
would have expected
AN/AD…if absent in only
one ear, you might have
suspected a vestibular
Schwannoma. Either way
an ABR is called for, not
just an MRI.
• If emissions were absent,
and reflexes were present
you would have expected
an aidable condition.
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Note normal bone!! This
Is an example of how
audiological triage can
help minimize
other errors.
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50
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30
60
Disc 80% 76%
70
80
Tympanometry?:
Reflexes?:
Emissions?:
90
100
110
Right
Left
bone
How the Triage Works:
• If emissions were
normal and reflexes
were PRESENT, while
tymps were normal
you would NOT have
expected a conductive
loss, and should have
suspected a collapsed
canal or wax- clogged
insert phones.
• If emissions were
absent, and
reflexes were
ABSENT or
ELEVATED you
would have
expected the mild
conductive loss
you see. Also other
options.
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Lt
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60
Disc 80% NR
70
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Emissions?:
90
WITH MAXIMUM NB
MASKING IN RIGHT
100
110
Right
Left
Reflexes BOTH IPSI and
Contra??:
How the Triage Works:
• If emissions were
normal on both sides
and reflexes were
absent only when
stimulating the poor
ear, while tymps are
normal you would have
expected AN/AD, done
an ABR in both ears
and looked also for
neural problems.
• If emissions were
absent on the poor
ear only, and
reflexes were
present when
stimulating the
good ear, you
would have
expected a dead
ear and …
Key Questions About Auditory
Dys-synchrony
• What is it?
• What does it sound like and how does it differ from
regular hearing loss or Scheibe types of deafness?
• What Causes it?
• How many people are there like this?
• What will happen to my child/our patients who have
this diagnosis?
• What can I do to manage this disorder?
Interim Recommended Codes:
• Neuropathy 355.9
• Abnormal Auditory Perception:
388.4
• If present add:
–Peripheral Neuropathy: 356.9
What is it? The Combined
Observation of...
• Absent or Abnormal Auditory Brainstem
Responses WITH SALIENT Cochlear
Microphonics.
• Normal Otoacoustic Emissions…WHICH
SOMETIMES DISAPPEAR SPONTANEOUSLY
AND MAY NOT BE PRESENT AT TIME OF
TEST
• ABSENT MIDDLE EAR MUSCLE REFLEXES
• Audiograms ranging from normal to totally deaf
Normal Emissions and Absent ABRs
are a Paradox Only if You Already
Thought You Knew Why the Sky Was
Dark at Night and...
• ..were
taught or told that
ABRs and Emissions are
infallibly objective hearing
tests
• THEY ARE NOT
Neither ABR Nor Otoacoustic
Emissions Are Hearing Tests
• Let’s review the
Physiology to dissect the
physiologic basis of
audiologic practice and to
see how a “paradox” of
normal emissions and
absent ABRs occurs.
Basic Review of Physiologic
Principles
• The Four Electrical Events in the Cochlea
– The endocochlear potential
– The cochlear hair cell potential or cochlear
microphonic
– The compound action potential or Wave I of
the ABR
– The summating potential
• Oto-Acoustic Emissions
Charles I. Berlin, Ph.D.
Staff of the Kresge Lab
at LSUMC Dept of ORL
The Endocochlear Potential
Gene expressions #s
1
4
3
2
5
CM (AC) and
Summating Potential DC
Pam Beck
• 216-292-6213
• Two organizations:
• National Cued Speech Association ny
• Cued Language Network of America utah
VIDEO DEMONSTRATIONS….
PRAY
FOR COMPUTER AND PROJECTOR
COMPATIBILITY.
Important reminders
• Many AN patients have already lost their
emissions but can be diagnosed properly by the
combined use of middle ear muscle reflexes with
Cochlear Microphonics, ABR and EcochG.
• Everyone who has residual hair cell function will
have a cochlear microphonic that inverts with
polarity.
• THE PRESENCE OF THE INVERSION ALONE
DOES NOT MAKE THE DIAGNOSIS.
Normal Polarity Inversion of the
Cochlear Microphonic in a Newborn
but not opposite polarity
neural responses.
Berlin, LSU Kresge 95
A Normal ABR on the Left, a Potential
Trap or Misdiagnosis of Central Brain
Disorder on the Right
Reverse the Click Polarity and What
Looked Like an ABR is Revealed as
a Cochlear Microphonic
Reverse the Click Polarity
at Least Once IN A
SECOND COMPLETE
AVERAGE to Separate CM
from AP
Hence Our Request of Our
Colleagues to Consider ...
•
•
•
•
Tympanometry
Reflexes
Emissions on all new diagnostic patients
This yields 6 possible results, one of which raises
a high index of suspicion for AN/AuDys:
ABSENT REFLEXES AND NORMAL
EMISSIONS
• PLEASE DON’T SKIP THE REFLEXES!!
So You Can See That
Otoacoustic Emissions Only
Test Hair Cells…Not Hearing...
• …and we can test neural synchrony and inner hair cells
with ABR and related tests.
• When both tests are normal, you are likely to have normal
hearing, provided there are no upstream problems.
• If the two tests conflict, where the emissions are normal
and the Neural Response can’t be well recorded, you have
Auditory Dys-synchrony.
• If the emissions are gone, and you have large cochlear
microphonics and absent ABR you are alos likely to have
AN/AD but the reflexes should also be absent or severely
elevated.
How Does Normal Outer
Hair Cell Function Act Like
a Biological Hearing Aid?
Human Middle Ear moving 1.25 mm
In response to 120 dB 200 Hz. Stroboscopic
Illumination (courtesy Prof. Kirikae)
Hair Cell in Action from Jonathan
Lear Ashmore
Displacement of the Chinchilla Basilar
Membrane Relative to the Stapes
Adapted from Ruggero in Berlin, 1996
10000
1000
3dB
20dB
60dB
80dB
100
10
1
5k
6k
7k
8k
9k
10k
Idealized Gain Function of a Hearing Aid Which Would Do
Somewhat the Same Thing in the Intensity Domain and Whose
Compression Knee Begins at 40 dB Input
Adapted from Berlin, 1996
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40dB
60dB
90dB
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250Hz 500Hz 1000Hz 2000Hz 3000Hz 4000Hz 6000 Hz
Demonstrations of Splitting
the study of the organ of Corti
by emissions and ABR
From University of Wisconsin Web
Page
Demonstration of Polarity
Sensitive Inner Hair Cell
From The University of Wisconsin
Web Page
See Gateway.nlm.nih.gov
for access to the National Library
of Medicine
and visit our web page at
csd.bcs.usf.edu/berlin-russell
for access to some of the
patient films. Your FLASH
MEMORY will have the
animations.
Normal and Pathological Temporal
Bones and a simulation of what
speech might sound like through
their audiograms.
From University of
Wisconsin Web Page and
Berlin, C. (1996) “Hair Cells
and Hearing Aids”
Causes are Many and Might
Include...
• The inner hair cells can be absent or disabled
• Jaundice…mild …probably coupled with a gene for
hypersensitivity. The jaundice leads to a deposit, called
kernicterus, which can interfere with inner hair cell access
to the nerve fibers
• Genetic predisposition..this runs in families
• Let’s see more
The Final Common Path
Probably Involves
Failure of inner hair cells to
communicate synchronously
to primary single units of the
auditory nerve
Studying Inner vs. Outer Hair Cell
function will reveal the nature of
the hearing loss
• The pure tone audiogram
alone may often be insufficient
to reveal the function of inner
vs. outer hair cells without
reference to reflexes,
emissions and ABR.
We have all seen these cases and
often mis-diagnosed many of them
as having…
• CENTRAL AUDITORY PROCESSING
DISORDERS
• VERBAL AGNOSIA
• WORD DEAFNESS.
• We must check all of these patients for
missing reflexes and absent ABRs but
present emissions or at least large
cochlear microphonics.
• What will happen to
my child or patient
when they get this
diagnosis?
A Continuum of Auditory DysSynchrony
No overt delays
or auditory
complaints until
adulthood or
until first ABR
1
Bizarrely inconsistent auditory
responses, best in quiet, poorest
in noise. Audiograms can be
misleading or fluctuate. ABR
always desynchronized, reflexes
absent. Visual phonetic
language usually works best
until implantation, unless family
prefers cultural Deafness
5
Total Lack
of Sound
Awareness
10
How do cochlear implants help?
• Watch post-op movie.
We all have these patients…
• Find them before it’s too late to be sure by
using….
• A.
• B.
• C.
• D.
• Before behavioral audiometry which can
often be misleading or delay accurate
diagnosis or proper management.
Modify Your ABR Protocol
• Use one positive and one negative polarity
click to isolate and identify the CM
• The presence of a CM inversion alone does
not make the diagnosis. You need to show
absence of Latency-Intensity Function, and
ABSENT MIDDLE EAR MUSCLE REFLEXES
as well as normal emissions
Using one positive and one negative polarity click to
uncover AN/AuDys . Note Normal auditory function with
CM reversal and Normal Latency -Intensity Function on
the Left... Auditory Dys-synchrony with only CM reversal
and no latency-intensity function on the Right.
Normal
ONLY CM
Twin JO’s ABR at LSU in 1993
Shows All Waves Are Cochlear
Microphonics
Note
polarity
inversion
and no
latency
shift
showing
this to be
a CM
Twin JO Compared to His Brother JA
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0
10
JO’s SRT was 10 dB
20
30
JO right
JO left
JA right
JA left
40
50
60
70
80
90
100
JA’s SRT
was 55-60 dB
Patient Whose Cochlear
Microphonic Masqueraded as an
ABR and Gradually Disappeared
Type PL...Loss of CM Over
Time
3/93
95
85
4/93
85
8/94
95
85
Type M Patient Who Maintains
Emissions But Behaves Deaf
Patient FB: ABR absent at birth, Emissions not available.
Diagnosed as Deaf and reportedly raised at a school for the Deaf
without speech and with ASL only. No ABR at age 12, but a normal
emissions, immittance, pure tone audiogram and awareness of
many sounds. Limited speech, language and reading skills.
Type PR
dB Hearing Level
Frequency
0
10
20
30
40
50
60
70
80
90
100
125 250 500
100 200 300 400 600 800
0 0
0 0
0 0
FB Age 5
Right
75 85 95 95 95
FB Age 5
Left
80 90 90 95 95
FB Age 8
Right
65 65 70 65 70 75 65 60 55
FB Age 8
Left
60 60 65 70 65 60 55 50 65
FB Age 12 15 10
Right
5
5
10 15 10 15 15
FB Age 5
Right
FB Age 5
Left
FB Age 8
Right
FB Age 8
Left
FB Age 12
Right
FB Age 12
Left
Latest Data courtesy of
parents and Jack Katz
Ph.D. With the University of
Buffalo Audiology Service
Patient DV: No ABR, Normal Otoacoustic Emissions, No Middle Ear
Muscle Reflexes. Audiogram shifts but emissions remain normal and ABR
remains absent. Patient Developed Charcot-Marie Tooth Disease; sister has
normal audiogram, abnormal ABR, no efferent suppression, but no
symptoms as yet.
Frequency
LEFT
AGE 13
0
10
Type WPN
dB Hearing Level
20
RIGHT
AGE 13
30
40
50
LEFT
AGE 21
60
70
80
90
100
250
500
1000
2000
4000
8000
LEFT AGE
13
10
20
20
25
35
65
RIGHT
AGE 13
10
10
10
10
40
65
LEFT AGE
21
55
50
20
80
30
75
RIGHT
AGE 21
20
40
50
40
95
85
RIGHT
AGE 21
How Many People Are There
Like This?
Roughly 1 in 1000 are born Deaf.
At least 10-12% of the Deaf have Auditory DysSynchrony from our survey of close to 1,000
students in schools for the Deaf. Probably
there are many more because the results
undergo change over time.
After Cochlear Implantation
(45 of 50 Verified as Successful So
Far in Our Data Base as of March
2009)...
• Auditory verbal and oral therapies are ideal
• Total and sudden removal of any and all prior manual
assistance is generally counterproductive
• Children who depend upon vision for language can
benefit from interleaving of auditory with visual
support
• This is what makes Cued Speech so valuable…it
supports spoken language phonology which allows
CI users to know what it was they missed
SELF-DESCRIBED “PROFOUNDLY DEAF” FRENCH-BORN USER OF
CUED SPEECH, AS YET UNCONFIRMED AN/AD.
NOTE GOOD PROSODY AND VOICE CONTROL
FRENCH ACCENT, ETC. COURTESY JUDY CURTIN.
How to Guide Families Through
Such an Unpredictable Course?
• What is your Goal?
• Mine is to help parents raise a literate
taxpayer and position them so that whatever
choices they make for their child, they cannot
have made a serious error
• Parents are in charge and must become
expert in their child’s management
No Matter What Choices You
Make…Remember That...
• Children learn language by
eavesdropping and imitating Language
here means receptive vocabulary and
comprehension much more than
speaking
• So you must offer systems and tools
that the parents and other family
members can use easily and will
facilitate language learning
What Has NOT Worked and What
Mistakes Have I Made Since I Saw
My First AN Patient in 1982
• Hearing aids and Auditory Verbal Therapy have been
disproportionately unsuccessful with these children
ESPECIALLY IF ONE ADHERES TO THE AVT
PRINCIPLES YEAR AFTER YEAR (blocking the
face) BECAUSE THE AUDIOGRAM IS SO GOOD
AND THE TRAINING “SHOULD WORK”.
• FM systems have been at best marginal
• Trying to teach these most (BUT NOT ALL) of these
patients to talk without some form of visual language
has been singularly unsuccessful despite their
“normal hearing” by otoacoustic emissions
What Has Been Effective For the
Families With Whom I Have
Worked
• Sign language, with or without the adoption of
Deaf Culture values, is very useful but rarely
produces good spoken language.
• Sign language with speech, while difficult, will
help because of the hearing the child has and
will facilitate two-way communication
• Cued Speech has great benefits, for teaching
sounds OF ANY LANGUAGE and phonology
and will not conflict with signs.
• Auditory Verbal Therapy is ideal AFTER
implantation, but may cause serious
problems before.
For Children Who Get Implants
and Need to “Catch Up” On
English Phonology and Reading...
• The Fast ForWord™ series of
programs, designed around
Neuroscience Training Principles,
has been remarkably effective
(here and with CAPD as well)
Final Take Home Message to My
Audiology , Otolaryngology AND
EDUCATIONAL Colleagues
• Check Tympanometry, Emissions and reflexes on all
new or difficult diagnostic patients. Normal emissions
and ABSENT reflexes should be a red flag calling for
ABR regardless of the behavior.
• Emissions and reflexes which are not coherent with
audiological data require re-examination.
• During the ABR use one positive and one negative
polarity click to isolate and identify the CM.
• Parents will report “surprising hearing
moments”…honor and respect their reports and learn
from them.
• Parents “IN DENIAL” of their child’s hearing loss may
really have children with AN/AD
Acknowledgments
• Colleagues who participated in these studies
include:
• Linda Hood, PhD., Bronya Keats, PhD, Li Li,
MD, Diane Wilensky, M.S., Shanda Brashears,
MCD, Patti St. John, MCD, Liz Montgomery, MA,
Harriet Berlin , MS, Thierry Morlet, PhD, Jennifer
Jeanfreau-Taylor, MCD, Kelly Rose, MA.,, JM
Huang MD. Stefan Frisch, PhD, Jon Shallop,
PhD, Ben Russell, Michelle Arnold.
• Support from NIH, BMDR 1549, and Private
Foundations, including Marriott, Oberkotter,
Lions, LSUHSC, and others.