[PDF] - Learning Objectives 1. I de ntify the pre va le nc e o f Co lo PDF Document

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Auditory Neuropathy/Auditory Dyssynchrony

Christine Yo shina g a -I ta no , Ph.D. Unive rsity o f Co lo ra do , Bo ulde r De pa rtme nt o f Spe e c h, L a ng ua g e & He a ring Sc ie nc e s Alb e rt Me hl, M.D. K a ise r Pe rma ne nte , Co lo ra do Cha pte r Cha mpio n Vic kie T ho mpso n, Ph.D. Unive rsity o f Co lo ra do Me dic a l Ce nte r Ma rio n Do wns Ce nte r E HDI Sta te Co o rdina to r K ristin Uhle r, Ph.D. Unive rsity o f Co lo ra do De nve r Me dic a l Ca mpus Childre n’ s Ho spita l

T

he a utho rs ha ve no c o nflic t o f inte re sts to disc lo se

Learning Objectives

1. I

de ntify the pre va le nc e o f Co lo ra do c hildre n ide ntifie d with a udito ry ne uro pa thy/ a udito ry dyssync hro ny (AN/ AD) a fte r unive rsa l ne wb o rn he a ring sc re e ning .

2. De sc rib e the c ha ra c te ristic s o f c hildre n with

AN/ AD: NI CU ve rsus we ll-b a b y, unila te ra l ve rsus b ila te ra l (N==67)

3.De sc rib e the de ve lo pme nta l o utc o me s o f a

po pula tio n o f a Co lo ra do po pula tio n o f c hildre n with b ila te ra l AN/ AD (N=37)

4. I

de ntify c ha ra c te ristic s o f c hildre n with AN/ AD a nd CI suc c e ss a nd c hildre n with AN/ AD a nd CI s who a re una b le to de ve lo p spo ke n la ng ua g e .

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2002‐2009 State of Colorado

I

nc ide nc e o f HL in 610,829 infa nts sc re e ne d fro m a b irth po pula tio n o f 626,701

T

he sc re e ning ra te wa s 97.5% a nd the fo llo w-thro ug h ra te ra ng e d fro m 83 to 89%

873 infa nts we re dia g no se d with SNHL

in this time pe rio d

67 infa nts we re dia g no se d with AN
Of c hildre n with SNHL

the inc ide nc e o f AN wa s 8.7%

Pre va le nc e o f AN wa s a b o ut 1

in 10,000

Pre va le nc e o f SNHL

wa s 1.6 pe r 1000

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Children with AN

21% we re Unila te ra l AN (14/ 67)
79% or 53 we re bila te ra l (53/ 67)

NICU (Newborn Intensive Care Unit

60 we re b o rn in the NI

CU (90%)

Most infa nts from NICU ha ve

bila te r a l AN

11 we re unila te ra l (18%)
49 we re b ila te ra l (82%)

Well baby nursery

7 we re b o rn in the we ll-

b a b y nurse ry (10%)

3 we re unila te ra l (43%)
4 we re b ila te ra l (57%)

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Developmental data about ANSD

39 o f the c hildre n in this b irth c o ho rt

ha ve de ve lo pme nta l da ta in the b irth thro ug h thre e a g e ra ng e

2 o f the 11 (18%) a re c hildre n with

unila te ra l UANSD

37 o f 53 c hildre n ha ve b ila te ra l

ANSD (70%)

Proportion of Children with significant cognitive disability (32%)

32% (12/ 37) ha ve sig nific a nt c og nitive disa bility

in a ddition to he a r ing loss

Almo st 1 in e ve ry 3 c hildre n
9 o f the se 12 c hildre n o r 75% ha ve sig nific a nt

c o g nitive a nd ne uro lo g ic a l invo lve me nt with de ve lo pme nta l q uo tie nts ra ng ing fro m 10 to 55 (9/ 37 o r 1 in 4)

3/ 4 o f the c hildre n with c o g nitive disa b ility

ha ve se ve re / pro fo und invo lve me nt a nd multiple o the r issue s

Additional disabilities including cognitive disabilities ‐ 57%

9 a dditio na l c hildre n ha d no rma l c o g nitive

de ve lo pme nt b ut o the r disa b ilitie s inc luding visio n, mo to r/ o rtho pe dic , se ve re he a lth issue s

57% (21/ 37) of the popula tion of c hildre n

with bila te ra l AN ha ve a dditiona l disa bilitie s

a nd the va st pro po rtio n o f the se issue s a re se ve re / pro fo und c o g nitive de la ys, in ma ny c a se s a lso ne uro lo g ic a l issue s.

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Normal cognitive function – 68%

25 of 37 or 68% of the c hildre n ha d c og nitive

de ve lopme nta l quotie nts within the norma l r a ng e .

9/ 37 ha d no rma l c o g nitive q uo tie nts a nd

a dditio na l disa b ilitie s (24%)

9/ 25 Nine c hildre n o f the 25 with c o g nitive

de ve lo pme nta l q uo tie nts in the no rma l ra ng e o r 36% o f c hildre n with no rma l c o g nitio n ha d a dditio na l disa b ilitie s

Language Quotients

21/ 37 c hildre n ha d la ng ua g e q uo tie nts within

the no rma l ra ng e Re c a ll tha t 25/ 37 c hildre n ha d no rma l c o g nitive q uo tie nts

57% of the c hildre n ha d la ng ua g e quotie nts

within the norma l ra ng e

Re c a ll tha t 57% o f the c hildre n ha d he a ring lo ss

plus a dditio na l disa b ilitie s

37% o f the se c hildre n with a dditio na l disa b ilitie s

ha d c o g nitive disa b ilitie s

Appro xima te ly 16% o f the c hildre n with no rma l

c o g nitive sc o re s did no t ha ve la ng ua g e q uo tie nts in the no rma l ra ng e -

Hispanic/Latino

32% (12/ 37) c hildre n ide ntify a s

Hispa nic / L a tino

83% or 10 of the se 12 c hildre n a re

Spa nish- spe a king in the home

indic a ting tha t o ne o f e ve ry fo ur c hildre n with b ila te ra l AN in the sta te

f Co lo ra do is b o rn into a fa mily tha t

do e s no t spe a k E ng lish a s a na tive la ng ua g e .

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Gender

35% a re fe ma le (13/ 37)
65% a re ma le (24/ 37)
T

he inc ide nc e o f b ila te ra l AN wa s two time s g re a te r fo r ma le s tha n fo r fe ma le s.

Cochlear Implants

4/ 37 o r 10.8% o f the c hildre n

re c e ive d a c o c hle a r impla nt/ s

5/ 37 we re no t a mplifie d – 14%
28 or 76% use d he a ring a ids

Early Intervention Services

All 37 fa milie s re c e ive d we e kly

e a rly inte rve ntio n se rvic e s fro m a n e a rly inte rve ntio n pro vide r with e ithe r a de a f e duc a tio n, spe e c h/ la ng ua g e pa tho lo g y o r a n a udio lo g y de g re e .

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Sign Language

31 o f 38 fa milie s c ho se to re c e ive

we e kly sig n la ng ua g e instruc tio n fro m a de a f o r ha rd o f he a ring na tive / flue nt sig n la ng ua g e instruc to r (82% )

1 o f the c hildre n use d Cue d

Spe e c h

PHYSIOLOGY – Gardner‐Berry, 2012

N=142 CI

s o ve r 10 ye a r pe rio d

Sydne y Co c hle a r I

mpla nt Ce nte r,

16% ha d a bnorma litie s on the ir CT

sc a ns, whic h

inc lude d Mo ndini de fo rmitie s, wide inte rna l a udito ry me a tus, dyspla stic a pic a l turn, a nd a b no rma l ve stib ule a nd la te ra l se mi-c irc ula r c a na ls.

20% of the c hildre n with bila te ra l AN a nd 6% of the

c hildr e n with unila te r a l AN ha d c ompr

mise d

a uditory ne rve s.

A tota l of 43% of the c hildr

e n ha d disa bilitie s in a ddition to HL .

The Infant Monitor of vocal Production (IMP IMP)

Ro b yn Ca ntle Mo o re , PhD RI DBC Re nwic k Ce ntre / Unive rsity o f Ne wc a stle Sydne y, Austra lia .

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IMP IMP Précis

Crite rio n re fe re nc e d instrume nt
Clinic a l e va lua tio n & pa re nt e duc a tio n to o l
Suc c e ssive me a sure o f infa nt, pre -ling uistic

vo c a l c o mpe te nc e

Dia g no stic a id to ha b ilita tio n pro g ra mming

Typical infant vocal development

(Kuhl, 2004; Lewkowicz & Hansen‐Tift, 2012 *

Perception Production

1 2 3 4 5 6 7 8 9 10 11 12 MONTHS Universal speech production Language specific speech production First words Non-speech sounds Vowel-like sounds ‘Canonical babbling’ Universal speech perception Language specific speech perception Phonetic contrasts all languages Language specific vowels Language specific sound combinations Native language consonants

* Visual attention shift to mouth seeks

redundant audio-visual speech cues Social attention shift back to eyes Visual-social attention to eyes

Stages of Infant Vocal Production

Adapted from: Oller (2000) Nathani, Ertmer, & Stark (2006)

Vowel-like sounds Non-speech sounds ‘Canonical babbling’ Language specific speech production First words

Kuhl (2004)

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IMP IMP probe‐questions

Interpreting the IMP

IMP

TYPICAL

IMP IMP : infant (ANSD) Case Study 102

Innate Transition to A-P Integrity of A-P Loop Loop

“Lily””

11.5

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IMP IMP : infant (ANSD) Case Study 102 IMP IMP : infant (ANSD) Case Study 36

Innate Transition to A-P Integrity of A-P Loop Loop

“Mary””

9

IMP IMP : infant (ANSD)

Case Study 36

“Mary” 9

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IMP IMP : infant (ANSD) Case Study 35

Innate Transition to A-P Integrity of A-P Loop Loop

“Ian” 1 7

19 14 19 14

IMP IMP : infant (ANSD) Case Study 35

“Ian” 1 7

IMP IMP : infant (ANSD) Case Study 135

Innate Transition to A-P Integrity of A-P Loop Loop

“Cameron” 1 1

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IMP IMP : infant (ANSD)

Case Study 135

“Cameron” 1 1

IMP IMP : difference in progress

(ANSD)

(Kuhl, 2004).

1 2 3 4 5 6 7 8 9 10 11 12 Language specific speech production First words Non-speech sounds Vowel-like sounds ‘Canonical babbling’ MONTHS Language specific speech perception Language specific vowels Language specific sound combinations Native language consonants

MARY 6mth HAge LILY 8mth HAge IAN 14mth HAge Perception Production CAMERON 5mth Hage / CI

Re gistration

T his tra ining is fre e o f c ha rg e , ho we ve r you are

re quire d to re g iste rb e fo re yo u c a n a c c e ss the

tra ining mo dule s. We a lso a sk tha t yo u sha re da ta c o lle c te d using the I MP via the o nline fo rm, a va ila b le in the tra ining we b site . Go to http:/ / www.r

idbc r e nwic kc e ntre .c om/ imp

to re g iste r. Onc e re g iste re d, yo u will re c e ive a n e ma il c o nta ining yo ur lo g in c re de ntia ls.

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Contact

Robyn Ca ntle Moore , PhD

RI DBC Re nwic k Ce ntre Unive rsity o f Ne wc a stle , NSW

robyn.moore @ridbc .org .a u

RIDBC Re nwic k Ce ntr e for Re se a r c h a nd Pr

fe ssiona l

Educ a tion Administe re d b y the Ro ya l Institute fo r De a f a nd Blind Childre n in a ffilia tio n with T he Unive rsity o f Ne wc a stle , Austra lia

Treatment options

De ve lo ping An Ac tio n Pla n

The importance of effective Counseling

Ne e d to he lp pa re nt o ve rc o me fe e ling s o f

he lple ssne ss a nd c o nfusio n due to unc e rta intie s:

With unkno wns, o utc o me s, tre a tme nt pla ns a nd va ria b illitie s F inding c o mfo rt in ma king c ho ic e s tha t ma y c ha ng e

Wo rk with pa re nts to de ve lo p a n a c tio n pla n
Ga the r da ta / a udio lo g ic & de ve lo pme nta l
Ne e d to e sta b lish a n e ffe c tive te a m o f

pro fe ssio na ls

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Amplification considerations

No a mplific a tio n
He a ring Aids
Co c hle a r I

mpla nta tio n

Co nside r c o g nitive c o mpe te nc y o f the

c hild to use the a udito ry pe rc e ptua l info rma tio n a nd a udito ry a c c e ss pro vide d b y the c o c hle a r impla nt.

Co nside r the visua l c o mmunic a tio n

de ve lo pme nt o f the c hild

Co nside r the a udito ry skill de ve lo pme nt o f

the c hild

Communication approaches/opportunities: Visual

Sig n la ng ua g e – visua l c o nc e ptua l

c o mmunic a tio n

Re q uire s visua l a b ility (e .g . c o rtic a l b lindne ss
r se nso ry b lindne ss)
Re q uire s mo to r a b ility (e .g . se ve re c e re b ra l

pa lsy)

Re q uire s visua l ling uistic a b ility (e .g . a utism

a nd diffic ulty with visua l a tte ntio n, visua l inte rpre ta tio n, visua l symb o lic g e sture o r pla y) – sig ns a re c o nc e ptua l

Communication

pportunities/approaches: visual
Cue d Spe e c h – visua l c o de fo r spe e c h re a ding
Re q uire s visua l a b ility (e .g . b lindne ss, o the r visua l

impa irme nt)

Re q uire s mo to r a b ility (e .g . c e re b ra l pa lsy o r o the r

sig nific a nt g ro ss mo to r disa b ility)

Re q uire s visua l ling uisitic a b ility (e .g . visua l

a tte ntio n, visua l inte g ra tio n o f c ue a nd spe e c h re a ding )

Cue s re q uire inte g ra tio n o f spe e c h re a ding c ue s

a nd spe e c h/ mo to r

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Communication Approaches/opportunities: Auditory/Oral

Audito ry/ Ora l- spo ke n la ng ua g e c o mmunic a tio n
Re q uire s a udito ry a c c e ss (c o nsiste nt a nd sta b le

a c c e ss-fluc tua tio n o f thre sho lds, g o o d da ys/ b a d da ys)

Re q uire s a udito ry a tte ntio n (a b ility to a tte nd

a udito ra lly)

Re q uire s a udito ry inte g ra tio n (inte g ra tio n o f so unds

to ve rb a l wo rds- me a ning )

Ca n b e suc c e ssful with he a ring a ids, witho ut HAs,

with re so lve d AN o r with c o c hle a r impla nts

Developing a Plan

Amplific a tio n
F

unc tio na l Audito ry Skill Se t

Co mmunic a tio n
L

a ng ua g e

Spe e c h
Co g nitio n

Key to a successful plan is ongoing assessment and flexibility

Consiste nt T

e am Communic ation

Par

e nt and Child Ce nte r e d Cle ar Obje c tive s

Conne c tions to R

e sour c e s

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Assessment And Adaptations

Ob ta in b a se line da ta
Re te st to me a sure ra te o f

pro g re ss

Go a l: Atte mpt to ma inta in

de ve lo pme nt c o mme nsura te with c o g nitive a g e – a dditio na l disa b ilitie s c o mplic a te s de ve lo pme nta l pro g re ss Functional Auditory Skill Development

Clo se ly o b se rve a nd mo nito r liste ning skills in

a va rie ty o f c o nditio ns. Cha ng e s ma y b e no te d:

q uie t vs. no ise
music a nd sing ing
time o f da y
we e kly a nd e ve n mo nthly
a ide d a nd una ide d
L
o k fo r c o nsiste nc y a nd q ua lity o f

re spo nse s

Audito ry b e ha vio rs ma y no t b e hie ra rc hic a l

pre -impla nta tio n fo r AD c hildre n

Auditory Skill Development

Mo nito r with tria l a mplific a tio n
Awa re ne ss vs. spe e c h disc rimina tio n
Co rtic a l a udito ry e vo ke d po te ntia ls sho w

pro mising use fo r fitting o f a mplific a tio n with AN

With a c o c hle a r impla nt, e xpe c t

hie ra rc hic a l a udito ry skill de ve lo pme nt

Allo w time fo r spo nta ne o us re c o ve ry
Mo nito r de ve lo pme nt o f spe e c h & la ng ua g e
I

de ntify a udito ry disc rimina tio n skills vs. pure to ne he a ring le ve ls

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Tools to Measure Functional Auditory Skill Development

DASL

, De ve lo pme nta l Appro a c h to Suc c e ssful L iste ning

Audito ry Skills Che c klist
I

nfa nt Mo nito r o f Vo c a l Pro duc tio n

CASL

L S Co tta g e Ac q uisitio n Sc a le s fo r L iste ning , L a ng ua g e a nd Spe e c h- So unds a nd Spe e c h

L

ittle E a rs Audito ry Que stio nna ire

Che c klist o f Audito ry Co mmunic a tio n Skills

Creating a Functional Developmental Profile

Asse ss a t re g ula r inte rva ls – e ve ry 3-6

mo nths

E

xpe c t de ve lo pme nta l g a ins a t a ra te tha t is c o mme nsura te with tha t c hild’s c o g nitive skills – with the e xc e ptio n o f multiply disa b le d c hildre n who ma y ha ve e xtre me diffic ulty le a rning la ng ua g e a t a no rma l ra te

Re vie w da ta o n ANAD/ ANSD - %

c o g nitive de la y, % o the r disa b ilitie s

Types of assessment

Pa re nt/ c a re g ive r re po rt – pa re nt

q ue stio nna ire s

Dire c t o b se rva tio n o f the c hild
Ob se rva tio n o f c hild’s inte ra c tio n

with a pa re nt

Vide o ta pe d inte ra c tio n
Clinic ia n-a dministe re d a sse ssme nts
Multi-disc iplina ry – a ll de ve lo pme nta l

do ma ins

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Carlos

We ll – b a b y nurse ry
Pa re nts – b o th ha d a c o lle g e de g re e
Se ve re he a ring lo ss b ila te ra lly – pre -impla nt
Spa nish-spe a king ho me
F

irst c o c hle a r impla nt – a b o ut 20 mo nths

Se c o nd c o c hle a r impla nt – 3 ye a rs, 11

mo nths

Case 6 =CARLOS

ANSD
NRT

a b se nt with first CI

Slo w pro g re ss in spo ke n a nd visua l la ng ua g e

syste ms

P1s pre se nt prio r to impla nting se c o nd side
One ye a r la te r due to po o r o utc o me s, P1s we re

a b se nt whe n re pe a te d

Se c o nd CI

a c tiva te d 12/ 2010

NRT

pre se nt

Ma king sig nific a nt g a ins in visua l

c o mmunic a tio n

Re spo nding to hig h fre q ue nc y so unds
Minima l a udito ry spo ke n la ng ua g e pro g re ss

Carlos – LENA data – quality of his spoken language daily diet

Adult wo rd c o unt – 23,990 95th%ile
Co nve rsa tio na l T

urns – 674 72nd%ile

Child Vo c a liza tio ns -

2312 57th%ile

***AVA Sta nda rd Sc o re – 73.9 4th%ile
Hig h q ua ntity o f vo c a liza tio ns b ut the y we re

no t spe e c h-like vo c a liza tio ns – no me a ning ful spo ke n la ng ua g e

He use d his vo c a liza tio ns fo r c o nve rsa tio na l

turn-ta king .

Vo c a l pro duc tio ns we re no t de ve lo ping

no rma lly

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IS SOUND REACHING THE CORTEX – CORTICAL Auditory Evoked Potentials – P1s

Ca rlo s ha d P1 te sting – c o rtic ia l inde x o f

a udib ility

T

ime 1 – P1 wa s within no rma l limits with CI

Ho we ve r, b e ha vio ra lly, Ca rlo s wa s

re spo nding inc o nsiste ntly to so und

T

ime 2 – P1 wa s a b se nt – with CI

T

ime 3 – P1 wa s pre se nt, the n, a b se nt, the n, pre se nt – fluc tua te d within se ssio n

Eddie

Ag e o f I

D: 18 mo .

Rig ht UE

c o ng e nita l a mputa tio n

L

e ve l E d: so me c o lle g e

NR ABR – Unkno wn de g re e – initia l

a udio

CI

: 20 mo nths o f a g e

Se c o nd CI

23 mo nths o f a g e

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Eddie – AT AGE LEVEL STANDARDIZED TESTS

CE

L F P2, Clinic a l E va lua tio n o f L a ng ua g e F unda me nta ls

Se nte nc e Struc ture Sc a le d Sc o re : 10
Wo rd Struc ture Sc a le d Sc o re : 9
E

xpre ssive Vo c a b ula ry Sc a le d Sc o re : 10

Co re L

a ng ua g e Sta nda rd Sc o re : 98

Pe rc e ntile : 45%
I

nte llig ible spe a ke r

Ma instre a m suc c e ss – a t a g e le ve l
Witho ut a dditio na l se rvic e s
Audito ry dyssync hro ny – CI

wo rke d we ll fo r a udito ry a c c e ss

What do we know?

Ne uro pa thy vs. Dys-sync hro ny
if AN – ne ura l site o f le sio n wo uld indic a te tha t CI

is no t b e ne fic ia l

I

f AD – the n CI sho uld b e b e ne fic ia l

Curre ntly the re is no de finitive te st to diffe re ntia te

the se two c a te g o rie s

Curre nt a sse ssme nt pro c e dure s c ha ra c te rize

a udito ry skill de ve lo pme nt –

Co urse o f the c o nditio n is unpre dic ta b le
a re the y pro g re ssing with HAs o r witho ut,
is the re fluc tua tio n – g o o d he a ring da ys a nd b a d

he a ring da ys, g o o d he a ring time s-b a d he a ring time s

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Auditory Neuropathy/Auditory Dyssynchrony

Co mple x c hildre n with hig hly individual

c ha ra c te ristic s

Ma ny c hildre n ha ve c o mplic a te d b irth

histo rie s

Mo st o f the se c hildre n ha ve a dditio na l

disa b ilitie s

I

t is po ssib le to pla n inte rve ntio n fo r the se c hildre n so tha t the y a re a b le to de ve lo p la ng ua g e skills c o mme nsura te with the ir c o g nitive a b ilitie s