Early Hearing Detection & Intervention (EHDI) Status ... · EHDI SYSTEMS IN THE EUROPEAN AREA...

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Early Hearing Detection & Intervention (EHDI)Status, Challenges, Directions

Martyn Hyde PhD

Web: IHP.mtsinai.on.caEmail: mhyde@mtsinai.on.ca

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Program Goal

• Every child with significantly impaired hearing

• shall have full and equal access to

• prompt and effective services to optimize

• their development of communication skills

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What the words really mean

• Every: no child shall be missed• Full: all barriers to access minimized• Equal: no child has limited access• Prompt: as soon as the impairment occurs• Effective: evidence-based, highest quality,

appropriate to individual needs/characteristics• Development: no quick fix, complex, lengthy• Communication skills: language development,

early literacy, cognitive & social development, readiness for school…..

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Why a program for early hearing?

• Hearing underpins oral language development• Family knowledge underpins ANY language dev.• Language underpins literacy, school readiness

• Brain development underlying auditory perception & early language is EXPLOSIVE during infancyUse it or lose it (or never fully develop it…)

• Universal family right to know?• Universal child right to communicate, to hear?

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Why NEWBORN SCREENING ?

• Early enhancement of H and CD are crucial• Early enhancement requires earlier diagnosis• Earlier diagnosis requires even earlier detection

BUT

• Most hearing impairment invisible in young infants• CANNOT be reliably detected behaviourally!• Modern physiological tests can detect it at birth• Access to babies easiest during birth admission

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Why UNIVERSAL screening?

• Hearing impairment more common (~2/1000) than any other disorder for which newborns are screened

• Only about half of all newborns with impaired hearing have observable risk indicators (5-10% are at risk)

• Targeted high-risk hearing screening violates the equal access and universal rights principles

• Universal screening IS practicable and IS NOT costly, given the ensuing benefits to child, family, society

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What happened before EHDI?

• Medical referral was hopelessly ineffective

• Public & professional awareness was minimal

• AVERAGE age at detection was 2-3 years

• Many children not diagnosed until school age

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012345678

Mea

n A

ge o

f Dia

gnos

is

(yea

rs)

Screened Referred with highrisk factors

Referred with nohigh risk factors

Referral Group

mild moderate mod-severe severe profound

Age at diagnosis, by severity and route to diagnosis, N=613 with HAs (Ontario)

Durieux-Smith & Whittingham, J Sp Lang Pathol Audiol, 2000

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EHDI IS EFFECTIVE!

• Better early hearing (HAs, ADs, CIs)

• Better language development outcomes

• Better family communication strategies

• More informed family decision-making

• Medical benefits (management, etiology, syndrome detection, surveillance, genetic counselling, etc)

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Distribution of age at hearing aid fitting for hearing impaired newborns before and after NHSP introduced

Age of hearing aid fitting in weeks

0

20

40

60

80

100

0 100 200 300 400 500

Perc

entil

e of

dis

trib

utio

n fo

r aid

ed c

hild

ren

NHSP 2003/4

Before NHSP

(NHSP eSP data Feb 2005, n=228; Davis et al 1997 n=495)

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Kennedy C et al

NEJM 2006, 354;20:2131-41

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UNHS status in Canada

• NB, ON, PEI, YK implemented

• BC, NS announced 2005

• QB draft PH proposal

• AB, MN, NF, SK partial or NICU-only

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EHDI SYSTEMS IN THE EUROPEAN AREAEHDI SYSTEMS IN THE EUROPEAN AREA

IMPLEMENTED (>85%)IMPLEMENTED (>85%)

PARTIALLY IMPLEM.PARTIALLY IMPLEM.

ADV. PLANNINGADV. PLANNING

PILOTSPILOTS

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Why is the IHP admired worldwide?

• Government recognition and support• Quality and dedication of personnel

• Evidence-based, family-centered• Completeness• Centralized design, evaluation, development• Regional implementation & adaptation• Strong protocols & standardization

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Standard protocols are CRUCIAL

• Driven by evidence: only one best approach

• Every child/family entitled to standard of care

• Diverse practices undermine program evaluation and contribution to knowledge

• Deviations must be known, justified, approved and accounted for in program reporting/QM

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The domino effect problem

• Initial screening coverage 95%• Compliance to re-screening 95%• Test sensitivity 95%• Compliance to diagnostics 95%• Follow-up service uptake 90%• NET PROGRAM PERFORMANCE 73%

• Continuous quality improvement CRITICAL!

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IHP screening tests & protocol

• Automated Distortion Product OtoacousticEmissions (ADPOAE, ‘AuDX’)

• Automated Auditory Brainstem Response (AABR, ‘ABaer’)

• No risk: AuDX > ABaer > ABaer > Dx• At-risk: ABaer > Dx

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Screening

• Protocols are strong and evidence-based• Newborn coverage is very good (>98%)• Overall net refer rates to Dx are good (<2%)

BUT

• World best practice targets now 99% & <1% • High refer rate variability – why?• Follow-up compliance probably ~ 85% - why?

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Variable referral to diagnostics from 32 IHP regions in 2006

0

0.5

1

1.52

2.5

3

3.5

4

100 1000 10000 100000

number screened

Dx a

cces

sed

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No-risk DPOAE refer % by screener caseload

0%

5%

10%

15%

20%

25%

30%

35%

40%

45%

50%

0 1000 2000 3000 4000

number screened

refe

r rat

e

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WBN DPOAE % refer vs age

0

5

10

15

20

25

8 12 16 20 24 28 32 36 40 44 48 52 56 60 64

hours after birth

% r

efe

r

5

8

26

56

26

9

20

11 6

21

5

32

Mt. Sinai Hospital, TorontoNov 04 - May 05N= 3,564Refer 5.7% to AABR

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Refer rates on 1 and 2 ears as a function of site, well babiesIn hospital based screen

screening site

WIR

WIG

WAS

WAL

TRA

TGH

STO

STH

SHE

SHA

SAL

ROT

ROC

RDB

PRE

PIWOXF

NST

NOT

NOR

NNG

NEH

NCH

MIL

MAN

MAC

LEE

HILHAV

HAR

GRI

ESX

ESS

ERH

ELC

ELA

EKH

DON

DEW

CRO

CRE

COV

COL

CMP

CMH

CMA

CHS

CHE

CAL

BSS

BRA

BOL

BEH

BED

BAR

AVO

.080

.070

.060

.050

.040

.030

.020

.010

0.000

-.010

FINAL1

FINAL2

Refer rate vs site- hospital based screening- well babies

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Improving screening

• Understanding best performance & practices • Stronger quality management• Standard province-wide training• Better guidelines/support materials (web etc)• More experience sharing/problem solving

• Improving critical messaging to families:Confidence, acceptance, concern vs compliance

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High-risk surveillance

• Very comprehensive protocol

• Better definition of risk indicators & priorities• Better surveillance testing protocols• Better information about yield vs risk• Better family access & compliance strategies

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Confirmation & diagnostic tests

• Rigorous, definitive assessment protocol• Strong audiologist training & decision support

• Technical/procedural optimization• Data management & new evidence pooling• Information & messaging to families• Linkages & synergy with other service

providers, eg medical, family support, etc

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Medical links & actions

• Etiologic investigation, HA ADP process, IHP compliance promotion, new risk discovery and prompt IHP referral

• No standard medical protocol in Ontario• No standard fast-track / info exchange – ad

hoc local arrangements• Complicated interface with OHIP services• Stronger physician support & engagement

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Family Support

• Psychological support• Information about CD options and services• Empowerment of families

• Clarity & consistency of role, best practices• Better training, decision support, QM• Family needs, cultural adaptations, timely and

understandable messages, appropriate & unbiased info, linkages & synergy, consistency

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Hearing Aids

• World-leading protocols• Strong training, decision support, QM

• Limited funding• Cumbersome ADP-linked process• Better program models conceivable

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Communication Development

• Strengthened services for auditory-oral, auditory-verbal, ASL and dual programs

• Strong links with pre-school speech-language services

• Evolving synergies in Early Years Programs

• Limited evidence base for optimization, option selection, inter-option transition, maximization of family engagement and early literacy dev.

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On the horizon

• Major advances in geneticsScreening, diagnostics, prognostics, treatment

• Better cytomegalovirus (CMV) prevention

• Improvements in screening, diagnostic and assistive device technologies

• Improvements in language development procedures and strategies