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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

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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 (

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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

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NST

NOT

NOR

NNG

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MIL

MAN

MAC

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ESX

ESS

ERH

ELC

ELA

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DON

DEW

CRO

CRE

COV

COL

CMP

CMH

CMA

CHS

CHE

CAL

BSS

BRA

BOL

BEH

BED

BAR

AVO

.080

.070

.060

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.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

Early Hearing Detection & Intervention (EHDI)�Status, Challenges, DirectionsProgram GoalWhat the words really meanWhy a program for early hearing? Why NEWBORN SCREENING ? Why UNIVERSAL screening?What happened before EHDI?Age at diagnosis, by severity and route to diagnosis, N=613 with HAs (Ontario)�� Durieux-Smith & Whittingham, J Sp Lang PathEHDI IS EFFECTIVE!UNHS status in CanadaEHDI SYSTEMS IN THE EUROPEAN AREAWhy is the IHP admired worldwide?Standard protocols are CRUCIALThe domino effect problemIHP screening tests & protocolScreeningVariable referral to diagnostics from 32 IHP regions in 2006Improving screeningHigh-risk surveillanceConfirmation & diagnostic testsMedical links & actionsFamily Support Hearing AidsCommunication DevelopmentOn the horizon