1
Early Hearing Detection & Intervention (EHDI)Status, Challenges, Directions
Martyn Hyde PhD
Web: IHP.mtsinai.on.caEmail: [email protected]
2
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
3
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…..
4
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?
5
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
6
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
7
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
8
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
9
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)
10
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)
11
Kennedy C et al
NEJM 2006, 354;20:2131-41
12
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
13
14
EHDI SYSTEMS IN THE EUROPEAN AREAEHDI SYSTEMS IN THE EUROPEAN AREA
IMPLEMENTED (>85%)IMPLEMENTED (>85%)
PARTIALLY IMPLEM.PARTIALLY IMPLEM.
ADV. PLANNINGADV. PLANNING
PILOTSPILOTS
15
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
16
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
17
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!
18
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
19
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?
20
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
21
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
22
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
23
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
24
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
25
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
26
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
27
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
28
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
29
Hearing Aids
• World-leading protocols• Strong training, decision support, QM
• Limited funding• Cumbersome ADP-linked process• Better program models conceivable
30
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.
31
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