Universal vs high risk screening

Neonatal Hearing Screening-
Universal vs High Risk Screening-
Experience in HK
Dr. Barbara CC Lam
Consultant
Honorary Clinical Associate Professor
Department of Paediatrics and
Adolescent Medicine
Queen Mary Hospital
8 October 2005

Introduction
• Severe congenital hearing impairment
(HI) is an important handicap affecting
1-3 per 1,000 live birth
• The prevalence of moderate to profound
hearing loss was 2 - 4 % of NICU infants
• Infants with risk factors accounts for less
than ~ 50% of cases
• ~ 165 infants per year are born with
significant HI in HK per year

Hearing is an important sensory input
• Auditory stimulus perceives in the first
few months of life forms the basis of
speech, linguistics and cognitive
development
• The language ability, the social,
emotional, comprehensive and motor
development of HI are adversely affected

Early Intervention Improves Outcome
• Yoshinaga-Itano showed age of the diagnosis of
HI was the only significant variable to affect
the language skill of HI child.
• Several prospective studies showed that the
prognosis for intellectual, language and speech
development in the HI child can be improved
significantly when the diagnosis is made early
and intervention begins before 6 month of age
• The first year of life, especially the first 6
months is most critical
Robinshaw 1995 Apuzzo & Yoshinaga-Itano 1995
Moeller 1996 & Yoshinaga-Itano 1998

Past Situation in Hong Kong
• Since 1978, Maternal and Child Health Centre
(MCHC) provide ‘universal’ hearing screening
using Behavioural Distraction Test for infants
around 6-9 months
• Most HA birthing hospitals perform hearing
test (BAEP) for high risk infants
• The usual age at diagnosis of hearing
impairment especially for those without risk
factors is usually at 2 year and most infants
receive treatment and education after 2 years

Recent Change in Hong Kong
• Most realized the importance of early diagnosis
and early intervention
• Some HA birthing hospitals pilot UNHS
• Maternal and Child Health Centre (MCHC)
replace the distraction test by Infant Hearing
screening ( AOAE) for babies registered before 2
months through the early infant hearing &
surveillance programme since Aug 2003
• There is a lack of a coordinated territory wide
policy on hearing screening and the lack of
territory wide registry with tracking and
monitoring system

What is the most appropriate
and cost effective newborn
hearing screening programme
for Hong Kong ?

Newborn Hearing Screening
Strategies
• High risk screening
• Universal screening

High Risk Indicators
America Academy of Paediatrics Joint
Committee on Infants Hearing 1990
1. Family history of HI
2. Congenital infection
3. Craniofacial anomalies
4. Low Birth weight (1500 grams or less)
5. Severe neonatal jaundice

High Risk Indicators
6. Ototoxic medications in toxic range
7. Bacterial meningitis
8. Severe depression at birth
9. Mechanical ventilation for or > 5 days
10. Syndrome known to include
sensorineural and/or conductive
hearing loss

High Risk Screening
• ~ 5 - 10% of all babies born will exhibit
one or more of these indicators
• 2.5 - 10% of these infants confirmed to
have permanent congenital hearing
impairment (PCHI)
• At least 50% of infants with PCHI do not
have any of the risk factors

High Risk Multicentre Hearing
Screening 1 year Project in HK
1999-2000
• Multicentre project involving 5 HA hospitals
• Two stage distortion products OAE
• Conventional auditory brainstem response for
failed screening and babies with neurological
abnormalities
Chan KY et al
(Sponsored by The Save Children Fund(HK)

Protocol of High Risk Infant
Screening Project
High Risk Infants
OAE (14-28 days)
Family history, asphyxia,
meningitis, NNJ, congenital
infection, PPHN etc
N
Abn
Repeat OAE (

Summary of High Risk Infant
Live Birth 19,922
Screening Result
Risk Factor 546 ( 2.7%)
OAE 533 ( 97%)
2nd OAE 70
failed
ABR 46 ( 8.6%)
failed
ENT 30
*Hearing Loss 22 ( 4%)
Default 13 (2.4%)
CNS risk Additional
ABR 210 ( 39%)
Pass OAE but failed
ABR - 13
ENT 13
Default 54
* Hearing Loss 2 ( 0.3 %)

Distribution of Risk Factors

Multicentre High Risk Infants
Hearing Screening Project
1. 1.2 per thousand (24 out of 19,922 LB) have
HI - high risk approach detect less than 50%
of cases
2. 2.4% of at risk infants have moderate to
profound HI
3. HI infants were identified before age 4-6
months and all of them had received
appropriate intervention before age of 9
months

Multicentre High Risk Infants
Hearing Screening Project
4. OAE may miss some case with CNS defects
5. No significant HI was detected at age 9
months in those infants who passed
newborn screening
6. Default rate for OAE is 2.4%, for ABR for
those with CNS risk factors is 26% -
screening best to be performed before
hospital discharge & coupled with a robust
tracking system

Criteria for Public Health
Universal Screening:-
1. Easy-to-use screen tests - high sensitivity to
minimize unnecessary referrals
2. The condition being screened for is not
otherwise detectable by clinical means
3. Interventions are available to correct the
condition once detected
4. Early screening, detection, and intervention
result in improved outcomes
5. The screening program is documented to be
in an acceptable cost-effective range

Universal Newborn Hearing Screening
• Congenital Hearing Loss fulfills the
established Criteria for Universal
Screening
• A universal newborn hearing screening
with a coverage rate of more than 95% is
a more vigorous approach to achieve the
aim of identifying most, if not all babies
with congenital hearing loss at an early
age to enable timely habilitation

Universal Newborn Hearing Screening
• Hospital based vs Community based?
• Which screening tools ?
– AABR ? OAE ? OAE and AABR

UNHS Projects in Hong Kong
I. First Pilot hospital based UNHS - 2 stage
OAE ( TYH)- 1999
II. Multicentre hospital based UNHS -
Comparing different screening devices and
protocols - 2001
III. Pilot study of Community based UNHS by 2
stage OAE in 4 MCHC -2001
IV.
Regional UNHS -2 stage AABR and cost
effectiveness study – 2003-2005

Pilot UNHS- 2 stage OAE in TYH
OAE Screen OAE Screen OAE Screen
Day 1-4 Day 5-14 Day 21-30
Infants 806 913 872
Screened
Failure 59% 21% 3.9%
Rate
PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

Pilot UNHS- 2 stage OAE in TYH
• Bilateral hearing loss is 0.28
• High parental acceptance of UNHS – Coverage
rate is 99.3%
• Unacceptably high false positive rate ( 20%) in
first 2 week due to ear debris
• Refer rate for diagnostic audiological test is 3.5%
PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

Questionnaires study on Parental
Acceptance of Newborn Hearing Screening
• 91% consider newborn hearing screening
desirable
• 82% favor pre-discharge screening
• 56% do not have a sound knowledge on
hearing developmental milestone, undesirable
to rely on parental self surveillance to detect HI
in their babies
PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

Hospital based Multi-centre UNHS –
Screening Device and Protocol
• 3 different screening protocols
– 2-stage AABR (Algo model 2e Color
Newborn Screener)
– 2-stage AABR (Biologic 2 in 1 screener)
– 2 stage OAE-AABR (Biologic 2 in 1 screener)
• Pre-discharge screening

Hospital based UNHS- Comparisons
for 3 Different Screening Protocols
BWY Young, BCC Lam CM Wong et al 2002
AABR- OAE- ABear-
AABR(Algo) ABear ABear( Bio)
Failed first Test 5.3% 29.1% 15.1%
Final Refer Rate 0.6% 5.0% 3.8%
Average Screening 11 11 17.5
Time (min.)
Disturbance score 1.2±1.7 0 ±0.7 1.5 ±1.8

Hospital-based Multicentre UNHS
BWY Young, BCC Lam CM Wong et al 2002
• High coverage rate of 95% - UNHS is feasible
before discharge from birthing hospital
• The 2 stage AABR screening yielded the lowest
refer rate - 0.6%
• The time spent in the 3 screening methods are
comparable
• OAE screening cause the least disturbance to
the babies, followed by AABR

Pilot Study of Community Based
UNHS in MCHC
KY Chan, SSL leung HKJ Paedatr 2004
• 4 MCHC in August 2000 to July 2001
• Infants with first MCH attendance at 2
weeks to 2 months
• 2 Stage OAE, re-screened 2 weeks later if
failed the first screening

Pilot Study of Community based
UNHS in MCH
• Uptake rate 77% of attendees at MCH
• Overall coverage rate not known
• Failure rate: 2nd screen 3.8%
• Out of 3,949 screened, 4 cases of Bilateral HI -
1 per 1000 babies
• Mean age of HI identification 85 days (vs 9-12
month for distraction test)
KY Chan, SSL leung HKJ Paedatr 2004

QMH & PYNEH
Universal Newborn Hearing
Screening
A regional 2-stage AABR and cost
effective analysis

UNHS Screening Protocol - 2-stage AABR – AABR
All babies born in QMH
& PYNEH
No
Distribute information sheet
when baby is stable and >35 weeks
Stable
and >35
weeks
Yes
Distribute information sheet
on the first day of life
Pass
1st AABR
screening
Fail
Fail
1st AABR
same / next day
after newborn
examination
Pass
2nd AABR
Screening on the
same / next day
Pass
Discharge and encourage to
attend MCHC for on going
surveillance
Fail

UNHS Screening protocol - 2-stage AABR – AABR
Counseling by Paediatrician
Refer to audiologist for diagnostic BAER
Paediatric follow-up appointment 4 weeks later
Diagnostic
BAER
Fail
Pass
Follow-up once
by paediatrician
Follow-up by
paediatrician
Follow-up by
ENT Surgeon
Refer to Special
Education Unit for
early habilitation

Can be used in Nursery
Environment ( 5-20 min)

UNHS- QMH & PYNEH
2 stage AABR- 2003-2005
Total birth 14,604
Babies screened 14,560
Coverage rate 99.7%
Failed 2nd screen 1.5%
Confirmed HI 76 (0.5%)
Bilateral HI 36
Unilateral HI 40

UNHS- QMH & PYNEH
2 stage AABR- 2003-2005
Time Median
1st screening 22 hrs
2nd screening
41 hrs
Diagnostic BAER
43 days
ENT
66 days
Special ed.
158 days
Hearing aid
198 days

Recommended Parameters
for Effective UNHS
Parameters Study AAP
High Coverage Rate 99.7% ≥ 95%
Good Sensitivity (RR) 1.5% ≤ 4%
Zero false negative √
Bilateral HL ≥ 35dB √
Before discharge √
√
√
√

Cost
No. Per baby Cost Average
cost/birth
Equipment 2 109,161 18
Staff hours 5,098 20 min. 130-105/hr 40
Consumable 3,028 0.25 75 12
$
Total 70

UNHS: Cost Analysis
• Capital cost of equipment
• Manpower & consumable cost
• Cost for confirmatory test
• Life long quality of life
• Differences in life-time learning
capabilities
• Education cost

Long term Cost analysis
Yea
r
Capital cost
$
Annual
Recurrent
cost
$
Additional
Education cost
(EETC)
$
Cumulative
cost
$
Cumulative
Education
Savings
$
Cost
(-spending/
+saving)
$
1 1,228,500 5,440,758 2,581,508 9,250,766 / -9,250,766
2 / 5,440,758 7,744,523 22,436,047 / -22,436,047
3 / 5,440,758 7,744,523 35,621,328 / -35,621,328
4 / 5,440,758 7,744,523 48,806,609 / -48,806,609
5 / 5,440,758 7,744,523 61,991,890 / -61,991,890
6 / 5,440,758 7,744,523 75,177,171 8,199,565 -66,977,606
7 / 5,440,758 7,744,523 88,362,452 24,598,695 -63,763,757
8 / 5,440,758 7,744,523 101,547,733 49,197,390 -52,350,343
9 / 5,440,758 7,744,523 114,733,014 81,995,650 -32,737,364
10 / 5,440,758 7,744,523 127,918,295 122,993,475 -4,924,820
11 / 5,440,758 7,744,523 141,103,576 172,190,865 +31,087,289

Cost Effectiveness
• Long term cost analysis showed that
the cost of UNHS could be offset by
savings from reduced burden on
special education
• Positive gain at 10-11 years after
implementation.

Hospital vs Community Based
- Coverage Rate
Hospital based:
TYH 2 stage OAE
98.9% LB
QMH PYNEH 2 stage AABR 99.7%
MCH based:
Distraction test
60% at 9M
2 Stage OAE 72% of all
attendees

Hospital vs Community Based
High Sensitivity
• Recommended parameter - Refer rate for
diagnostic test Target < 4%
• Refer rate for diagnostic test
– Hospital based AABR program 1.5%
– MCH based OAE program 3.8%

Hospital vs Community Based -
Early Referral
• Recommended Parameter - Target 100%
before 3 months or shortly after birth
• Mean age of referral for diagnostic test
– Hospital based AABR program - 41hours
– MCHC based OAE program - 54 days

Hospital vs Community Based -
Parental Acceptance
• Screening test is completed before
discharge
• Child back to China
• High parental satisfaction
• Less anxiety due to lower and earlier
referral for diagnostic test

Hospital VS Community Based
Cost Analysis
Hospital based AABR MCH based OAE
8 centres ($M) 44 centres ($M)
Capital cost 1.2 3.2
of equipment
Annual labour cost 2.38 2.83
Annual consumables 2.5 0.8
Cost of diagnostic test 0.5 12
Cost per deaf child 0.33 0.95
identified

Available Supporting Facilities!
• Hospital based UNHS
– Available specialist support and
expertise including audiologist,
paediatrician and ENT surgeon for
counseling and further audiological
evaluation

Universal Hospital Based Predischarge
Newborn Hearing should be Introduced
• Efficacy of a screening program
Maximal coverage
Good sensitivity (low refer rate)
High specificity (low false negative rate)
High patient acceptance
Cost effective

The Way Forward -
Collaborative model
Combine the specific competencies of the 2
involved parties

Role of Community Centre
• Establish and maintain a central registry
and monitoring system
• Establish and maintain a tracking
program that monitor all referrals and
miss
• To provide mop up service for out-ofhospital
births
• Ongoing surveillance for late onset
hearing impairments

Summary
• Our UNHS studies demonstrated
comparable incidence of HI among
local infants at 3 to 5 per thousand
• A hospital based UNHS ensure high
coverage and early detection
• 2 stage AABR achieve a low refer
rate , high parental acceptance at an
acceptable costs

Summary
• Newborn hearing screening program
– Good screening method
– Tracking and follow up system
– Early identification and intervention
– Ongoing evaluation for late onset HI
• A territory wide hospital based UNHS
should be implemented to enable early
detection and intervention leading to
improved language and learning outcome
in HI children

Acknowledgement
• HA High Risk Infants
Hearing Screening Program
– CB Chow
– KY Chan
– B CC Lam
– W Wong
– TF Fok
– CW Law
– WH Lee
– KC Wong
– KN Yuen
– CC Shek
– E Wong
• Multicentre Universal
Newborn Hearing Project
– B Young
– BCC Lam
– Y Hui
– E Wong
– W Yeung
– T Wong
– M Tong
– CB Chow
– W Wong
– P Chan

Acknowledgement
• TYH pilot universal
newborn hearing
screening
– P K Ng
– Barbara Lam
– C Y Yeung
– Winnie Goh
– Yau Hui
• QMH & PYNEH
Regional UNHS
– BWY Young
– BCC Lam
– W Yeung
– Y Hui
– D Au

Thank You !

Risk Indicates for Infants with Late
Onset/Progressive Hearing Loss
• Neonatal risk factors including hyperbilirubinemia
requiring exchange transfusion
• Syndromes associated with hearing loss such as
Usher’s syndrome
• Neurodegenerative disorders such as Friedreich’s
ataxia
• Head trauma
• Recurrent persistent otitis media with effusion for
more than three months

Risk Indicates for Infants with Late
Onset/Progressive Hearing Loss
• Caregiver concern about hearing, speech or
language and/or developmental delay
• Family history of permanent hearing loss
• Stigmata associated with permanent hearing loss
• Postnatal infections associated with hearing loss,
such as bacterial meningitis
• In-utero infections including cytomegalovirus,
herpes, rubella, syphilis, and toxoplasmosis

Recommended Parameters of an Effective
UNHS Program
- JCIH 2000 Position Statement
1. Screening
2. Tracking and Follow Up
3. Identification and Intervention
4. Evaluation

Current Recommendation on
Hearing Loss
1. Universal detection of hearing loss in infants
before 3 months
2. Appropriate intervention no later than 6
month
3. Targeted at permanent bilateral or unilateral,
sensory or conductive hearing loss, average
30-40 dB or more in the frequency region
important for speech recognition

Current Recommendation on
Hearing Loss
4. Infants who pass NHS, but have risk
indicators for auditory disorders should
receive ongoing audiologic and medical
surveillance and monitoring for 3 years
5. Infants and family rights are
guaranteed through informed choice,
decision marking and consent

JCIH Recommends Benchmarks for
Screening, Identification and Intervention
• Within 6 months of initiation of a screening
program must screen a minimum of 95% of
infants during birth admission or before 1 month
of age
• Referral rate for audiologic and medical
evaluation after screening should be 4% or less
within 1 year of the programme
• Document efforts to follow up on 95% of infants
who do not pass the screen and actual FU of 70%
or more of infants

More Cost Effective !
Cost Analysis- Assumptions
• Annual livebirths in HK 55,000
• Annual livebirth in HA hospitals = 40,000
• Private hospitals provide their own UNHS service
• Incidence of PCHI = 3 per 1,000 livebirths
• Annual PCHI cases = 165
• No. of MCHC providing hearing screening service
= 42 out of the 50 MCHCs
• No. of birthing hospitals in HK = 9
• Gazette cost of ABR = $1,600

Recommended Parameters of an Effective UNHS
Program - JCIH 2000 Position Statement
1) Screening
– Testing both ears, with a minimum of 95%
coverage rate
– Able to detect significant bilateral HL of ≥ 35
dB in the better ear
– Low false-positive rate of ≤ 3% and low
referral rate for further assessment of < 4%
– Ideally should have false-negative rate of zero

Recommended Parameters of an Effective UNHS
Program - JCIH 2000 Position Statement
1) Screening
– Screening methods should be noninvasive,
quick and easy to perform
– Screening should be conducted before
discharge from the hospital whenever is
possible
– Each birthing hospital should have an
established UNHS program

Recommended Parameters of an Effective UNHS
Program - JCIH 2000 Position Statement
2) Tracking and Follow Up
– Aim at 100% follow-up with a minimum of 95%
successful follow-up
– Establish and maintain a central monitoring
system for all hearing screening programs
– Establish and maintain a tracking program that
monitors all referrals and misses

Recommended Parameters of an Effective UNHS
Program - JCIH 2000 Position Statement
3) Identification and Intervention
– Aim at 100% of infants with significant
congenital hearing loss being identified by 3
months of age and having appropriate and
necessary intervention initiated by 6 months of
age
– The Paediatrician should coordinate and ensure
access for all affected children to appropriate
expert services

Recommended Parameters of an Effective UNHS
Program - JCIH 2000 Position Statement
4) Evaluation – ongoing and regular by central
monitoring and tracking
– The performance of UNHS programs
– Tracking and follow-up system
– Intervention services to ensure that sufficient
expert services are available for affected children
and the outcomes from interventions provided
are effective

Distribution of Risk Factors for
Moderate – Severe Hl
Risk factors Moderate Severe Profound Auditory
Hearing loss Hearing loss Hearing loss neuropathy
Family history of deafness 1 (HA)
Ear malformation 1 1
Cleft palate 1
Multiple craniofacial abn 1
Low birth weight 3 (2HA) 1
Severe asphyxia
1 (HA)
Down syndrome 1 1
CHARGE association
1 (HA)
syndrome
Total 13, HA - Hearing aid (5)

Comparison of Language Development
for the Screen and Non-screen HL
Children
Semin Neonatol Yoshinaga-Itano 2001
Screen
Non-screen
Identification by 6 month 84% 8%
Language development 56% 24%
Normal - LQ ≥ 80
No. of types of consonant 4.5 0
(50th %)
Total no. of words (50th%) 95.5 14.5
No. of different words 30 7

Newborn Hearing Screening Milestones
1989 Project to assess the feasibility of
screening infants in nurseries for
HL (TEOAE & ABR) in the U.S.A.
1992 Consensus conference on NHS
recommended that every newborn
be screened in the 1st month of life
1993 Rhode Island was the first state to
achieve universal newborn hearing
screening

Newborn Hearing Screening Milestones
1999 APA published its policy
statement ” newborn and infant hearing
loss- detection and intervention”
1999 First pilot study of UNHS in TYH
maternity hospital in HK
2000 JCIH position statements expand the
concept to early detection and
Interventions through integrated
interdisciplinary systems of UNHS,
evaluation and family centered
interventions

Newborn Hearing Screening milestone
2003 U.S.A. 37 states have
legislation mandating
UNHS and all states
are developing hearing
screening systems
2002 Published studies of UNHS
in Taiwan, Japan and China

Cost-effectiveness and Test-performance
Factors in Relation to UNHS
M. Gorga Research Review 2003
• The costs of UNHS exceeds the benefits at
the initiation of programme
• After 4 years of operation, UNHS program
will result in a net savings to society
• The saving increase rapidly, reaching a
maximum annual benefit of 7 billion dollars
75 years after initiation