1. 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

2. 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 % 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

3. 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

4. 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 Apuzzo & Yoshinaga-Itano 1995
Moeller 1996 & Yoshinaga-Itano 1998

5. 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

6. 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

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

8. Newborn Hearing Screening Strategies
High risk screening
Universal screening

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

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

11. High Risk Screening
~ % of all babies born will exhibit one or more of these indicators
% 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

12. 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)

13. Protocol of High Risk Infant Screening Project
OAE (14-28 days)
Repeat OAE (<2 wks)
MCHC Distraction test at 6-9 months
ABR
ENT & Audiologist
ED for intervention
Family history, asphyxia,
meningitis, NNJ, congenital
infection, PPHN etc N
Abn

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

15. Distribution of Risk Factors

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

17. Multicentre High Risk Infants Hearing Screening Project
OAE may miss some case with CNS defects
No significant HI was detected at age 9 months in those infants who passed newborn screening
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

18. Criteria for Public Health Universal Screening:-
Easy-to-use screen tests - high sensitivity to minimize unnecessary referrals
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

19. 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

20. Universal Newborn Hearing Screening
Hospital based vs Community based?
Which screening tools ?
AABR ? OAE ? OAE and AABR

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

22. Pilot UNHS- 2 stage OAE in TYH
OAE Screen OAE Screen OAE Screen
Day Day Day 21-30
Infants
Screened
Failure % % %
Rate
PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

23. 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

24. 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

25. 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

26. 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 % % %
Final Refer Rate % % %
Average Screening
Time (min.)
Disturbance score   1.8

27. 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 %
The time spent in the 3 screening methods are comparable
OAE screening cause the least disturbance to the babies, followed by AABR

28. 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

29. 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 month for distraction test)
KY Chan, SSL leung HKJ Paedatr 2004

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

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

32. 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
Follow-up once by paediatrician
Follow-up by paediatrician
Follow-up by ENT Surgeon
Refer to Special Education Unit for early habilitation
Pass
Fail

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

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

36. UNHS- QMH & PYNEH 2 stage AABR- 2003-2005
Time Median
1st screening hrs
2nd screening hrs
Diagnostic BAER days
ENT days
Special ed days
Hearing aid days

37. Recommended Parameters for Effective UNHS
Parameters Study AAP
High Coverage Rate %  95%
Good Sensitivity (RR) %  4%
Zero false negative  
Bilateral HL  35dB  
Before discharge  

38. Cost No. Per baby Cost Average cost/birth $ Equipment 2 109,161 18
Staff hours 5, min /hr
Consumable 3,
Total

39. 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

40. Long term Cost analysis

41. 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 years after implementation.

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

43. 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%

44. 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

45. 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

46. Hospital VS Community Based Cost Analysis
Hospital based AABR MCH based OAE
8 centres ($M) 44 centres ($M)
Capital cost
of equipment
Annual labour cost
Annual consumables
Cost of diagnostic test
Cost per deaf child
identified

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

48. 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

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

50. 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-of- hospital births
Ongoing surveillance for late onset hearing impairments

51. 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

52. 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

53. 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

54. 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

55. Thank You !

56. 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

57. 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

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

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

60. 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

61. 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

62. 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

63. 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

64. 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

65. 2) Tracking and Follow Up
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

66. 3) Identification and Intervention
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

67. 4) Evaluation – ongoing and regular by central monitoring and tracking
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

68. 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 (HA)
Ear malformation
Cleft palate
Multiple craniofacial abn 1
Low birth weight (2HA)
Severe asphyxia (HA)
Down syndrome
CHARGE association (HA)
syndrome
Total 13, HA - Hearing aid (5)

69. 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% %
Language development 56% %
Normal - LQ  80
No. of types of consonant
(50th %)
Total no. of words (50th%)
No. of different words

70. 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

71. Newborn Hearing Screening Milestones
APA published its policy statement ” newborn and infant hearing loss- detection and intervention”
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

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

73. Cost-effectiveness and Test-performance Factors in Relation to UNHS M
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