NEONATAL INFANT HEARING SCREENING IN SOUTH AFRICA

1
NEONATAL / INFANT HEARING SCREENING IN SOUTH
AFRICA
the dogmas of the quiet past are inadequate to
the stormy present As our case is new, so we
must think anew, and act anew (Abraham Lincoln,
1846)
DEPARTMENT OF COMMUNICATION PATHOLOGY

• De Wet Swanepoel
• dswanepoel_at_postino.up.ac.za

2
OUTLINE

• Importance of early identification
• Definition history of screening
• UNHS in developed contexts
• UNHS in South Africa

3
WHY EARLY IDENTIFICATION?
4
BASIC ASSUMPTION That early detection followed by
early intervention maximize the benefits the
child, the family, and society will receive
(Diefendorf, 2002469)
3 FACTS The expenditure of financial,
technological, and human resources involved in
the implementation of UNHS is justified by 3
facts emerging from research (White, 2002)
5
Why is Early Identification of Hearing Loss so
Important?

• Hearing loss is the most frequent birth defect.

6
Rate Per 1000 of Permanent Childhood Hearing Loss
in UNHS Programs

• Sample Prevalence
• Site Size Per 1000
• Rhode Island (3/93 - 6/94) 16,395 1.71
• Colorado (1/92 - 12/96) 41,976 2.56
• New York (1/95 - 12/97) 69,761 1.95
• Texas (1/94 - 6/97) 52,508 2.15
• Hawaii (1/96 - 12/96) 9,605 4.15
• New Jersey (1/93 - 12/95) 15,749 3.30

7
Incidence per 10,000 of Congenital
Defects/Diseases
8
Why is Early Identification of Hearing Loss so
Important?

• Hearing loss is the most frequent birth defect.
• Undetected hearing loss has serious negative
  consequences.

9
Reading Comprehension Scores of Hearing and Deaf
Students
Grade Equivalents
Age in Years
Schildroth, A. N., Karchmer, M. A. (1986). Deaf
children in America, San Diego College Hill
Press.
10
Effects of Unilateral Hearing Loss
Normal Hearing
Unilateral Hearing Loss
Math
Keller Bundy (1980)
(n 26 age 12 yrs)
Language
Math
Peterson (1981)
(n 48 age 7.5 yrs)
Language
Social
Bess Thorpe (1984)
(n 50 age 10 yrs)
Math
Blair, Peterson Viehweg (1985)
Language
(n 16 age 7.5 yrs)
Math
Culbertson Gilbert (1986)
Language
(n 50 age 10 yrs)
Social
Average Results
0th
10th
20th
30th
40th
50th
60th
Math 30th percentile
Percentile Rank
Language 25th percentile
Social 32nd percentile
11
Effects of Mild Fluctuating Conductive Hearing
Loss
Teele, et al., 1990
194 children followed prospectively from 0-7
years.
)
Days child had otitis media between 0-3 years
assessed during normal visits to physician.
)
Data on intellectual ability, school achievement,
and language competency individually
)
measured at 7 years by "blind" diagnosticians.
Results for children with less than 30 days OME
were compared to children with more than
)
130 days adjusted for confounding variables.

Effect Size for
Outcome Measure
Less vs. More OME
WISC-R Full Scale
.62
Metropolitan Achievement Test
Math
.48
Reading
.37
Goldman Fristoe Articulation
.43
Teele, D.W., Klein, J.O., Chase, C., Menyuk, P.,
Rosner, B.A., and the Greater Boston Otitis media
Study Group (1990).
Otitis media in infancy and intellectual
ability, school achievement, speech, and language
at age 7 years.
The Journal


of Infectious Diseases
,
162
, 685-694.
12
Why is Early Identification of Hearing Loss so
Important?

• Hearing loss is the most frequent birth defect.
• Undetected hearing loss has serious negative
  consequences.
• There are dramatic benefits associated with early
  identification of hearing loss.

13
Yoshinaga-Itano, et al., 1996
Compared language abilities of hearing-impaired
children identified
6
before 6 months of age (n 46) with similar
children identified after 6
months of age (n 63).
All children had bilateral hearing loss ranging
from mild to profound,
6
and normally-hearing parents.
Language abilities measured by parent report
using the Minnesota
6
Child Development Inventory (expressive and
comprehension scales)
and the MacArthur Communicative Developmental
Inventories
(vocabulary).
Cross-sectional assessment with children
categorized in 4 different
6
age groups.
Yoshinaga-Itano, C., Sedey, A., Apuzzo, M.,
Carey, A., Day, D., Coulter, D. (July 1996).
The effect of early


identification on the development of deaf and
hard-of-hearing infants and toddlers
. Paper presented at the
Joint Committee on Infant Hearing Meeting,
Austin, TX.
14
Expressive Language Scores for Hearing Impaired
Children Identified Before and After 6 Months of
Age
35
30
25
20
Language Age in Months
15
10
Identified BEFORE 6 Months
5
Identified AFTER 6 Months
0
13-18 mos
19-24 mos
25-30 mos
31-36 mos
(n 15/8)
(n 12/16)
(n 11/20)
(n 8/19)
Chronological Age in Months
15
Vocabulary Size for Hearing Impaired Children
Identified Before and After 6 Months of Age
300
250
200
Vocabulary Size
150
100
Identified BEFORE 6 Months
50
Identified AFTER 6 Months
0
13-18 mos
19-24 mos
25-30 mos
31-36 mos
(n 15/8)
(n 12/16)
(n 11/20)
(n 8/19)
Chronological Age in Months
16
Boys Town National Research Hospital Study of
Earlier vs. Later
129 deaf and hard-of-hearing children assessed 2x
each year.
)
Assessments done by trained diagnostician as
normal part of early intervention program.
)
6
Identified lt6 mos (n 25)
5
Identified gt6 mos (n 104)
4
3
Language Age (yrs)
2
1
0
0.8
1.2
1.8
2.2
2.8
3.2
3.8
4.2
4.8
Age (yrs)
Moeller, M.P. (2000).
17
Why is Early Identification of Hearing Loss so
Important?

• Hearing loss is the most frequent birth defect.
• Undetected hearing loss has serious negative
  consequences.
• There are dramatic benefits associated with early
  identification of hearing loss.

18
DEFINITION HISTORY
GENERAL DEFINITION OF SCREENING Process of
filtering cases into two groups. The first group
has an adequately high probability of having a
given disease or condition to warrant referral
for further testing. The second group has an
adequately low probability of having the disease
or disorder and therefore does not merit the
expense, inconvenience or risk of diagnostic
testing (Lutman, 2000367)
GOAL OF A SCREENING PROGRAM To identify
asymptomatic individuals with an increased
likelihood of having the target disorder, so that
diagnostic-testing procedures can be applied only
to that subset of individuals (Roush, 200133).
19
DEFINITION HISTORY

• 1893 Sachs APR
• 1940s Ewings Behavioural responses
• 1940s 1950s - Various techniques (e.g. teacup
  spoon, paper crumpling and cowbells! Frodings
  gong 133 dB SPL APR in 96 of 2000 babies!)
• 1950s Wedenberg Modern-day Father of newborn
  screening
• 1950s 60s Hardy group List of etiological
  factors for SNHL
• 1960s 70s Downs APRITON

20
MOTHER OF NHS
21
DEFINITION HISTORY

• 1972 US JCIH High Risk Register
• 1970s Crib-o-gram, Auditory Response Cradle
• 1980s ABR
• Late 1980s OAE
• 1994 JCIH 1993 NIHCS 1998 ECS
• 2000 JCIH

22
UNHS IN DEVELOPED CONTEXTS

• USA UK
• Austria
• Poland
• Flemish Belgium
• Singapore
• Canada (Ontario)

JCIH Year 2000 Position Statement
23
JCIH 2000
Endorses the early detection of, and
intervention for infants with hearing loss (early
hearing detection and intervention, EHDI) through
integrated, interdisciplinary state and national
systems of universal newborn hearing screening,
evaluation, and family-centered intervention
(JCIH, 200010) The aim of endorsing these
services is to ensure the maximum linguistic and
communicative competence and literacy development
for children who are deaf or hard of hearing
(JCIH, 200010)
24
JCIH 2000
8 PRINCIPLES Components, Benchmarks and Quality
indicators

• Hearing screening
• Confirmation of hearing loss referred from UNHS
• Early intervention
• Continued surveillance of infants and toddlers
• 5 6. Protection of infants and families
  rights
• 7 8. Information Infrastructure

25

• HEARING SCREENING

• COMPONENTS
• PERSONNEL
• PROGRAM PROTOCOL DEVELOPMENT
• SCREENING TECHNOLOGIES
• SCREENING PROTOCOLS

26
OAE vs AABR

• DIFFERENCES
• TIME OAE generally faster but Gabbard et al.
  (1999) showed similar times at approx. 12 min.
  ND (2002286) 10 to 20 min for AABR
• COST-EFFICIENCY Equipment similar but AABR
  more disposables
• AUDITORY NEUROPATHY OAE will most likely miss AN
  OAE does not test neural components
• CONDUCTIVE LOSSES AABR less sensitive
• YOUNGER NEONATES Less likely to pass OAE than
  AABR

COMBINED OAE AND AABR UNITS AVAILABLE NOW
27
SCREENING PROTOCOL
PROTOCOL SPECIAL CONSIDERATION FOR AN 42 of
AN cases are genetic 10-20 are
toxic-metabolic, particularly
hyperbilirubinemia         Reports of AN even
with low bili levels when compounded by
low-birth weight, prematurity etc. Colorado UNHS
program indicates that all AN cases were from
the NICU THEREFORE Use risk factors to improve
identification of neural hearing loss and screen
at risk (NICU) infants with AABR preferably
28
2. CONFIRMATION OF HL

• COMPONENTS
• AUDIOLOGICAL EVALUATION
• MEDICAL EVALUATION

29
3. EARLY INTERVENTION

• COMPONENTS
• EARLY INTERVENTION PROGRAM DEVELOPMENT
• AUDIOLOGIC HABILITATION
• MEDICAL AND SURGICAL INTERVENTION
• COMMUNICATION ASSESSMENT AND INTERVENTION

30
4. CONTINUED SURVEILLANCE

• COMPONENTS
• RISK INDICATORS FOR NEONATES (BIRTH THROUGH AGE
  28 DAYS)
• RISK INDICATORS FOR NEONATES OR INFANTS (29 DAYS
  THROUGH 2 YEARS)

31
HIGH RISK REGISTER (HRR)
RISK INDICATORS FOR USE IN NEONATES WHERE UNHS IS
NOT YET AVAILABLE (Targeted screening)
32
HIGH RISK REGISTER (HRR)
Only identifies 50 of the paediatric population
with congenital hearing impairment (Northern
Downs, 2002275) 9 of newborns have one or more
risk-factor (Mahoney Eichwald, 1987161) NB for
Audiologist to have an in-depth understanding of
risk factors Used for

• Targeted NHS programs
• Monitor for progressive HL
• Assist in interpreting developmental history of
  children for evaluation (Northern Downs,
  2002275)

33
5 6 PROTECTION OF INFANTS AND FAMILIES
RIGHTS
34
7 8 INFORMATION INFRASTRUCTURE
35
EXPANSION OF UNHS

• JCIH goal reaching developing contexts
• Downs (2000) - The western world will soon see
  most newborns enrolled by hearing screening
  programs and these developed countries must now
  extend their expertise to developing countries

36
UNHS IN SOUTH AFRICA
South African Position Statement (2002)
South African context characteristics
Audiological services in SA
37
SA POSITION STATEMENT
The SA position statement advocates early
detection of infants with hearing impairment
followed by early intervention provided by
integrated interdisciplinary Provincial and
District Health Systems (DHS) health care
services (HSPS, 20021). The rationale is to
ensure optimum, cost effective solutions for
individuals identified with hearing loss to
enable persons to communicate effectively,
thereby allowing maximum habilitation or
rehabilitation of the individuals capabilities
and potential, to secure their full participation
in, and contribution to, society and the
countrys economy (HSPS, 20021).
38
SA POSITION STATEMENT
GUIDELINES SUMMARIZED IN 4 GOALS

• Screening for hearing loss should identify
  infants at risk for hearing loss that impacts on
  development.
• The types of hearing loss targeted by these
  programs are uni- or bilateral, conductive or
  sensori-neural, and greater than 30 dB in the
  speech frequencies (0.5 4 kHz).
• All infants should receive ongoing monitoring of
  the development of auditory behaviour and
  communication skills, and other sensory and motor
  milestones, through developmental screening
  programs at Primary Health Care clinics.
• Quantifiable goals and quality indicators must be
  determined for the monitoring and evaluation of
  EHDI programs with periodic reviews to assure the
  quality of the programmes.

39
SA POSITION STATEMENT
1. ACCESS TO HEARING SCREENING

• SCREENING OF AT-RISK NEONATES / INFANTS
• OBJECTIVE TESTS ONLY (OAE AABR)
• SCREENING PERSONNEL NURSES LAY VOLUNTEERS
• AUDIOLOGISTS SUPERVISE PROGRAMME MANAGER
• ENSURE ALL SOCIO-ECONOMIC LEVELS OF SOCIETY HAVE
  ACCESS TO SCREENING AND BENEFITS OF EI
• HEARING SCREENING IN WELL-BABY NURSERY AT
  DISCHARGE FROM NICU 6-WEEK IMMUNIZATION CLINICS
• 2005 SCREENING TECHNOLOGY AT MCH CLINICS
• 2010 98 OF NEONATES/INFANTS SCREENED FOR HL

40
SA POSITION STATEMENT
1. ACCESS TO HEARING SCREENING

• INFANTS NOT SCREENED MUST BE SCREENED AT MCH
  CLINICS
• HL DIAGNOSIS MUST TAKE PLACE BEFORE 3 MONTHS OF
  AGE
• PARENT EDUCATION REGARDING COMMUNICATION OPTIONS
  BY AGE 3-MONTHS MULTIPROFESSIONAL TEAM
  FRAMEWORK
• REFERRAL AND INTERVENTION BEFORE 6 MONTHS
• ONGOING AUDIOLOGICAL AND MEDICAL MONITORING FOR
  AT-RISK INFANTS FOR 3 YEARS
• HRR AND BOA NOT RECOMMENDED AS STAND-ALONE
  SCREENING METHODS

41
SA POSITION STATEMENT
1. ACCESS TO HEARING SCREENING

• NOISE-EMITTING DEVICES (e.g. RATTLES WHISTLES)
  NOT ENDORSED AND SHOULD BE DISCONTINUED
• COMMUNITY BASED DEVELOPMENTAL SCREENING
  INCORPORATING COMMUNICATION MILESTONES AT PRIMARY
  CARE LEVEL WITHIN DISTRICT HEALTH SERVICES
• SUPPORTING INFRASTRUCTURE AND SERVICES AT
  REGIONAL LEVELS FOR DIAGNOSTIC AUDIOLOGICAL
  ASSESSMENTS AND INTERVENTIONS

42
SA POSITION STATEMENT
2. CONFIRMATION OF HEARING LOSS

• INFANTS WHO FAIL SCREENING MUST BE REFERRED FOR
  COMPREHENSIVE AUDIOLOGIC ASSESSMENTS AND
  SPECIALIST MEDICAL EVALUATIONS
• BENCHMARKS AND QUALITY INDICATORS NEED TO BE
  DEVELOPED

43
SA POSITION STATEMENT
3. EARLY INTERVENTION

• GOAL FACILITATION OF DEVELOPMENTALLY
  APPROPRIATE LANGUAGE SKILLS EMPOWERMENT OF
  FAMILIES TO ASSUME THE PRIME ROLE IN HABILITATION
• PRINCIPLES FROM INTEGRATED NATIONAL DISABILITY
  STRATEGY, NATIONAL REHABILITATION POLICY,
  DEAFSAS EI POLICY AND RELEVANT INCLUSIVE
  EDUCATION POLICIES ARE ENDORSED
• THESE ARE

• Early intervention should be family oriented
• Assistive device technology should be readily and
  immediately accessible
• Medical and surgical intervention for otitis
  media and cochlear implantation should be
  available
• Primary focus of early intervention should be
  development of a communication mode suited for
  the individual

44
SA POSITION STATEMENT
3. EARLY INTERVENTION

• FREE HEALTH CARE FOR CHILDREN UNDER SIX YEARS
  OLD MUST ALSO INCLUDE REHABILITATION AND
  PROVISION OF ASSISTIVE DEVICES
• WITHOUT PROMPT INTERVENTION NEONATAL HEARING
  SCREENING IS UNETHICAL AND SHOULD NOT BE
  UNDERTAKEN

45
SA POSITION STATEMENT
4. CONTINUED SURVEILLANCE

• CONTINUED SURVEILLANCE OF INFANTS AND CHILDREN
  UNDER 6 YEARS
• RISK INDICATORS MUST BE WIDELY PUBLICISED,
  ESPECIALLY IN PHC CONTEXT
• IT IS EQUALLY IMPORTANT TO MONITOR PROGRESSIVE
  AND DELAYED ONSET HEARING LOSS

46
SA POSITION STATEMENT
5 - 6. INFANTS FAMILIES RIGHTS

• RIGHT TO CHOOSE PREFERRED COMMUNICATION MODE
  PROTECTED BY SA CONSTITUTION
• VARIOUS CHARTERS BILLS PROTECT THE RIGHTS OF
  PERSONS WITH DISABILITIES
• CLIENT AND PARENT AUTONOMY SHOULD BE RESPECTED
  AT ALL TIMES

47
SA POSITION STATEMENT
7 - 8. INFORMATION INFRASTRUCTURE

• NATIONAL DATABASE STANDARDIZED METHODOLOGY,
  REPORTING PROGRAMME EVALUATION
• EACH PROVINCE REPORT NO. OF LIVE BIRTHS, NO. OF
  NEWBORNS SCREENED
• NB INFO TO COLLATE

• No. of birthing hospitals in each province
• No. of live births in each province
• No. of infants screened for hearing loss before
  discharge
• No. of infants referred for audiologic evaluation
  before one month of age
• No. of infants whose hearing has been evaluated
  before 3 months
• No. of infants with permanent congenital hearing
  loss
• Mean, median, and minimum age of diagnosis
• No. of infants with permanent hearing loss
  receiving intervention by 6 months

48
SA POSITION STATEMENT
7 - 8. INFORMATION INFRASTRUCTURE

• DEVELOP MECHANISMS FOR FURTHER ID FOLLOW-UP
  FOR INFANTS WHO ARE MISSED
• ONGOING MONITORING OF HR INFANTS IDENTIFY HLs
  ASSOCIATED WITH MENINGITIS AND TB
• THIS DATA WILL PROVIDE ESTIMATIONS OF HL
  PREVALENCE PER PROVINCE AND REGION WILL PROVIDE
  NATIONAL BENCHMARKS AND QUALITY INDICATORS

49
SA CONTEXT
CHALLENGES

• 3rd World Additional barriers
• Low socio-economic levels
• Paucity of accessible health care
• Inadequate resources
• Ignorance and the absence of regular screening
  programs for ear disease

50
SA CONTEXT
SOME SPECIFIC CHALLENGES

• GENERAL POPULATION CHARACTERISTICS
• ECONOMIC INFRASTRUCTURE
• LEVEL OF EDUCATION IN SA
• CHILDREN IN SOUTH AFRICA
• HIV/AIDS AND CHILDREN

51
SA CONTEXT
CHILDREN IN SOUTH AFRICA

• Six out of every 10 children live in poverty,
  mostly in rural areas
• The Infant Mortality Rate in 1998 was 45.4 per
  1000 live births. For Africans it was 47,
  Coloureds 18.8 and whites 11.4
• The Under-Five Mortality Rate was 59.4 per 1000.
  For Africans it was 63.6, Coloureds 28.2 and
  whites 15.3
• 63 of children were fully immunized and only
  2.2 had no immunizations
• Nearly a quarter of children under 5 years old
  are stunted, and 1 in 10 is underweight for
  his/her age

52
SA CONTEXT
CHILDREN IN SOUTH AFRICA

• One third of children under 5 years have a
  Vitamin A deficiency and 1 in 10 is anaemic
• 21 of children under five die from diarrhoea,
  and 10 from acute respiratory infections
• The pass rate for the Grade 12 examinations in
  2000 was 57.9
• At least half a million children have moderate to
  severe disabilities and need access to specialist
  services
• 42 of children under 7 years of age live only
  with their mother and 20 do not live with either
  parent

53
SA CONTEXT
CHILDREN HIV / AIDS

• 1 in 9 South Africans are infected with HIV 11
  of total population
• In 1998, 22.8 of women attending antenatal
  clinics in public health facilities were HIV
  positive. The rate of increase among teenagers 15
  19 years was 65.4 from the previous year. At
  the end of 2002 an estimated HIV prevalence rate
  of 26.5 amongst sexually active women aged from
  15 to 49 was reported.
• Approximately one third of children born to
  HIV-positive mothers are infected and an
  estimated one in seven will acquire it through
  breast-feeding. Most of these children develop
  AIDS and die within a few years of birth.
• Projections for the year 2005 suggests that there
  will be 1 million orphaned children and by 2010
  there will be 2 million orphaned due to HIV/AIDS

54
SA CONTEXT
CHILDREN HIV / AIDS

• Infants living with HIV/Aids are also susceptible
  to other infections and neurological
  complications that can compromise auditory
  function and may impact auditory development.
• Infants born to HIV positive mothers are at risk
  for a congenital hearing loss or for developing a
  hearing impairment shortly after birth Prone to
  MEE
• Ototoxic medications taken prenatally for
  treatment of HIV related diseases may cross the
  placenta and damage the fetal ear structure
  development
• Increase in infants and young children living
  with HIV will therefore also have a profound
  affect on the prevalence of hearing disorders
  across the population
• Causing a significant impact on the health care
  system priorities.

55
AUDIOLOGY IN SA
CRITICAL EVALUATION OF SERVICES

• RESEARCH
• PREVENTION
• SCREENING
• EVALUATION
• INTERVENTION
• SCHOOLS

56
AUDIOLOGY IN SA
RESEARCH

• EXTREME DEARTH OF LOCAL RESEARCH
• IMPORTANCE RATIONALE
• RECENT FUTURE STUDIES
• Incidence of HL in VLBW infants in Kalafong
  Hospital
• MCH clinics in Hammanskraal
• NICU infants in Kalafong Hospital

57
CONCLUSIONS
2 RECENT ASSETS IN IMPLEMENTING WIDESPREAD
SCREENING IN SA

• SA Hearing Screening Position Statement (2002)
• Community service year for all audiology graduate
  students

58
CONCLUSION
100 years later

• UTILIZE INTERNATIONAL KNOWLEDGEBASE AND APPLY
  WITHIN THE CONTEXT OF SOUTH AFRICA
• INITIATE COMPREHENSIVE CONTEXTUAL RESEARCH
• A PROACTIVE POSITION IN DEVELOPMENT OF THE
  PROFESSION SPECIFICALLY THE FUNCTION OF
  PROVIDING SERVICES TO THE YOUNGEST AND MOST
  FRAGILE POPULATION ACROSS ALL SOUTH AFRICAN
  PEOPLES

59
A sense of urgency, and the feeling of
irreplaceable time being squandered have to
permeate all our efforts with young children
(Ross, 199069)