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Semiramis Zizlavsky, MD ORL-HNS,PhD Faculty of Medicine, Universitas Indonesia, Jakarta

  1. : Medical Doctor , Faculty of Medicine Universitas Indonesia, Jakarta

1991 : ENT Specialist , Faculty of Medicine ,Universitas Indonesia, Jakarta

2013 : PhD Program, Faculty of Medicine, Universitas Indonesia, Jakarta

2002 : Work in Cipto Mangunkusumo Hospital, Jakarta

Lecturer at Faculry of Medicine, Universitas Indonesia, Jakarta

Author / co Author in both Indonesian and international journals.

Interest in hearing and communication disorders in children

Currently also on the cochlear implant team

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Situation of Newborn Hearing Screening in Jakarta

Semiramis Zizlavsky, Ronny Suwento

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1 to 1.5 per 1000 live births

Severe or profound permanent bilateral hearing loss (PBHL)

serious condition in newborns

Background

1 to 2 per 1000 newborns

bilateral mild to moderate hearing loss, or unilateral hearing loss of any degree

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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hearing loss

sensorineural

conductive

mixed

most common form of hearing loss in the neonates 🡪 50% genetic cause

Lawrensia S, Gomez Pomar E. Newborn Hearing Screening. [Updated 2023 Jul 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560930/

Background

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Lawrensia S, Gomez Pomar E. Newborn Hearing Screening. [Updated 2023 Jul 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560930/

Hearing Impairment

impact on

  • child development (language or speech) development
  • poor academic performance
  • personal-social maladjustments
  • emotional disturbances

Prompt detection and appropriate intervention within the first six months of age

Background

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What is newborn hearing screening ?

​

Newborn hearing screening……..

programs are designed to identify hearing loss in infants shortly after birth

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History of Newborn Hearing Screening

  • 1960 : Froeding ( auropalpebral reflex)
  • 1965 : Marion Downs (Startle response)
  • 1970 : Invention of ABR
  • 1978 : Invention of OAE
  • 1983 : UNHS program by ABR (USA)
  • 1986 : Invention of AABR
  • 2015 : OAE & AABR in Cipto Mangunkusumo Hospital

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Joint Committee on Infant Hearing [JCIH], 2019) :

  • NHS is the standard of care in hospitals nationwide.
  • Primary purpose of NHS : to identify newborns who are likely to have hearing loss and who require further evaluation.
  • Secondary objective : to identify newborns with medical conditions that can cause late-onset hearing loss and establish a plan for continued monitoring of their hearing status

Benchmarks recommended by NHS programs :

  • Hearing screening completion by 1 month of age,
  • Audiological diagnosis of any hearing loss by 3 months of age,
  • HA selection & fitting within 1 month of confirmation of hearing loss
  • Early intervention services by 6 months of age.

INTRODUCTION

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Screening�

Quick

Cheap

Not Invasive

Accurate, reproducible

​

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Principles of Screening�

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Benefits of Newborn Screening

​

    • IDENTIFICATION

​

    • EARLY INTERVENTION
    • REDUCED MORBIDITY
    • FAMILY PLANNING

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Risk of Newborn Hearing Screening�

  • Parent anxiety (false positive )
  • Missed diagnosis ( false negative)
  • Unanticipated outcome

- range of reactions

- maternal feelings of guilt

- financial problem

- cultural sensitivity

​

​

​

​

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Hearing Screening

Prerequisites

    • Background noise of screening room should be as minimal as possible.
    • Baby should be well fed, not hungry and crying
    • Baby should be quiet and still. Ideal 🡪 sleeping
    • Having the baby swaddled during testing will help to keep the body movements

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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Mechanical ventilation for 5 days

Ototoxic medications

Premature birth

Low birth weight

Admission to NICU >7 days

Low APGAR score

Lawrensia S, Gomez Pomar E. Newborn Hearing Screening. [Updated 2023 Jul 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560930/

Risk Factors Newborn Hearing Loss

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Causes of Permanent Bilateral Hearing Loss

Intrauterine infections TORCH (Toxoplasma, rubella, cytomegalovirus, herpes)

congenital infections

genetic abnormalities

craniofacial problems

50% of newborns with PBHL have no identifiable risk factor

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

Etiology

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Intervention before the age of 6 months

better school outcomes, improved language and communication skills by 2–5 years

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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Hearing Screening

Targetted

congenital permanent bilateral, unilateral sensory, or permanent conductive hearing loss

Universal Hearing Screening

include neural hearing loss (e.g., “auditory neuropathy/dyssynchrony”)

in infants admitted to the neonatal intensive care unit (NICU)

  1. Joint Committee on Infant Hearing. (2007). Year 2007 position statement: Principles and guidelines for early hearing detection and intervention. Available from www.asha.org/policy.
  2. Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

All new born babies and infants

All infants irrespective of risk factors born in facilities should be screened before discharge

Also cover all births including home births.

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Booth test used alone or combination

sensitivity 89%–100%

specificity 92%–100%

    • OAE or AABR 🡪 early identification of permanent bilateral hearing loss (PBHL)
    • Should be accompanied by diagnostic and management services

WHO 2024 recommendation for UNHCS

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

Where costs permit or for infants in the NICU,

AABR screening is recommended

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Principle of Universal Newborn �Hearing Screening

1

Screen by

Month of Age

6

Month of Age

Intervene by

3

Month of Age

Diagnose by

Risk Factors For Early Childhood Hearing Loss

Admission to NICU > 5 days

Bilirubin levels at or above exchange levels

Aminoglycoside administration >5 days

Asphyxia or hypoxic ischemic encephalopathy

In utero infections ; CMV, herpes, rubella, syphilis, toxo

Craniofacial malformations or syndromes

Meningitis

Infections with blood culture positivity

Caregiver concern

Consanguinity or family history of hearing loss

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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TIMING TO TEST

INTERVENTION

< 6 month

HA CI

DIAGNOSIS (OAE,AABR/ABR)

3 month

RISK FACTORS

IDENTIFICATION (OAE)

BEFORE DISCHARGE

2 days

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WHO Suggested Algorithm For Screening Protocol

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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Hearing Screening Protocol

in Cipto Mangunkusumo Hospital

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Oto-acoustic emissions (OAE)

Result “pass” or “refer” displayed on the unit screen

These emissions picked up by microphone in the probe, analysed by the screening unit

sound stimuli transmitted through middle ear to inner ear where outer hair cells of cochlea produce active response

Small probe placed in ear canal, delivers sound stimuli into auditory system

Sound vibrations emitted from the normal cochlear amplifier to ear canal where acoustic energy recorded

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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Oto-acoustic emissions (OAE)

  • Main reason for false-positive outcomes with OAE are :
    • the external auditory canal (e.g. collapse of the ear canal and the presence of debris)
    • the middle ear (e.g. presence of amniotic fluid and mucus)

​

  • These condition 🡪 high ambient noise level

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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Otoacoustic Emmision (OAE)

​

Measures sound waves produced in the inner ear

PASS

REFER

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Automated Auditory Brainstem Response (AABR)

  • an auditory evoked potential that originates from the auditory nerve

​

  • detect impairment on the level of cochlea, auditory nerve, and auditory pathway in the brainstem

​

  • obtained by placing disposable surface electrodes on the forehead and recording brain wave activity in response to sound

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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Automatic Auditory Brainstem Response (AABR)

PASS

REFER

Measures how the hearing nerve responds to sound

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Counselling After The Test

  • “pass,” 🡪 inform them, it confirms that sound is reaching the beginning of the inner part of the ear. 🡪 periodical follow-up and stimulation.

​

  • “refer” 🡪 counsel the family, the test needs to be repeated to confirm the hearing status.

​

  • Inform about age-appropriate hearing and language development and communication skills regardless of the screening results

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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NORMAL MILESTONES OF

HEARING AND LANGUAGE

Inform parents and caregivers about age-appropriate hearing & language development

and communication skills regardless of the screening results

Universal newborn screening: Implementation guidance. New Delhi: World Health Organization, Regional Office for South-East Asia; 2024. Licence: CC BY-NC-SA 3.0 IGO.

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NHS IN DEVELOPED COUNTRIES

NHS – USA 2019�Over 98% of U.S. newborns were screened for hearing loss; Almost 6,000 U.S. infants born in 2019 were identified early with a permanent hearing loss;

Total Screened 3,545,388

Total Not Pass 61,475 (1,7%)�Hearing Loss 5,934 (9,7%)

Prevalence per 1,000 screened : 1,7

​

Data Source: 2019 CDC EHDI Hearing Screening & Follow-up Survey (HSFS)

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NEWBORN HEARING SCREENING (NHS) �IN INDONESIA

  • Not yet implemented as a National Programme even though it is available:
      • Health Technology Assessment (HTA) since 2006 (revised 2010) / HTA Indonesia_2010_Buku Panduan Tatalaksana Bayi Baru Lahir Di Rumah Sakit
      • Pedoman Nasional PK Congenital Hearing Loss ( incl NHS guideline recommendation)

@Local NHS Programme : Jakarta , Bandung, Semarang, Surabaya, Bali

​

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  • NOT A NATIONAL PROGRAMME

Good example : Philiphine has Republic Act No 9709 for NHS Nat.Programme)

  • Limited and maldistribution of facilities
  • Manpower limitations and distribution
  • Follow up system in 3 months age should be done in primary/ secondary health � fascilities ( Nat.Health Insurance / JKN patient) hearing screening results were sometimes missing for infants admitted to the neonatal intensive care unit (NICU) or transferred from one birthing facility to another,

​

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NEWBORN HEARING SCREENING (NHS) �IN INDONESIA : Constrains

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NHS IN JAKARTA

Population (2023) : 11,34 million

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Newborn Hearing Screening

GOVERNMENT HOSPITAL

​

Cipto Mangunkusumo Hospital

​

​

PRIVATE HOSPITAL

  1. Pantai Indah Kapuk Hospital
  2. Pondok Indah Hospital
  3. Hermina Jatinegara Hospital
  4. Eka Hospital
  5. Bunda Hospital

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NHS IN CIPTO MANGUNKUSUMO NATIONAL HOSPITAL, Jakarta

Start : 2003

  • Universal NHS : OAE’s , before hospital discharge,
  • Discontuned : lack of coordination w/ other stakeholder, limited manpower

​

Restart : 2008

  • Targeted NHS (NICU): OAE’s + AABR ; following NICU discharge
  • National Health Insurance (JKN)
  • Comprehensive Pediatric Audiology Assessment ( 3 month of Age): �ABR ( Click & Tone Burst), ASSR

​

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Newborn Hearing Screening with Hearing Impairment Risk �ENT Department – Cipto Mangunkusumo Hospital �February 2015-September 2017

Screening 1850 baby

REFER

120 baby (6,48%)

PASS

1.730 baby (93,5%)

Follow Up

105 baby (5,67%)

Normal

91 baby (4,91%)

Conductive hearing loss

1 baby (0,05%)

Sensorineural hearing loss

13 baby (0,70%)

Ronny Suwento et al. Targeted NHS in Cipto Mangunkusumo National Hospital Jakarta . PIN X PERHATI KL Nov 2018

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BENCHMARK NHS

  1. The number of newborns completing ‘screening’ by the end of the 1st month of age : ≥95% of the newborns.
  2. The number of newborns who are referred for follow up at pediatric audiologic centers by 3 months of age (the referral rate) = ≤4% of the newborns.

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Targeted Newborn Hearing Screening

in Cipto Mangunkusumo Nat. Hospital 2019 - 2024

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UNIVERSAL NEWBORN HEARING SCREENING �IN HERMINA HOSPITAL – EAST JAKARTA�2019 - 2024

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Hearing Screening Card in RSCM

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Hearing Screening Card in RSCM

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Audiologist

Nurses who have received training

Doctor

Who can do the hearing screening?

RSCM

  • Audiologist
  • ENT Specialist

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Difficulties Hearing Screening in RSCM

Screening program

    • Not all health facilities have OAE and AABR
    • It’s not a national program
    • Human resources
    • The refer rate value is above the benchmark value ( > 4%)
    • no referral data platform between health institutions

Hearing Screening Evaluation

    • Patients din't come back for comprehensive ped.audiology test at 3 months of age because
      • Parents not well educated�House is far away 🡪 need costs�Busy�Parents feel its good enough after first screening results

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Conclusion

☺Screening is part of a system of

follow-up, diagnosis, treatment and

evaluation.

​

☺Timely and appropriate intervention

have lasting effects on outcomes.

​

☺ Family involvement : important

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Thank you