GUIDELINES

Consensus Statement of the Indian Academy of Pediatrics on Newborn

Hearing Screening
ABRAHAM PAUL, #CHHAYA PRASAD, $SS KAMATH, *SAMIR DALWAI, ‡MKC NAIR AND @WAHEEDA PAGARKAR; for
the National Consultation Meeting for Developing IAP Guidelines on Neurodevelopmental Disorders under the aegis
of IAP Childhood Disability Group and the Committee on Child Development and Neurodevelopmental Disorders
From Child Care Centre, Cochin Hospital; #Max Super Speciality Hospital, Chandigarh; $Welcare Hospital, Vytilla; *New Horizons
Group, Mumbai; ‡Kerala University, Thrissur; India, and @Audiovestibular Medicine, Hackney ARK and Royal National Throat
Nose and Ear Hospital, London.
Correspondence to: Dr Samir Dalwai, Director, New Horizons Child Development Centre, Mumbai. [email protected].
Received: September 13, 2016; Initial Review: September 19, 2016; Accepted: May 15, 2017.

Justification: Hearing impairment is one of the most critical sensory impairments with significant social and psychological
consequences. Evidence-based, standardized national guidelines are needed for professionals to screen for hearing impairment during
the neonatal period.
Process: The meeting on formulation of national consensus guidelines on developmental disorders was organized by Indian Academy of
Pediatrics in Mumbai, on 18th and 19th December, 2015. The invited experts included Pediatricians, Developmental Pediatricians,
Pediatric Neurologists and Clinical Psychologists. The participants framed guidelines after extensive discussions.
Objective: To provide guidelines on newborn hearing screening in India.
Recommendations: The first screening should be conducted before the neonate’s discharge from the hospital – if it ‘fails’, then it should
be repeated after four weeks, or at first immunization visit. If it ‘fails’ again, then Auditory Brainstem Response (ABR) audiometry should
be conducted. All babies admitted to intensive care unit should be screened via ABR. All babies with abnormal ABR should undergo
detailed evaluation, hearing aid fitting and auditory rehabilitation, before six months of age. The goal is to screen newborn babies before
one month of age, diagnose hearing loss before three months of age and start intervention before six months of age.
Keywords: Assessment, Auditory brainstem response audiometry, Deafness, Hearing loss, Otoacoustic emission, Prevention.

Published online: June 04, 2017. PII:S097475591600067

H
earing impairment is one of the most critical impairment if remediated at birth is 300-700 words; if re-
sensory impairments with significant social mediated at 6 months is 150-300 words and if remediated at
and psychological consequences. Failure to 2 years is 0-50 words, respectively; as compared to
detect children with congenital or acquired vocabulary of a 3-year-old child with typical hearing which
hearing loss may result in lifelong deficits in speech and is 500-900 words.
language acquisition, poor academic performance and
personal-social and behavior problems [1,2]. Deficits in In view of the above, standard guidelines for screening
speech and language lead to lack of stimulation, which newborns for hearing loss are urgently needed. The
adversely affects the structure of the synaptic junction. meeting on formulation of National consensus guidelines
Lack of auditory stimulation leads to retrograde on developmental disorders was organized by the Indian
degeneration in the cell body and axon [3]. Academy of Pediatrics in Mumbai, on 18th and 19th
December, 2015. The invited experts included
Apart from the biological evidence, the data on Pediatricians, Developmental Pediatricians, Pediatric
congenital disabilities indicate that hearing loss has a Neurologists and Clinical Psychologists. The participants
substantially high incidence with congenital hearing loss framed guidelines after extensive discussions and review of
affecting 30 per 10,000 children [4]. Significant hearing literature. Thereafter, a committee was established to
loss is the most common disorder, occurring in 1 to 2 review and finalize the points discussed in the meeting.
newborns per 1000 in the general population, and 24% to
46% of newborns admitted to neonatal intensive care unit Subsequent sections include the points of consensus on
[5,6]. Vocabulary of a 3-year-old child with hearing screening of newborn hearing.

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PAUL, et al. CONSENSUS STATEMENT ON NEWBORN HEARING SCREENING

RECOMMENDATIONS syndrome, Alport syndrome, Neurofibromatosis

syndrome, Achondroplasia, CHARGE syndrome, Brachio
Early Screening
Oto Renal syndrome, Chudley McCullough syndrome and
Critical period for identification and remediation of Golden Har syndrome [8].
hearing loss is before the age of 6 months. Since the
Screening for Newborn Hearing Loss
pediatrician is the primary care provider for the child
during the first few days of life, it is the sole responsibility In India, majority of hospitals do not conduct universal or
of the pediatrician (or the primary physician) to evaluate high risk screening. In such a situation, a centralized
the child for hearing loss (or ensure referral for the same). facility catering to all hospitals in a city is a practical
It has been observed that practice of neonatal screening option. A two-stage screening protocol can be made, in
has dramatically lowered the age of diagnosis of deafness which infants are screened first with otoacoustic
from 1½ - 3 years to less than 6 months of age. Screening emissions (OAE). Infants who fail the OAE are screened
should ideally be ‘universal’ i.e., everybody is screened with auditory brainstem response (ABR). In this two tier
and at a minimum, screening should be ‘targeted’ i.e., screening program, the second tier being ABR (which is
‘high risk’ babies are screened. more expensive) is required only for a select few, making
the program more practical and viable.
Causes of hearing loss are summarized in Box 1. These
can be classified as: Conductive, Cochlear (i.e. Sensory: The Child Health Screening and Early Intervention
defect in the cochlea and Neural: defect in the 8th cranial/ Services Program (Rashtriya Bal Swasthya Karyakram)
auditory nerve), Retrocochlear (i.e. defect at the level of under National Rural Health Mission initiated by the
auditory nerve, brainstem auditory pathway or both) and Ministry of Health and Family Welfare of Government of
Central (i.e. defect in the auditory area in cerebral cortex). India has included congenital deafness as one of the
conditions to be included for early identification and
With respect to current guidelines, sensorineural
remediation. It involves screening of infants and children
hearing loss is most relevant and cochlear causes of
under age 18 years by a mobile team and provision of
sensorineural hearing loss are more common. Many risk
appropriate treatment at District Early Intervention
factors for hearing loss have been identified and are listed
Centres (DEICs). This ambitious scheme is likely to
in Box 2.
streamline the management of hearing disabilities [9].
Congenital rubella syndrome, Usher syndrome and
Otoacoustic emissions (OAEs) are quicker methods
Jervell and Lange-Nielsen (JLN) syndrome have been
(as compared to electrophysiologic methods like ABR) for
noted to be associated with hearing loss; few other
assessing hearing in newborns via a simple set-up.
syndromes include Treacher-Collins syndrome, Apert
Otoacoustic emissions (OAEs) are sounds of cochlear
origin recorded in the auditory meatus (ear canal),
BOX 1 CAUSES OF HEARING LOSS produced by the action of healthy outer hair cells. The
• Causes in ear canal/Conductive (e.g., congenital
atresia, wax, foreign body, trauma, external otitis, BOX 2 RISK FACTORS FOR HEARING LOSS [7]
stenosis
• Family history of hereditary childhood
• Causes in middle ear/Conductive (e.g., acute and sensorineural hearing impairment
chronic otitis media, perforation of tympanic
membrane, congenital defects, trauma, • Intrauterine infection (TORCH)
malformations either hereditary or familial) • Craniofacial anomalies
• Causes in the cochlea/Cochlear (e.g., ototoxic • Birth weight less than 1500 gram
drugs, stay in neonatal intensive care unit due to • Hyperbilirubinemia at a serum level requiring
jaundice or other causes, neonatal infections, exchange transfusion
head injury, noise); and
• Ototoxic medications used in multiple courses, or
• Causes in auditory nerve/Retrocochlear (e.g., in combination with loop diuretics.
problems in cochlear nerve, auditory pathway or
• Bacterial meningitis
cortex like tumors, trauma, de myelination).
• APGAR scores 0-4 at 1 minute or 0-6 at 5 minute
• Intrauterine infections (tetanus, toxoplasma,
rubella, cytomegalovirus and herpes or TORCH • Mechanical ventilation for 5 days or longer
group of infections) can be classified as cochlear • Stigmata of other findings associated with a
or retrocochlear causes of Sensorineural hearing syndrome known to include sensorineural and/or
loss. conductive hearing loss.

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emissions themselves serve no purpose and are simply a communication skills are chosen. They should be
leakage of energy from the ear. Hearing is facilitated by provided basic training in hearing screening and also
hair cell activity in the cochlea and more specifically, the skills to gather information on high-risk criteria, if any,
activity of outer hair cells. There are three rows of outer from parents/hospital staff/hospital records. This
hair cells (OHCs) and one row of inner hair cells that sit on training is to be conducted over one day.
the basilar membrane, sandwiched by the tectorial
• The screening personnel should visit each hospital
membrane on top. This forms the organ of Corti. There are
daily/on alternate days/twice a week/weekly
around 12000 motile OHCs working together to provide
depending upon the number of births in that particular
mechanical assistance to sound energy, amplifying the
hospital. Daily screening may be carried out in
travelling wave to overcome the viscous nature of the
hospitals which have more than 200 births, alternate
cochlear fluid. As the ‘W’ shaped steriocilia are stimulated
day screening in hospitals with 100-200 births and
by fluid moving over them, it causes the cells to alternately
twice weekly or weekly screening in hospitals with
contract and release, providing a pumping action. This
births less than 100 per month.
mechanical system provides the frequency tuning within
the cochlea. The inner hair cells are also stimulated and • All screeners should maintain a register of all cases
deflected by fluid flow; and at a specific threshold, the screened and those with abnormal results. Neonates
inner cells release a neurotransmitter which causes the with abnormal screening results should be evaluated. It
auditory nerve to transmit a signal to the brain. is the duty of the screeners to call back all abnormal
cases for follow up, with the help of a coordinator.
Cochlear damage is almost always apparent in the loss
(Number of hospitals covered by a screener depends
of outer hair cells. This is true regardless of the etiology –
on the number of cases in a particular hospital and
congential progressive hearing loss, ototoxic drugs,
proximity of the hospitals)
presbyacusis (Sensorineural hearing loss with aging), as
well as noise-induced hearing loss. With damaged OHCs, • If abnormal OAE is detected, it is repeated at 6 weeks
there is no amplification or frequency tuning, thus the child on the 1st immunization visit. If again abnormal, ABR
will not only suffer a threshold shift but also have is done for confirmation followed by full audiological
problems with frequency discrimination. evaluation and remediation with hearing aids
(cochlear implant may be required in cases of
OAE test is performed via a small probe placed in the profound hearing loss or poor response to hearing
child’s ear canal; click sounds are delivered and response aids).
is detected (Web Appendix 1). The child must be quiet
[10]. • All NICU babies undergo ABR testing to rule out
auditory dyssynchrony/ auditory neuropathy.
Recommendations on Screening
• In babies with abnormal ABR, detailed enquiry is
• A two-stage screening protocol with OAE as the first made to identify and record any risk factors. Any baby
screen, followed by ABR for those who fail the OAE missing screening before hospital discharge is called
screen [11]. for OAE test on the first immunization visit.
• It is advisable that all hospitals with level-3 neonatal • All babies with abnormal ABR should undergo
care have OAE and ABR facilities. If not feasible, a detailed ENT evaluation hearing- aid fitting and
centralized hearing screening with a portable OAE is auditory rehabilitation before 6 months of age.
suggested and all abnormal cases can be referred for Systematic evaluation for ruling out syndromic
ABR to the nearest centre. associations such as ophthalmic, paediatric and
cardiac assessments should be conducted.
• The program is to be coordinated by an audiologist and
weekly assessment meeting is to be convened with the • Children with neonatal meningitis should be treated as
staff to discuss and sort out the issues, if any (held by a special category and need investigations including
the convenor). Usual issues could include non- imaging and intervention like cochlear implant (if
compliance by parents to bring the child for repeat needed) on a semi-emergency basis. Delay can result
OAE or ABR. This usually can be tackled by phone in cochlear ossification which may preclude
calls made by screening personnel, coordinator, or in subsequent intervention like a cochlear implant.
rare instances by the convenor himself. A medical
The goal is to screen newborn babies before 1 month
social worker can be involved for problem-solving.
of age, diagnose hearing loss before 3 months of age and
• Personnel with basic knowledge in computer and good start intervention before 6 months of age. Hurdles

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KEY MESSAGES
• Hearing loss should be screened preferably before 1 month of age.
• Universal neonatal screening rather than targeted ‘high risk’ screening is ideal.
• If abnormal OAE detected, it is repeated at 6 weeks or on the first immunization visit. If again abnormal, ABR
is done for confirmation followed by full audiological evaluation and remediation with hearing aids.
• All NICU babies should undergo ABR testing to rule out auditory dys-synchrony/ auditory neuropathy.

experienced in the screening process include: less investing for the screening equipment. Follow up of
motivated pediatricians; lack of awareness among parents/ positive cases and drop-outs are made easier with the
community; non-compliance by the family for evaluation, central reporting and monitoring system. With unified
and stigma attached to hearing aids. strength of pediatricians, IAP city/ district branches could
take initiative to replicate this model in their respective
CONCLUSIONS
towns or districts and by collaborating with government
As normal hearing is critical for speech and language agencies involved in implementation of Rashtriya Bal
development, it is recommended that during first 6 Swasthya Karyakram.
months of life, clinicians identify infants with hearing Newborn hearing screening will help to identify
loss, preferably before 3 months of age. Other important hearing loss at an earlier age and alleviate the double
issues are: tragedy of inability to hear and speak. Forming a
• Evaluate infants before discharge from nursery, consensus and national level guidelines for hearing
especially high risk babies screening is very important to construct a healthy
independent society. Early intervention is mandatory for
• Universal neonatal screening and not targeted ‘high best prognostic outcomes.
risk’ screening is ideal since about 50% of infants with
hearing loss have no known risk factors for hearing REFERENCES
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followed up (if presence of language delays, Psychol Psychiatry. 2010;51:77-83
infections, head trauma, stigmata of syndromes, 2. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL.
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intrauterine infections, neurofibromatosis type II) hearing loss. Pediatrics. 1998;102:1161-71.
3. Dominguez M, Becker S, Bruce I, Read H. A spiking
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the other newborn disorders combined, which can be hearing loss: Spontaneous firing, synchrony and tinnitus.
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4. Wynbrandt J, Ludman MD. The Encyclopaedia of Genetic
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otoacoustic emission. Pediatrics. 2005;116:933-98.
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Universal Newborn Hearing Screening (UNHS) has in Kuwait. Electromyogr Clin Neurophysiol. 2007;47:305-
become a standard practice in most developed countries. 13.
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The identification of all newborns with hearing loss before
Statement: Principles and Guidelines for Early Hearing
six months has now become an attainable and realistic Detection and Intervention Programs. Pediatrics. 2007;
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cater to all hospitals in the district is worth replicating [12]. Malformation 6th Edition, Philadelphia, USA: Elsevier;
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9. Rashtriya Bal Swasthya Karyakram. Available from http:// Convener: Dr Samir Dalwai, Mumbai.
nrhm.gov.in/nrhm-components/rmnch-a/child-health-
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Bhattacharya, Mumbai; Anuradha Sovani, Mumbai; Bakul
background.html. Accessed on February 6, 2015.
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Mumbai; Kate Currawalla, Mumbai; Kersi Chavda, Mumbai;
function, and use. Br Med Bull. 2002;63:223-41.
Madhuri Kulkarni, Mumbai; Monica Juneja, New Delhi;
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Monidipa Banerjee, Kolkata; Mamta Muranjan, Mumbai;
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Nandini Mundkar, Bangalore; Neeta Naik, Mumbai; P
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ANNEXURE I Pune.
Participants of the National Consultative Meet for Invited but could not attend the meeting: MKC Nair, Thrissur;
Development of IAP National Consensus Guidelines on Pratibha Singhi, Chandigarh; Jeeson Unni, Ernakulam, Cochin;
Newborn Hearing Screening Manoj Bhatvadekar, Mumbai.

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WEB APPENDIX I DETAILS OF SCREENING TESTS

The two available techniques are Otoacoustic emissions (OAE) and Auditory Brain-stem response (ABR).
Otoacoustic emissions (OAE): Probe is kept in the ear and the machine is switched ‘on’ when click sounds are produced.
Sound waves travel from the external ear to the inner ear and causes outer hair cells of the cochlea to move, producing
sound detected by the machine probe at the external ear canal. This indicates that the baby has a normal cochlear (inner
ear) function.
Cost of instrument: OAE Screener Otoportlite (Otodynamics, UK) cost INR 2.95 lakh + 5% Tax; can be purchased
locally.
Purchase and maintenance: Of the four basic parts of the OAE instrument (main body, probe, probe tips and couplers),
it is the couplers which require replacement most often, when the debris in the ear canal or wax obstructs it. Couplers
cost around INR 500. Probe tips may require frequent changes, cost INR 50. Main instrument and probe last long
enough for two to three years. In addition, the batteries need to be replaced.
OAE may be affected by debris or fluid in the external and middle ear, decreased tympanic mobility, delayed cochlear
maturation - resulting in referral rates of 5% to 20% when screening is performed in the first 24 hours after birth. Hence,
OAE should be done on the day of discharge or 72 hours after birth. Referral rates <3% may be achieved when screening
is performed during first 48 hours after birth. In a two step system using OAE as the first step, referral rates of 5% to 20%
for repeat screening with ABR or OAE may be expected.
Due to automated OAEs, screening can be conducted by anyone after baseline training. However, the person should be
educated till graduate level or preferably from medical field, so he/she can communicate results to caregivers.
Auditory Brainstem Response (ABR): ABR is an electro-physiologic measurement that is used to assess auditory
function from the eighth nerve through the auditory brainstem; via placing disposable surface electrodes high on the
forehead, mastoid and the nape of the neck.

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