3. Concept:
⢠Hearing impairment is one of the most critical sensory impairments with
significant social and psychological consequences.
⢠Failure to detect children with congenital or acquired hearing loss may result in
lifelong deficits in speech and language acquisition, poor academic performance
and personal-social and behavior problems
⢠The data on congenital disabilities indicate that hearing loss has a substantially high
incidence with congenital hearing loss affecting 30 per 10,000 children
⢠Significant hearing loss is the most common disorder, occurring in 1 to 2 newborns
per 1000 in the general population, and 24% to 46% of newborns admitted to
neonatal intensive care unit
4. Concept:
⢠In this direction, the Universal newborn/infant hearing screening program has been
initiated worldwide for early detection of hearing loss in young babies due to its
high prevalence rates
⢠As per the recommendation of the WHO (2009),facilitating early hearing detection
and intervention is crucial in the first month of life.
⢠The first 3 years of life is considered the most intensive and critical period for
acquiring speech and language skills in newborns and infants, and accordingly,
emphasis is laid on early detection programs like UNHS
⢠Without UNHS, infants with hearing loss are typically identified with an
established language delay
⢠Hearing loss in infants : invisible disability
5. Concept:
⢠Significant expressive language deficit, noted well beyond one year of age, has been
the primary diagnostic feature in young children with hearing loss.
⢠Thus, in unscreened children, as is the current situation in many countries, the
average age at diagnosis is approximately 24 months.
⢠This will have a negative impact on intervention
⢠Without early intervention, children with hearing loss demonstrate predictable
irreversible deficits in communication and psychosocial skills, cognition and
literacy
⢠The impact on the childâs speech and language is directly proportional to the
severity of hearing loss and the time delay in diagnosis and intervention
6. Concept:
⢠Thus hearing screeningâŚ
⢠Hearing screening applies to a large population with no apparent signs or symptoms of the target
disorder.
⢠Screening program can be defined as a designed approach that separates disease group from
clinically normal group.
⢠In any screening program there four sets of result can be expected when administered on target
population.
⢠In case of hearing screening they are,
⢠true positive (presence of hearing loss),
⢠true negative (absence of hearing loss)
⢠false positive (though in the absence of hearing loss, screening instrument indicate presence of
hearing loss),
⢠false negative (though in the presence of hearing loss, screening instrument indicate absence of
hearing loss).
Thus screening hearing test should have higher
sensitivity (true positive) and specificity (true
negative).
8. History
⢠Historically, clinical screening for hearing loss in infants and young children was limited to
observation of the behavioural response to a sound, such as a ringing bell, introduced out of direct
vision of the child
⢠Later , behavioural observational audiometry (BOA) and high-risk register (HRR) (Northern &
Downs, 1974) were included as part of the screening protocol.
⢠In order to improve the sensitivity and specificity of a screening protocol, and facilitate better
identification of having hearing loss in newborns, various modifications in screening protocols have
been introduced worldwide.
⢠A few of these modifications have been specific to the HRR criteria (JCIH, 2000,2007) inclusion of
a two stage Otoacoustic emission screening along with automated auditory brainstem response
(AABR) (2011); three-stage screening of Transient Evoked Otoacoustic Emission (TEOAE) &
AABR ( 2015)
HRR screening resulted in around 50% of
congenital HL being undetected
10. Present Scenario
⢠The Joint Committee on Infant Hearing (JCIH 2000, 2019) recommends that all
infants be screened no later than 1 month of age, diagnosed by 2 months of age, and
enrolled in early intervention programs no later than 3 months of age.
⢠The basis for this recommendation is to maximize social, emotional, and linguistic
outcomes for children who are deaf or hard of hearing
⢠The JCIH recommended a screening protocol with automated auditory brainstem
response(AABR) and/or evoked otoacoustic emission (TEOAE) as they were
reported to yield successful outcomes in the early detection of hearing loss (Korres,
et al., 2008).
⢠Further, studies also indicated that a two- stage screening with the use of TEOAE &
AABR helped in reducing the false positive and referral rates (Iwasaki, et al.,
2003 Tatli, et al., 2007).
11. Present Scenario
⢠JCIH in its most recent revision, it expanded the target hearing loss as permanent
bilateral, unilateral sensory, or permanent conductive hearing loss to include neural
hearing loss (e.g., Auditory Neuropathy Spectrum Disorder [ANSD]).
⢠It also established separate screening & rescreening protocols for well baby &
neonatal intensive care units (NICU), specifying that babies in the NICU for 5 days
or more should be screened with Automated Auditory Brainstem Response (A-
ABR) technology.
12. Present Scenario
⢠Both AABR and OAE techniques are portable, inexpensive, automated and
reproducible
Otoacoustic emissions (OAE)
⢠Principle: Transient evoked otoacoustic emissions (TEOAEs) are frequency-
dispersive responses arising within the cochlea. Since OAE evaluates hearing from
the middle ear to the outer hair cells of the inner ear, it is used to screen for
sensorineural hearing loss (SNHL) but cannot detect auditory neuropathy (AN).
⢠Technique: The OAE screener consists of small microphone that is placed in the
ear canal of the infant. The screener sends stimuli in form of clicks or tones and also
receives the reflected sound from the cochlea. The device measures the signal-to-
noise ratio to make sure that recordings are accurate.
13. Present Scenario
⢠Automated Auditory Brainstem Response (AABR)
Principle: It is an electro-physiologic measurement that is used to assess auditory
function from the eighth nerve to the primary auditory cortex of the brain in response
to a click stimulus.
The AABR method produces a simple âpassâ or âfailâ result without requiring
interpretation. It is important to note the difference between AABR and ABR; ABR
being a diagnostic test which provides quantitative data (e.g, waveforms) that must be
interpreted by a trained audiologist, thereby determining the degree and the site of the
hearing loss.
Technique: AABR equipment measures the surface signals by placing electrodes on
the forehead and the mastoid, and on the nape of the neck. Most commercially
available devices effectively screen infants younger than 6 months.
14.
15. ⢠Two-step screening
A two-step screening means that if any ear fails the first screen, a repeat screening
should be done on both ears within a specified time frame. The repeat screening in
such cases should be done prior to discharge of the infant from the hospital
Two-step screening: What is the evidence? A large community-based trial showed
that a 2-step screening approach (OAEs followed by ABR for those who failed the
first test) 4 yielded a sensitivity of 0.92 and specificity of 0.98
Present Scenario
21. Hurdles
1. Loss to follow-up and loss to
documentation. Although loss to
follow-up has improved from
almost 50% in 2006 to 35.3% in
2011, state EHDI programs
continue to work diligently to
reduce this percentage
So, healthcare providers that involved
in UNHS program must provide more
counselling to the caregivers in order
to avoid loss to follow up for hearing
examination
⢠Ensuring correct identification of the
primary care provider before
discharge from the birthing hospital.
⢠Acquiring a second contact phone
number before discharge.
⢠Scripting the message given to
families when an infant does not pass
the initial screening test.
⢠Scheduling a follow-up appointment
(rescreening or diagnostic) before the
family leaves the hospital and
stressing its importance to the family.
⢠Calling the family to verify the
follow-up appointment
Solution
22. Hurdles
2. The shortage of professionals
with skills and expertise in
pediatrics and hearing loss,
continue to work on education and
training within their respective
professional communities.
⢠Invite more applicants in hospitals ,
primary care clinics etc
Solution
23. Hurdles
3. Lack of knowledge about
importance of early intervention
among medical professionals
the ultimate challenges was to
persuade pediatricians regarding the
importance of applying newborn
hearing screening test in children
without any risk factors
Make the professional aware about 50%
missing case and its consequences
Solution
24. Hurdles
4. Timely referral for diagnosis of and intervention for suspected hearing loss in infants and
children. Barriers include the lack of support in rural areas, finances of the parents, cultural
and linguistic obstacles, etc
5. The lack of uniform performance and national standards for the calibration of OAE or A-
ABR instrumentation.
6. The inability of state tracking systems to follow individual infants with suspected or
confirmed hearing loss through the EHDI program.
7. there is also a challenge in societies with a lower socioeconomic status, included not
attending to the re-screening date, deficiency of information of caregivers around the
indications and the impacts of hearing impairment
25. Hurdles
8. On the other hand, the challenges facing hearing screening in newborns in developing
countries are great.
Finding the resources to implement solutions for the detection and treatment of newborns is
a major problem. Most developing have a high birth rate with heavily dense populations.
Hearing impairment prevalence rate in the newborns is estimated to be higher in
developing countries considering the relatively higher rate of exposure to risk factors
Standardizing screening and intervention programs remains an important goal to establish
national newborn hearing screening programs in developing countries.
Also, it needs to consider the local culture and be acquainted with local resource limitations
and strengths.
27. ⢠India faces the challenge of a very large population and a high annual birth rate
⢠Moreover, 75% of the population live in rural areas and over 50% of births occur at
home and are frequently attended by a trained birth attendant.
⢠In India, although Newborn screening programs have been initiated, it is not widely
spread across the country due to inadequate professionals in the field of Speech and
Hearing.
⢠However, India also has a well-developed health care delivery system, right down to
the grassroots/village level, and a well-established immunization programme
28. ⢠In 2006, India launched the National Programme for Prevention and Control of
Deafness. (NPPCD)
⢠Currently running in over 60 districts of the country
⢠Aim: to identify babies with bilateral severe-profound hearing losses by 6 months
of age & initiate rehabilitation by 9 months of age
⢠Under this programme, the following two-part protocol for infant hearing
screening is being implemented:
29. 1. Institution-based screening â
to screen every baby born in a hospital or admitted soon after birth using OAE. Those
who fail the test are re-tested after 1 month.
Those who fail the second screening are referred for ABR testing at the tertiary-level
centres
2. Community-based screening
to screen babies who are not born in hospitals. Such screening is carried out using a brief
questionnaire & behavioural testing. The screening is performed when the baby attends
for immunization at 6 weeks of age & onwards.
A trained health care worker at the subcentre administers immunization & conducts the
hearing screening. The protocol is repeated at every immunization. Any baby failing the
screening is referred for formal OAE screening to the district hospital, and if they fail
OAE they are then sent for ABR testing.
30. The programme includes:
⢠training of existing human resources using standardized training programs & other
materials
⢠provision of the equipment required for behavioral testing & for OAE at the
respective centers
⢠provision of suitable audiological personnel for diagnosis & for rehabilitation at the
district hospitals
⢠creating awareness of the importance of detecting childhood hearing loss amongst
parents and the general population through the use of posters, flipcharts, fliers,
handouts and other suitable materials
⢠provision of a referral slip to aid patient compliance and simplify the visiting
process.
31. ⢠Once an individual is identified as hearing impaired, they are referred for
hearing aid fitting and for suitable therapy at the district hospital.
⢠Identified problems include the need for patients/parents to make repeated
visits and to visit different centres.
⢠In addition, even though OAE is provided at all centres, there is a shortage of
centres where ABR is done.
⢠There is also a shortage of audiological personnel and a heavy burden placed
on health care workers.
32. ⢠As a part of the newborn screening programs, various protocols have been evaluated
in our country.
⢠In one such reported study (Vignesh et al., 2015), a two â stage protocol with
DPOAE and AABR was found to significantly reduce referral rates of newborns in
newborn screening programs. Further, they also stated that AABR reduces the false
positive responses resulting in increasing the efficiency of a screening program.
⢠Similarly, another study was carried out using a three stage protocol including
High risk register, TEOAE and Screening ABR ( Savithri et al., 2015). It was
concluded that rescreening using TEOAE significantly reduces the false positive
response and referral rate for ABR. Attempts to improve the efficacy of the NBS
protocol continue across the country.
33. ⢠The Department of Prevention of Communication Disorders (POCD) at the All
India Institute of Speech and Hearing (AIISH), Mysore is actively involved in
carrying out various extension activities such as screening, diagnostic and
rehabilitation services for communication disorders.
⢠As a part of secondary prevention, the institute is extensively involved in newborn
screening program for communication disorders.
Editor's Notes
Đe outcome of false positive result is costlier because patient has to undergo unnecessary detailed diagnostic evaluation. It consumes time and financially expensive for the patients. tĐus screening hearing test should have higher sensitivity (true positive) and specificity (true negative).