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Hearing is a vital part of newborns contact with his environment

and is crucial for the development of speech and language in a naturalistic


fashion1, first year of life being the most important in this developmental
process2. Hearing loss very early in life can affect the development of speech
and language, social and emotional development, behaviour, attention and
academic achievement. Even mild or unilateral involvement may have
detrimental effect on the development of a young child and on school
performance3. No disability affects an infant to communicate as severely as
hearing impairment1. The severity of these hearing disabilities is generally
related to the length of time the hearing loss is left untreated. Hence the policy
of wait and watch cannot be adopted with hearing impairment, hoping that the
child will grow out of it4. Early identification of hearing impairment improves
prognosis, hence screening programmes have been widely and strongly
advocated3.
Significant hearing loss is one of the most common major
abnormalities present at birth and if left untreated, will impede speech, language
and cognitive development5. The incidence of significant bilateral hearing loss
in neonates is 1-3 cases per 1000 live births and 2-4 per 100 infants surviving
neonatal intensive care6. Data from the Colorado newborn screening programme
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suggest that if hearing impaired infants are identified and treated by 6 months of
age , these children ( with the exception of those with bilateral profound
impairment ) should develop the same level of language as their age-matched
peers who are not hearing impaired3 .Thus early intervention enhances the
potential of most hearing impaired children to become adults who are fully
independent, participating and contributing members of society4.
Hearing loss falls into four major categories6:
1. Sensorineural Loss as the result of abnormal development or damage to
cochlear hair cells ( sensory end organ) or auditory nerve.
2. Conductive Loss is the result of interference in transmission of sound
from external auditory canal to the inner ear .the most common cause for
conductive hearing loss is fluid in the middle ear. Less common are
anatomic causes such as microtia, canal stenosis, or stapes fixation that
often occur in infants with craniofacial malformations.
3. Auditory Dyssynchrony or Auditory Neuropathy---- In this less
common type of hearing loss, the inner ear or cochlea appears to receive
sounds normally; however, the transfer of the signal from the cochlea to
the auditory nerve is abnormal. The etiology of this disorder is not well
understood,

however

patients

with

severe

hyperbilirubinemia,

prematurity, hypoxia and immune disorders are at increased risk.

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4. Central Hearing Loss--- In this type of hearing loss there is intact


auditory canal and inner ear and normal neurosensory pathways but
abnormal auditory processing at higher levels of the central nervous
system.
ETIOLOGY3 :
Genetic causes3:
Genetic causes of Conductive hearing loss include those with craniofacial
anomalies like Pierre Robin syndrome, Treacher Collins syndrome, Klippel-Feil
syndrome, Crouzon & Brachio-otorenal syndromes & Osteogenesis imperfecta.
Malformation of ossicles and middle ear structures and atresia of external
auditory canal are the other causes of conductive hearing loss.
Genetic causes of sensorineural hearing loss (SNHL) account for almost
50% of cases of SNHL. These may be associated with other abnormalities, may
be part of a named syndrome, or may exist in isolation. Autosomal dominant
causes, which account for 10% of cases, include Waardenberg syndrome,
Brachio-otorenal syndrome,etc. Autosomal recessive causes both syndromic &
non-syndromic account for 80% of all cases of genetic causes of SNHL.
Examples are Usher, Pendred, Alstrom, Type 4 Bartter, Jervell and Lange~3~

Nielsen syndrome, Biotinidase deficiency etc. Non-syndromic causes include


mutations of connexin-26 & -30 genes.
Chromosomal abnormalities such as Trisomy 13-15, Trisomy 18 and
Trisomy 21 also can be accompanied by hearing impairment.
Non-Genetic Causes3:
Middle ear effusion is the most common cause of conductive hearing loss.
Sensorineural hearing loss the recognized risk factors account for about
50% of cases of moderate to profound SNHL. Examples are congenital
infections

(CMV

most

common),

birth

weight<1500gm,

neonatal

hyperbilirubinemia, ototoxic medications, bacterial meningitis, perinatal


asphyxia, mechanical ventilation >5 days.
Screening programmes for hearing impairment may be either
universal or high risk population based7. The problem with using high risk
criteria to screen is that 50% of cases of hearing impairment will be missed,
either because the infants are hearing impaired but do not meet any of the high
risk criteria, or because they develop hearing loss after the neonatal period.
Hence the American Academy Of Pediatrics endorses the goal of universal
detection of hearing loss in infants before 3 months of age3. Prior to the
universal screening, the average age at which children were found to have a
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hearing loss is 2-3 years. Children with mild-to-moderate hearing loss were
often not identified until 4 years of age. Children identified when they are older
than 6 months can have speech and language delays. Children identified when
they are younger than 6 months do not have these delays and are equal to their
hearing peers in terms of speech and language 8. Until mandatory screening
programmes are established universally, many hospitals will continue to use
high risk criteria to screen for hearing loss3. The screening of infant at risk is
selective and considered as first step towards introduction of universal hearing
screening9.
The currently acceptable methods for physiologic hearing screening in
newborns are Brainstem Auditory Evoked Response (BAER) and evoked
otoacoustic emissions (EOAEs).EOAEs are used to assess cochlear integrity
and are physiologic measurements of the response of the outer hair cells to
acoustic stimuli. They serve as a fast objective screening test for normal
preneural cochlear function. To measure EOAEs, a probe assembly is placed in
the ear canal, tonal or click stimuli are delivered, and the EOAE generated by
the cochlea is measured with a microphone. EOAEs are fast, efficient, and
frequency-specific measurements of peripheral auditory sensitivity. However,
the effectiveness of the test is reduced by contamination with low-frequency
ambient noise in a busy nursery, vernix in the ear canal, or any middle ear
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pathology. EOAEs are not a sufficient screening tool in infants who are at risk
for neural hearing loss (eg, auditory neuropathy/dyssynchrony). Any infant in
the NICU or in the hospital for more than 5 days should undergo an ABR
screening so that the presence of auditory neuropathy is not missed. Cochlear
function, and therefore EOAE measurements are usually normal in infants and
children

with

this

type

of

hearing

loss8.

BAER

measures

the

electroencephalographic waves generated by the auditory system in response to


clicks through three electrodes placed on the infants scalp 6. This has been
recommended for newborn hearing assessment because it is objective,
correlates well with hearing, can detect mild and moderate hearing losses as
well as severe to profound losses, permits ear specific information, has good
performance statistics (sensitivity and specificity), is stable over time, is
unaltered by sleep/ sedation as the response is physiological, and can be done at
any age4.

Assessment of the Auditory System by EOAEs and BAER8

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Auditory Structure

EOAEs

BAER

Outer ear

Yes

Yes

Middle ear

Yes

Yes

Inner ear

Yes

Yes

Auditory nerve

No

Yes

Auditory brainstem

No

Yes

The BAER occurs as a result of synchronous neural activity


originating in the auditory nerve and brainstem pathways, arising in the first 10
milliseconds after an auditory stimulus. It is facilitated by a rapid click stimulus
presented through headphones and recorded via surface electrodes applied to
locations on the skull (vertex and mastoid)10. The responses are summed up and
recorded as a graphic display with vertex positive peaks noted and designated
as waves l-V.In infants waves I,III,V are easily identifiable. The absolute
latencies as well as interpeak latencies are higher than adults. It is always
prudent to record the response for atleast 15 milliseconds instead of 10
milliseconds that is done for adults11. The waves are described by their
amplitude and latency characteristics. The units for latency and amplitude are
usually milliseconds and micro volts, respectively12. Wave V has proven to be
the most prominent and robust component of the response pattern6. The five

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waveform peaks reflect neurotransmission in the Auditory Response Pathway


and give information regarding hearing sensitivity for each ear 7. It is
worthwhile to mention here that BAER tests only electrophysiological integrity
of auditory pathway from cochlea to midbrain and not a test for hearing per se,
since it does not test conscious perception of sound11.

Screening programmes for hearing impairment may be either universal or


high risk population based. A universal approach was first reported in 1961 in

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UK, where health visitors screened for hearing in the respective homes using a
behavioural observation technique7.
James Coplan, Department of Pediatrics, State University of New York,
revived records of 1000 children seen for evaluation of developmental delay
from July 1979 to December 1985. Forty six children with permanent hearing
loss were identified with mean age at diagnosis in cases of profound hearing
loss not less than twenty four months. In forty percent of the subjects author
was the first to diagnose hearing impairment14.
P M Watkin, M Baldwin and G Mc Enery ,1991, performed hearing
screening on 322 at risk neonates out of 10686 live births by ABR and found
that neonatal at risk screening programme was effective in terms of both yield
and cost. The mean age at which hearing aids were fitted was 6 months in the
children identified by the neonatal screen15.
R J McClelland, D R Watson, V Lawless, H G Houston and D
Adams,1992, screened 405 neonates by brain stem evoked response admitted to
the baby unit, Special care baby unit and children's audiology department,
Belfast. 85 children failed the screening test, 62of whom were followed up.
Five children had severe bilateral sensorineural impairment and 12 had
conductive impairment requiring surgical intervention. A further 18 had severe

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neurological disorder detected. The sensitivity of screening was 100% and


specificity was 88%16.
Watson D. R et al, 1996, reported findings of seven year study evaluating
ABR as basis of hearing screening procedure in high risk neonates. A Special
Care Baby Unit (SCBU) population of 417 infants with diverse clinical
backgrounds and treatment histories was tested for hearing impairment at birth
using ABR audiometry. Some 332 passed the original screen at 30 dBnHL test
level in both ears. Of the failure group, 18 did not survive and 32 had some
degree of hearing impairment confirmed, nine of which were sensorineural in
origin and concluded that ABR technique offers excellent sensitivity and
specificity for the detection of significant hearing loss in the test population.
They also found that it could reduce the detection of permanent hearing loss by
seven months17.
P.K. Misra et al,1996, studied the brainstem auditory evoked response
(BAER) abnormalities and their reversibility in neonates with birth asphyxia. 30
term Neonates with 5-min Apgar < 6 and hypoxic ischemic encephalopathy
(HIE) underwent BAER testing with follow up at 3 months. An equal number
of normal term neonates served as controls. Results: 13 out of 30 (43.3%)
neonates with birth asphyxia showed some abnormality in BAER wave form.
Abnormalities in BAER were significantly associated with stages of HIE and
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duration of neurological abnormalities more than 5 days. On follow up of 16


cases at 3 months of age, BAER abnormalities reverted back to normal in all the
neonates. The Denver Developmental Screening Test (DENVER II) was suspect
in 4 cases but the BAER was normal18.
Hulya Blgen et al,1997, performed the auditory brain stem responses
(ABR) of neonates at high risk for hearing impairment at Marmara University
Hospital Neonatal Intensive Care Unit. One hundred fifty-four high-risk
neonates underwent screening by ABR carried out according to the US Joint
Committee on Hearing 1994 Position Statement. One hundred and fourteen
infants passed the first ABR test while 40 patients failed. The number of
patients with sensorineural and conductive hearing deficit was 5 and 35,
respectively. Fifteen of the infants who failed had bilateral hearing loss. During
follow-up, 6 of these 40 infants (15%) failed subsequent tests. Two of these
babies had bilateral involvement, and the rest had unilateral involvement. The
infant with bilateral sensorineural hearing loss had cochlear implantation.
Seventeen of the 40 infants passed the second ABR (42%). Three patients did
not survive and 11 (27%) were lost from the follow-up. It was concluded that
early diagnosis of hearing loss in high risk neonates is important for the
implementation of hearing aids early in infancy so as to prevent acoustic
deprivation19.
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Judith A. Mason* and Kenneth R. Herrmann,1997,from the Departments


of Audiology Services and Pediatrics, Kaiser Permanente Medical Center,
Honolulu, Hawaii reported findings of a five year study conducted on 10 372
infants. Universal hearing screening by automated auditory brainstem response
was done in the nursery. Successful screening in the nursery was achieved for
96% of infants. The failure rate was 4%. The incidence of bilateral loss
requiring amplification was 1.4/1000. The false-positive rate was 3.5% after the
initial screening and .2% when a two-stage screening procedure was used. The
incidence of congenital bilateral hearing loss in the well population was 1/1000,
and in the neonatal intensive care unit population, 5/100020.
Christiane Meyer et al, 1998, conducted the ABR screening on the high risk
population as defined by Joint Committee on infant hearing besides some other
criteria like maternal drug abuse, PPHN, ICH, periventricular leukomalacia.
Out of 777 high risk neonates enrolled,41 (5.3%) infants exhibited pathologic
A-ABR results (16 bilateral and 25 unilateral). Meningitis or sepsis, craniofacial
malformations, and familial hearing loss were independent significant risk
factors. Follow-up examinations in 31 infants revealed persistent hearing loss
in 18 infants (13 infants sensorineural, 5 from mixed disorders), 7 requiring
amplification. Their study helped to determine the incidence and risk factors for

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hearing disorders in a selected group of neonates and feasibility of selective


hearing screening21.
K Y Chan, F Lee, C B Chow et al, 1998, Princess Margaut Hospital,
Hong Kong, performed ABR testing in high risk neonates before the age of
three months. From a cohort of 6,127 live births in Princess Margaret Hospital,
309 (5%) were identified as being at risk of hearing impairment. The at risk
infants were screened at postconceptual age of 37 weeks or before age of 3
months when they were in stable condition by measuring the auditory brainstem
response (ABR). Thirty-five (11%) of 309 at risk infants failed the ABR test.
Twenty-six infants were further evaluated by ABR examination, distraction test
and impedence test. Twelve were confirmed to have moderate to severe hearing
loss. Their risk factors were as follows - 4 babies with Down syndrome, 2 with
severe ear malformation, 1 preterm baby, 1 baby with severe asphyxia, 3 with
severe neonatal jaundice with serum bilirubin of >= 340 mol/L and 1 with
elevated netromycin level. The overall rate of hearing impariment of at risk
neonates was 3.8% and the incidence of moderate to severe deafness for the
cohort was 1.9 per 1000 live births. Auditory training was provided to all
severely hearing impaired infants before one year of age22.
V.K. Agrawal, Rakesh Shukla, P.K. Misra, R.K. Kapoor and G.K.
Malik,1998, Department of Pediatrics and Neurology, King George's Medical
~ 13 ~

College, Lucknow, determined the initial Brainstem Auditory Evoked Response


(BAER) abnormalities in neonates with hyperbilirubinemia. Seventeen out of
thirty (56.7%) neonates with hyperbilirubinemia showed abnormalities on
initial BAER. Abnormalities in BAER correlated significantly with bilirubin
level. After therapy abnormalities reverted back to normal in 10 cases but
persisted in 7 out of 17 (41.17%) cases with initial abnormal BAER.
Development screening at 1 yr was abnormal in 3 infants all of whom had
persistent abnormalities in BAER23.
Van Riper LA, Kileny PR., 1999, Department of Otolaryngology, Head
and Neck Surgery, University of Michigan Medical Center, USA, screened
2,103 newborns presenting with one or more risk indicators for significant
congenital hearing loss or delayed onset/progressive sensorineural hearing loss
to determine the outcome of a high-risk newborn auditory brainstem response
hearing screening program. One hundred fourteen (5.4%) infants were
diagnosed with bilateral hearing loss. Twenty-three infants (1%) presented with
unilateral hearing loss. Sixty seven (49%) of the 137 infants diagnosed with
hearing loss presented with greater than moderate hearing loss. Nine (13.4%) of
these 67 patients presented with delayed onset hearing loss that was diagnosed
at appointments subsequent to the initial screening. The largest percentage of
diagnosed hearing loss was found in the "craniofacial anomalies" category. It
~ 14 ~

was concluded that auditory brainstem response hearing screening of newborns


at risk for significant hearing loss is a clinically efficient and cost effective
approach to early detection of significant hearing loss24.
A Zamani, K. Daneshjou, A. Ameni and J. Takand, Department of
pediatrics, Imam Khomeini Hospital, School of Medicine Tehran university of
Medical Sciences performed a cross sectional study on 230 neonates who were
at risk of hearing loss between September 2000 and February 2002. Hearing
was examined before the 3rd month by auditory brainstem responses (ABR).
Eighteen neonates (8%) had sensorineural hearing loss24. They found significant
statistical relationships between hearing loss and craniofacial anomalies (P
value <0.000001), the neonates age during hospitalization (P value < 0.005),
hyperbilirubinemia (P value < 0.01), using artificial ventilation (P value < 0.05)
and use of ototoxic drugs (P value < 0.05)25.
Abdullah A et al, screened all the newborns delivered at Hospital
University Kebangsaan Malaysia over a nine-month period, between April to
December 2003, with a portable otoacoustic emission(OAE) before discharge.
At the age of two months, a second OAE test was repeated on newborns who
failed the initial test. Those who failed the second test were re-tested at three
months of age. When these infants failed the third OAE test, a brainstem

~ 15 ~

evoked response (BSER) test was performed. The prevalence of hearing loss in
this study was 0.42 percent (16/3,762)26.
Alwan M. Maisoun, Siraj M. Zakzouk, 2003, Security Forces Hospital,
Riyadh, screened one thirty newborns for hearing loss by using otoacoustic
emissions, tympanometry and ABR. Ninety-six of the infants had known risk
factors such as prematurely, low birth weight, hyperbilirubinemia, asphyxia and
congenital abnormalities. Out of 130 infants 80 passed the OAE test, 50 had to
be rescreened, and 19 had ABR. Only 13 were found with moderate to severe
hearing loss, 13.5% of at the risk infants27.
Pimol Srisuparp MD,Ruemporn Gleebbur RN, Sopapan Ngerncham
MD, Jintana Chonpracha RN, Jeeranan Singkampong RN ,2005, performed
hearing screening on all neonates admitted to the Division of Neonatology,
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol
University, who met the high-risk criteria according to Joint Committee of
Infant Hearing 1994, American Academy of Pediatrics. The infants were
screened with one-step protocol using an automated OAE/ABR device .Five
hundred and seven infants were identified to be at-risk in 18-month study
period. The prevalence of pathologic hearing screening test was 6.7% with
unilateral and bilateral pathologic results in 13 and 21 infants (2.6% and 4.1%).

~ 16 ~

Only craniofacial anomalies and mechanical ventilation > 5 days were shown to
be independent significant risk factors (42-fold and 4-fold increased risk)28.
M. AL-Harbi, N. Barakat and M. AL-Khandary,2006, screened one
hundred and five newborns with risk factors for hearing impairment at
Maternity Hospital-Kuwait from January 2006 to December 2006 . Forty nine
(46%) infants had pathological ABR, sixteen (15.2%) infants had unilateral
affection and 33 (31.4) infants had bilateral hearing affection. Forty (38%)
infants had pathological TEOAE, 15 (14.3%) infants had unilateral affection
and 25 (23.8%) had bilateral affection. Sepsis/meningitis and intraventricular
hemorrhage were independent significant risk factors for hearing impairment29.
P Nagapoornima, A. Ramesh et al, St. Johns Medical College Hospital,
Bangalore, 2006, performed screening on a cohort 1769 neonates by transient
evoked otoacoustic emissions. 1490 neonates were without any risk factors and
279 neonates were at risk for hearing loss. 3 out of 279 at risk neonates were
found to be hearing impaired, while 7 out of 1490 neonates had some degrees of
hearing loss30.
M John, A. Balraj, M Kurien undertook a cross sectional study to
evaluate the possible burden of hearing loss among neonates born at tertiary
hospital in southern India(Christian Medical College Vellore, Tamil Nadu) in

~ 17 ~

2009. Five hundred neonates were screened with automated distortion product
otoacoustic emission (aDPOAE) for hearing loss, 9.2% of whom had one or
more high risk factors. Although 6.4% had hearing loss at initial assessment,
only 1.6% had hearing loss on retesting with aDPOAE. Retesting with OAE
before an automated Auditory brainstem response (aABR) helped to exclude
patients without hearing loss. The frequency of moderate to moderately severe
hearing loss in this study was 0.6%. necessitating introduction of screening
programmes for deafness atleast in high risk newborns31.
R. G. Aiyer and Bhavin Parikh, 2009, recorded auditory brainstem
responses (ABR) 30 normal and 60 high-risk neonates with gestational age
between 30 and 45 weeks at Sayajirzo General Hospital and Medical College,
Baroda. The normative data of normal group as regard to age, sex and various
parameters of ABR were compared with high-risk group. 12 of the high risk
neonates showed mild to moderate hearing impairment (absent replicable wave
V at 3060 dB HL) and 2 of them showed severe to profound hearing
impairment (absent replicable wave V at 70 dB HL). 9 of the failed group
were reevaluated within 3 months and several times thereafter if the abnormal
responses persisted. 2 (3.3%) infants showed persistent hearing loss, which was
confirmed later by behavioral audiometry32.

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1. To estimate the incidence of neonatal hearing loss in high risk neonates


admitted in G B Pant Hospital.
2. To determine the risk factors predictive of hearing impairment in
neonates.

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A hospital based prospective study was undertaken in the department of


Pediatrics, Government Medical College Srinagar, at G B Pant Hospital, which
is a tertiary care referral hospital of Government Medical College Srinagar for
Children. The study was done over a period of one year from August 2009 to
July 2010. The sample size of the study group compromised of all the neonates
admitted in G B Pant Hospital and considered to be high risk patients for
hearing loss. The following were considered as risk factors3:
1. Family History of hereditary childhood sensorineural hearing loss.
2. Intrauterine infections (TORCH).
3. Craniofacial anomalies, including those with morphologic abnormalities
of the pinna and ear canal.
4. Birth weight < 1500 g.
5. Hyperbilirubinemia at a serum level requiring exchange transfusion.
6. Ototoxic medications, including but not limited to the aminoglycosides,
used for more than five days or multiple courses or in combination with
loop diuretics
7. Bacterial meningitis.
8. Apgar scores of less than four at one minute or less than six at fifth
minute.
9. Needing mechanical ventilation for more than five days.
10. Stigmata or other findings associated with a syndrome known to include
sensorineural and/or conductive hearing loss.

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Neonates with one or more of the above risk factors were screened
for hearing impairment using Brainstem Auditory Evoked Response (BAER)
before the age of 3 months using the Medlec Synergy (USA). The BAER
result is not affected by sedation or general anesthesia. Children less than
four months of age slept for long enough period of time after feeding to
allow a BAER to be done. For awake neonates, a 20mg/kg of triclofos was
given orally for sedating them. The morphology of the response and wave
and interwave latencies was examined in respect to age-appropriate forms.
An initial test using a stimulus intensity of 70 dB was done. Failure to
produce wave V indicated hearing impairment. If wave V was present,
repeated tests at sequential reductions of 10 dB established the hearing
threshold. Intensity of 30dB was taken as normal threshold for wave V 33.
Subsequently, the latency-intensity curve of wave V was studied, in addition
to V-l interpeak interval. In sensorineural hearing impairment the latencyintensity curve of wave V shifted to the right and the slope became steeper 33.
Follow up BAER after one month was performed only in those cases where
initial BAER was abnormal. Babies who tested abnormal on the follow-up
were referred for detailed audiology diagnostic work up.

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STATISTICAL ANALYSIS
The data obtained was tabulated and the variables were analysed for
their association with the outcome by applying the Fishers exact test, Chi~ 22 ~

square test, correlational analysis and calculation of p-value and Odds ratio.
The statistical package for social sciences (SPSS) software program 10.0 and
GraphPad Instat were used.

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