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International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086

Contents lists available at ScienceDirect

International Journal of Pediatric Otorhinolaryngology


journal homepage: www.elsevier.com/locate/ijporl

Delayed cochlear implantation in congenitally deaf children—identifying


barriers for targeted interventions
Steven A. Gordon, Susan B. Waltzman, David R. Friedmann *
Department of Otolaryngology-Head and Neck Surgery, New York University Grossman School of Medicine, New York, USA

A R T I C L E I N F O A B S T R A C T

Keywords: Objectives: Age of cochlear implantation (CI) is an important predictor of language development in those with
Delayed cochlear implantation congenital sensorineural hearing loss. Despite universal newborn hearing screening initiatives and the known
Early implantation benefits of early CI, a subset of congenitally deaf children continue to be evaluated for cochlear implants later in
Universal newborn hearing screening
childhood. This study aims to identify the barriers to early cochlear implantation in these children.
Barriers to care
Barriers to cochlear implantation
Methods: A retrospective review was conducted for all pediatric cochlear implants aged 3 years or older per­
formed at a single academic institution between 2013 and 2017. Children implanted before the age three, those
with a prior unilateral cochlear implant, and those with progressive or sudden hearing loss were excluded.
Variables included newborn hearing screen results, age at hearing loss diagnosis, time of initiation and docu­
mented benefit of hearing aids, age of implantation, pre/post-implantation evaluation scores, and reason for
delayed referral for cochlear implantation.
Results: Thirty-one patients were identified meeting these inclusion criteria. Twenty-one children were subject to
UNBS in the U.S. Fourteen of those children failed their newborn hearing screening. Average age at implantation
was 6.2 years. Four reasons were identified for increased age at cochlear implantation. Two categories represent
delays related to (1) Amplification continually prescribed even though the range of hearing loss and speech
development assessment suggests CI may have been more appropriate well before referral (N = 13) (2) Patients
were not subject to newborn hearing screening and/or timely diagnosis of their hearing loss (N = 8). In other
cases, patients were appropriately fit with hearing aids until evidence that they derived limited benefit and then
referred for CI (N = 8). Lastly, in a few cases, records were indeterminate with regards to the timing and
appropriate diagnosis of their hearing loss (N = 2).
Conclusion: Understanding the reasons for delayed cochlear implantation in congenitally deaf children might
allow the development of targeted interventions to improve outcomes. Specifically, those children who were not
referred before age 3 despite use of amplification with limited benefit offer one potential target population for
earlier CI.

1. Introduction child’s success with cochlear implantation, this delay in access to


auditory input can contribute to worse oral language outcomes, and
Cochlear implantation for children with congenital severe-profound negatively influence other domains including long-term academic per­
sensorineural hearing loss can facilitate the development of oral speech formance, social interaction [18], employment, and quality of life [19].
and language. Factors associated with improved speech perception Despite this knowledge, many children are still implanted after the
outcomes for children after cochlear implantation (CI) have been well age of 3. There are a number of possible contributing factors, perhaps
studied [1–3] and include age at implantation [4–7]. Studies [8–14] the most obvious of which is a delay in diagnosis such that children with
describe a “sensitive period” of maximal neuroplasticity of the central hearing loss are not identified until speech milestones are missed. To
auditory system [15–17] that occurs early in life with closure of the address this, a number of countries including the United States have
neural gate around three years of life. While many factors contribute to a implemented universal newborn hearing screening (UNBHS) to

* Corresponding author. Division of Otology, Neurotology and Skull Base Surgery Department of Otolaryngology – Head and Neck Surgery, New York University
Grossman School of Medicine, 530 First Avenue, Suite 7Q, New York City, NY, 10016, USA.
E-mail address: david.friedmann@nyulangone.org (D.R. Friedmann).

https://doi.org/10.1016/j.ijporl.2022.111086
Received 29 September 2021; Received in revised form 1 February 2022; Accepted 15 February 2022
Available online 22 February 2022
0165-5876/© 2022 Elsevier B.V. All rights reserved.
S.A. Gordon et al. International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086

facilitate earlier age at diagnosis and treatment of hearing loss. These


programs, mandated nationally but implemented on the state level over
the last 20 years, have demonstrated improved rates of detection and
early referral for audiological assessment, enrollment into early inter­
vention (EI) programs, and treatment [20–22]. While these programs
have helped children receive implants earlier in life, the average age of
CI in the United States for children is 3 years of age [23].
It is unknown which factors account for the observed variability in
age at implantation of children with severe-profound hearing loss. The
current study was undertaken to understand reasons for late-
implantation in children from a referral Cochlear Implant Center. The
longer-term goal of this work is to assist in identifying targeted in­
terventions for modifiable barriers.

2. Methods

A retrospective review was performed of all children who underwent


cochlear implantation at a single academic institution between the years
2013–2017 (N = 650). Based on age at implantation, we excluded all
children implanted before the age of three years, the defined critical
period for intervention for congenital hearing loss in children [4,6,7,17,
24]. For those children implanted after this critical period (n = 111),
individual chart abstraction was performed. Additional exclusion Fig. 1. Flow diagram of patient selection.
criteria were those patients who underwent sequential CI older than 3
years but received their first implant before this age. Also excluded were (1) Children were appropriately fit with hearing aids until evidence
those with documented progressive or sudden hearing loss, who were that they derived limited benefit and then referred for CI (N = 8).
appropriately evaluated for implantation based on the timing of these These children were implanted at an average age of 6.5 years
changes in their hearing (n = 81). The remaining cohort were implanted (range 3.5–12).
between 3 and 14 years of age (n = 31). 2) Amplification was continually prescribed even though the range
Demographic information as well as audiometric data were recorded of HL and speech development assessment suggests CI may have
including age at diagnosis, age at implantation, pre-implantation and been more appropriate well before referral (N = 13). This may
post-implantation speech perception scores using age-appropriate have been related to patient, family or provider factors as
speech perception testing including, Phonetically Balanced Kinder­ detailed below. The average age of cochlear implantation for this
garten Test (PBK) [25]; Glendonald Auditory Screening Procedure group was 6.3 (range 3.4–12.5).
(GASP) [26]; Multisyllabic Lexical Neighborhood Test (MLNT) [27]; (3) Patients not subjected to newborn hearing screening and/or
Lexical Neighborhood Test (LNT) [27]; AzBio [28]; The Hearing in Noise timely diagnosis of their hearing loss (N = 8) often because they
Testing in Children (HINT C) [29]; The Infant-Toddler Meaningful had emigrated from somewhere where this testing was not
Auditory Integration Scale (IT-MAIS) [30]; and The Early Speech available. The average age of at implantation was 5.5 years
Perception (ESP) test [31]. If available, information related to UNBHS (range 3.2–10.3 years).
outcomes were included. Age at hearing aid fitting were also noted. (4) Records were indeterminate with regards to the timing and
Detailed discussion with the audiologists involved in the care of these appropriate diagnosis of their hearing loss (N = 2). The two
patients elucidated the explicit factors related to the timing of children were implanted at ages of nearly 6 and 9 years old.
implantation.

3. Results
3.1. Delayed referral (GROUP 2)
Thirty-one children implanted after the age of 3 met our inclusion
criteria and were included for analysis (see Fig. 1). The individual pa­ Based on individual chart abstraction and discussion with the
tient demographics and outcomes are described in Table 1. Fourteen of treating audiologists, the children with delayed CI referral despite
the twenty-one children subject to UNBS reportedly failed their newborn limited demonstrable benefit from amplification was inferred to occur
hearing screening. Overall, 6 children were found to have cochlear related to three underlying factors—1) those related to family dynamics;
malformations—3 with Mondini malformation and an enlarged vestib­ 2) structural/socioeconomic; and 3) inadequate guidance from prior
ular aqueduct and 3 with cochlear nerve dysplasia. Nine children providers.
received simultaneous bilateral implantation and another five under­
went bilateral implantation in a sequential manner. For those obtaining 3.2. Patient/family factors
a single implant, there were 9 children with right-sided CI and 8
receiving a left. Twenty-two of the thirty-one implants were Cochlear In this cohort, the majority (7/13) of delays in cochlear implantation
brand, 8 Advanced Bionic, and 1 Med-El. Mean age at first hearing aid were a result of family hesitancy. For example, Patients 3 and 17 grew
was 2.8 years old (range 4 months–6 years). Overall, the mean age at up in conservative Middle Eastern and East Asian families. Parents
cochlear implantation in this cohort was 6.2 years old (range 3.1–13.5 initially denied their child’s diagnosis, contributing to the delayed im­
years). plantation. Specifically, Patient 3 failed her newborn hearing screening
Individual chart abstraction was performed to determine what fac­ and the family waited nearly two years before following up. She has
tors impacted on the age at implantation. In doing so, 4 themes were three older siblings with hearing loss that were similarly delayed in
identified: treatment. The family reported they were worried about the cultural
stigma associated with a diagnosis of hearing loss and ultimately
required multiple referrals for cochlear implantation.

2
Table 1

S.A. Gordon et al.


Individual Preoperative and Postoperative Assessment for Children with Delayed Cochlear Implantation After Age 3.
# Age Aided Age Implanted Laterality Abnormal Newborn Cata Preoperative Speech Testingc Post Operative Speech Post Operative Speech Testingc Post Operative Speech Testingc
(years) (years) Anatomyb Screen Testingc 1 year 2 years
3 months

1 4.5 5.0 BIL none 3 Severe to Profound Loss BIL mild HL, moved out of
state
2 4.5 6.2 BIL none 3 ESPT unable to complete past IT-MAIS 25/40
training
3 2 13.5 L then R fail 2 PBK words: CNC words:
R HA- 24%, L HA- 8%, B/L- 20% R CI-66%, L CI-60%, B/l-80%
HINT C SENT (noise) AZ BIO (quiet)
R HA- 36%, L HA- 0%, B/L- 34% R CI-94%, L CI-86%, B/l-98%
AZ BIO(noise)
R CI-81%, L CI-68%, B/l-79%
4 2.5 3.7 R none 3 GASP words: PBK-1/2 list:
R HA- 0%, L HA- 83% R CI - 48%, L HA- 56%,
MLNT words: B/l-74%
R HA: DNT, L HA- 76%, B/l- 87%
5 0.5 4.9 R fail 2 LNT words: LNT words: HINT C Sentences (quiet):
R HA-0% Bilateral- 84% R CI-80% Bilateral- R CI-94% Bilateral- 100%
84% HINT C Sentences (noise):
R CI-93% Bilateral- 94%
6 5.5 6.1 BIL EVA, Cochlear N unknown 3 could not perform the training ESPT: Cat 3 8/17
Deficiency subtest correct
7 6 6.1 R then L pass 4 LNT words: LNT words: CNC words:
(2017) Bilateral: 56% CI-64% Bilateral: 84% R CI-96%, L CI-88%, B/l-96%
HINT sent (quiet)
R CI-90%, L CI-96%, B/l-100%
HINT sent (noise)
3

R CI-94%, L CI-92%, B/l-96%


8 4 7.9 R pass 1 PBK words: PBK words Able to repeat LING sounds
R HA: 4%, L HA 32%, B/l- 52% R CI 0% “mom notes improvement and
HINT C (quiet) HINT-C (quiet) hearing from the CI time"
R HA: 0%, L HA 83%, B/l- 79% R CI 0%
9 3 8.2 R Mondini, EVA BIL fail 2 AZ BIO Quiet: AZ BIO Quiet:
R HA-0%, L HA-92%, B/l-88% R CI-83%, L HA-94%,

International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086


AZ BIO Noise: B/l-98%
R HA-0%, L HA-86%, B/l-87% AZ BIO Noise:
R CI-78%, L HA-91%,
B/l-99%
10 1 10.3 BIL none 3 ESPT Cat 1 ESPT: Cat 2 8/17 ESPT: Cat 2 4/12 correct
correct “Loves to wear device"
11 2.8 3.2 R none 3 IT-MAIS: 0/40 IT-MAIS: 25/40 IT-MAIS: 32/40
“always responds to name in quiet
and sometimes in noise"
12 none 3.5 BIL Mondini, EVA BIL pass 1 Unable to perform Little Ears Able to repeat LING sounds. Only
Auditory Questionnaire wearing 1.7 h on R and 1.0hrs on L
13 0.4 3.8 L fail 2 Unable to perform Little Ears ESPT: Cat 1
Auditory Questionnaire
14 3.8 4.1 L none 3 Unable to perform ESPT for pattern HINT C Sent (quiet):
perception R HA- CNT, L CI - 82%, B/L- 79%
HINT C Sent (noise):
R HA- CNT, L CI - 70%, B/L- 80%
15 3 4.7 BIL pass 2 MLNT Words: MLNT Words: HINT C SENT (quiet)
R HA- 20%, L HA- 27%, B/l- 29% R CI - 80%, L CI - 86%, B/l- 63% R CI - 92% L CI - 96%, B/L- 96%
HINT C SENT (noise)
R CI - 72%, L CI - 85%, B/L- 94%
(continued on next page)
S.A. Gordon et al.
Table 1 (continued )
# Age Aided Age Implanted Laterality Abnormal Newborn Cata Preoperative Speech Testingc Post Operative Speech Post Operative Speech Testingc Post Operative Speech Testingc
(years) (years) Anatomyb Screen Testingc 1 year 2 years
3 months

16 4.5 4.7 BIL Born 23 wks fail 2 BIL Profound No Follow-up


17 5 5.3 L fail 2 MLNT Words: MLNT Words: LNT words:
R HA- 71, L HA- NR L CI - 67%, R HA- 84%, L CI - 88%, B/L- 96%
18 2.4 5.6 BIL pass 2 HINT C SENT (quiet) HINT C SENT (quiet) AZ-BIO (quiet)
R HA- 32%, L HA- 42%, B/L- 56% R CI - 76%, L HA- 42%, R CI - 100%, L CI - 98%, B/L- 100%
B/L- 96% AZ-BIO (noise)
R CI - 95%, L CI - 94%, B/L- 99%
19 1.7 7.3 R fail 1 HINT C SENT (quiet) HINT C SENT (quiet)
R HA- 82% L HA- 70%, B/L- 98% R CI - 94% L HA- 96%,
HINT C SENT (noise) B/L- 100%
R HA- 17%, L HA- 49%, B/L- 80% HINT C SENT (noise)
R CI - 92%, L HA- 92%,
B/L- 100%
20 3 8.8 L fail 2 AZ-Bio (quiet) AZ-Bio (quiet) AZ-Bio (quiet) AZ-Bio (quiet)
R HA- 73%, L HA- 61%, B/L- 76% R HA- 70%, L CI - 68%, R HA- 67%, L CI - 84%, B/L- 89% R HA- 73%, L CI - 84%, B/L- 92%
B/L- 88% AZ-Bio (noise) AZ-Bio (noise)
AZ-Bio (noise) R HA- 68%, L CI - 87%, B/L- 93% R HA- 29%, L CI - 68%, B/L- 65%
R HA- 58%, L CI - 65%,
B/L- 84%
21 1.8 3.7 R then L (12/ fail 2 Profound loss b/l: IT-MAIS 0/40 MLNT Words HINT C SENT (quiet)
17) R CI-66%, L HA- 0% R CI - 96%, L CI - 88%, B/L- 100%
HINT C SENT (noise)
R CI - 98%, L CI - 84%, B/L- 98%
4

22 4 7.9 L none 4 MLNT Words: LNT Words (easy):


R HA- 47%, L HA- 40%, B/L- 46% R HA- 28%, L CI - 88%, B/L- 84%
23 4 10.0 L fail on L 2 AZ-BIO (quiet): AZ-BIO (quiet):
pass on R R HA-100%, L HA: 0%, B/l- 92% R HA-100%, L CI: 94%, B/l- 100%
AZ-bIO (noise): AZ-bIO (noise):
R HA-83%, L HA: 0%, B/l- 84% R HA-98%, L CI: 84%, B/l- 93%

International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086


24 1.5 3.1 L pass 1 ESPT unable to complete training HINT C SENT (quiet)
Unable to consistently repeat Ling R HA- 78% L CI - 94%, B/L- 92%
speech sounds or words HINT C SENT (noise)
R HA- 74%, L CI - 98%, B/L- 94%
25 0.5 3.4 L Hypoplastic cochlea fail 2 IT-MAIS 0/40 IT-MAIS 18/40
26 0.3 3.4 BIL L: CSF leak; Mondini fail 1 IT-MAIS 9/40 MLNT Words: HINT C SENT (quiet)
R: common cavity rarely responds to name in quiet, R CI - 27%, L CI - 73%, B/L- 63% R CI - 6%, L CI - 69%, B/L- 92%
never in nopise Common Phrases (quiet) HINT C SENT (noise)
R CI - 30%, L CI - 45%, B/L- 65% R CI- DNT, L CI - 60%, B/L- 86%
27 0.4 4.0 R EVA, Mondini fail 1 LNT Words (easy): AZ-BIO (quiet):
R HA- 56% R CI - 98%
LNT Phonemes (easy) AZ-bIO (noise):
R HA- 79% R CI - 97%
28 3 5.4 R none 3 CNT PBK words or phonemes on R Left USA post op
29 4.5 5.5 R unknown 2 ESPT: CAT 3 AZ-BIO (quiet):
MLNT Words: R CI - 97%
R HA- 33%, L HA- 13%, B/L- DNT AZ-bIO (noise):
R CI - 96%
(continued on next page)
S.A. Gordon et al. International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086

A similar reluctance was seen from the family of Patient 17, who had

Legend: BIL= Bilateral; EVA = Enlarged Vestibular Aqueduct; CSF= Cerebrospinal Fluid; R = Right; L = Left; HA= Hearing Aid; CI= Cochlear Implant; CAT= Category; IT-MAIS = The Infant-Toddler Meaningful Auditory
Integration Scale; GASP = Glendonald Auditory Screening Procedure; PBK= Phonetically Balanced Kindergarten Test; ESPT = The Early Speech Perception Test; LNT = Lexical Neighborhood Test; MLNT = Multisyllabic
a failed newborn hearing screening and then a subsequent ABR at 4
months indicating a profound loss in the left ear and a moderate loss in

R CI - 77%, L CI - 100%, B/L- 100%


R CI - 100%, L CI - 100%, B/L- 99%

R CI - 21%, L CI - 97%, B/L- 100%


R CI - 98%, L CI - 99%, B/L- 97%
the right ear. The parents chose not to pursue amplification for over four
Post Operative Speech Testingc years due to their perceived cultural stigma, believing hearing loss to be
an “unacceptable disability.” They eventually returned for care and
implantation after the patient began experiencing more difficulty
hearing in social and educational settings with evident speech delay.
AZ-Bio (noise)

AZ-Bio (noise)
AZ-Bio (quiet)

AZ-Bio (quiet)
Denial of a child’s diagnosis represents another explanatory theme
seen with the families of Patients 20, 21, and 29. Patient 20 failed his
2 years

newborn hearing screening, but was appropriately aided and enrolled in

Age appropriate speech testing determined by audiologist at time of preoperative and post-operative assessment. If blank, testing was not collected/performed or lost to follow up.
educational support services including speech and language therapy,
and assistive listening technology. Despite numerous early CI referrals,
he was not implanted until 8 years of age, because of the family’s belief
Post Operative Speech Testingc

that their child’s hearing would spontaneously improve. However, the


child had such a positive experience post implantation that his family
pursued early CI for their second child with profound hearing loss prior
to 12 months of age.
Similarly, patient’s 21 and 23 passed their newborn hearing
screenings, but were later diagnosed with severe to profound hearing
loss at age 12 months and 24 months respectively, when their parents
1 year

noticed they were not developing speech and language. Both patients
were immediately fit with bilateral amplification and referred for CI.
Post Operative Speech

Despite a recommendation for CI, the family of patient 21 waited


another 11 months prior to evaluation citing concerns about the safety
of surgery. The family never fully accepted the child’s hearing loss and
Lexical Neighborhood Test; HINT C= The Hearing in Noise Testing in Children; CNC= The Consonant Nucleus Consonant Word Test.

continually expressed a resolve that her hearing would suddenly


3 months
Testingc

improve with time. Likewise, the family of patient 23 delayed amplifi­


cation by 6 months followed by an additional year-long delay after
amplification prior to CI evaluation despite multiple referrals. While the
parents had two previous children diagnosed and appropriately treated
B/L- 81%

B/L- 79%

B/L- 98%

B/L- 92%

for hearing loss, they were unable to process the reality of the diagnosis
Cata Preoperative Speech Testingc

of a third child with the same condition.


R HA- 59%, L HA- 59%,

R HA- 67%, L HA- 73%,

R HA- 93%, L HA- 70%,

R HA- 65%, L HA- 72%,

Socioeconomic/structural factors in the child’s environment were


the key influence in delayed implantation for Patients 16 and 23. Child
16 was born at 23 weeks and had a prolonged six month NICU stay at an
HINT C (noise)

HINT C (noise)
HINT C (quiet)

HINT C (quiet)

outside hospital contributing to his profound hearing loss. Despite this


history, he was not formally diagnosed with bilateral profound hearing
loss until nearly three years of age, he was not aided until he was four,
and then implanted at almost five years of age. Much of this delay was a
result of the child being moved between family members in different
1

cities with heavy involvement and support by social services. His follow
up is limited because he left the New York region immediately after
Newborn

implantation.
Screen

pass

A similar dynamic was seen with Patient 23 who failed her newborn
fail

hearing screening in the left ear, but passed in the right. She was born
and had all of her care at the public city hospital affiliated with our
institution. Her parents reported they had no concerns about her hearing
and didn’t follow up with ENT or audiology after her newborn screening
Anatomyb
Abnormal

Descriptive Category related to delayed implantation.

until she started Pre-K at age 4. At this point, she was formally diagnosed
with profound hearing loss in the left ear and moderate hearing loss in
the right ear and fit with a hearing aid only in the right ear. In this re­
view, she was categorized as delayed management because of the sig­
Laterality

R, then L

nificant interval prior to amplification fitting, however, she was


Anatomy considered normal if blank.

appropriately transitioned from amplification to CI.


L

3.3. Delayed referral by providers


Age Implanted

There were four children—Patients 9, 13, 15, and 25—whose


(years)

treating providers delayed referral of the child for CI long after they met
11.1

12.0
Table 1 (continued )

criteria. For instance, Child 9 was diagnosed with profound loss on the
right and moderate hearing loss on the left from an ABR at age 3 and on
Age Aided

imaging found to have bilateral enlarged vestibular aqueduct (EVA) and


(years)

dysmorphic cochlea. Some providers may be reluctant to refer when one


31 0.5
30 3

ear is doing well even with a “threatened contralateral ear.” Per chart
#

review and discussion with our audiologists, there was no hearing


b
a

5
S.A. Gordon et al. International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086

change or identifiable event that resulted in the CI referral. Similar investigation into barriers to access and utilization to pedi­
A similar delay was seen for child 15. He was born full-term and atric hearing healthcare have been designed to examine the impact of
passed a newborn hearing screening. Hearing loss was ultimately diag­ broader social disparities on the care of children with hearing loss
nosed between the ages of 2–3 years after two surgeries for myr­ [38–47]. For instance, Bush et al. have identified rural zip code and
ingotomy and tympanostomy tubes. He attained expressive language at distance from cochlear implant centers as significant barriers to hearing
age 2.5 years but had continued difficulty with speech, resulting in a healthcare [38–41]. There has been conflicting data regarding insurance
formal diagnosis of hearing loss at age 3 when he was fit with amplifi­ status and barriers to hearing rehabilitation with a group from Case
cation bilaterally. His provider did not refer him for over 1.5 years for CI Western showing that socioeconomic status and Medicaid insurance did
despite meeting criteria. not impact age of cochlear implantation, however, they did note that
Child 13 and 25 had a provider/system failure that contributed to children with Medicaid insurance missed significantly more
their delayed implantation. Both children failed their newborn hearing post-operative follow up visits [42]. In contrast, a recent study analyzing
screening, had subsequent ABR testing suggestive of bilateral profound delayed cochlear implantation in the state of Florida found children with
hearing loss, and were found to have congenital inner ear anomalies on Medicaid were significantly more likely to be implanted after the age of
follow up CT scan. Patient 13 had marked bilateral cochlea dysplasia, 2 compared to children with private insurance [44]. Moreover, this
narrowed internal auditory canals, and absent semi-circular canals. This group determined that children who identified as either Black or His­
child was not implanted until nearly four years old because she was panic were significantly more likely to have delayed implantation
initially denied coverage for implantation by her insurance carrier. compared to their White counterparts [44].
Child 25’s CT scan demonstrating bilateral hypoplastic cochlear and the The current study takes a more in-depth examination into delayed
family was incorrectly told by the provider that the child was not a cochlear implantation and identified four main categories of patients
candidate for CI because of his anatomy. who were implanted after the age of three years, excluding those with
progressive or sudden hearing loss as mentioned above. The children
3.3.1. Newborn hearing screening not performed (GROUP 3) who had amplification continually prescribed even though loss range
An overview of those patients not subject to infant hearing screening was on the range for CI; and those patients who arrived in the US later in
because they were born outside the U.S. is described in Table 2. Five out life seeking medical care—not subject to infant screening and timely
of eight of these patients are children that had the potential to be helped diagnosis, offer opportunities for intervention. In this cohort, the ma­
sooner after moving to the US and implanted earlier, had they been jority (7/13) children had delays in care as a result of family/internal
properly referred. The remaining three patients were appropriately factors. Cultural norms, family stigma, and parental acceptance of
diagnosed and referred for evaluation of implant candidacy in a timely diagnosis had major impacts in how these children and families inter­
manner and were implanted within one year of arrival to the U.S. acted with the health care system. Providers must incorporate this
knowledge and develop the necessary cultural competency to under­
4. Discussion stand the rationale for family resistance to implantation or denial of
diagnosis in order to help properly navigate these at-risk children
In order to develop proper language skills, children require access to through the medical system.
acoustic-phonetic cues [32–36]. However, children born with severe to Similarly, for those not subject to infant screening because they were
profound hearing loss lack access to this information, which can limit born outside the U.S., we lack a safety net to ensure these children are
their communication skills and hinder their development of spoken accurately diagnosed and appropriately managed in a timely fashion. In
language [37]. Cochlear implants, when performed within the critical or many cases, such children remain in the United States, and in addition to
“sensitive period” for neuroplasticity—typically before age 3—can the personal consequences on their development, there are important
potentially mitigate many of these adverse consequences. While the downstream consequences on their educational and vocational oppor­
need for early cochlear implantation in children with prelingual hearing tunities and potential economic burden on society [48]. While the
loss has been well established, many children are continually implanted introduction of newborn hearing screening programs has helped to
after this “sensitive period.” This study attempts to understand and accelerate detection of children with profound bilateral SNHL, as this
categorize barriers to early cochlear implantation in order to identify review demonstrates, early treatment is hampered by the complex de­
targeted solutions to mitigate delayed implantation in the future. livery of hearing health care.

Table 2
Timeline of hearing healthcare interaction among children born outside the US and not subject to universal newborn screening (category 3): From birth to cochlear
implantation.
Patient # Native County Hearing Loss Newborn screen Age at Age at Diagnosis Age at Hearing Aid Age at
Arrival to US Implantation

1 Nigeria Severe to Profound Bilaterally No 4 years 2 years 4 year 5 yearsa


6 months
2 Yemen Severe to Profound Bilaterally No 1 year 4 years 4 years 6 years 2 monthsa
6 months 6 months
4 Congo Right Profound No 2 years 2 years 2 years 3 years 7 monthsb
Left Moderate 6 months
6 China Severe to Profound Bilaterally No 5 years 5 years 5 years 6 years 1 monthsa
6 months 6 months
10 Honduras Severe to Profound Bilaterally No 7 years 1 year 1 year 10 years 4monthsa
11 India Right Profound No 8 months 2 years 2 years 3 years 2 monthsb
Left Severe 8 months
14 Guyana Severe to Profound Bilaterally No 2 Years 2 years 3 years 4 years 1 monthsb
6 months 8 months
28 Canada Right Profound No 5 Years 3 years 3 years 5 years 4 monthsb
Left Severe (left only)
a
Bilateral Simultaneous Cochlear Implantation.
b
Unilateral Cochlear Implantation.

6
S.A. Gordon et al. International Journal of Pediatric Otorhinolaryngology 155 (2022) 111086

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implantation in the spoken language development of children with severe to
after birth but before closure of the critical period independent of
profound hearing loss, J. Speech Lang. Hear. Res. 50 (2007) 1048–1062.
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