AJA
Research Article
Understanding Hearing and Hearing Loss
in Children With Down Syndrome
Emily Nightengale,a Patricia Yoon,a Kristy Wolter-Warmerdam,a Dee Daniels,a and Fran Hickeya
Purpose: This study evaluated the prevalence of permanent
and transient hearing loss, the use of hearing aids as a
recommendation, and middle ear dysfunction in children with
Down syndrome (DS) through a large multiage and ethnically
diverse sample, using current audiologic testing practices.
Method: Retrospective analysis of data collected on
308 children with DS (168 boys, 140 girls; average age =
5.99 ± 4.88 years) who received an audiological evaluation
during 2013 as part of their medical care at a large pediatric
hospital.
Results: Permanent hearing loss was identified in 24.9% of
the children, among whom bilateral (75.4%) and conductive
(33.3%) hearing losses occurred most often. Of children
C
hildren with Down syndrome (DS) are at a higher
risk for hearing loss as compared with their typi-
cally developing peers. According to Centers for
Disease Control and Prevention data, 1.4 per 1,000 new-
borns and 5 per 1,000 children from 3 to 7 years of age
are identified as having hearing loss (Centers for Disease
Control and Prevention, National Center on Birth Defects
and Developmental Disabilities, Division of Birth Defects
and Developmental Disabilities, 2013a). DS is the most fre-
quently occurring chromosomal disorder and leading cause
of intellectual disability in the United States. With an inci-
dence of 1 per 691 live births, an estimated 6,000 infants
with DS are born in the United States each year (Parker
et al., 2010). Incidence of hearing loss in children with
DS is significantly greater, reported to be as high as 78%,
with a majority exhibiting conductive hearing loss. Conser-
vative estimates of congenital hearing loss in children with
DS range from 15%–20% (Tedeschi et al., 2015). As with
typically developing children, hearing loss can be associ-
ated with poor language and long-term developmental
a
Children’s Hospital Colorado and University of Colorado, Aurora
Correspondence to Emily Nightengale:
Emily.nightengale@childrenscolorado.org
Editor-in-Chief: Sumitrajit Dhar
Editor: Ryan McCreery
Received February 1, 2017
Revision received May 3, 2017
Accepted May 5, 2017
https://doi.org/10.1044/2017_AJA-17-0010
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with DS, 22%–30% experienced a transient hearing loss,
with a high incidence of middle ear pathologies from infancy
until early adulthood. There were no statistical differences
between ethnicity and permanent/transient hearing loss
diagnosis. Twenty-three percent were current hearing aid
users or had them recommended in a treatment plan.
Conclusions: The prevalence of hearing loss and abnormal
middle ear status is high in the pediatric population with
DS. Audiologic evaluations should follow the American
Academy of Pediatrics practice guidelines to monitor this
high-risk population, and amplification should be considered
as an appropriate intervention option if repeated audiologic
examinations reveal hearing loss.
outcomes (Austeng et al., 2013; Balkany, Mishke, Downs,
& Jafek, 1979; Dahle & McCollister, 1986; Lau, Ko, &
Cheng, 2015; Raut et al., 2011; Roizen, Wolters, Trent, &
Blondis, 1993; Shott, Joseph, & Heithaus, 2001). These
outcomes can be especially detrimental to a child with DS
who already has an existing developmental delay.
Abnormal middle ear status is much more common
in individuals with DS. Otitis media with effusion (OME)
has a reported prevalence of 20% in 2-year-olds (Paludetti
et al., 2012), but in children with DS a peak prevalence has
been reported at 1 year of age (67%–93%) and again at 6–
7 years of age (60%; Barr, Dungworth, Hunder, McFarlane,
& Kubba, 2011; Maris, Wojciechowski, Heyning, &
Boudewyns, 2014). Of patients with DS, 43% to 83% re-
quired pressure equalization tube placement to treat con-
ductive hearing loss secondary to middle ear effusion and
chronic ear infections (Park, Wilson, Stevens, Harward, &
Hohler, 2012; Shott et al., 2001).
The anatomical structure of the ear in children with
DS has some typical characteristics that may predispose
them to hearing deficits (Fausch & Roosli, 2015; Ramia,
Musharrafieh, Khaddage, & Sabri, 2014; Rodman & Pine,
2012). Ear canals tend to be stenotic, and hypotonia leads
to poor eustachian tube function. Conductive hearing loss
may be a result of cerumen impaction in stenotic ear canals,
recurrent middle ear effusion and otitis media, eardrum per-
foration, retraction pockets, cholesteatoma, abnormalities
Disclosure: The authors have declared that no competing interests existed at the time
of publication.
1
 of the mastoid, and abnormalities of the ossicular chain such
as the structure of the incudomalleolar joint and/or incudos-
tapedial joint (Fausch & Roosli, 2015). Sensorineural hear-
ing loss is also seen in this population, with current research
showing significant inner ear abnormalities on computed
tomography scans (Blaser et al., 2006). A narrow internal
auditory canal has been identified in nearly 25% of individ-
uals with DS. Malformations of the inner ear, primarily in
the vestibular organ, have been documented in approxi-
mately 75% of patients with DS (Intrapiromkul, Aygun,
Tunkel, Carone, & Yousem, 2012).
Treatment of hearing loss can be more complicated
in children with DS. Evidence suggests that pressure equal-
ization tubes are not as effective in treating OME in children
with DS as compared with age-matched, typically develop-
ing children (Iino, Imamura, Harigai, & Tanaka, 1999).
Children with DS are also at a higher risk for complications,
including persistent otorrhea and eardrum perforations due
to lack of a lamina propria layer in the membrane (Iino
et al., 1999; Ramia et al., 2014; Rodman & Pine, 2012; Shott,
2006). In addition, surgical management does not always
ensure return to normal hearing (Iino et al., 1999). A child
with DS and conductive hearing loss despite surgical man-
agement or one who is not a candidate for ventilation tube
surgery due to ear size, comorbidities, or parent refusal, for
example, may be managed with amplification to allow
consistent access to speech-language cues in order to sup-
port speech-language development.
Whereas prior studies identify hearing loss as a prob-
lem for children with DS, limitations in the literature exist
such as small participant numbers, data collection span-
ning many years, disparities in subject age range, lack of
ethnic or race information, outdated research with assumed
older audiologic testing, no report of using a test assistant,
and lack of a standardized definition for hearing loss with
potential for error in variables such as severity, frequencies
affected, laterality, and permanence (Austeng et al., 2013;
Balkany et al., 1979; Barr et al., 2011; Dahle & McCollister,
1986; Iino et al., 1999; Intrapiromkul et al., 2012; Lau et al.,
2015; Maris et al., 2014; Raut et al., 2011; Roizen et al., 1993;
Shott et al., 2001; Tedeschi et al., 2015). Recommendations
for intervention and habilitation are often lacking (Austeng
et al., 2013; Lau et al., 2015; Park et al., 2012; Roizen et al.,
1993; Tedeschi et al., 2015). Current literature has also not
examined the potential effects of ethnicity with regard to hear-
ing loss in children with DS. Ethnic, racial, and language
differences can affect a child’s access to care (Avila &
Bramlett, 2013; Burgos, Schetzina, Dixon, & Mendoza, 2005;
Flores et al., 2016; Weinick & Krauss, 2000). Prior research
has suggested that many occurrences of hearing loss are
due to a lack of early medical surveillance and aggressive
intervention in the population with DS (Manickam, Shott,
Heithaus, & Shott, 2016; Shott et al., 2001). Further investi-
gation is warranted to determine whether ethnicity affects
permanent or transient hearing loss in this population.
It is reported that only 5%–10% of the patient popu-
lation is in compliance with the longstanding American
Academy of Pediatrics (AAP) published practice guidelines
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for monitoring hearing acuity in children with DS (Herzig
et al., 2016). These guidelines recommend testing every
6 months until the age of 3 years, then annually through
childhood. Hearing loss from 2 to 4 years of age adds a
significant burden to speech-language development in chil-
dren with DS and has a particular long-term negative im-
pact beyond the course of the disease itself (Laws & Hall,
2014; Whiteman, Simpson, & Compton, 1986). Youth with
DS may suffer additional detrimental effects on cognitive
abilities such as poorer performance in sentence imitation,
language comprehension, speed of word processing, and
sensitivity to brief acoustic cues as compared with age-matched
peers with intellectual impairment (Marcell, 1995). Greater
compliance with these guidelines is important to ensure
optimal speech-language development.
This study analyzes clinic data for a large, diverse
population of children with DS, focusing on hearing loss
and interventions. Our investigation was developed to ad-
dress current gaps in the literature in order to improve
identification, long-term outcome, and clinical care. The
objective was to evaluate the prevalence of (a) permanent
and transient hearing loss, (b) hearing aid use as a recom-
mendation, and (c) middle ear dysfunction in children
with DS.
Method
Design
A retrospective analysis was performed of audiologi-
cal clinical data on 308 children with DS who were treated
at a large pediatric academic medical hospital in 2013.
Patients were identified and data were extracted from the
Sie Center for Down Syndrome/Audiology Clinic Database,
which was populated by two clinic documentation (doc)
flowsheet forms within the electronic medical record. Data
collected included results from audiometric testing, nor-
mal or abnormal findings, and testing related behaviors.
Patients were excluded from analysis if their audiologist
did not attempt frequency-specific audiologic assessment
(i.e., newborn hearing screens, hearing aid functional test-
ing, tympanometry only) or if their doc flowsheet was min-
imally complete, defined as information missing on 20%
or more of the variables. The study was approved by the
Institutional Review Board of Children’s Hospital Colorado
(CHCO), University of Colorado School of Medicine.
Participants
All participants (168 boys, 140 girls; mean age at
evaluation = 5.99 ± 4.88 years) met inclusion criteria of
having DS, having had at least one audiological test per-
formed at CHCO between January 1, 2013, and December
31, 2013, and having received care at the hospital between
the ages of 1 day and 22 years. Overall, 81.2% (n = 250)
of children in the study were also patients who had received
care in the Sie Center for Down Syndrome at CHCO. Our
population with DS (trisomy 21 nondisjunction = 95.8%;
translocation = 2.6%; mosiac = 1.6%) reflects the percentages
 seen in the genetics literature (Centers for Disease Control
and Prevention, National Center on Birth Defects and
Developmental Disabilities, Division of Birth Defects and
Developmental Disabilities, 2013b).
The 308 children underwent a total of 582 audiological
evaluations during the year. Overall, 64.5% of participants
were evaluated to comply with AAP guidelines for surveil-
lance or monitoring of hearing loss in children with DS, 26.3%
were evaluated due to suspected hearing loss or ear problems,
9.5% were seen for a postoperative audiologic assessment,
6.9% were evaluated for an intraoperative assessment, and
4.0% were seen for a hearing aid evaluation. Newborn
hearing-screen data were available for 160 participants;
77.5% (n = 124) passed this initial evaluation, 14.4% (n = 23)
were referred for both ears, and 8.1% (n = 13) were referred
for one ear. As reported in Table 1, study participants
mirrored the diversity of the hospital patient population.
Measures
Audiologic Evaluation
Hearing evaluation data were extracted from two clinical
doc flowsheets embedded in the hospital’s electronic medical
records. To classify results, standardized parameters were
established for the audiologists completing the doc flow-
sheets. The audiologic evaluation was categorized as normal,
essentially normal, abnormal, or inconclusive. To qualify as
a normal evaluation, a child’s responses had to be obtained
at 20 dB HL or better across the speech frequency spectrum.
Tympanometry
Tympanometry testing (226 Hz or 1000 Hz probe
tones) results were coded as normal or abnormal using the
Jerger classification system: A = normal eardrum move-
ment, B = flat tracing/restricted eardrum movement, C =
retracted eardrum movement, As = restricted/shallow ear-
drum movement, Ad = hypermobile eardrum movement,
abnormal–other (large ear canal volume suggesting ear-
drum perforation, abnormal with 1000-Hz probe tone),
pressure equalization tube, inconclusive, could not test, or
did not test (Jerger, 1970). For tympanograms with large
ear canal volumes, the results were placed either in the
pressure equalization tube category or in abnormal–other
(suggesting eardrum perforation).
Defining Hearing Loss
Hearing loss was divided into 11 response categories:
normal hearing, essentially normal hearing (responses at
25 dB HL), conductive hearing loss, conductive hearing loss
for at least one ear (normal unmasked bone conduction
results obtained), sensorineural hearing loss, mixed hearing
loss, mixed hearing loss for at least the better-hearing ear
(only unmasked bone conduction results obtained), auditory
neuropathy, unknown/inconclusive, could not test, and not
applicable/did not test. During the audiology evaluation pro-
cess, if air conduction responses/thresholds were obtained
at elevated levels suggesting hearing loss, then bone conduc-
tion testing was attempted. If bone conduction responses/
thresholds were obtained in the normal range or were better
than air conduction responses by 10–15 dB, then a conduc-
tive component was suspected.
Children were defined as having a permanent hearing
loss if they had a sensorineural component (greater than or
equal to 25 dB HL) in one or both ears or a conductive hear-
ing loss not related to middle ear effusion without medical
treatment (e.g., surgery, antibiotics). This category includes
children with persistent eardrum perforations or conductive
hearing loss in the presence of normal eardrum movement.
Unless previously identified, those with permanent conductive
hearing loss did not include children with Type B, C, or As
tympanograms at time of diagnosis. Additional options within
data tracking included in progress and cannot determine.
Procedure
Audiologic Evaluations
Audiology data entered into the electronic medical
record by audiologists as part of a clinic quality improvement

Table 1. Participant and hospital-wide patient comparison in 2013.

Audiological evaluation Overall patients with Down

study participants syndrome at hospital
Demographics n % n %

Gender
Male 168 54.4 877 54.1
Female 140 45.5 745 45.9
Race
White, non-Hispanic 149 48.4 807 49.8
Hispanic and/or Latino 104 33.8 493 30.4
Black, non-Hispanic 15 4.9 74 4.6
Asian 4 1.3 29 1.8
American Indian or Alaskan Native 2 0.6 3 0.2
Native Hawaiian/Pacific Islander 0 0.0 1 0.1
Other 9 2.9 39 2.4
More than one race 4 1.3 63 3.9
Unknown 21 6.8 113 7.0
Totals 308 100.0 1622 100.0

Nightengale et al.: Hearing and Hearing Loss in Children With DS 3

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 investigation were used. Hearing was assessed using one
or more of the following techniques depending on age and/
or developmental capabilities: behavioral observation, visual
reinforcement audiometry, conditioned play audiometry,
standard audiometry, natural-sleep auditory evoked poten-
tial (AEP) procedures, and sedated AEP procedures. Evalu-
ations occurred (a) in the audiology clinic, (b) in conjunction
with a child’s otolaryngology clinic appointment, (c) at bed-
side with the child as an inpatient, or (d) in the operating
room/procedure center. A test assistant was used for 75.1%
of the evaluations.
Testing results. Following each audiologic encounter,
the audiologist completed an electronic medical record
form describing the child’s evaluation and testing behavior.
Investigation guidelines regarding reporting were pro-
vided by the principle investigator to the audiology team
at CHCO. Information on the type of audiological test
and results, reason for evaluation, test reliability, and clini-
cal recommendations were included. Data were collected
on reported level of hearing loss (in the sound field and/
or one or both ears), hearing loss configurations (in the
sound field and/or one or both ears), completion of results
across the speech spectrum, tympanometry results for both
ears, and whether permanent hearing loss had been previ-
ously diagnosed for each ear. Essentially normal evaluations
included responses at 25 dB HL, and an abnormal evalua-
tion included responses 30 dB HL and greater. At times the
evaluations were inconclusive or could not be completed;
the specific reason was collected. Data were coded for later-
ality unless ear-specific testing could not be completed, in
which case sound field data were entered. Evaluation in-
cluded tympanometry testing at 226-Hz or 1000-Hz probe
tones. If the hearing evaluation was abnormal, the audi-
ologist included recommendations such as use of a hearing
aid. The primary investigator of this study reviewed and
verified data entered by participating audiologists to ensure
accurate completion.
Background information. A separate history/background
doc flowsheet was completed for each child and when a
child’s history had changed during a subsequent testing
encounter (i.e., pressure equalization tubes, hearing aids,
sedated AEP evaluations). This electronic form was pop-
ulated using electronic medical record review and captured
demographics, newborn hearing-screen results, history of
middle ear effusion, history of pressure equalization tubes,
history of natural-sleep AEP evaluations and/or sedated
AEP evaluations, use of hearing aids/amplification, and
other pertinent medical history. Information was entered
by a research assistant trained on audiological chart review.
Data Collection
Data from the doc flowsheets were then collected
and managed using Research Electronic Data Capture
(REDCap) tools hosted at the University of Colorado
(Harris et al., 2009). REDCap is a secure, web-based ap-
plication designed to support data capture for research
studies, providing (a) an intuitive interface for validated
data entry, (b) audit trails for tracing data manipulation
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and export procedures, (c) automated export procedures
for seamless data downloads to common statistical pack-
ages, and (d) procedures for importing data from external
sources. To ensure data accuracy, all audiological testing
and outcomes were reviewed by the principal investigator,
a board-certified audiologist with expertise treating and
working with children with DS.
Statistical Analysis
Descriptive statistics and chi-square tests were per-
formed on demographic and clinical characteristics in the
data set. Results are presented as mean ± standard devia-
tion, or percentage where appropriate. Chi-square tests
were run to determine the significant associations between
ethnicity and permanent or transient hearing loss. Data
were analyzed by the statistical package SPSS 23.0. All
analyses were conducted at an α-level .05 significance.
Children were omitted from an analysis if the variable of
investigation had an “unknown” or “missing data” value.
Results
Of the 308 children evaluated, 111 (36.0%) were
diagnosed with some degree of hearing loss in 2013. Of
these, 35 ears were sensorineural, 103 were conductive,
and 28 were mixed. Sixty-four children had inconclusive
results with available data.
Confirmed Permanent Hearing Loss
Descriptive statistics for characterizing confirmed
permanent hearing loss are presented in Table 2. Reduced
subject numbers were used in calculating this information
because the permanent hearing loss variable (yes or no)
was added several months after data collection began. Data
were collected for this variable on 229 unique patients. A
total of 24.9% (n = 57) of participants were defined as hav-
ing permanent hearing loss in 2013; the determination of
loss for 10.9% was unable to be identified with available
data (7% were still undergoing evaluation and 3.9 % had
inconclusive results).
Overall, 27.7% of non-Hispanic/Latino patients had
permanent hearing loss compared with 21.8% Hispanic/
Latino patients. A chi-square test for association was
Table 2. Incidence and characteristics of confirmed permanent
hearing loss.
Results n %
Laterality of permanent hearing loss, by patient
Bilateral 43 75.4
Unilateral 13 22.8
Unilateral and in progress on other 1 1.8
Type of hearing loss, by ear
Conductive 38 38.0
Sensorineural 34 34.0
Mixed 28 28.0
 Table 4. Tympanogram results.
conducted between ethnicity and presence of permanent
hearing loss. All expected cell frequencies were greater
Results n %
than 5. There was not a statistically significant association
between ethnicity and permanent hearing loss, χ2(1) = 1.367, Normal results—Type A (both ears) 110 35.3
p = .242. Abnormal results total 124 39.7
Bilateral abnormal results 60 19.2
Unilateral abnormal results 64 20.5
Pressure equalization tube 56 17.9
Transient Hearing Loss Did not test 9 2.9
Inconclusive 6 1.9
Presumed transient hearing loss, defined as children Could not test 3 1.0
with an abnormal hearing evaluation and at least one Abnormal type reported (by ear)
abnormal tympanogram but without confirmed perma- Abnormal—Type B 94 51.1
nent hearing loss, occurred in 29.7% (n = 51) of patients. Abnormal—Type C 46 25.0
Abnormal—Type Other 30 16.3
Again, no statistically significant correlation was found Abnormal—As 14 7.6
between ethnicity and transient hearing loss, χ2(1) = 1.529,
p = .216.
One hundred thirty-one children completed more
than one audiologic evaluation in 2013. Results from par- Hearing Aids
ticipants without a permanent hearing loss were compared Data were run to determine how many children did
over time to determine consistency between tests. Evalua- not have hearing loss confirmed but still had a hearing aid
tion results changed for 32.5% (n = 40) of the children recommended. Of the 308 participants, 55 (17.9%) patients
(Table 3). In this group, 22.1% of patients had at least one were current hearing aid users and 17 (5.5%) had hearing
normal and one abnormal evaluation. aids recommended by the end of 2013, including 12 patients
without confirmed permanent hearing loss.
Descriptive data were evaluated on the 57 children
Tympanometry Results with confirmed permanent hearing loss in at least one ear
Of the 308 children, 119 (38.6%) had a history of to determine hearing aid use/recommendations: 57.9% (n =
middle ear effusion, with the first indication presenting at 33) were current hearing aid users, 35.1% (n = 20) had
a mean age of 4.43 ± 5.61 years. Table 4 summarizes tym- hearing aids recommended, 5.3% (n = 3) were deemed to
panometry data for each patient in 2013. To capture not need a hearing aid, and recommendation or status
testing results details from the 2013 visits, only the most was unknown for one patient (1.8%).
recent data are included for patients who had more than
one test.
Changes in middle ear function were examined in the Discussion
91 children who had results from more than one tympano- When the Sie Center for Down Syndrome at CHCO
metry test in 2013 (Table 5). These data exclude patients opened in 2010, more referrals were made to the Audiol-
who had pressure equalization tubes and just one other ogy Department than any other hospital department, illu-
result. minating the dire need for audiologic involvement in the
As seen in Figure 1, children with DS experience OME care of this patient population. The purpose of this study
throughout childhood and into early adulthood. Data include was to better characterize hearing loss and management
results per ear reported by percentage of abnormal results in a large diverse population, utilizing recent patient data
and total sample number for each age category. Cases clas- that reflects current practice in the specialty of pediatric
sified as normal, pressure equalization tubes, inconclusive, audiology.
did not test, and could not test were excluded. We did not see a difference in overall prevalence of
Due to the frequent occurrence of middle ear pathol- hearing loss when utilizing a larger sample size compared
ogies, ear tubes are often recommended for children with with prior studies; however, differences in type of hearing
DS. One hundred eighty-one (58.8%) participants have had loss, both conductive and sensorineural, were observed.
a history of pressure equalization tubes from birth to current. Thirty-six percent of children in our study experienced

Table 3. Hearing evaluation results for unique patients with more Table 5. Tympanometry evaluation results for unique patients with
than one test. more than one test.

Results n % Results n %

Always abnormal 57 43.5 Always abnormal 35 38.5

Normal sometimes 29 22.1 Normal sometimes 30 33.0
Normal always 26 19.8 Normal always 24 26.4
Inconclusive with normal or abnormal 11 8.4 Inconclusive 2 2.2

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 Figure 1. Percentage of abnormal tympanograms per ear as a
function of age.
hearing loss, which is consistent with other recent results
at 34%–36% (Austeng et al., 2013; Lau et al., 2015; Raut
et al., 2011). Children with DS have a much higher inci-
dence of multiple types of hearing loss (sensorineural, con-
ductive, mixed) when compared with the general pediatric
population. Prior studies have reported a wide range of in-
cidence from 16%–72.2% for conductive hearing loss and
18%–27.8% for sensorineural hearing loss (Austeng et al.,
2013; Lau et al., 2015). Our study found a prevalence of
33.3% conductive, and 29.8% sensorineural hearing loss.
The usage of our larger sample size may provide a more
accurate view of the prevalence in different types of hear-
ing loss in this population. We were able to further charac-
terize this hearing loss as being permanent in 24.9% of
patients and transient in at least 29.7%.
Another significant finding was that of middle ear
dysfunction throughout the span of childhood into young
adulthood, not just in early childhood. This highlights the
need for close audiologic monitoring at routine intervals
throughout the entire span of childhood. Older studies had
inconsistent age ranges due to lack of available partici-
pants (Roizen et al., 1993). More recent literature, and this
study, have attempted to correct for this disparity in age
range. In line with the “2011 Clinical Report—Health
Supervision for Children With Down Syndrome” (Bull &
Committee on Genetics, 2011), we agree with the recom-
mendation of audiologic testing at least every 6 months
until the age of 3 years, and then annually through child-
hood for individuals with DS.
Almost all (93.0%) children with a permanent hear-
ing loss either were already hearing aid users or were rec-
ommended for a trial of amplification. For children with a
permanent hearing loss where no medical and/or surgical
treatments are available or recommended, hearing aids
should be turned to as a viable intervention without fur-
ther delay.
Seventeen percent of current hearing aid users or
those with hearing aid recommendations with DS did not
have a confirmed permanent hearing loss. In our analy-
sis, we came across a number of children (43.5%, n = 57)
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without permanent hearing loss who had repeated abnor-
mal evaluations. Only seven of these individuals were cur-
rent hearing aid users or had hearing aids recommended.
Given the concern for further developmental difficulties
secondary to hearing loss in this population, we would ad-
vocate a low threshold for amplification in children who
fail repeated hearing evaluations.
There were limitations to our study that are inherent
in any retrospective review. Because this was an observa-
tional analysis, we were not able to control for parameters
that may have affected outcomes such as different reporting
styles of multiple audiologists. Although all audiological
testing and outcomes were reviewed by a second board-
certified audiologist with expertise in treating and working
with children with DS, variability is inevitable. One might
speculate that children with more complex medical needs
will seek out services from a pediatric hospital, thus creat-
ing a population bias; however, all patients with DS meet-
ing inclusion criteria were included in the study. Though
this type of retrospective analysis could potentially be prone
to sampling error due to patients with existing known mid-
dle ear disorders, 90.0% of our participants were referred
for audiologic evaluation for AAP guideline monitoring or
due to suspected unconfirmed issues.
Further investigation will help to better serve this
population. Areas that may be helpful to focus on include
assessment of audiologic test behaviors and support for
consistent hearing aid use. These are lacking in the existing
literature and would provide valuable insight into the test-
ing expectations for various ages of children with DS and
help with programming to assist parents and caregivers
with hearing aid compliance.
On the basis of the results from our experience initi-
ating the Sie Center for Down Syndrome at CHCO, the
high percentage of 2013 referrals for Hispanic and Latino
patients for audiologic testing warrant further investigation
regarding audiology service accessibility. Additional infor-
mation is needed to determine whether a DS clinic can
provide an entryway for health access such as ear, nose,
and throat evaluations and procedures. Although statistical
significance was not found in variables associated with per-
manent and transient hearing loss, we did see a trend sug-
gesting that referred Hispanic and Latino children with DS
were less likely to have permanent hearing loss. To our
knowledge, this information has not been previously re-
ported in the literature. This discrepancy was less evident
between the two groups for transient hearing loss.
Children with DS are at a greater risk for permanent
and transient hearing loss as compared with their typically
developing peers. Long-term consequences of late or failed
diagnosis, particularly in this population, can significantly
affect quality of life, including school performance, speech
and language, behavioral challenges, community engage-
ment, and safety. Therefore, ongoing audiologic monitoring
and otologic management is highly recommended for this
group into adulthood. The “2011 Clinical Report—Health
Supervision for Children With Down Syndrome” provides
appropriate guidelines that are consistent with the findings
 from this investigation (Bull & Committee on Genetics,
2011). Our results indicate the strong need to educate pri-
mary physicians, parents, and teachers regarding the im-
portance of referral, early identification, and, when needed,
use and compliance of amplification to help these children
in their speech-language development, psychosocial devel-
opment, and educational achievement.
Acknowledgments
We would like to thank the Global Down Syndrome Foun-
dation and the Anna and John J. Sie Foundation for their finan-
cial support of research conducted at the Sie Center for Down
Syndrome at Children’s Hospital Colorado.
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