INT J LANG COMMUN DISORD, MAY–JUNE 2014,

VOL. 49, NO. 3, 333–342

Research Report
Early hearing loss and language abilities in children with Down syndrome
Glynis Laws† and Amanda Hall‡§
†Department of Experimental Psychology, University of Bristol, Bristol, UK
‡Centre for Hearing and Balance Studies, University of Bristol, Bristol, UK
§University Hospitals Bristol NHS Foundation Trust, Bristol, UK
(Received December 2012; accepted November 2013)

Abstract
Background: Although many children with Down syndrome experience hearing loss, there has been little research
to investigate its impact on speech and language development. Studies that have investigated the association
give inconsistent results. These have often been based on samples where children with the most severe hearing
impairments have been excluded and so results do not generalize to the wider population with Down syndrome.
Also, measuring children’s hearing at the time of a language assessment does not take into account the fluctuating
nature of hearing loss in children with Down syndrome or possible effects of losses in their early years.
Aims: To investigate the impact of early hearing loss on language outcomes for children with Down syndrome.
Methods & Procedures: Retrospective audiology clinic records and parent report for 41 children were used to
categorize them as either having had hearing difficulties from 2 to 4 years or more normal hearing. Differences
between the groups on measures of language expression and comprehension, receptive vocabulary, a narrative task
and speech accuracy were investigated.
Outcomes & Results: After accounting for the contributions of chronological age and nonverbal mental age to
children’s scores, there were significant differences between the groups on all measures.
Conclusions & Implications: Early hearing loss has a significant impact on the speech and language development
of children with Down syndrome. Results suggest that speech and language therapy should be provided when
children are found to have ongoing hearing difficulties and that joint audiology and speech and language therapy
clinics could be considered for preschool children.

Keywords: Down syndrome, hearing, language, vocabulary, speech.

What this paper adds?

What is already known on this subject?
Many individuals with Down syndrome have speech and language impairments. Around 40–80% of individuals with
Down syndrome also have hearing impairments. In young children the most common cause of hearing difficulties
is otitis media with effusion. Past studies provide inconclusive evidence for the effects of hearing loss but have often
excluded children with more severe hearing impairments from samples.

What this paper adds?

Children who have hearing difficulties from 2 to 4 years may go on to have lower scores on tests of receptive vocabulary,
language comprehension and expression, and speech accuracy than children who have no hearing difficulties. Clinical
implications are that speech and language therapy could be important from the first identification of ongoing hearing
difficulties in preschool children and that joint audiology/speech and language clinics could be considered.

Introduction is usually due to trisomy 21 when cells contain three

rather than two copies of chromosome 21. Nonverbal
Down syndrome (DS) affects around 1 in 1000 live IQs range from 30 to 70 (Glenn and Cunningham
births in the UK (Morris and Alberman 2009) and 2005) and the diagnosis carries risks for other

Address correspondence to: Glynis Laws, Department of Experimental Psychology, University of Bristol, 12a Priory Road, Bristol BS8 1TU,
UK; e-mail: glynis.laws@bristol.ac.uk
International Journal of Language & Communication Disorders
ISSN 1368-2822 print/ISSN 1460-6984 online  C 2014 Royal College of Speech and Language Therapists

DOI: 10.1111/1460-6984.12077
334
developmental delays and difficulties, including hear-
ing losses (e.g., Davies 1996). Most individuals with
DS also experience speech and language impairments
although the severity of these is variable (e.g., Abbeduto
et al. 2007). Since children with good hearing at the
time of a language assessment can nevertheless show ev-
idence of language impairments, one might conclude
that these are not attributable to hearing loss. The lan-
guage profile associated with DS is rather similar to
that of specific language impairment (SLI) in otherwise
typically developing children (Caselli et al. 2008, Eadie
et al. 2002, Laws and Bishop 2003, 2004). Hearing loss
is ruled out in the diagnosis of SLI and so it seems
plausible that linguistic deficits might also account for
language impairments in DS. However, given the na-
ture of the hearing difficulties associated with DS, it is
important to consider whether early hearing loss is a
contributory factor.
Hearing losses affect 40–80% of individuals with
DS (Dahle and McCollister 1986, Davies 1996, Marcell
and Cohen 1992, Roizen 1997). In young children, the
most common cause is conductive loss due to episodes
of ear infection and otitis media with effusion (OME).
For example, a recent prospective study of a community
based sample reported a prevalence of OME in children
with DS of 93% at age 1, remaining at 68% by age
5 years (Barr et al. 2011). In around half of these cases
OME was associated with severe conductive hearing
impairment with a small percentage treated with hearing
aids or grommets (pressure equalisation tubes).
When researchers studying language development
in DS have measured hearing, it has either been tested
directly, using pure tone audiometry to establish hear-
ing thresholds (e.g., Chapman et al. 1991), or indirectly,
using speech discrimination tasks (Jarrold and Baddeley
1997, Keller-Bell and Fox 2007). Neither approach takes
into account the fluctuations in hearing associated with
periods of OME or allows for children’s hearing histo-
ries to be considered as well as current hearing status.
Children with good hearing at the time of a language as-
sessment could have had hearing problems due to OME
when younger. Maturational accounts of language de-
velopment in DS suggest that maximum growth in lan-
guage takes place before the age of 7 years (Fowler 1990).
Persistent hearing losses throughout a critical period for
the acquisition of language (Lenneberg 1967) have po-
tential to interfere with this development.
Language development in DS
The development of language in DS has been widely
researched (for reviews see, for example, Abbeduto et al.
2007, Chapman 1997, Fowler 1990, Laws and Bishop
2004, Roberts et al. 2007, Rondal 1995, Tager-Flusberg
1999, Ypsilanti and Grouios 2008). The main features
Glynis Laws and Amanda Hall
are marked variation in the severity of impairments and
an uneven profile of strengths and weaknesses across
the language system. Atypical profiles emerge early in
development as language production fails to keep pace
with cognitive development (Miller 1999). Expressive
language continues more slowly than nonverbal men-
tal age (MA) throughout childhood and adolescence
(Chapman et al. 1991, 1998, Miller 1999). Receptive
vocabulary may develop in line with nonverbal MA
(Fowler 1995, Laws and Bishop 2003, Miller 1999,
Vicari et al. 2000) or in advance of that predicted by
nonverbal MA (Glenn and Cunningham 2005, Rosin
et al. 1988). However, most individuals develop poor
understanding and use of grammar (e.g., Chapman
et al. 1991, Fowler 1990, 1995, Gunn and Crombie
1996, Laws and Bishop 2003, Rosin et al. 1988, Tager-
Flusberg 1999). Syntax comprehension is poor and
slows, especially during adolescence (e.g., Abbeduto
et al. 2003, Chapman et al. 2002, Laws and Gunn
2004), and there are difficulties with the acquisition
of morphology (e.g., Chapman et al. 1998, Eadie
et al. 2002, Laws and Bishop 2003, Rutter and Buckley
1994).
In addition to language impairment, many individu-
als with DS have speech production difficulties and poor
intelligibility (Kumin 1994). Anatomical anomalies and
differences in speech-motor control contribute to articu-
lation difficulties (Miller and Leddy 1999). There is de-
bate about whether phonological development is simply
delayed (van Borsel 1996) or whether inconsistencies
in the production of speech sounds are indicative of
underspecified phonological representations (Dodd and
Thompson 2001). Although poor speech might be ex-
pected to be linked to language development, Cleland
et al. (2010) found no significant correlations between
the speech of children and adolescents with DS and their
language or cognitive abilities.
Relationship between hearing and speech and
language measures in DS
Systematic reviews of prospective studies have concluded
that OME has little to no long term association with
typical speech and language development (Roberts et al.
2004, Vernon-Feagans et al. 2003). It is unclear how
these results apply to children with DS but, in children
with existing risks to language development, the im-
pact of OME may be greater than when no other risks
are present (Vernon-Feagans et al. 2003). For example,
Paradise et al. (2000) found that OME contributed to
lower language abilities in typical development but only
in less advantaged children where home environment
was a risk factor for both OME and language. Other
risks to language development in individuals with DS
include the learning disability (Glenn and Cunningham
Early hearing loss and language abilities in children with DS
2005) and verbal short term memory deficits (Jarrold et
al. 2006).
Searches on PubMed and Europe PubMed Central
show no prospective studies to have tracked hearing
status alongside language development in a population
with DS. The children in Barr et al.’s (2011) prospective
study of hearing, described above, attended clinics held
jointly by audiology and education services but language
outcomes were not investigated. Roizen et al. (1993)
measured auditory brain stem responses of 47 children
with DS aged 2 months to 3.5 years and identified 38%
with normal hearing (NH) and 62% with unilateral or
bilateral hearing losses of varying severity (HI). A lan-
guage scale completed by parents showed no differences
between the children with NH and HI. However, given
the delays in language development associated with
DS, and the fact that 57% of the children were under
12 months, it was rather early to determine any effects
of hearing loss.
Most information on the association between
hearing and speech or language comes from stud-
ies where the main focus has been on establishing
syndrome-specific language features (e.g., Barnes et
al. 2009, Caselli et al. 2008, Dodd and Thomp-
son 2001, Chapman et al. 1991, 2000) or where
hearing has been measured as a control vari-
able in experiments (e.g., Jarrold and Baddeley
1997). Some studies have excluded individuals with
hearing loss to avoid masking the effects of the syn-
drome or to ensure that children can complete study
measures. The exclusion criteria adopted by researchers
has varied, which could partly explain the inconsistency
of evidence for the effects on language development. Se-
lective samples also mean that the results of studies do
not generalize to the wider population with DS.
Some studies have restricted samples to individu-
als with good hearing. Barnes et al. (2009) compared
phonological accuracy and speech intelligibility in boys
with fragile X syndrome to that for boys with DS aged
4 to 16 years who had passed a hearing screen at 25
dB. Mean phonological accuracy in connected speech
samples for the boys with DS was 70%, which was
significantly poorer than 89% accuracy achieved by a
comparison group of MA-matched typically developing
children. Keller-Bell and Fox (2007) also limited study
to children that passed a hearing screen at 25 dB. Speech
discrimination of 8 children with DS, aged 5 to 12 years,
was compared with that of nonverbal MA-matched typ-
ically developing children. The groups differed in the
discrimination of two of five speech contrasts. Only
one contrast contributed to variance on measures of
speech accuracy, vocabulary and mean length of utter-
ance (MLU) but these correlations were calculated across
all participants; the association in DS was not investi-
gated separately.
335
Studies that have retained children with mild HI and
excluded only those with more severe HI (that is with
hearing thresholds over 40 dB in the better ear) have re-
ported mixed findings. Abbeduto et al. (2003) compared
the receptive language skills of adolescents and young
adults with DS and fragile X syndrome and reported no
significant correlations between hearing thresholds and
receptive language scores. Miolo et al. (2005) also found
no association between hearing thresholds and receptive
vocabulary and sentence comprehension by adolescents
with DS. However, hearing thresholds did predict par-
ticipants’ use of grammatical morphemes. In other stud-
ies with the same inclusion criterion, hearing has made a
small contribution to variance in language scores (Chap-
man et al. 1991, 2000). Chapman et al. (1991) studied
48 individuals with DS, aged 5 to 20 years. Mean hear-
ing thresholds predicted 4% of the variance in receptive
vocabulary and syntax scores. In a similar group, Chap-
man et al. (2000) reported that mean hearing thresholds
predicted 6–8% of the variance in MLU derived from a
narrative sample.
Marcell and Cohen (1992) compared the hearing
capabilities of adolescents and young adults with DS
to those of individuals with other intellectual disabili-
ties. The study established that hearing difficulties were
more prevalent in the group with DS but found no as-
sociation between hearing and vocabulary and grammar
within the group. However, participants with no speech
and those who could not understand test instructions
had been excluded. If these difficulties were due to poor
hearing, the study could have underestimated any asso-
ciation in the wider population.
When hearing status has not been a selection cri-
terion, some effects of poor hearing on language are
apparent but results are still inconsistent (Cairns and
Jarrold 2005, Jarrold and Baddeley 1997, Laws 2004,
Laws and Gunn 2004). Jarrold and Baddeley (1997)
controlled for speech discrimination when studying the
short-term memory of children and adolescents with
DS. Discrimination scores were significantly correlated
with receptive vocabulary but there were no signifi-
cant correlations with expressive vocabulary or verbal
comprehension. Cairns and Jarrold (2005) also found
no significant correlations between speech discrimina-
tion and word or nonword repetition. Laws (2004) in-
vestigated the relationship between hearing thresholds
and MLU in 30 adolescents with DS. There was no
significant correlation between average hearing thresh-
olds and MLU but participants with average thresh-
olds above 39 dB in the better ear had not provided
intelligible narratives and were excluded from the anal-
ysis. Across the whole study sample, average hear-
ing thresholds were significantly correlated with recep-
tive vocabulary and word repetition (Laws and Gunn
2004).
336
In sum, the evidence for a link between hearing loss
and speech and language difficulties is inconsistent. This
is partly explained by the exclusion of individuals with
worse hearing from some study samples. The inclusion
of individuals with more severe HI in research samples
seems more likely to reveal a link between hearing and
language progress in this population. However, it is also a
possibility that any relationship depends on the effect of
hearing losses experienced when children were younger,
which is not captured by concurrent measurement of
hearing and language at later ages.
Outline of the study
This study used retrospective collection of audiology
clinic data and parent report to investigate the associa-
tions between children’s hearing histories from age 2 to 4
years and their speech and language abilities. Language
assessments were available from two ongoing studies that
had shared measures of expressive language and com-
prehension, receptive vocabulary, and speech accuracy.
Parents completed questionnaires about their children’s
hearing and gave consent for researchers to contact audi-
ology clinics to obtain children’s records. This approach
was taken as it is recommended that all children with
DS have regular audiological assessments, which should
be at least yearly for the first 5 years of life (Down Syn-
drome Medical Interest Group 2007). Audiology test re-
sults and clinic observations, supplemented by parents’
questionnaire responses, were used to group children in
terms of early hearing status. Speech and language abil-
ities of children who had had hearing difficulties from
ages 2 to 4 years were compared with those who had
had either no hearing loss or mild fluctuating hearing
loss.
Method
Participants
Fifty-one children with DS had been recruited to one of
two ongoing studies of language and reading develop-
ment. Three children were excluded early on; one moved
to a special school (mainstream education was an inclu-
sion criterion for school-age children), and two were
withdrawn by parents concerned about them missing
school for assessments. Of the remaining children, 42/48
(82.4%) parents gave consent for audiology records to
be accessed by researchers (six parents did not return
an additional consent form). Children attended audi-
ology clinics across the South of England and South
Wales (Bristol, Gloucester, Bath, Portsmouth, Newport
and Cardiff ). Audiology services were contacted and
hearing records were obtained for 39 of the 42 children.
Although audiology data were not returned by one NHS
Glynis Laws and Amanda Hall
Trust (Cardiff ), parents of two children from this centre
provided information. Data were unobtainable for one
other child due to missing audiology notes. Results are
based on audiology and language data for 41 children
(15 boys).
Parents confirmed that children spoke English as
their first language and had received no additional diag-
noses of autism or other neurodevelopmental disorders.
Most parents reported a diagnosis of trisomy 21 but two
girls had mosaicism and one boy was reported to have a
translocation.
Measures
Nonverbal ability was assessed using the Leiter Inter-
national Performance Scale—Revised (Roid and Miller
1997). This test minimizes the effects of hearing difficul-
ties or speech production problems because the exam-
iner uses pantomime to demonstrate the tasks and the
child is required to give only nonverbal responses. Four
subtests provided a brief IQ score. MA equivalent scores
(Leiter MA) were calculated for use in the analyses.
Hearing information came from two sources. (1)
Parents’ questionnaires provided information about:
children’s current or past hearing problems; whether
any hearing loss was considered to be mild, moderate
or severe; whether the diagnosis was conductive hear-
ing loss, sensori-neural hearing loss or a combination
of diagnoses; details about hearing aids and the fitting
and removal of any grommets; and the number of ear
infections that their child had experienced in each year
since birth, as far as they could remember. (2) Audiol-
ogy departments provided copies of all available hearing
tests performed, as well as a summary of all treatment
received (hearing aids or grommets). For each child,
the results of all hearing tests were examined. Due to
the fact that the hearing results were obtained from
retrospective clinical records, there was variation across
and within children in the number of tests performed,
the type of hearing test used, the frequencies measured
and the ages at which the tests were performed. It was
therefore not possible to generate a standard measure of
hearing history directly comparable across children. A
pragmatic approach was therefore taken as follows. The
average hearing threshold (binaural or better ear results,
across the frequency range assessed) was calculated for
each test. If the results were noted to be unreliable or
supra-threshold they were excluded. For each child the
hearing threshold data were plotted according to age ob-
tained and the pattern of the hearing results from 2 to
4 years of age was used to categorise hearing history. Data
across this age range were selected because the availabil-
ity of reliable data from year 1 was patchier. Results were
categorised as:
Early hearing loss and language abilities in children with DS 337
r Satisfactory history of hearing: hearing thresholds Table 1. Mean (SD) CA at the time of language assessments,
consistently less than 30 dB HL over the number Leiter MA and language test results (N = 41)
of tests measured.
r History of moderate or worse hearing loss: hearing Mean SD Range
CA (months) 77.46 22.62 45–133
thresholds consistently over 40 dB HL, or grom- Leiter Brief IQ 63.78 14.46 42–95
mets/hearing aids in situ, over the number of tests Leiter MA (months) 43.37 9.16 24–62
measured. RDLS Comprehensiona 33.71 12.39 4–55
r History of mild/fluctuating hearing loss: hearing RDLS Expressiona 13.71 8.39 0–29
BPVS IIa 30.83 13.89 2–66
thresholds between 30 and 40 dB HL over the age
BPVS IIb 40.80 11.58 28–78
range measured or hearing thresholds fluctuating MLUw 4.42 1.85 1–7.14
between normal and mild/moderate levels over Goldman–Fristoec 48.45 26.09 0–97
the age range measured or hearing loss present Notes: a Raw score.
but only one or two tests measured. b
Age equivalent (months).
c
Percentage consonants correct (PCC).
Goldman–Fristoe N = 31; MLUw N = 15 (of 24 children offered the task); CA =
Language assessments took place in a quiet room in chronological age; MA = mental age; RDLS = Reynell Development Language Scales;
children’s schools or in a room provided by the parent BPVS II = British Picture Vocabulary Scale, Second Edition; MLUw = mean length of
utterance in words.
support group except for two children assessed at home
by parents’ request. Although children were drawn from
two separate studies, the same test procedures were fol- correctly and a point is awarded for each correct pro-
lowed. Assessors ensured that children who needed spec- duction. The percentage of sounds produced correctly
tacles had them in place and that children with hearing was used as a measure in the analyses.
aids had them switched on. Apart from this, assessors
were not aware of children’s hearing histories.
Procedures
Receptive vocabulary was assessed using the British
Picture Vocabulary Scale II (BPVS II; Dunn et al. 1997). Ethical approval was obtained from the Ethics Com-
For each item, the child is shown four pictures and asked mittee of the Department of Experimental Psychology,
to choose the picture that matches a word spoken by the University of Bristol and from Gloucestershire NHS Re-
examiner. Raw scores were used in the analyses because search Ethics Committee (REC). Additional REC ap-
some children fell outside the range of test norms so proval for requesting audiology data from NHS clinics
that raw scores could not be converted to standardized was obtained after submitting a Notice of Substantial
scores. Amendment. The Research and Development depart-
Language abilities were assessed using the Reynell ment of each NHS Trust also approved procedures. Chil-
Developmental Language Scales (RDLS; Edwards et al. dren were recruited through schools or parent support
1997) that provide a clinical assessment for children groups. All parents gave consent after being provided
aged 18 months to 7 years. The scales cover expressive with information.
language and comprehension, and include items prob-
ing vocabulary and grammar. Raw scores were used in
Results
the analyses.
Mean length of utterance in words (MLUw) was Table 1 describes mean CA and test results for all par-
derived from a narrative task offered to children taking ticipants at the time of the language assessments. Girls
part in one of the two main studies (n = 24). A researcher did better than boys on all language measures but the
went through the picture storybook Frog, Where Are differences were not statistically significant.
You? (Mayer 1969) to familiarize children with the story, Children’s scores increased with CA and Leiter MA
and then returned to the beginning and asked them (table 2). CA and Leiter MA were treated as covariates
to tell the story. Narratives were recorded, transcribed in the analyses, where appropriate.
and agreed by two researchers and coded according to
the conventions of the Systematic Analysis of Language
Early hearing difficulties and language abilities
Transcripts (SALT; Miller 2008). SALT software was
used to calculate MLUw. Two categorical variables were derived from the audi-
The Goldman–Fristoe Test of Articulation, Second ology data. First, children were grouped according to
Edition (GF2; Goldman and Fristoe 2000) was used as whether or not they had received treatment for hearing
a measure of speech accuracy. Children name a series difficulties (fitting of grommets or hearing aids) before
of pictures, each designed to elicit specific consonants the age of 4 years. Second, children were grouped ac-
or consonant clusters. The examiner decides whether cording to the severity of hearing loss experienced from
the target sound(s) in each word have been produced 2 to 4 years: mainly no hearing loss; mainly mild hearing
338
Table 2. Bivariate and partial correlations between CA, Leiter
MA and speech and language scores
CA (CA contro-
lling for
Leiter MA)
RDLS Comprehension 0.723∗∗ (0.576∗∗ )
RDLS Expressive 0.641∗∗ (0.337∗ )
BPVS II raw scores 0.691∗∗ (0.392∗ )
MLUw 0.582∗ (0.57∗ )
GF2 PCC 0.381∗ (0.166)
Note: ∗ p < 0.05, ∗∗ p < 0.01 (one-tailed).
loss or fluctuating hearing loss; or mainly more severe
or non-fluctuating hearing loss. These categories were
merged to provide a group with HI (N = 16) consisting
of children who had received treatment plus the chil-
dren who had recorded more serious hearing difficulties
from age 2 to 4 years whether or not they had been
treated. The remaining children formed a group with
NH (N = 25) consisting of children who had not re-
quired grommets or hearing aids, and all of whom had
either no hearing loss or no more than mild, fluctuating
loss from ages 2 to 4 years. There was no statistically
significance difference in the proportions of children
with HI from the two main study samples, chi-square
(1) = 0.79, p > 0.05. Table 3 describes the NH and HI
groups. There were no significant differences between
them in CA, nonverbal IQ, sex distribution, history of
heart defects or level of mothers’ education. It is worth
noting that one girl with mosaicism and the boy with a
translocation were placed in the HI group.
Table 4 describes the two groups’ nonverbal MAs
and scores on all language assessments.
Univariate analyses of variance (ANOVA) were used
to investigate group differences on all measures, treat-
ing CA and Leiter MA as covariates where indicated.
ANOVA of RDLS Comprehension scores, treating CA
Glynis Laws and Amanda Hall
as a covariate, confirmed significant main effects of CA,
F 1, 38 = 52.725, p = 0.000 ηp 2 = 0.581, and hearing
Leiter MA group membership, F 1, 38 = 17.139, p = 0.000, ηp 2 =
(Leiter MA contro- 0.311. ANOVA of RDLS Expressive scores, treating CA
lling for CA) and Leiter MA as covariates, showed no significant effect
0.536∗∗ (0.119) of CA, F 1, 37 = 3.929, p = 0.055, ηp 2 = 0.096, but
0.627∗∗ (0.291) there were significant effects of Leiter MA, F 1, 37 =
0.688∗∗ (0.333∗ ) 7.098, p = 0.011, ηp 2 = 0.161, and hearing group, F
0.739∗∗ (0.17)
0.459∗∗ (0.320∗ ) 1, 37 = 7.678, p = 0.009, ηp 2 = 0.172. ANOVA of
BPVS II scores, treating CA and Leiter MA as covari-
ates, showed significant main effects of CA, F 1, 37 =
5.532, p = 0.026, ηp 2 = 0.126, Leiter MA, F 1, 37 =
10.978, p = 0.002, ηp 2 = 0.229, and hearing group
membership, F 1, 37 = 9.351, p = 0.004, ηp 2 =
0.202. ANOVA of arc-sine transformations of propor-
tions of GF2 consonants correct, treating Leiter MA as a
covariate, confirmed that Leiter MA made a significant
contribution to variance, F 1, 28 = 7.613, p = 0.01,
ηp 2 = 0.214, and hearing group membership also con-
tributed significantly to scores, F 1, 28 = 6.832, p =
0.014, ηp 2 = 0.196.
To avoid the possibility of Type I error following
the application of multiple tests, p values were or-
dered and compared with modified p values follow-
ing the Holm–Bonferroni calculation (Holm 1979).
All reported effects remained statistically significant. In
sum, these analyses confirmed that the HI group had
significantly poorer scores than the NH group on all
measures.
Children from one of the studies contributing data
to this investigation had been offered a story narrative
task (n = 24). Of the eight children in the HI group
offered the task, only two could provide a narrative com-
pared with 13/16 children in the NH group, χ (1) =
7.2, p = 0.007. The narrative task depends on speech
production as well as expressive language abilities. The
two children from the HI group who managed the task

Table 3. Description of NH and HI groups

Variables NH (n = 25) HI (n = 16) t(39) p Cohen’s d

CA (months) 79.44 (23.00) 74.38 (20.85) 0.695 0.491 0.22
Leiter Brief IQ 63.36 (15.95) 64.44 (12.26) −0.230 0.819 −0.07

χ (1)
Sex of child
Boys 7 8 2.035 0.154 0.52
Girls 18 8
Heart defectsa
Yes 9 6 0.035 0.851 0.07
No 12 7
Maternal educationa
To 16 or 18 6 3 0.000 1 0.00
Beyond 18 years 16 8
Notes: a n = 34.
NH = children who had received no treatment and recorded no more than mild, fluctuating loss from 2 to 4 years; HI = children who had received grommets or hearing aids, or
recorded more severe hearing losses from 2 to 4 years.
Early hearing loss and language abilities in children with DS
Table 4. Mean nonverbal ability, language and speech accuracy unadjusted raw scores for children who had satisfactory hearing (NH)
or early hearing impairment (HI)
Variables NH group (n = 25)
Leiter MA 43.12 (9.10)
RDLS Comprehensiona 38.24 (10.73)
RDLS Expressiona 15.88 (8.68)
BPVS IIa 34.40 (14.13)
Goldman–Fristoeb 58.28 (21.36)
Notes: a Raw score.
b
Percentage consonants correct (PCC).
c
d.f. = 29.
MA = mental age (months); RDLS = Reynell Development Language Scales; BPVS II = British Picture Vocabulary Scale, Second Edition. For Goldman–Fristoe, n = 18 for NH
group, n = 13 for HI group.
did better on the speech and expressive language tests
than the other six children in the group. Mean per-
centages of consonants correct were 67.5 and 17.5, and
mean RDLS Expressive scores were 20.5 and 8.17, re-
spectively. Within the group of 15 children who had
provided a narrative, RDLS Expressive scores were sig-
nificantly correlated with MLUw, r (15) = 0.67, p =
0.007, but the correlation between Goldman–Fristoe
scores and MLUw fell short of statistical significance, r
(15) = 0.50, p = 0.06. After controlling for CA, Leiter
MA and Goldman–Fristoe scores, the partial correlation
between RDLS Expressive scores and MLUw was not
statistically significant, r (10) = 0.39, p = 0.22. Af-
ter controlling for CA, Leiter MA and RDLS Expressive
scores, the partial correlation between Goldman–Fristoe
scores and MLUw was close to zero, r (10) = 0.003,
p = 1.
Discussion
This study used retrospective audiological data to in-
vestigate the impact of early hearing difficulties on later
language abilities of children with DS. A total of 16/41
(39%) children had experienced more severe hearing
difficulties from 2 to 4 years. This is comparable with
the results of Barr et al.’s (2011) study where OME was
associated with significant hearing impairment in 47%
of cases at age 3 and in 38% at age 4, a small proportion
of whom were treated with grommets or hearing aids.
The speech accuracy and language test scores of chil-
dren with hearing difficulties from 2 to 4 years were sig-
nificantly below those for the group that had had NH at
this age. Leiter MA as well as CA accounted for signif-
icant variation in test scores, confirming low nonverbal
cognitive ability as a risk factor for language difficulties.
Once this variation was accounted for there remained
statistically significant group differences on measures of
language comprehension, expressive language, receptive
vocabulary and speech accuracy.
Mean speech accuracy in both study groups (58%
and 35% respectively) was below the mean of 71% re-
339
HI group (n = 16) t(39) p 95% CI Cohen’s d
43.75 (9.55) −0.212 0.833 −6.6 to 5.4 0.07
26.63 (11.74) 3.261 0.002 4.4−18.8 1.04
10.31 (6.86) 2.166 0.036 0.4−10.8 0.69
25.25 (11.84) 2.149 0.038 0.5−17.8 0.69
34.85 (26.64) 2.718c 0.011 5.8−41.1 0.87
ported by Barnes et al. (2009) for boys with DS with
good hearing. Participants from the NH group who
had provided a speech accuracy measure were some-
what younger than Barnes et al.’s sample (mean CA was
about 7.5 compared with 9.5 years), which may explain
their lower mean score. However, it seems likely that
early hearing difficulties have contributed to the addi-
tional deficits in speech accuracy evident for the HI
group.
This study could be seen as adding to the inconsis-
tent reports in the literature describing the relationships
between hearing and language abilities in DS. How-
ever, it is worth noting that no exclusions were made
on the basis of hearing so results are more representa-
tive of the population with DS than some other studies.
Also, basing our measure on children’s hearing from 2 to
4 years allowed us to investigate the effects of hearing dif-
ficulties experienced at a critical period for language de-
velopment. Whereas typically developing children may
compensate for early OME and hearing difficulties, this
may not be the case for children with DS given their
additional difficulties (Vernon-Feagans et al. 2003).
The group differences in speech and language have
clinical and practical significance. After allowing for CA
and MA, effect sizes were small but should be considered
in the context of understanding the language develop-
ment of children who are already significantly delayed
by other effects of the syndrome, including learning dis-
abilities. The mean receptive vocabulary age equivalent
for the children with HI was 7 months below that for
the group with NH. This might not seem very much
until one considers that the mean vocabulary age for
the NH group was already 3 years behind that expected
for CA. Practical significance of additional vocabulary
delay could be that children may struggle to read since
receptive vocabulary is a predictor of literacy in typical
development and in children with DS (Laws 2010).
A subgroup of school-aged children were offered a
narrative task. Most children with NH provided a story
narrative but only two of the eight children from the HI
group managed the task. Providing a narrative requires
340
adequate speech as well as expressive language abilities,
and scores for both abilities were significantly lower
in the HI group. Expressive language was associated
with MLUw but there was no significant association be-
tween speech accuracy and MLUw, and no statistically
significant correlation between expressive language and
MLUw once speech was taken into account. However,
given the small sample size and the lack of longitudinal
speech and language data, no safe conclusion could be
drawn about how these abilities contribute to MLUw.
Whatever the nature of the relationships within chil-
dren who could manage the task, the poor narrative
skills of children who had had early hearing difficulties
is of concern, especially since productive speech capa-
bility of individuals with DS is even more challenged by
conversation than by narrative (Chapman et al. 1998).
Vernon-Feagans et al. (2003) argue that, for typically de-
veloping children, pragmatic use of language may be at
risk from OME in the longer-term even if other aspects
of children’s language catch up.
Limitations and future research
There were no measured differences between the groups
that offer alternative explanations for the speech and
language differences between them. Children with more
cognitive difficulties find it more difficult to respond to
a hearing test and are therefore more likely to present
with a hearing loss because results are supra-threshold.
However, tympanogram results were consistent with the
findings, and we did not include results in the audio-
logical analysis when the audiologist had noted that
results were not threshold. There was also no significant
difference between the groups on the measure of non-
verbal cognitive ability. The categorization of hearing
difficulty was based on the available hearing test data.
In cases where there were not enough data available
to distinguish between severe or moderate hearing loss
or satisfactory hearing, the less severe categories were
used to avoid misclassifying a child with hearing loss.
This approach could have diluted the size of the effects
observed.
We did not measure hearing at the time of the lan-
guage assessments so we could not investigate possible
effects of hearing difficulties on the day, which would
have been useful given the inconsistent evidence from
other research. Future prospective study of children’s
OME and hearing alongside the development of speech
and language should allow researchers to differentiate
between the effects of past and current hearing loss. We
chose a priori to use the clinical cut-off of moderate or
worse to categorize hearing difficulties as ‘significant’.
Future research could focus on examining the level of
Glynis Laws and Amanda Hall
hearing loss that has an impact. It is worth noting that
the NH group included some children with mild or
less persistent losses so this study has not explored the
possible effects of these.
Conclusions and implications
The evidence that severe early hearing difficulties may
impact on children’s language development confirms
that language difficulties in children with DS should
not be attributed entirely to linguistic deficits such as
those found for children with SLI, nor to the learning
difficulties associated with DS. Linguistic and cogni-
tive factors are clearly important for the development of
children with DS since children who had NH also have
delayed language but early hearing difficulties may add a
significant burden. Options for treatment of hearing loss
include grommets or hearing aids but recent guidance
describes the evidence for the effectiveness of these treat-
ments in children with DS as weak (National Collabo-
rating Centre for Women’s and Children’s Health 2008).
Notably, in our study, treated children were included in
the group with hearing loss yet we still observed an effect
on language. The guidance acknowledges the particular
problems for assessment and management of OME in
children with DS and the need for research to evaluate
treatment outcomes.
The provision of speech and language therapy for
preschool children with DS is variable across the UK
and many families and parent groups employ their own
therapists. The results of this study suggest that speech
and language therapy services should be involved as soon
as a child is diagnosed with ongoing hearing difficulties.
To this end, holding joint clinics with audiologists could
be helpful (e.g., Pappas et al. 1994). These could pro-
vide the opportunity for parents to receive early advice
on supporting the speech and language development
of a child following the diagnosis of persistent hearing
difficulties. Joint working could also provide opportu-
nity for more informative, prospective, research to study
hearing and speech and language development in this
population.
Acknowledgements
This research was completed with the support of the Wellcome Trust
(grant numbers 07250 and 08/RPM/4351076520). Alison Fisher,
Stephanie Guillaume, Frances Lombard and Joanna Nye contributed
to language assessments. Philippa Hough contributed to data entry.
The authors thank the audiology staff who sent data: Adrian Dighe,
Janine Matthews, Elizabeth Midgley, Chris Till, Anne Thomas and
Alison Watson; and acknowledge the contributions of children, their
parents and teachers. Declaration of interest: The authors report
no conflicts of interest. The authors alone are responsible for the
content and writing of the paper.
Early hearing loss and language abilities in children with DS
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