language disorders in children with congenital hypothyroidism
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Language Disorders in Language Disorders in Children with congenital Children with congenital
hypothyroidismhypothyroidism
Abd El Baky, A,A, Abd El Baky, A,A,
Mammdoh, HM.,Mammdoh, HM.,
Zaky EA,Zaky EA,
Abd El Wahab, MM,Abd El Wahab, MM,Unit of Phoniatrics, Department of Unit of Phoniatrics, Department of Otolaryngology, Otolaryngology, Minia UniversityMinia University..
The thyroid hormones are a major physiological regulator The thyroid hormones are a major physiological regulator
of the brain development. Cell differentiation, migration of the brain development. Cell differentiation, migration
and gene expression are altered as a consequence of the and gene expression are altered as a consequence of the
thyroid hormone deficiency or excess. thyroid hormone deficiency or excess.
Thyroid hormone deficiency when established during the Thyroid hormone deficiency when established during the
critical period of neuronal differentiation produces critical period of neuronal differentiation produces
permanent and severe alteration in the anatomy and permanent and severe alteration in the anatomy and
function of the nervous system (function of the nervous system (cretinismcretinism) ) ((Bernal & Bernal &
Nunez, 1995).Nunez, 1995).
Congenital hypothyroidism Congenital hypothyroidism (CH)(CH) is the deficiency of is the deficiency of
thyroid hormone. Which may be severe and its thyroid hormone. Which may be severe and its
symptoms develop in early weeks of life, in others, symptoms develop in early weeks of life, in others,
lower degree of deficiency occurs, and manifestations lower degree of deficiency occurs, and manifestations
may be delayed for months may be delayed for months (Dessault & Walker, 1993).(Dessault & Walker, 1993).
The prevalence of congenital hypothyroidism is The prevalence of congenital hypothyroidism is
1/40.0001/40.000 infants worldwide infants worldwide ..
Congenital hypothyroidism is Congenital hypothyroidism is twicetwice as common in girls as common in girls
as in boys as in boys (Delange, 1989)(Delange, 1989) . .
Thyroid hormone deficiency does not cause major Thyroid hormone deficiency does not cause major
changes in the gross anatomy of the brain, but it changes in the gross anatomy of the brain, but it
impairs the cytoarchitechture of the neocortex inimpairs the cytoarchitechture of the neocortex in
the cerebellum the cerebellum ( ( Glorieux et al, 1988).Glorieux et al, 1988).
Also, there are changes in the cortical patterns of Also, there are changes in the cortical patterns of
lamination and axonal projection lamination and axonal projection ((Bargagna, 1989).Bargagna, 1989).
Several steps of the brain development seem Several steps of the brain development seem
to be slow down by the deficiency of thyroid to be slow down by the deficiency of thyroid
hormones whereas glial cell formation (gliosis) hormones whereas glial cell formation (gliosis)
and the neuronal cell death are increased. and the neuronal cell death are increased.
T3 regulates the level of neurotrophine to T3 regulates the level of neurotrophine to
promote the development of the cerebral promote the development of the cerebral
cortex cortex (Noven & Arenes, 2006).(Noven & Arenes, 2006).
Magnetic resonance imaging was Magnetic resonance imaging was
reported that most patients with CH reported that most patients with CH
had mild cerebral cortical atrophy in had mild cerebral cortical atrophy in
the frontal and parietal lobes and the frontal and parietal lobes and
identified a delay in myelination in identified a delay in myelination in
patients with untreated congenital patients with untreated congenital
hypothyroidism hypothyroidism (Alves et al., 2004).(Alves et al., 2004).
The neonatal screening programs and early
treatment of CH has been effective in
preventing the serious brain damage and
mental retardation; it is still controversial
whether these patients achieve normal
cognitive skills and language development
or not (Heyerdal et al, 1991).
Also, with early recognition and treatment of CH, with early recognition and treatment of CH,
these children are slower in development than the these children are slower in development than the
normal children. they concluded that congenital normal children. they concluded that congenital
hypothyroidism affect the brain development hypothyroidism affect the brain development
before birth. Some of these effects last after birth before birth. Some of these effects last after birth
even if the replacement therapy is given regularly even if the replacement therapy is given regularly
since birth.since birth.
Glorieux et al, (1988)Glorieux et al, (1988) studied the mental studied the mental
development of the hypothyroid infants and they development of the hypothyroid infants and they
found that there was no difference between the found that there was no difference between the
global quotient in the 12 months. But at age of 18 global quotient in the 12 months. But at age of 18
months and 36 months, the hypothyroid group had months and 36 months, the hypothyroid group had
scientifically lower mean global quotient than the scientifically lower mean global quotient than the
control group. This was mainly in the performance control group. This was mainly in the performance
as well as the in hearing and language aquestion. as well as the in hearing and language aquestion.
Also Also Fuggle et al (1988)Fuggle et al (1988) reported that reported that
congenital hypothyroidism may be associated congenital hypothyroidism may be associated
with hearing and language impairment. with hearing and language impairment.
The problems in receptive language, auditory The problems in receptive language, auditory
processing and reading may persist in processing and reading may persist in
hypothyroid children, particularly if their hypothyroid children, particularly if their
treatment with l-thyroxin is delayed into the treatment with l-thyroxin is delayed into the
third week of life. third week of life.
On psychometric tests, the hypothyroid On psychometric tests, the hypothyroid
group was observed to perform group was observed to perform
scientifically poorer than the normal child scientifically poorer than the normal child
at 3 years of age but seemed to catch up at 3 years of age but seemed to catch up
by age 5 and did not differ on an by age 5 and did not differ on an
expressive language test given at ago of 7 expressive language test given at ago of 7
years years ((Rovet et al,1992Rovet et al,1992).).
Aim Of the workAim Of the work
The objective of this study is to The objective of this study is to
estimate the size of language disorder estimate the size of language disorder
and detect the factors influencing and detect the factors influencing
language acquisition in the children language acquisition in the children
with congenital hypothyrodism. with congenital hypothyrodism.
Subjects and MethodsSubjects and Methods This study included This study included 6060 children. children.
TheThe( ( study groupstudy group)) included included 4040 children who complained from children who complained from
congenital hypothyrodism. This group were collected from el congenital hypothyrodism. This group were collected from el
Minia Insurance Hospital –Pediatric unit. The range of age of Minia Insurance Hospital –Pediatric unit. The range of age of
this group from this group from 3 to 83 to 8 years. years.
The results from the study group were compared to another The results from the study group were compared to another
group group ((control groupcontrol group)), which included , which included 2020 normal children . The normal children . The
range of age of this group from range of age of this group from 3 to 83 to 8 years Children of both years Children of both
groups were matched in the age and sex.groups were matched in the age and sex.
subject
G1G1: the children with congenital : the children with congenital
hypothyroidism with controlled level T3 and hypothyroidism with controlled level T3 and
TSH .TSH .
G2G2: the children with congenital : the children with congenital
hypothyroidism with uncontrolled level T3 and hypothyroidism with uncontrolled level T3 and
TSH.TSH.
G3G3: the children with normal thyroid function.: the children with normal thyroid function.
MethodsMethods
Each child of the 3 groups was Each child of the 3 groups was
subjected to the protocol of language subjected to the protocol of language
assessment, which applied in assessment, which applied in The The
Unit of Phoniatrics-El Minia University Unit of Phoniatrics-El Minia University
Hospitals.Hospitals.
ResultsResults
70 68
71
10
20
30
40
50
60
70
80
90
100
G1 G2 G3
Non-statistical significant difference was obtained between G1 , G2 and G3 as
regard the age (P<0.05).
Age with months
8
14
5
13
119
0
2468
101214161820
G1 G2 G3
male
female
Non-statistical significant difference was obtained between G1 , G2 and G3 as regard sex (P<0.05).
I.QI.Q
7265
82
50
60
70
80
90
100
G1 G2 G3
A statistical significant differences were obtained between
G1 and G2 as regard the I.Q (P>0.05).
A high statistical significant differences were obtained
between G1 and G3 as regard the I.Q (P>0.05).
Mental ageMental age
62
43
75
20
30
40
50
60
70
80
90
100
G1 G2 G3
A statistical significant differences were obtained between G1
and G2 as regard the mental age (P>0.05).
A high statistical significant differences were obtained
between G1 and G3 as regard the mental age (P>0.05).
Age with months
Social ageSocial age
58
38
70
20
30
40
50
60
70
80
90
100
G1 G2 G3
A statistical significant differences were obtained between G1 and G2 as regard the social age (P>0.05).
A high statistical significant differences were obtained between G1 and G3 as regard the social age (P>0.05).
Age with months
Language ageLanguage age
43
23
57
10
20
30
40
50
60
70
80
G1 G2 G3
A statistical significant differences were obtained between G1
and G2 as regard the language age (P>0.05).
A high statistical significant differences were obtained
between G1 and G3 as regard the language age(P>0.05).
TSH levelTSH level
1.6
14.3
0.61
3
5
7
9
11
13
15
17
19
G1 G2 G3
A statistical significant differences were obtained between G1
and G2 as regard the TSH level(P>0.05).
A high statistical significant differences were obtained
between G1 and G3 as regard the TSH level (P>0.05).
T4 levelT4 level
1.20.6
1.8
0
1
2
3
4
5
G1 G2 G3
A statistical significant differences were obtained between G1
and G2 as regard the T4 level (P>0.05).
A high statistical significant differences were obtained
between G1 and G3 as regard the T4 level (P>0.05).
The audiological assessmentThe audiological assessment::
Non-statistical significantNon-statistical significant difference was obtained difference was obtained
between the two groups as regard the audiological between the two groups as regard the audiological
evaluation (P. evaluation (P. << 0.05). 0.05).
II: CorrelationsII: Correlations::
A high A high positivepositive significant correlation was obtained significant correlation was obtained
between the language , mental, social age and IQ in between the language , mental, social age and IQ in
correlation with the level of correlation with the level of T4T4 in G1 and G2 (r=0.76). in G1 and G2 (r=0.76).
A high A high negativenegative significant correlation was obtained significant correlation was obtained
between the language , mental, social age and IQ in between the language , mental, social age and IQ in
correlation with the level of correlation with the level of TSHTSH in G1 and G2 (r=-0.87). in G1 and G2 (r=-0.87).
DiscussionDiscussion
This study aimed at investigating the language This study aimed at investigating the language
developmental outcomes of the children with congenital developmental outcomes of the children with congenital
hypothyroidism (hypothyroidism (study groupstudy group) in comparison to the ) in comparison to the
children who with normal thyroid functional (children who with normal thyroid functional (control control
groupgroup). The two groups were matched as possible in ). The two groups were matched as possible in
their sociodemographic data (their sociodemographic data (age and sexage and sex) in order to ) in order to
show the effect of the hypothyroidism in the language show the effect of the hypothyroidism in the language
abilities of their children.abilities of their children.
In this study, there was In this study, there was significantsignificant difference difference
between the hypothyroid and normal thyroid between the hypothyroid and normal thyroid
functional children as regard the functional children as regard the IQ , mental and IQ , mental and
social agesocial age. Moreover , There was . Moreover , There was high high
significantsignificant difference between the controlled difference between the controlled
hypothyroidism and non- controlled hypothyroidism and non- controlled
hypothyroid functional children as regard the hypothyroid functional children as regard the IQ IQ
, social and mental age, social and mental age..
This result matched to This result matched to Glorieux et al, (1988)Glorieux et al, (1988) who who
found that, the hypothyroid group had scientifically found that, the hypothyroid group had scientifically
lower mean global quotient than the control group. lower mean global quotient than the control group.
They said that their results may represent the They said that their results may represent the
manifestations of brain damage occurs even with early manifestations of brain damage occurs even with early
detection and treatment of hypothyroidism. Also, detection and treatment of hypothyroidism. Also,
Koibuchi and Chin, ( 2000)Koibuchi and Chin, ( 2000) found that the children with found that the children with
severe CH, had mean IQ scores 10 to 22 points below severe CH, had mean IQ scores 10 to 22 points below
that of their siblings .that of their siblings .
AlsoAlso, Nacfau et al (2005), Nacfau et al (2005) found that the CH children found that the CH children
who were being treated for hypothyroidism starting who were being treated for hypothyroidism starting
before the age of before the age of 2 years2 years. Their IQ scores lay within the . Their IQ scores lay within the
normal range ( 71- 122; mean 92.4), but 77% showed at normal range ( 71- 122; mean 92.4), but 77% showed at
least one sign if impaired brain function. least one sign if impaired brain function. ClumsinessClumsiness
was found in 33%, was found in 33%, behaviour disordersbehaviour disorders in 23%, in 23%,
language disorderslanguage disorders in 20%, in 20%, learning disorderslearning disorders in 26%, in 26%,
squintsquint in 53%, in 53%, nystagmusnystagmus in 10%, and in 10%, and minor motor minor motor
disordersdisorders in 50%. in 50%.
In the other handIn the other hand, Heyerdal et al, (1991) , Heyerdal et al, (1991) found that found that
even with early recognition and treatment of even with early recognition and treatment of
congenital hypothyroidism, these children are slower congenital hypothyroidism, these children are slower
in development than the normal children. they in development than the normal children. they
concluded that congenital hypothyroidism affect the concluded that congenital hypothyroidism affect the
brain development before birth. Some of these effects brain development before birth. Some of these effects
last after birth even if the replacement therapy is last after birth even if the replacement therapy is
given regularly since birth.given regularly since birth.
The results obtained from the Arabic language test The results obtained from the Arabic language test
revealed a revealed a significant differencesignificant difference between the between the
hypothyroidism and normal thyroid functional children hypothyroidism and normal thyroid functional children
as regard the as regard the language agelanguage age. Moreover , There was . Moreover , There was high high
significantsignificant difference between the controlled difference between the controlled
hypothyroidism and non- controlled hypothyroid hypothyroidism and non- controlled hypothyroid
functional children as regard functional children as regard language agelanguage age. These . These
results are in agreement with those ofresults are in agreement with those of Glorieux et al, Glorieux et al,
(1988)(1988) , Fuggle et al (1988), Fuggle et al (1988) and and Rovet et al,(1992Rovet et al,(1992),),
Miller et al ( 2002)Miller et al ( 2002) revealed that the treated revealed that the treated
hypothyroid group had significant increased hypothyroid group had significant increased
verbal memory retrieval. Results suggest that verbal memory retrieval. Results suggest that
specific memory retrieval deficits associated specific memory retrieval deficits associated
with hypothyroidism can resolve after with hypothyroidism can resolve after
replacement therapy with levothyroxine.replacement therapy with levothyroxine.
But But our result not matched toour result not matched to Denman (1984)Denman (1984) who who
found that the CH group did not differ from control found that the CH group did not differ from control
children on tests of children on tests of phonological processingphonological processing
(WJRMT Word Attack), (WJRMT Word Attack), writingwriting (test of written (test of written
language ( TOWL), or language ( TOWL), or spellingspelling ( WRAT_R spelling). ( WRAT_R spelling).
The results of the supplementary tests also The results of the supplementary tests also
indicated no difference in indicated no difference in languagelanguage and and memorymemory
tasks.tasks.
Rovet et al., (1995)Rovet et al., (1995) indicate that the indicate that the
neuropsychological functioning of neuropsychological functioning of
children with congenital children with congenital
hypothyroidism (CH) is affected by hypothyroidism (CH) is affected by
factors associated with the disease factors associated with the disease
and its treatment. and its treatment.
Their nonverbal or visuospatial intellectual abilities Their nonverbal or visuospatial intellectual abilities
were sensitive to the were sensitive to the etiologyetiology of hypothyroidism, of hypothyroidism,
whereas several aspects of memory were sensitive whereas several aspects of memory were sensitive
to the to the timingtiming of thyrotropin (TSH) normalization. of thyrotropin (TSH) normalization.
With regard to With regard to etiologyetiology, children with athyreosis , children with athyreosis
scored significantly below the other groups on scored significantly below the other groups on
indices of nonverbal intelligence and arithmetic indices of nonverbal intelligence and arithmetic
achievement. achievement.
Cohen (1997)Cohen (1997) concluded that the important of the age at concluded that the important of the age at
normalization of TSH affected selective aspects of normalization of TSH affected selective aspects of
memory and attention functioning. Specifically, children memory and attention functioning. Specifically, children
with CH who normalized by with CH who normalized by I to 2 monthsI to 2 months of age, scored of age, scored
higher than those normalizing later on indices of higher than those normalizing later on indices of
immediate and delayed visual memory and attention. immediate and delayed visual memory and attention.
While, children who normalized by While, children who normalized by 3 months3 months of age of age
performed better than those normalizing past this age performed better than those normalizing past this age
on indices of global memory, visual memory and on indices of global memory, visual memory and
learning.learning.
The study revealed that, A high The study revealed that, A high positive significant positive significant
correlationcorrelation was obtained between the language , was obtained between the language ,
mental, social age and IQ in correlation with the mental, social age and IQ in correlation with the
level of level of T4T4 in the study group, Also, A in the study group, Also, A high negative high negative
significant correlationsignificant correlation was obtained between the was obtained between the
language , mental, social age and IQ in correlation language , mental, social age and IQ in correlation
with the level of with the level of TSHTSH in the study group. in the study group.
This result is matched to This result is matched to Kooistra et al Kooistra et al
(1995)(1995) showed Significant correlations showed Significant correlations
between the start of therapy and both between the start of therapy and both
motor scores and performance IQ scores at motor scores and performance IQ scores at
the age of 7 years in children with severe the age of 7 years in children with severe
hypothyroidism. hypothyroidism.
Also, Also, Soon and Song (2001)Soon and Song (2001) observed that children's observed that children's
thyroid hormone levels at the time of testing were thyroid hormone levels at the time of testing were
positively correlated with indices of memory, positively correlated with indices of memory,
language, speed of processing, and fine motor language, speed of processing, and fine motor
performance, whereas TSH levels were negatively performance, whereas TSH levels were negatively
associ ated with receptive language and recognition associ ated with receptive language and recognition
memory skills. memory skills.
Conclusion& RecommendationConclusion& Recommendation
There is evident that the children with congenital There is evident that the children with congenital
hypothyroidism show poorer language development hypothyroidism show poorer language development
in comparison to normal children, moreover the in comparison to normal children, moreover the
children with uncontrolled congenital children with uncontrolled congenital
hypothyroidism show worsen in language, mental, hypothyroidism show worsen in language, mental,
social age and IQ in comparison to that children with social age and IQ in comparison to that children with
controlled hypothyroidism and normal children.controlled hypothyroidism and normal children.
The need for diagnosis and treatment of children The need for diagnosis and treatment of children
with congenital hypothyroidism as soon after birth with congenital hypothyroidism as soon after birth
as possible, preferably no later than the second as possible, preferably no later than the second
week of life .it is also important to screen week of life .it is also important to screen
hypothyroid children for hearing problems and to hypothyroid children for hearing problems and to
provide remediation in language. provide remediation in language.
The present study provide several challenges for the The present study provide several challenges for the
behavioral pediatrician who may have children with behavioral pediatrician who may have children with
congenital hypothyroidism in his or her practice. It is congenital hypothyroidism in his or her practice. It is
important to monitor these children frequently and important to monitor these children frequently and
closely to ensure thyroid hormone levels closely to ensure thyroid hormone levels
maintained .maintained .
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