Accepted Manuscript

Title: Dystrophic changes in masticatory muscles, related chewing problems

and malocclusions in duchenne muscular dystrophy

Author: L. van den Engel-Hoek, I.J.M. de Groot, L.T. Sie, H.W. van Bruggen,
S.A.F. de Groot, C.E. Erasmus, N. van Alfen

PII: S0960-8966(16)30010-4
DOI: http://dx.doi.org/doi: 10.1016/j.nmd.2016.03.008
Reference: NMD 3165

To appear in: Neuromuscular Disorders

Received date: 10-1-2016

Accepted date: 31-3-2016

Please cite this article as: L. van den Engel-Hoek, I.J.M. de Groot, L.T. Sie, H.W. van Bruggen,
S.A.F. de Groot, C.E. Erasmus, N. van Alfen, Dystrophic changes in masticatory muscles, related
chewing problems and malocclusions in duchenne muscular dystrophy, Neuromuscular
Disorders (2016), http://dx.doi.org/doi: 10.1016/j.nmd.2016.03.008.

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Dystrophic changes in masticatory muscles, related chewing problems and malocclusions in
Duchenne muscular dystrophy

L. van den Engel-Hoek, PhD1

I.J.M. de Groot, MD PhD1
L.T. Sie, MD PhD2
H.W. van Bruggen, DDS PhD3
S.A.F. de Groot, SLT1
C.E. Erasmus, MD PhD4
N. van Alfen, MD PhD4

1
Donders Centre for Neuroscience, Department of Rehabilitation, Radboud university
medical center, Nijmegen, The Netherlands

2
Department of Pediatric Neurology, Juliana Children's Hospital/Haga Teaching Hospital, The
Hague, The Netherlands

3
Department of Cariology, Endodontology & Pedodontology Academic Centre for Dentistry
Amsterdam (ACTA), University of Amsterdam and VU University Amsterdam, Amsterdam,
The Netherlands

4
Donders Centre for Neuroscience, Department of Neurology, Radboud university medical
center, Nijmegen, The Netherlands

Corresponding author:
Lenie.vandenEngel-Hoek@radboudumc.nl
Radboud university medical center, Nijmegen,
Geert Grooteplein 10, 6525 GA Nijmegen, the Netherlands,
internal post 818 Phone: 0031-24 3615187
Fax 003124 361 98 39

Page 1 of 18
Abstract

Dysphagia in Duchenne muscular dystrophy (DMD) worsens with age, with increasingly

effortful mastication. The aims of this study were to describe mastication problems in

consecutive stages in a group of patients with DMD and to determine related

pathophysiological aspects of masticatory muscle structure, tongue thickness, bite force and

dental characteristics. Data from 72 patients with DMD (4.3 to 28.0 years), divided into four

clinical stages, were collected in a cross sectional study. Problems with mastication and the

need for food adaptations, in combination with increased echogenicity of the masseter

muscle, were already found in the early stages of the disease. A high percentage of open

bites and cross bites were found, especially in the later stages. Tongue hypertrophy also

increased over time. Increased dysfunction, reflected by increasingly abnormal echogenicity,

of the masseter muscle and reduced occlusal contacts (anterior and posterior open bites)

were mainly responsible for the hampered chewing.

In all, this study shows the increasing involvement of various elements of the masticatory

system in progressive Duchenne muscular dystrophy. To prevent choking and also nutritional

deficiency, early detection of chewing problems by asking about feeding and mastication

problems, as well as asking about food adaptations made are essential and can lead to

timely intervention.

Keywords

Duchenne muscular dystrophy

Chewing problems

Tongue hypertrophy

Malocclusion

Masticatory muscles

Page 2 of 18
1. Introduction

Duchenne Muscular Dystrophy (DMD) is a severe, progressive, X-linked neuromuscular

disorder caused by mutations in the dystrophin gene. The dystrophic changes are caused by

a disruption of the muscle fiber architecture, followed by replacement of myofibers by fat

and fibrosis. Most patients are diagnosed around the age of 5 years. The disorder is

characterized by hypertrophic calves and a deep lordosis, waddling gait and absence of

dystrophin in muscle biopsy [1]. Muscle strength deteriorates and ambulation becomes

increasingly difficult. Most boys require the use of a wheelchair before their teens [2]. With

further progression, muscles of the upper limbs, neck, heart and respiration also become

involved. Oral muscles become affected as well, causing dysphagia in the later stages of the

disease [3, 4]. Studies examining dysphagia in DMD have shown that dysphagia worsens with

age with increasingly effortful mastication reported by patients [5]. Mastication is defined as

the process of chewing food for swallowing and digestion with the crushing of solid food

between the occlusal surfaces of the post-canine teeth [6, 7]. Masticatory movements are

executed with the masseter, temporalis, medial and lateral pterygoid muscles, together with

the submental muscles, i.e. the digastric, mylohyoid and geniohyoid muscles [6]. The tongue

and cheeks are essential for manipulating food in the oral cavity. Diseased muscles due to a

neuromuscular disorder negatively influence oral functions and orofacial morphology, and

from these changes malocclusions such as anterior and posterior open bites or cross bite

may occur [8, 9].

A previous study by us showed increased tongue thickness in DMD, which initially occurs

without structural tissue changes and thus can be considered hypertrophy. In the advanced

stage of the disease, found hyperechogenic tongue muscles in combination with abnormal

Page 3 of 18
tongue thickness can be considered pseudohypertrophy [4]. Eckardt and Harzer reported

involvement of the masticatory muscles in DMD as well [10]. Other studies found a reduced

maximum bite force in older DMD patients [3, 9, 11], and showed the high prevalence of

malocclusions [8, 9, 12-15]. Together with significantly reduced mandibular movements

these factors are influencing masticatory performance in boys and young adults with DMD

[9, 16].

Masticatory difficulties (especially biting and eating hard and sticky food) are responsible for

decreased fragmentation of food and larger food chunks predispose patients to

complications like choking, asphyxiation or dysphagia caused by post swallow residue [4, 17,

18]. With a better understanding of the nature and clinical course of mastication problems in

DMD, related problems with oral food handling can be detected early and so possibly be

prevented or mitigated. In the study of van Bruggen et al. (2014) no information on structure

of masticatory muscles was collected [9]. To gain insight in the extent of these problems, we

started a new study to collect data in a cross-sectional study in boys and adults with DMD.

Complaints of mastication, the presence of malocclusions and maximum bite force were

assessed. In addition masticatory muscle structure and tongue thickness were measured.

The aims of this study were (a) to describe mastication problems in consecutive stages in a

group of patients with DMD; (b) to determine related pathophysiological aspects of

masticatory muscle structure, tongue thickness, bite force and dental characteristics

2. Patients and Methods

2.1 Patients

The study was conducted at the Radboud university medical center in Nijmegen, The

Netherlands, between March 2012 and January 2015. Data from 72 DMD patients were

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collected during their annual visit to the interdisciplinary outpatient clinic of the pediatric

and adult center for neuromuscular disorders. Only patients with an established diagnosis of

DMD were included. Patients who were totally depended on tube feeding were excluded.

The scores on the Motor Function Measure Scale (MFM) [19] and information on their motor

abilities were used to divide the patients in four clinical stages: early ambulatory stage (EAS),

late ambulatory stage (LAS), early non- ambulatory stage (ENAS) and late non-ambulatory

stage (LNAS) [1].

This study was approved by the Committee on Research Involving Human Subjects of

Arnhem and Nijmegen. Eligible patients and participants or, if they were <12 years their

parents, received an information letter. After signing the informed consent, they were

invited to participate in the study.

2.2 Procedures

2.2.1 Quantitative muscle ultrasound

In previous studies we have shown that structural oral muscle changes caused by

neuromuscular disorders can be detected by muscle ultrasound, and that quantitative

muscle ultrasound (QMUS) can be used to quantify the changes in muscle architecture [20].

QMUS was used to measure the echogenicity of the masticatory muscles (the masseter

muscle and temporalis muscle) and compared with our own laboratory reference values of

healthy participants in the same age range (see for graphical elucidation figure 1A and 1B).

All measurements were performed using a broadband linear 10–5 MHz transducer using a

Z.one convertible ultrasound system (Zonare Medical Systems; Mountain View, California).

Patients were measured while sitting with a neutral head and neck position, with a closed

mouth and relaxed cheeks. Images were stored as DICOM image files on a computer with a

Page 5 of 18
resolution of 800-600 pixels for offline analysis, with a dedicated Matlab program QUMIA

[21]. Three measurements were taken of the left muscles, and results were averaged offline

to minimize variation in echogenicity. The QUMIA software compared the data with

reference values and the result was described as a z-score (i.e., the amount of standard

deviations below or above the mean of normal values), taking into account the influencing

factors of age and weight. Tongue thickness, including the geniohyoid and genioglossus

muscles, was measured from the raphe of the mylohyoid muscle to the upper boundary of

the tongue following the protocol previously described [4, 20]. Tongue thickness data were

also compared with normal values and described as z-scores.

2.2.2 Maximum bite force

The maximum bite force was measured using the VU University Bite Force Gauge (VU-BFG).

The VU-BFG is a hand-held device which uses a load cell to measure maximal voluntary bite

force in kilograms, which are converted to Newton (N) [22]. Based on the study by Roldan et

al. we used the VU-BFG centrally between the incisors to obtain the maximal bite force

measurements [23]. Patients were asked to bite three times, with approximately one minute

between to rest. The highest (i.e. peak) value of each DMD patient was used as Maximum

Voluntary Bite Force (MVBF). Data were compared with the MVBF of 46 healthy participants

in the same age range (5 to 30 years divided into 4 age groups, which were comparable with

the mean age of the DMD stages).

2.2.3 Questionnaire

Information was collected on complaints on feeding and swallowing, using a semi-structured

questionnaire [4]. For this study the items on chewing and food modifications were selected

for further analysis. For the different items, see table 3.

2.2.4 Dental examination

Page 6 of 18
After the mastication surfaces of the upper and lower teeth were brought into contact, the

occlusal relationship was assessed regarding the absence or presence of posterior cross bite

and regarding the anterior and posterior occlusal contacts, i.e. the touching of opposing

teeth of mandible and maxilla (to score into normal, anterior open bite, posterior open bite)

[7] (figure 2). Because the aim of this study was the assessment of mastication anterior cross

bites were not included in the data collection.

2.3 Statistical analysis

Data were analyzed with SPSS 20.0 for Windows (SPSS Inc., Chicago, IL, USA). The level of

significance was set at p = .05.

One-way analysis of variance was performed to analyze differences between the four DMD

stage groups, in each of the following variables, separately: the mean z-scores of

echogenicity of the masseter and temporalis muscle, the mean z-score of tongue thickness,

and the mean MVBF. The independent variable was the DMD stage (EAS, LAS, ENAS, LNAS).

The estimated mean z-score and mean MVBF in each DMD stage (with the 95 % confidence

interval (CI)) is presented. Data of the MVBF per stage were compared with the data of the

healthy participants with a one sample T-test. Correlation between DMD stages and items of

the questionnaire on masticatory problems and food adaptation, and between DMD stages

and occlusal contacts and occlusal relationships were analyzed with Spearman’s rho.

Correlation between malocclusions and tongue thickness was analyzed with Spearman’s rho.

Multiple regressions were calculated to predict problems with mastication, based on the z-

score of the echogenicity of the masseter and temporalis muscle, the MVBF, occlusal

relationships and occlusal contacts.

Page 7 of 18
3. Results

Seventy-two patients participated in the study, divided according to the four stages: EAS (N =

13, age range 4.3 - 9.0 years ), LAS (N = 17, age range 7.6 - 19.3 years), ENAS (N = 26, age

range 8.9 - 20.6 years) and LNAS (N = 16, age range 15.6 - 28.0 years). The patient

characteristics are depicted in table 1.

Data of the muscle ultrasound measurements and bite force are depicted in table 2. There

was a significant effect of the DMD stage on the echogenicity of the masseter muscle,

(F(3,68) = 3.1, p = 0.03). The mean echogenicity of the masseter muscle showed a gradual

increase from normal (z-score < 2) in the AS, to abnormal in the consecutive stages

(respectively mean z-scores of 2.2, 2.4 and 3.3) (figure 1C and 1E for examples). There was

no significant effect of the DMD stage on the echogenicity of the temporalis muscle, (F(3,67)

= 2.9, p = 0.07). The mean echogenicity of the temporalis muscle was slightly increasing over

time, but even in the LAS the mean z-score remained < +2 (figure 1D and 1F for examples).

There was a significant effect of the DMD stage on tongue thickness (F(3,66) = 12.02, p =

0.024), with a gradual increase from normal values (z scores < +2) in the EAS and LAS to

mean abnormal values (z scores > +2) in the advanced stages.

There was also a significant effect of the DMD stage on the MFBV (F(3,63) = 3,36, p = 0.024).

A significant difference (ranging from p = 0.00 tot p=0.001) was found for all stages between

the MFBV data of the healthy participants and data of the DMD patients.

A significant positive correlation between DMD stages and items of the questionnaire on

masticatory problems (rs 0.53, p = 0.00) and items on food modification (rs 0.54, p = 0.00)

were found (table 3). More problems with mastication were found in the ENAS and LNAS

than in the AS and LAS. The need to cut small pieces of food, especially when eating meat,

was already reported in 32.1% in the EAS and in 50% in the LAS. In the advanced stages of

Page 8 of 18
the disease, the need for food modifications was reported by 50.7% in the ENAS and 93.8%

in the LNAS (table 3).

Significant positive correlations were found between the four DMD stages and the deviant

occlusal contacts (rs 0.32, p = 0.007) and deviant occlusal relationships (rs 0.48, p = 0.00).

Almost half of the patients in all stages had anterior open bites. Posterior open bite was

found only in the ENAS and LNAS. Posterior cross bite showed a gradual increase over time

from 38.5% in the AS to 93.8% in the LNAS (table 3).

Tongue thickness was significantly positive correlated with deviant occlusal contacts (rs 0.34,

p = 0.006) and with deviant occlusal relationships (rs 0.47, p = 0.00).

When mastication problems were predicted it was found that occlusal contacts (Beta =

0.545, p = 0.002) and the z-score of the echogenicity of the masseter muscle (Beta = 0.132, p

= 0.021) were significant. The overall model fit was R2 = 0.23. The z- score of the temporalis

muscle, the MVBF and occlusal relationships were not significant predictors for mastication

problems.

4. Discussion

This study assessed the mastication problems of DMD patients and determined related

pathophysiological aspects of masticatory muscle structure, tongue thickness, bite force and

dental characteristics. This larger study confirms the preliminary results from the study of

van Bruggen et al (2014), which further acknowledge their validity. We also add new

information about the structural changes of the masticatory muscles during the course of

the disease [9]. In this cohort of 72 DMD patients we found early involvement of the

masseter muscle and the need to cut hard foods in small pieces as a modification because of

chewing difficulties already in the early stages of the disease. Both highlight the use of

Page 9 of 18
structured questionnaires in the feeding and swallowing assessment of boys and adults with

DMD, that cover questions about feeding and mastication problems but also explicitly ask

about compensations strategies already performed.

To our knowledge this is the first study that shows the increasing involvement of various

elements of the masticatory system, i.e masticatory muscles, tongue and dental

characteristics in consecutive stages of DMD, and the negative influence on mastication of

the diseased masseter muscles in combination with diminished occlusal contacts, i.e.

anterior and posterior open bites.

In animals, the mdx mouse is a recognized model to study DMD [24]. Masseter muscles in

mdx mice were found to have a mild type of dystrophy, compared to the diaphragm and

gastrocnemius muscles, indicating differences in susceptibility of various muscles to

muscular dystrophy [24]. Spassov et al. (2010) found that the mdx oral muscles were

unequally affected by the disease progress. The tongue was the least implicated muscle and

the masseter and temporalis muscle had a higher degree of collagen proliferation,

suggesting a greater limitation [25]. The same is found in studies of patients with DMD.

Kiliaridis et al. (1998) described a higher echogenicity of the masseter muscle in DMD

patients [26]. With QMUS used in the current study we were able to show the increasing

echogenicity in the masseter muscle starting in the LAS. In a previous study we found that

muscles of the submental muscle group and tongue were only affected in the LNAS [4]. This

highlights the varied involvement of the disease in oral muscles.

MRI of the oropharyngeal region can also provide information about facial and oral muscles

in patients with neuromuscular disorders, especially of the small and deeper lying muscles,

like the pterygoid muscles and pharyngeal wall muscles [27 -28]. Because no reference or

normal values are available for this, these images must be scored subjectively and the

10

Page 10 of 18
sensitivity for detection abnormalities is unknown. Quantitative muscle ultrasound using

population-based reference values is able to show the increased echogenicity in consecutive

stages of the disease more precisely. In addition, ultrasound is a portable, bedside technique

that requires no sedation.

The increasing hypertrophy of the tongue over time found in this group of DMD patients is

comparable to data of a previous study by our group [4]. Until now, there are no risk factors

known for the development of an enlarged tongue. Muscle hypertrophy is known from calf

enlargement in DMD as characteristic for the first stage of the disease. In our previous study

we found an increase in tongue thickness in the non-ambulatory stages in 70% of the

patients. We hypothesize that this is the same pseudohypertrophy phenomenon as occurs in

skeletal muscles in DMD.

The enlarged tongue protrudes over the anterior teeth and lies on the occlusal surfaces of

the mandibular teeth as the jaws are relaxed [14]. Ghafari et al. (1988) reported anterior

open bites, caused by the resting position of the tongue between the incisors, in 55% of the

DMD patients, which is also comparable to the findings in our study [29]. A high percentage

posterior open bites was found in the study of Morel-Verdebout et al, progressively in the

older DMD patients [12]. We found posterior open bites, especially in the LNAS. The

significant correlation between tongue thickness and occlusal contacts is in agreement with

our previous study [9] and shows the combined influence: the enlarged tongue influences

not only the occlusal relationships, but also the occlusal contacts by prohibiting contact of

the teeth of maxilla and mandibula. From other studies it is known that occlusal contacts are

influencing the masticatory performance, in combination with age, sex and orthodontic need

[18]. Several studies have shown that 50% of the masticatory performance is explained by

the number of occlusal contacts, and is highly correlated with the contact area of the post

11

Page 11 of 18
canine teeth (18). We found the occlusal contacts and the increased echogenicity of the

masseter muscles as significant influencing factors of the masticatory performance. In

addition, in our cohort there was an increasing percentage of cross bites from 38% (AS) to

93% (LNAS), which can be attributable to the increasing tongue thickness and low position of

the tongue, and demonstrating the influence of the disease on growth.

The reduced MVBF found in this study was comparable with the data, measured in DMD

patients by Ueki et al. (2007) and van Bruggen et al. (2015) [9, 11]. The low MVBF is caused

by the reduced muscle strength of the masticatory muscles, compatible with the findings in

skeletal muscle ultrasound, in which a reduced strength is clearly related to an increased

echogenicity [30]. Van der Bilt (2011) reported for example a mean bite force of 118N

necessary to penetrate natural foods like raw carrots [18], which is higher than the mean

MVBF found in the various stages in our study. This could be an explanation for the food

modifications already reported in the early stages.

As survival in DMD increases, insight in the nature and clinical course of mastication

problems has become even more essential. Early involvement of the masticatory muscles

and the reported chewing problems asks for early detection and treatment

recommendations. It is plausible that altered food choice, predominantly soft and easy to

chew, results in lower intakes of key nutrients like iron and fiber [18]. Therefore, regularly

evaluations by a dietician are important in the care for patients with DMD.

Furthermore, a four weeks’ mastication training for patients with DMD with chewing gum (3

times a day) showed an improvement of the masticatory performance [31]. The chewing

gum mastication was considered a low intensity training comparable with training for motor

abilities in DMD [32]. The pilot study showed improved masticatory performance both in

younger and older patients. Since we found mastication problems in the early stages it is

12

Page 12 of 18
recommended to pay attention to these problems starting in the AS and LAS, together with

measuring bite force and repeated dental examinations. Mastication training with low

intensity should be considered in these stages.

In general, in case of malocclusions orthodontic treatment is started often in combination

with exercises for holding the tongue in the mouth and avoiding mouth breathing (orofacial

myofunctional therapy (OMT)) [33]. However, studies on the effect of these exercises in

DMD lack. Because of the existing dental problems (malocclusions) as caused by the affected

masticatory muscles and tongue hypertrophy in DMD orthodontic treatment should be given

careful consideration [34]. Only a few case studies have reported the benefit of orthodontic

treatment in Becker muscular dystrophy and DMD [35, 36]. More research is needed to

assess the benefits of exercises of mouth closing in early stages or orthodontic treatment.

Conclusions

In DMD problems with mastication and the need for food adaptations are already found in

the early stages. The pathological degeneration of the masseter muscle along with reduced

occlusal contacts (anterior and posterior open bites) are mainly responsible for the

hampered chewing. In order to detect chewing problems even in the early stages questions

about feeding and mastication problems as well as about the food adaptations should be

asked, and seem to be highly essential. Possible dietary deficiency should be taken into

account and masticatory training should be considered early. More research is needed to

assess the results of OMT and orthodontic treatment in boys and young adults with DMD.

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Page 13 of 18
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 Figure 1 Ultrasound transducer placement and related images
(A) Measurement of m. masseter. The transducer was placed perpendicular to the ramus, with altering
the angle of scanning until the best echo as a horizontal line of the mandibular ramus was achieved.
(B) Measurement of m. temporalis. The transducer was placed at the height of the eye, with altering
the angle of scanning until the best echo was achieved, showing the muscle with related fibrotic tissue
(top of the picture D). (C) Ultrasound picture of the masseter muscle of a healthy person (16 years)*.
(D) Ultrasound picture of the temporal muscle of a healthy person (16 years)*. (E) Ultrasound picture
of the masseter muscle of a patient with DMD (16 years)*. (F) Ultrasound picture of the temporal
muscle of a patient with DMD (16 years)*.
*the region of interest to calculate the echogenicity is depicted by the dotted lines

Figure 2, Occlusal relationships and occlusal contacts

(A.) Occlusal relationship between the upper and lower masticating surfaces. (A.1.) No posterior cross
bite. (A.2.) Posterior cross bite. (B) Occlusal contacts, i.e. the touching of opposing teeth of mandible
and maxilla. (B.1.) Normal. (B.2.) Anterior open bite. (B.3.) Posterior open bite

Table 1 Patient characteristics per stage

DMD stage EAS N=13 LAS N=17 ENAS N=26 LNAS N=16
Mean age in years (range) 6.3 (4.3 – 9.0) 11.5 (7.6 – 19.3) 14.5 (8.9 – 20.6) 21.1 (15.6 – 28.0)
Prednisone use (%) 76.9 93.8 73.1 33.3
Prednisone used, but stopped (%) - - 23.1 6.7

DMD, Duchenne muscular dystrophy; EAS, early ambulatory stage; LAS, late ambulatory stage; ENAS, early non-
ambulatory stage; LNAS, late non-ambulatory stage

Table 2. The estimated mean z score (95 % confidence interval (CI)) of echogenicity and the
estimated mean maximum voluntary bite force (95 % confidence interval (CI)), by DMD stage
DMD stage
EAS LAS ENAS LNAS
Mean (CI) Mean (CI) Mean (CI) Mean (CI)
echogenicity
m. masseter 1.2 (0.7 - 2.4) 2.2 (1.3 - 3.2) 2.4 (1.7 - 3.1) 3.3 (2.2 - 4.4)
m. temporalis -0.6 (-1.3 - 1.2) 0.7 (0.1 - 1.3) 1.0 (0.3 - 1.6) 1.6 (0.6 - 2.6)

MBVF* Mean (CI) in N Mean (CI) in N Mean (CI) in N Mean (CI) in N

54.8 (38.9 – 70.5) 116.6 (84.9 – 148.2) 108.9 (90.5 – 127.3) 105.9 (70.2 – 141.5)

DMD, Duchenne muscular dystrophy; EAS, early ambulatory stage; LAS, late ambulatory stage; ENAS, early non-
ambulatory stage; LNAS, late non-ambulatory stage; MVBF, maximum voluntary bite force in Newton
* In healthy participants (N=46) mean MBFV ranged from 144.6N (5-10 years) to 180.2N (10-15 years), 268.2N
(15-20 years) and 267.1N (20-30 years)

Table 3. Correlation between DMD stages and items of the questionnaire, and dental
characteristics
DMD stage
EAS N=1 LAS N=17 ENAS N=26 LNAS N=16 rs (p)
N (%) N (%) N (%) N (%)
Items questionnaire:
Problems mastication: 0.53 (0.000)

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 No problems 9 (69.2) 8 (50.0) 11 (42.3) 1 (6.2)
< once a week 4 (30.8) 7 (43.8) 10 (38.5) 5 (31.2)
Once every day 0 (0) 1 (6.2) 5 (19.2) 5 (31.2)
Several times a day 0 (0) 0 (0) 0 (0) 5 (31.2)
Food modifications: 0.54 (0.000)
no modifications 10 (76.5) 8 (50.0) 11 (42.3) 1 (6.2)
only small slashed food 3 (32.1) 8 (50.0) 13 (50.0) 7 (43.8)
no tough or hard food 0 (0.0) 0 (0.0) 2 (7.7) 3 (18.8)
only soft food 0 (0.0) 0 (0.0) 0 (0.0) 5 (31.2)
Occlusal contacts and
relationships:
1
Occlusion : 0.32 (0.007)
Normal occlusion 6 (50.0) 9 (52.9) 9 (43.6) 3 (20.0)
Anterior open bite 6 (50.0) 8 (47.1) 14 (53.8) 8 (53.3)
Posterior open bite 0 (0.0) 0 (0.0) 3 (11.5) 4 (26.7)
Posterior cross bite 5 (38.5) 4 (25.0) 17 (70.8) 15 (93.8) 0.48 (0.000)

DMD, Duchenne muscular dystrophy; EAS, early ambulatory stage; LAS, late ambulatory stage; ENAS, early non-
ambulatory stage; LNAS, late non-ambulatory stage
1
in one patient (EAS) occlusion could not be identified

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