Postural control abnormalities related to sleep deprivation in patients with Marfan Syndrome

Abstract

BACKGROUND:

Marfan syndrome (MFS) is a rare autosomal dominant connective tissue disorder affecting virtually every organ. Sleep disturbances, associated to high collapsibility in upper airways, are common in MFS; daytime sleepiness could lead to reduction in attention and motor coordination, with detrimental effects on balance.

OBJECTIVE:

To evaluate otoneurological function in MFS patients, compared to healthy subjects, and to investigate possible correlations with sleep deprivation extent.

METHODS:

Forty-one MFS patients underwent a thorough otoneurological examination, video Head Impulse Test (vHIT), and static posturography. Sleep parameters were recorded by home monitoring. Daytime sleepiness and dizziness-related handicap were screened by means of Dizziness Handicap Inventory (DHI) and Epworth Sleepiness Scale (ESS). Results were compared with 49 healthy controls (HC).

RESULTS:

DHI and ESS scores were increased in MFS patients (p < 0,01). vHIT scores showed no between-group effect. Classical (surface and length) and frequency-domain posturographic parameters were significantly increased in MFS with respect to HC (p < 0,01). A positive correlation was found between ESS scores and posturographic parameters in MFS patients.

CONCLUSIONS:

An impaired postural control, related to the extent of sleep deprivation, was found in MFS patients. Such results could advocate for screening and treating sleep deprivation and balance dysfunctions in MFS patients.

Keywords

Marfan syndrome posturography sleep apnea sleep deprivation

1 Introduction

Marfan syndrome (MFS) is a rare, chronic connective tissue disorder, with autosomal dominant inheritance, caused by mutations in the gene coding for fibrillin-1 (FBN1). Its prevalence is estimated to be about 1 per 5000 individuals, without predilection for geography, gender, or race [20, 48]. Over one thousand mutations in FBN1 have been identified to cause MFS as well as a variety of other disorders, thus making MFS part of several conditions linked to abnormal signalling in the TGF-ß pathway, and encompassing many organs and systems, while ocular, skeletal and cardiovascular manifestations remain pivotal [38, 42]. MFS is associated with chronic fatigue, chronic pain, and subsequent psychological distress, compromising quality of life and autonomy of patients [49]. Psychological burden in MFS has been associated with the subjective perception of severity of disease rather than objective measures, and reduced quality of life was shown not to correlate with physical symptoms.

A number of peculiar craniofacial abnormalities (i.e. maxillary and mandibular retrognathia, arched palate and narrow nasal cavity), leading to oral breathing, have been associated with MFS [11, 32]. In turn, such features lead to upper airway collapsibility during sleep, repetitive episodes of airflow interruption, and obstructive sleep apnea (OSA), with subsequent arousals from sleep and overall sleep deprivation [10 , 44]. Sympathetic activation during these arousals leads to increased blood pressure, a process shown, among other effects, to worsen progression of aortic dilatation in MFS patients with sleep disturbances [25]. Moreover, chronic sleep fragmentation and deprivation may be related to subclinical changes in the central nervous system (CNS) which could lead to reduction in memory, attention and motor coordination [4 , 28].

In this light, previous research highlighted a possible relationship between sleep deprivation and postural control impairment in healthy subjects [28 , 39], with a lack of MFS literature on this topic. In fact, poor sleep quality leads to daytime sleepiness, usually measured by means of the Epworth Sleepiness Scale (ESS) [21], which in turn is associated with detrimental effects on balance, due to poor physical performance and cognitive function [46, 51]. In particular, focusing on the vestibular system, an increase in body sway, measured by posturographic parameters, and a deficit in vestibulo-ocular reflex (VOR), were found in a chronically sleep-deprived population affected by moderate-to-severe OSA [28]. Vestibular disorders and dizziness, in turn, are highly associated with postural imbalance and, consequently, with falls [3], due to increased body sway especially in those challenging situations represented by visual and somatosensory conflicts. Risk and incidence of falls are greater in patients with bilateral vestibular hypofunction[19, 43].

In this vision, given the relationships existing in MFS between craniofacial abnormalities, upper airway collapsibility and OSA [36], and recent evidences positing a connection between balance disorders and sleep deprivation, the aim of this study was to evaluate otoneurological function in MFS patients, compared to a cohort of healthy subjects, by the analysis of VOR gain and posturographic parameters, and to investigate possible correlations with the extent of sleep deprivation in such population, by means of ESS scores, and with the impact of balance-related symptoms on quality of life.

2 Materials and methods

2.1 Patient selection and home sleep apnea test

Following the American Academy of Sleep Medicine criteria [6], forty-one adults diagnosed as MFS patients and recruited at the Regional Referral Centre for MFS, and forty-nine healthy gender- and age-matched subjects, included in the Tor Vergata University Hospital database serving as controls (HC), underwent home sleep apnea testing. Patients were diagnosed with MFS according to the revised Ghent criteria [26]: in the absence of family history, MFS diagnosis was achieved in case of confirmed aortic root increase/dissection (Z-score≥2) and either ectopia lentis, FBN1 mutation or a high systemic symptom score. In the presence of family history, only one of the aforementioned signs/symptoms was considered sufficient for MFS diagnosis [26]. Eligible MFS subjects and HC were required to report negative history for malignancy, recent head trauma, neuropsychiatric disorders, infectious or otoneurological diseases. The peripheral blood was tested for the usual parameters and neurological disorders were excluded using the Mini Mental State Examination and Magnetic Resonance Imaging. No patient was pregnant or breastfeeding, and all subjects taking drugs possibly affecting vestibular and/or cochlear function were excluded.

The following parameters were obtained from the home sleep apnea test. The apnea–hypopnea index (AHI) is defined as the ratio between the sum of all apneas (>90% reduction in airflow for > 10 s) plus all hypopneas (>30% reduction in airflow > 10 s) associated with≥3% O₂ desaturation [6] and time (hours) in bed. In addition to AHI, also the following oxygen saturation (SaO₂) parameters were measured: mean SaO₂, time spent with SaO₂ < 90% (T < 90) and oxygen desaturation index (ODI) (number of oxygen desaturations≥3% per hour) [2].

The study adhered to the principles of the Declaration of Helsinki and all the participants provided written informed consent after receiving a detailed explanation of the study, which was approved by the Independent Ethical Committee of the University of Rome ‘Tor Vergata’.

2.2 Otoneurological testing

After a thorough clinical otoneurological examination [28, 29] including pure tone and impedance audiometry, binocular electro-oculography analysis with positional manoeuvres, Head Shaking Test, clinical Head Impulse Test and bithermal caloric testing, as well as limb coordination, gait observation and Romberg stance Test, all MFS and HC subjects underwent:

2.2.1 Video Head Impulse Test (vHIT)

For vHIT measurements the EyeSeeCamTM System (Interacoustics, Middelfart, Denmark) and the technique proposed in previous studies were used [8, 29]. Results were classified as abnormal if two conditions were met: abnormal gain according to the calculated normative data and/or presence of refixation saccades, revealed by visual inspection [8]. Following instructions of the software manufacturer (OtoAccess, Interacoustics), VOR median gain values of both sides recorded at 60 ms were extracted on an.xls file for raw analysis. According to previous experiences [8, 29], diagnosis of possible vestibular weakness was achieved in case of VOR gain below 0.84 and 0.87 for right and left side, respectively, calculated as the lower cut-off value of the gain-reference range (mean normal±2(standard deviations; SD) equal to 0.94±2(0.05) and 0.95±2(0.04) for right and left side respectively), incorporating 95% of healthy population, age- and gender matched with the current population of patients and including the above-mentioned normal volunteers in our laboratory.

2.2.2 Static posturography

Each patient was instructed to keep an upright position on a standardized platform for static posturography (EDM Euroclinic^®). The recording period was 60 s for each test (eyes closed or opened while standing on the stiff platform) and the sampling frequency in the time domain was 25 Hz [18 , 29]. The center of pressure displacement was monitored while performing the test. The posturographic parameters considered in our study were the trace length (length), the surface of the ellipse of confidence (surface) and the fast Fourier transform (FFT) elaboration of oscillations on both the X (right-left) and Y (forward–backward) planes [18, 28]. FFT elaborations of time-domain oscillations signals (X and Y) were acquired through a core function implemented in Matlab space [29]. Spectral values (power spectra, PS) of body oscillations were quantified on an.xls file, for every frequency from 0.0122 to 4.9927 Hz [30, 31]. As in previous studies [18 , 31] we subdivided the frequency spectrum into three groups: 0.0122–0.6958 Hz (low-frequency interval, L); 0.708–0.9888 Hz (middle-frequency interval, M); 1.001–4.9927 Hz (high-frequency interval, H).

2.3 Validated Questionnaires

All study subjects completed the following questionnaires:

The Dizziness Handicap Inventory (DHI) Italian version, including 25 items aimed at assessing functional (nine questions), emotional (nine questions) and physical (seven questions) limitations via a multiple choice scheme: ‘yes’ (4 points), ‘sometimes’ (2 points) and ‘no’ (0 points) [35];

The Italian validated version of the Epworth Sleepiness Scale (ESS) [47], which is a widely used test exemplifying eight daily situations at risk of sleepiness (rated with scores from 0 to 3, based on the likelihood of somnolence to occur).

2.4 Data handling and statistical analysis

Means and standard deviations (SDs) of all variables were calculated in HC and MFS groups. In order to assess that data were of Gaussian distribution, the D’Agostino K-squared normality test was applied (where the null hypothesis is that the data are distributed normally). A between-group analysis of variance (ANOVA) was performed, between MFS and HC, for all otoneurological variables and questionnaire scores. Age and gender were handled as continuous and categorical predictors, respectively. The significant cut-off level (α) was set at a p-value of 0.05. Bonferroni correction for multiple comparisons was used to test post-hoc significant main effects. Moreover, Spearman’s rank correlation was performed between questionnaires and otoneurological scores. Thus, given the sample size of this group and the two-tailed nature of the test, a significant cut-off level (α) was set at a p-value of 0.05 (STATISTICA version 7 for Windows).

3 Results

3.1 Patients and home sleep apnea test scores

Forty-one consecutive MFS patients affected by mild OSA (21 F; 20 M) were enrolled. The HC consisted of 49 right-handed BMI-, gender- and age-matched healthy individuals (25 F; 24 M) (Table 1). All enrolled participants in both groups completed the study.

Table 1
Demographic Variables and Sleep Apnea Test Scores in MFS and HC subjects

MFS (n = 41) HC (n = 49)

Age 39.22±11.08 39.73±8.82

Gender 21 F; 20 M 25 F; 24 M

AHI 10. 14±8.30 2.73±0.81

Mean SaO₂ 93. 07±2.57 95.92±0.59

T < 90 7. 15±16.23 0.73±0.54

ODI 4.38±4.66 1.95±0.66

	MFS (n = 41)	HC (n = 49)
Age	39.22±11.08	39.73±8.82
Gender	21 F; 20 M	25 F; 24 M
AHI	10. 14±8.30	2.73±0.81
Mean SaO₂	93. 07±2.57	95.92±0.59
T < 90	7. 15±16.23	0.73±0.54
ODI	4.38±4.66	1.95±0.66

Demographic and sleep apnea test data for MFS and HC groups. MFS: Marfan Syndrome. HC: healthy subjects. F: female. M: male. AHI: apnea/hypopnea index; SaO₂: oxygen saturation; T < 90: time spent with SaO₂ < 90%; ODI: oxygen desaturation index. Data are shown as mean±SD.

See Table 1 for MFS and HC demographic data and sleep apnea test results.

3.2 Validated questionnaires

A significant between-group effect was found for both ESS (p = 0.0072) and DHI (p = 0.011), with increased scores in MFS group with respect to HC (Table 2).

Table 2
Main effects in Questionnaire Scores and Otoneurological Data between MFS and HC subjects

MFS HC Significance

Mean SD Mean SD

Questionnaire Scores

DHI 12.58 15.98 2.71 1.67 F(1,88) = 27.823, p < 0.05

ESS 5.27 3.65 2.28 1.39 F(1,88) = 18.119, p < 0.05

Video Head Impulse Test

VOR gain - R 0.93 0.05 0.94 0.05 F(1.88) = 0.753, p = 0.38

VOR gain - L 0.94 0.05 0.95 0.05 F(1.88) = 2.019, p = 0.15

Classical Posturography

Surface - OE 519.01 271.56 383.16 120.38 F(1.88) = 11.938, p < 0.05

Surface - CE 1107.32 491.31 620.21 138.37 F(1.88) = 44.075, p < 0.05

Length - OE 578.30 132.43 497.36 103.29 F(1.88) = 6.602, p = 0.084

Length - CE 963.61 220.53 721.16 147.38 F(1.88) = 38.646, p < 0.05

FFT Analysis

Low Frequency – OE X 5.82 1.10 5.46 1.27 F(1.88) = 2.033, p = 0.16

Low Frequency – OE Y 4.62 0.89 4.38 1.14 F(1.88) = 1.271, p = 0.26

Low Frequency – CE X 6.27 1.82 4.43 1.71 F(1.88) = 24.176, p < 0.05

Low Frequency – CE Y 4.27 1.18 2.70 1.22 F(1.88) = 37.719, p < 0.05

Middle Frequency – OE X 0.33 0.07 0.31 0.09 F(1.88) = 0.540, p = 0.46

Middle Frequency – OE Y 0.23 0.05 0.20 0.05 F(1.88) = 3.568, p = 0.062

Middle Frequency – CE X 0.57 0.04 0.28 0.07 F(1.88) = 447.90, p < 0.05

Middle Frequency – CE Y 0.50 0.02 0.23 0.05 F(1.88) = 1012.1, p < 0.05

High Frequency – OE X 1.04 0.14 0.99 0.11 F(1.88) = 2.996, p = 0.086

High Frequency – OE Y 0.79 0.11 0.78 0.11 F(1.88) = 38.646, p = 0.52

High Frequency – CE X 1.44 0.13 1.11 0.17 F(1.88) = 102.43, p < 0.05

High Frequency – CE Y 1.23 0.09 1.01 0.15 F(1.88) = 71.119, p < 0.05

	MFS	HC	Significance
Questionnaire Scores
DHI	12.58	15.98	2.71	1.67	F(1,88) = 27.823, p < 0.05
ESS	5.27	3.65	2.28	1.39	F(1,88) = 18.119, p < 0.05
Video Head Impulse Test
VOR gain - R	0.93	0.05	0.94	0.05	F(1.88) = 0.753, p = 0.38
VOR gain - L	0.94	0.05	0.95	0.05	F(1.88) = 2.019, p = 0.15
Classical Posturography
Surface - OE	519.01	271.56	383.16	120.38	F(1.88) = 11.938, p < 0.05
Surface - CE	1107.32	491.31	620.21	138.37	F(1.88) = 44.075, p < 0.05
Length - OE	578.30	132.43	497.36	103.29	F(1.88) = 6.602, p = 0.084
Length - CE	963.61	220.53	721.16	147.38	F(1.88) = 38.646, p < 0.05
FFT Analysis
Low Frequency – OE X	5.82	1.10	5.46	1.27	F(1.88) = 2.033, p = 0.16
Low Frequency – OE Y	4.62	0.89	4.38	1.14	F(1.88) = 1.271, p = 0.26
Low Frequency – CE X	6.27	1.82	4.43	1.71	F(1.88) = 24.176, p < 0.05
Low Frequency – CE Y	4.27	1.18	2.70	1.22	F(1.88) = 37.719, p < 0.05
Middle Frequency – OE X	0.33	0.07	0.31	0.09	F(1.88) = 0.540, p = 0.46
Middle Frequency – OE Y	0.23	0.05	0.20	0.05	F(1.88) = 3.568, p = 0.062
Middle Frequency – CE X	0.57	0.04	0.28	0.07	F(1.88) = 447.90, p < 0.05
Middle Frequency – CE Y	0.50	0.02	0.23	0.05	F(1.88) = 1012.1, p < 0.05
High Frequency – OE X	1.04	0.14	0.99	0.11	F(1.88) = 2.996, p = 0.086
High Frequency – OE Y	0.79	0.11	0.78	0.11	F(1.88) = 38.646, p = 0.52
High Frequency – CE X	1.44	0.13	1.11	0.17	F(1.88) = 102.43, p < 0.05
High Frequency – CE Y	1.23	0.09	1.01	0.15	F(1.88) = 71.119, p < 0.05

Questionnaire and Otoneurological Scores for MFS and HC groups. MFS: Marfan Syndrome. HC: healthy subjects. DHI: Dizziness Handicap Inventory; ESS: Epworth Sleepiness Scale; VOR: vestibulo-ocular reflex; R: right ear; L: left ear; OE: eyes opened; CE: eyes closed; FFT: Fast Fourier Transform. Statistically significant between-groups differences are shown in bold. Exact p values of significant main effects are given in the text.

3.3 Otoneurological testing

Clinical otoneurological examination did not show any abnormality for all study subjects. In the field of classical posturography parameters, MFS patients demonstrated a significant increase in surface on both closed (p = 0.0054) and opened eyes (p = 0.014) conditions, and in length in closed eyes condition (p = 0.0062) only, when compared to HC (Fig. 1; Table 2). Regarding FFT results, a significant increase in PS values within low, middle and high frequency domains, both on X (p = 0.0091, p = 0.0073 and p = 0.0056, respectively) and Y planes (p = 0.0084, p = 0.0012 and p = 0.0067, respectively), in closed eyes conditions, was found with respect to HC (Fig. 2; Table 2).

Fig.1

Between-group effect in posturography parameters in MFS and HC subjects. Line indicates mean for each condition. Asterisks indicate statistically significant between-groups differences. OE: eyes opened. CE: eyes closed. All data are measured in mm.

Fig.2

Low-frequency interval power spectra differences in MFS and HC subjects. Lines indicate standard deviations. Asterisks indicate statistically significant between-groups differences.

Finally, there were no significant differences between MFS and HC with respect to VOR gain, calculated by means of vHIT.

3.4 Correlation analysis

Spearman’s rank correlation analysis yielded positive correlations, in MFS group, between ESS and classical posturography parameters (surface, in both opened and closed eyes conditions, and length, in closed eyes condition only) and between FFT data regarding low-frequency PS values (in closed eyes conditions and in X and Y planes) and ESS scores. See Fig. 3 and Table 3 for correlation plots and r values. Moreover, a positive correlation was also found between classical posturography parameters and low-frequency PS values as shown by FFT analysis. No significant correlation was found for HC between questionnaire scores and otoneurological variables.

Fig.3

Correlation plots between daytime sleepiness and posturography parameters in MFS subjects. 3A: Correlation between Epworth Sleepiness Scale (ESS) scores and Surface with eyes closed (CE) (r = 0.51); 3B: Correlation between ESS scores and low-frequency interval power spectra (PS) in the X plane in CE (r = 0.70); 3C: Correlation between ESS scores and low-frequency interval PS in the Y plane in CE(r = 0.71).

Table 3

Correlation Analysis between ESS and Posturographic Scores in MFS Subjects

	ESS	Surf – OE	Surf – CE	Length – CE	Low frequency PS X CE	Low frequency PS Y CE
ESS	1
Surf – OE	0.55	1
Surf – CE	0.51	0.98	1
Length – CE	0.66	0.59	0.56	1
Low frequency PS X CE	0.70	0.62	0.60	0.58	1
Low frequency PS Y CE	0.71	0.74	0.73	0.67	0.61	1

Correlation Analysis between Epworth Sleepiness Scale (ESS) scores and Posturographic parameters for MFS subjects, with significant Spearman’s rank correlation r values. MFS: Marfan Syndrome; OE: eyes opened; CE: eyes closed; PS: power spectra; Surf: surface of the ellipse of confidence.

4 Discussion

Considering that – aligning with previous literature [11 , 25] – MFS patients were found to mildly suffer from OSA and given those aspects positing influences between such disorder and balance control, the main finding in this study is the significant difference between MFS affected by mild OSA and HC in posturographic parameters. In our cohort of MFS patients, a significant between-group effect was found both in classical posturographic parameters, such as surface (with both eyes opened and closed) and length in eyes closed condition, and in frequency-domain analysis by means of FFT (Fig. 1-2). Another interesting finding is represented by the significant increase in DHI scores in our cohort of patients with respect to HC (Table 2), indicating subjective perception of dizziness-related handicap. Increased surface and length of body sway, as well as oscillations in the low-frequency domain, have been previously linked to vestibular dysfunction [1 , 52]. Although previous experiences demonstrated a decreased VOR gain during the moderate-to-severe stages of OSA [28], VOR analysis by means of vHIT and clinical otoneurological examination did not report significant abnormalities. This finding should be interpreted in line with previous studies demonstrating that the VOR gain reduction only may become evident with long-lasting deprivation, because of the covert effect related to redirecting attention when the sleep deprivation does not last long [2, 39]. Furthermore, these aspects - together with the amount of evidences related to the earlier changes in posturography parameters with respect to VOR gain, also after CPAP treatments [2 , 39] – reinforce those notions revealing that VOR gain — with respect to the vestibular frequency domain (indirectly related to vestibular-spinal reflex) — reacts more leniently to both sleep deprivation and those treatments directed to reduce hypoxic processes in the central nervous system [28]. On the other hand, maintaining an upright posture requires information from different systems, such as vision, the vestibular system, and proprioception, which – together – could be responsible for a general decrease in postural control without straightforward alterations in one subsystem. This is supported by the finding of increased PS values in MFS subjects, for the eyes closed condition, also in the middle- and high-frequency regions, usually linked to proprioceptive contribution to balance [7, 28], which appear to be impaired whether visual input is missing (Table 2). In this light, it is worth noting that also other numerous features affecting MFS (e.g. ocular, musculoskeletal and rheumatological manifestations) [11, 15] could lead to impaired postural control, as well as gait disturbances and recurrent joint pain. Finally, connective tissue diseases such as MFS are known to affect the constitutive elements of vessel walls [23], and the wide variability of MFS-related vascular lesions [13] could point to hypothesize, although this remains speculative, that neurovascular impairment affecting CNS pathways supervising postural control could be present.

Another interesting finding in this study is represented by the significant increase in ESS scores for MFS patients with respect to HC (Table 2). Such result is in accordance with present home sleep apnea test results (Table 1) and with previous research on MFS subjects focusing on OSA commonly affecting MFS [11 , 25]. In particular, such manifestation is possibly associated to craniofacial dysmorphisms inducing high collapsibility in upper airways [11 , 34]. In our cohort of MFS patients, sleep-disordered breathing is likely the cause for increased ESS scores, as such scale has demonstrated to be able to discriminate people with sleep disturbances from healthy subjects [21]. Chronic sleep deprivation is a well-known substrate of cardiovascular disease; in MFS patients, the severity of sleep apnea has been associated with increased aortic root dilatation and subsequent dissection, which is the main cause of premature death in these patients [25].

In this scenario, these findings are further supported by correlation analysis showing classical posturography parameters and ESS scores to be positively correlated in MFS group with those derived from FFT analysis (Fig. 3; Table 3). This finding confirms previous experiences linking subjective consequences of sleep deprivation to impaired postural control and vestibular contribution [28 , 39]. In fact, since a decreased ability to adapt to balance perturbations both under sleep deprivation and fatigue has been demonstrated on healthy subjects [5, 22], the integration of information from the visual, vestibular and somatosensory receptors and motor coordination are processes known to require attention and vigilance [9]. This is particularly clear when information from any of the sensory systems is not reliable [40], as it could happen in MFS, also due to visual and/or musculoskeletal impairment possibly affecting other sources of postural control (i.e. proprioceptive/somatosensory cues) which – together with chronic sleep restriction and decreased vigilance - may lead to inappropriate sensory integration and affect the efficiency of postural sway regulation [9].

In conclusion, the present study further highlights that MFS may potentially affect every organ or system. Such multisystem involvement has profound consequences including reduced quality of life, increased pain, modified lifestyle and severe fatigue [45]. Moreover, this condition is progressive, and its manifestations may unfold alongside aging. In fact, given the improvements in medical and surgical management leading to an increase in life expectancy of 30 years [50], it is possible that new features of the disease could be discovered and managed. In this light, postural control abnormalities, and their relationship with sleep deprivation, could represent an element worth investigating. Following these aspects, daytime sleepiness has been linked to increased risk of falls, as well as vestibular disorders and dizziness of any origin [3 , 51]. Since falls cause not only medical and social disabilities, such as injuries and loss of independence, but also decline quality of life by increased fear of falling, we believe that such an additional psychological burden could be avoided by evaluating postural control and sleepiness in MFS patients.

This study suffers from some limitations. In particular, sleep deprivation as measured by ESS showed different degrees of consistency with home sleep apnea test scores, which represents standard testing for OSA. Thus, further studies are needed to evaluate the extent of the relationship between postural control impairment and sleep disturbances in this cohort of patients. Furthermore, the various contributions to body oscillations calculated by means of FFT are partially debated in literature. In fact, while the main literature trend defines the lowest frequency range as related to vestibular (or visuo-vestibular) input [18 , 52], other range subdivisions exist. Although this debate could lead to some discrepancies regarding the exact contribution to balance control [5 , 17], present data have been generated with the same procedure previously published [29 –31] and results demonstrated to be in line with previous studies in which chronic sleep reduction and balance control abnormalities have been related to eachother [2 , 28].

Footnotes

Acknowledgments

None.

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	MFS		HC		Significance
	Mean	SD	Mean	SD
Questionnaire Scores
DHI	12.58	15.98	2.71	1.67	F(1,88) = 27.823, p < 0.05
ESS	5.27	3.65	2.28	1.39	F(1,88) = 18.119, p < 0.05
Video Head Impulse Test
VOR gain - R	0.93	0.05	0.94	0.05	F(1.88) = 0.753, p = 0.38
VOR gain - L	0.94	0.05	0.95	0.05	F(1.88) = 2.019, p = 0.15
Classical Posturography
Surface - OE	519.01	271.56	383.16	120.38	F(1.88) = 11.938, p < 0.05
Surface - CE	1107.32	491.31	620.21	138.37	F(1.88) = 44.075, p < 0.05
Length - OE	578.30	132.43	497.36	103.29	F(1.88) = 6.602, p = 0.084
Length - CE	963.61	220.53	721.16	147.38	F(1.88) = 38.646, p < 0.05
FFT Analysis
Low Frequency – OE X	5.82	1.10	5.46	1.27	F(1.88) = 2.033, p = 0.16
Low Frequency – OE Y	4.62	0.89	4.38	1.14	F(1.88) = 1.271, p = 0.26
Low Frequency – CE X	6.27	1.82	4.43	1.71	F(1.88) = 24.176, p < 0.05
Low Frequency – CE Y	4.27	1.18	2.70	1.22	F(1.88) = 37.719, p < 0.05
Middle Frequency – OE X	0.33	0.07	0.31	0.09	F(1.88) = 0.540, p = 0.46
Middle Frequency – OE Y	0.23	0.05	0.20	0.05	F(1.88) = 3.568, p = 0.062
Middle Frequency – CE X	0.57	0.04	0.28	0.07	F(1.88) = 447.90, p < 0.05
Middle Frequency – CE Y	0.50	0.02	0.23	0.05	F(1.88) = 1012.1, p < 0.05
High Frequency – OE X	1.04	0.14	0.99	0.11	F(1.88) = 2.996, p = 0.086
High Frequency – OE Y	0.79	0.11	0.78	0.11	F(1.88) = 38.646, p = 0.52
High Frequency – CE X	1.44	0.13	1.11	0.17	F(1.88) = 102.43, p < 0.05
High Frequency – CE Y	1.23	0.09	1.01	0.15	F(1.88) = 71.119, p < 0.05

Postural control abnormalities related to sleep deprivation in patients with Marfan Syndrome

Abstract

BACKGROUND:

OBJECTIVE:

METHODS:

RESULTS:

CONCLUSIONS:

Keywords

1 Introduction

2 Materials and methods

2.1 Patient selection and home sleep apnea test

2.2 Otoneurological testing

2.2.1 Video Head Impulse Test (vHIT)

2.2.2 Static posturography

2.3 Validated Questionnaires

2.4 Data handling and statistical analysis

3 Results

3.1 Patients and home sleep apnea test scores

Table 1 Demographic Variables and Sleep Apnea Test Scores in MFS and HC subjects MFS (n = 41) HC (n = 49) Age 39.22±11.08 39.73±8.82 Gender 21 F; 20 M 25 F; 24 M AHI 10. 14±8.30 2.73±0.81 Mean SaO2 93. 07±2.57 95.92±0.59 T < 90 7. 15±16.23 0.73±0.54 ODI 4.38±4.66 1.95±0.66

Footnotes

Acknowledgments

References

Table 1
Demographic Variables and Sleep Apnea Test Scores in MFS and HC subjects

MFS (n = 41) HC (n = 49)

Age 39.22±11.08 39.73±8.82

Gender 21 F; 20 M 25 F; 24 M

AHI 10. 14±8.30 2.73±0.81

Mean SaO₂ 93. 07±2.57 95.92±0.59

T < 90 7. 15±16.23 0.73±0.54

ODI 4.38±4.66 1.95±0.66