Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance

Background An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in patients with 22q11.2 deletion syndrome (22q11DS). Platybasia increases the depth of the velopharynx and is therefore postulated to contribute to velopharyngeal dysfunction. Our objective was to det...

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Main Authors: Nicole E Spruijt, Moshe Kon, Aebele B Mink van der Molen
Format: Article
Language:English
Published: Thieme Medical Publishers, Inc. 2014-07-01
Series:Archives of Plastic Surgery
Subjects:
Online Access:http://www.thieme-connect.de/DOI/DOI?10.5999/aps.2014.41.4.344
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author Nicole E Spruijt
Moshe Kon
Aebele B Mink van der Molen
author_facet Nicole E Spruijt
Moshe Kon
Aebele B Mink van der Molen
author_sort Nicole E Spruijt
collection DOAJ
description Background An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in patients with 22q11.2 deletion syndrome (22q11DS). Platybasia increases the depth of the velopharynx and is therefore postulated to contribute to velopharyngeal dysfunction. Our objective was to determine the clinical significance of platybasia in 22q11DS by exploring the relationship between cranial base angles and speech resonance. Methods In this retrospective chart review at a tertiary hospital, 24 children (age, 4.0-13.1 years) with 22q11.2DS underwent speech assessments and lateral cephalograms, which allowed for the measurement of the cranial base angles. Results One patient (4%) had hyponasal resonance, 8 (33%) had normal resonance, 10 (42%) had hypernasal resonance on vowels only, and 5 (21%) had hypernasal resonance on both vowels and consonants. The mean cranial base angle was 136.5° (standard deviation, 5.3°; range, 122.3-144.8°). The Kruskal-Wallis test showed no significant relationship between the resonance ratings and cranial base angles (P=0.242). Cranial base angles and speech ratings were not correlated (Spearman correlation=0.321, P=0.126). The group with hypernasal resonance had a significantly more obtuse mean cranial base angle (138° vs. 134°, P=0.049) but did not have a greater prevalence of platybasia (73% vs. 56%, P=0.412). Conclusions In this retrospective chart review of patients with 22q11DS, cranial base angles were not correlated with speech resonance. The clinical significance of platybasia remains unknown.
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spelling doaj.art-30eead4bf37449938b4c4e32460061782022-12-22T04:02:01ZengThieme Medical Publishers, Inc.Archives of Plastic Surgery2234-61632234-61712014-07-01410434434910.5999/aps.2014.41.4.344356Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech ResonanceNicole E Spruijt0Moshe Kon1Aebele B Mink van der Molen2Department of Plastic Surgery, University Medical Center, Utrecht, The NetherlandsDepartment of Plastic Surgery, University Medical Center, Utrecht, The NetherlandsDepartment of Plastic Surgery, University Medical Center, Utrecht, The NetherlandsBackground An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in patients with 22q11.2 deletion syndrome (22q11DS). Platybasia increases the depth of the velopharynx and is therefore postulated to contribute to velopharyngeal dysfunction. Our objective was to determine the clinical significance of platybasia in 22q11DS by exploring the relationship between cranial base angles and speech resonance. Methods In this retrospective chart review at a tertiary hospital, 24 children (age, 4.0-13.1 years) with 22q11.2DS underwent speech assessments and lateral cephalograms, which allowed for the measurement of the cranial base angles. Results One patient (4%) had hyponasal resonance, 8 (33%) had normal resonance, 10 (42%) had hypernasal resonance on vowels only, and 5 (21%) had hypernasal resonance on both vowels and consonants. The mean cranial base angle was 136.5° (standard deviation, 5.3°; range, 122.3-144.8°). The Kruskal-Wallis test showed no significant relationship between the resonance ratings and cranial base angles (P=0.242). Cranial base angles and speech ratings were not correlated (Spearman correlation=0.321, P=0.126). The group with hypernasal resonance had a significantly more obtuse mean cranial base angle (138° vs. 134°, P=0.049) but did not have a greater prevalence of platybasia (73% vs. 56%, P=0.412). Conclusions In this retrospective chart review of patients with 22q11DS, cranial base angles were not correlated with speech resonance. The clinical significance of platybasia remains unknown.http://www.thieme-connect.de/DOI/DOI?10.5999/aps.2014.41.4.344digeorge syndromeplatybasiavelopharyngeal insufficiency
spellingShingle Nicole E Spruijt
Moshe Kon
Aebele B Mink van der Molen
Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance
Archives of Plastic Surgery
digeorge syndrome
platybasia
velopharyngeal insufficiency
title Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance
title_full Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance
title_fullStr Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance
title_full_unstemmed Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance
title_short Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance
title_sort platybasia in 22q11 2 deletion syndrome is not correlated with speech resonance
topic digeorge syndrome
platybasia
velopharyngeal insufficiency
url http://www.thieme-connect.de/DOI/DOI?10.5999/aps.2014.41.4.344
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AT moshekon platybasiain22q112deletionsyndromeisnotcorrelatedwithspeechresonance
AT aebelebminkvandermolen platybasiain22q112deletionsyndromeisnotcorrelatedwithspeechresonance