Jamie L Perry1, Katelyn J Kotlarek2, Kelly Spoloric1, Adriane Baylis3,4, Lakshmi Kollara5, Jonathan M Grischkan6,7, Richard Kirschner8,9, David Gregory Bates10,11, Mark Smith11, Ursula Findlen12,13. 1. Department of Communication Sciences and Disorders, East Carolina University, Greenville, NC, USA. 2. Division of Communication Disorders, University of Wyoming, Laramie, WY, USA. 3. Section of Plastic and Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH, USA. 4. Clinical Plastic Surgery, The Ohio State University College of Medicine, Columbus, OH, USA. 5. Department of Clinical Health Sciences, Texas A&M University, Kingsville, TX, USA. 6. Department of Pediatric Otolaryngology, Nationwide Children's Hospital, Columbus, OH, USA. 7. Otolaryngology-Head & Neck Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA. 8. Plastic and Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH, USA. 9. Surgery at The Ohio State University College of Medicine, Columbus, OH, USA. 10. Department of Radiology, Nationwide Children's Hospital, Columbus, OH, USA. 11. Radiology at The Ohio State University College of Medicine, Columbus, OH, USA. 12. Division of Clinical Therapies, Audiology Department, Nationwide Children's Hospital, Columbus, OH, USA. 13. Clinical, Department of Otolaryngology, Head and Neck Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Abstract
PURPOSE: To investigate the dimensions of the tensor veli palatini (TVP) muscle using high image resolution 3-dimensional magnetic resonance imaging (MRI) of the soft palate among children with normal velopharyngeal and craniofacial anatomy and to compare values to individuals with a diagnosis of 22q11.2 deletion syndrome (22q11DS). We also sought to determine whether there is a relationship between hypoplasia of the TVP and severity of middle ear dysfunction and hearing loss. METHODS: Three-dimensional MRI were used to collect and analyze data obtained across 53 children between 4 and 12 years of age, including 40 children with normal velopharyngeal and craniofacial anatomy and 13 children with a diagnosis of 22q11.2 DS. Tensor veli palatini muscle length, thickness, and volume as well as bihamular distance were compared among participant groups. RESULTS: A Welch's t-test revealed that the TVP in participants with 22q11DS is significantly shorter (P = .005, 17.3 vs 19.0 mm), thinner (P < .001, 1.1 vs 1.8 mm), and less voluminous (P < .001, 457.5 vs 667.3 mm3) than participants without 22q11DS. Participants with 22q11DS also had a greater (P = .006, 27.7 vs 24.7 mm) bihamular distance than participants without 22q11DS. There was an inverse relationship between TVP abnormalities noted above and the severity of audiologic and otologic histories. CONCLUSION: The TVP muscle is substantially reduced in volume, length, and thickness in children with 22q11DS. These findings serve as preliminary support for the association of patient hearing and otologic severity and TVP dysmorphology.
PURPOSE: To investigate the dimensions of the tensor veli palatini (TVP) muscle using high image resolution 3-dimensional magnetic resonance imaging (MRI) of the soft palate among children with normal velopharyngeal and craniofacial anatomy and to compare values to individuals with a diagnosis of 22q11.2 deletion syndrome (22q11DS). We also sought to determine whether there is a relationship between hypoplasia of the TVP and severity of middle ear dysfunction and hearing loss. METHODS: Three-dimensional MRI were used to collect and analyze data obtained across 53 children between 4 and 12 years of age, including 40 children with normal velopharyngeal and craniofacial anatomy and 13 children with a diagnosis of 22q11.2 DS. Tensor veli palatini muscle length, thickness, and volume as well as bihamular distance were compared among participant groups. RESULTS: A Welch's t-test revealed that the TVP in participants with 22q11DS is significantly shorter (P = .005, 17.3 vs 19.0 mm), thinner (P < .001, 1.1 vs 1.8 mm), and less voluminous (P < .001, 457.5 vs 667.3 mm3) than participants without 22q11DS. Participants with 22q11DS also had a greater (P = .006, 27.7 vs 24.7 mm) bihamular distance than participants without 22q11DS. There was an inverse relationship between TVP abnormalities noted above and the severity of audiologic and otologic histories. CONCLUSION: The TVP muscle is substantially reduced in volume, length, and thickness in children with 22q11DS. These findings serve as preliminary support for the association of patient hearing and otologic severity and TVP dysmorphology.
Entities:
Keywords:
22q11.2 deletion syndrome; 3-dimensional reconstruction; eustachian tube dysfunction; magnetic resonance imaging; morphology; tensor veli palatini
Authors: A K Ryan; J A Goodship; D I Wilson; N Philip; A Levy; H Seidel; S Schuffenhauer; H Oechsler; B Belohradsky; M Prieur; A Aurias; F L Raymond; J Clayton-Smith; E Hatchwell; C McKeown; F A Beemer; B Dallapiccola; G Novelli; J A Hurst; J Ignatius; A J Green; R M Winter; L Brueton; K Brøndum-Nielsen; P J Scambler Journal: J Med Genet Date: 1997-10 Impact factor: 6.318
Authors: E Verheij; A L Kist; A B Mink van der Molen; I Stegeman; G A van Zanten; W Grolman; H G X M Thomeer Journal: Eur Arch Otorhinolaryngol Date: 2016-11-11 Impact factor: 2.503