title: Speech outcomes in children with 22q11.2 deletion syndrome following surgery for velopharyngeal insufficiency.
abstract: OBJECTIVE
  The purpose of this study was to identify prognostic factors associated with improved speech outcomes following surgical correction for velopharyngeal insufficiency (VPI) in pediatric patients with 22q11.2 deletion syndrome (22q11DS).
  
  
  METHODS
  Eighteen patients were identified via retrospective chart review of patients with 22q11DS between 2005 and 2014. Patient characteristics, medical histories, associated comorbidities, surgical procedures, and pre- and postoperative perceptual hypernasality (subjectively rated 1-5 with 5 being the most severe) were gathered for each patient.
  
  
  RESULTS
  12 patients (67%) experienced improvement in hypernasality following corrective surgery for VPI. Higher severity of hypernasality preoperatively was found to be indicative of a lower chance of improvement with VPI surgery. Of 8 patients with a preoperative hypernasality score of 5, 3 (38%) showed improvement in hypernasality postoperatively, while 9 out of 10 (90%) of patients with a preoperative hypernasality score less than 5 showed postoperative improvement. Females were also found to have worse speech outcomes compared to males.
  
  
  CONCLUSIONS
  22q11DS patients presenting with severely hypernasal speech preoperatively are less likely to show improvement in hypernasality following corrective surgery for VPI. Those patients with moderate hypernasality are most likely to benefit from surgery.
authors: Department of Otolaryngology, Washington University School of Medicine in Saint Louis, 4921 Parkview Place, Suite A, 11th Floor, St. Louis, MO, 63110, USA.
authors: Pawina
authors: P
authors: Jiramongkolchai
authors: Emory University School of Medicine, 100 Woodruff Circle, Atlanta, GA, 30322, USA.
authors: Manvinder S
authors: MS
authors: Kumar
authors: Department of Otolaryngology, Vanderbilt University Medical Center, 1215 21st Avenue South, Medical Center East, South Tower, Nashville, TN, 37232, USA.
authors: Derrick
authors: D
authors: Sowder
authors: Department of Otolaryngology, Vanderbilt University Medical Center, 1215 21st Avenue South, Medical Center East, South Tower, Nashville, TN, 37232, USA.
authors: Sivakumar
authors: S
authors: Chinnadurai
authors: Department of Otolaryngology, Vanderbilt University Medical Center, 1215 21st Avenue South, Medical Center East, South Tower, Nashville, TN, 37232, USA.
authors: Christopher T
authors: CT
authors: Wootten
authors: Department of Otolaryngology - Head & Neck Surgery, Emory University School of Medicine, 2015 Uppergate Drive, Room 222, Atlanta, GA, 30322, USA. Electronic address: steven.goudy@emory.edu.
authors: Steven L
authors: SL
authors: Goudy
mesh_terms: D002648:Child; D004062:DiGeorge Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D063868:Patient Outcome Assessment; D011379:Prognosis; D012189:Retrospective Studies; D013064:Speech Disorders / Q000209:etiology / Q000601:surgery*; D014681:Velopharyngeal Insufficiency / Q000209:etiology / Q000601:surgery*
keywords: 22q11.2 microdeletion syndrome; Cleft palate; DiGeorge syndrome; Speech articulation disorder; Velocardiofacial syndrome; Velopharyngeal insufficiency