Impact of Tongue Reduction on Overall Speech Intelligibility, Articulation and Oromyofunctional Behavior in 4 Children with Beckwith-Wiedemann Syndrome

Abstract

Objective: The purpose of this study was to determine the impact of partial glossectomy (using the keyhole technique) on speech intelligibility, articulation, resonance and oromyofunctional behavior. Patients and Methods: A partial glossectomy was performed in 4 children with Beckwith- Wiedemann syndrome between the ages of 0.5 and 3.1 years. An ENT assessment, a phonetic inventory, a phonemic and phonological analysis and a consensus perceptual evaluation of speech intelligibility, resonance and oromyofunctional behavior were performed. Results: It was not possible in this study to separate the effects of the surgery from the typical developmental progress of speech sound mastery. Improved speech intelligibility, a more complete phonetic inventory, an increase in phonological skills, normal resonance and increased motor-oriented oral behavior were found in the postsurgical condition. The presence of phonetic distortions, lip incompetence and interdental tongue position were still present in the postsurgical condition. Conclusion: Speech therapy should be focused on correct phonetic placement and a motor-oriented approach to increase lip competence, and on functional tongue exercises and tongue lifting during the production of alveolars. Detailed analyses in a larger number of subjects with and without Beckwith-Wiedemann syndrome may help further illustrate the long-term impact of partial glossectomy.

Single-Word Speech Intelligibility in Children and Adults With Down Syndrome

“…However, it cannot be assumed that all interventions affecting the tongue or oral cavity will lead to improved speech. Partial glossectomy does not necessarily result in improved speech intelligibility in either DS or Beckwith-Wiedemann syndrome (Margar-Bacal, Witzel, & Munro, 1987;Van Lierde et al, 2012). A variety of interventions have been introduced to improve appearance, reduce drooling, and facilitate swallowing in DS, but it is important to ensure that such treatments do not have deleterious effects on speech production.…”

Early Therapeutic Intervention in a Child with Beckwith–Wiedemann Syndrome in Inpatient and Outpatient Conditions — Case Report

“…The results of the study also show that after a surgery, comprehension of the speech improves, phonological skills develop, however training the correct position of the articulation organs is still necessary as the habit of the interdental tongue placement is persistent [30].…”

Current Status of Nursing Research on Beckwith Wiedemann Syndrome Based on Co-word Analysis

“…Some scholars suggest that there are 2 main criteria (abdominal wall defect, giant tongue, giant body) or a major standard plus 2 secondary criteria (mega kidney or kidney malformation, adrenal hypertrophy, genetic or chromosomal abnormalities, polyhydramnios, etc.) can be diagnosed, but more case support is needed to reach an agreement [14]. BWS is difficult to detect early in the birth examination and is easily missed.…”