Long-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with BeckwithWiedemann syndromeLong-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with BeckwithWiedemann syndrome
Faculty of Medicine and Health Sciences
Research group
Medical Genetics (MEDGEN)
Publication type
Human medicine
Source (journal)
International journal of pediatric otorhinolaryngology. - Amsterdam
74(2010):3, p. 309-3018
Target language
English (eng)
Full text (Publishers DOI)
The purpose of the present case study was to determine the long-term impact of partial glossectomy (using the keyhole technique) on overall speech intelligibility and articulation in a Dutch-speaking child with BeckwithWiedemann syndrome (BWS). Furthermore the present study is meant as a contribution to the further delineation of the phonation, resonance, articulation and language characteristics and oral behaviour in a child with BWS. Detailed information on the speech and language characteristics of children with BWS may lead to better guidance of pediatric management programs. The child's speech was assessed 9 years after partial glossectomy with regard to ENT characteristics, overall intelligibility (perceptual consensus evaluation), articulation (phonetic and phonological errors), voice (videostroboscopy, vocal quality), resonance (perceptual, nasometric assessment), language (expressive and receptive) and oral behaviour. A class III malocclusion, an anterior open bite, diastema, overangulation of lower incisors and an enlarged but normal symmetric shaped tongue were present. The overall speech intelligibility improved from severely impaired (presurgical) to slightly impaired (5 months post-glossectomy) to normal (9 years postoperative). Comparative phonetic inventory showed a remarkable improvement of articulation. Nine years post-glossectomy three types of distortions seemed to predominate: a rhotacism and sigmatism and the substitution of the alveolar /z/. Oral behaviour, vocal characteristics and resonance were normal, but problems with expressive syntactic abilities were present. The long-term impact of partial glossectomy, using the keyhole technique (preserving the vascularity and the nervous input of the remaining intrinsic tongue muscles), on speech intelligibility, articulation, and oral behaviour in this Dutch-speaking child with congenital macroglossia can be regarded as successful. It is not clear how these expressive syntactical problems demonstrated in this child can be explained. Certainly they are not part of a more general developmental delay, hearing problems or cognitive malfunctioning. To what extent the presence of expressive syntactical problems is a possible aspect of the phenotypic spectrum of children with BWS is subject for further research. Multiple variables, both known and unknown can affect the long-term outcome after partial glossectomy in a child with BWS. The timing and type of the surgical technique, hearing and cognitive functioning are known variables in this study. But variables such as children's motivation, the contribution of the motor-oriented speech therapy, the parental articulation input and stimulation and other family, school and community factors are unknown and are all factors which can influence speech outcome after partial glossectomy. Detailed analyses in a greater number of subjects with BWS may help further illustrate the long-term impact of partial glossectomy.