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Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder. Children born with BWS develop enlarged organs, including the tongue, a large body, and other signs. A woman with BWS was treated and followed for 30 years. Treatment consisted of tongue reduction, orthopedic and orthodontic treatment, orthognathic surgery, and retention. The patient was first treated when she was 5 years old. Her original orthodontic problems included macroglossia, anterior open bite, anterior crossbite, and a skeletal Class III jaw relationship caused by significant mandibular protrusion. The jaw-base relationships did not improve in the early preadolescent period after phase 1 of orthodontic treatment with a vertical chincap. With the growth spurt accompanying puberty, she developed a severe skeletal Class III jaw relationship and a constricted maxillary arch. Surgically assisted rapid maxillary expansion was performed at 23 years of age to correct the severe discrepancy between the maxillary and mandibular dental arch widths. Then, at 26 years, a LeFort I osteotomy, a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed after presurgical orthodontic treatment with extraction of the mandibular first molars. Both the facial profile and the occlusion were stable after 6 years of retention. This case report discusses the result of long-term observation of a patient with BWS who underwent tongue reduction, early orthodontic treatment, and surgical-orthodontic treatment.

Objective Owing to the complexity of the human language, numerous individuals develop different types of speech disorders. Some patients with cleft lip and/or palate (CLP) exhibit compensatory misarticulations (CMAs), which decrease speech fluency and interfere with the intelligibility of the speaker. The glottal stop (GS) is representative of CMAs in CLP patients. The glottal stop results from an occlusion or lack of airflow at the level of the glottis. In the context of speech therapy for CLP patients, intelligibility is usually used as an outcome measure. However, little is known about the behavioral and neurobiological mechanisms involved in the perception of speech produced by an individual with CMAs. We aimed to investigate the perception of GS compared to normal articulation (NA) using behavioral and neuroimaging experiments. Materials and methods Twenty healthy subjects without CMAs listened to auditory stimuli (NA and GS) and were asked to distinguish NA from GS using the button-press response during functional magnetic resonance imaging (fMRI). Results The reaction time for the perception of GS was significantly longer than that for NA. Additionally, the activation area and the percentage signal change in the auditory cortex were larger when the subjects listened to GS compared to NA. The auditory association area, which controls the higher-level processing of auditory signals, exhibited more intense bilateral activation in response to GS as compared to NA. Conclusions In subjects without GS, the perception of GS sounds may require more auditory attention and more complex auditory processing than the perception of NA sounds.