Rehabilitation in Franceschetti syndrome: an interdisciplinary approach using bone-anchored hearing aids

The purpose of the study was to determine the effectiveness of a concept of combined interdisciplinary rehabilitation for children with mandibulofacial dysostosis, developed at the Center for Facial Malformations. It consists of binaural implantation of bone-anchored hearing aids and gradual distraction of the mandible. After audiological testing and mandibular distraction on a phantom head designed with data from a spiral CT, the surgery was done in three steps: implanting the fixtures for BAHA and the bone-lengthening device, removing the device after six weeks and completing the BAHA implantation two months later. The distraction procedure and orthodontic treatment were performed on an outpatient basis. The results (six patients, ages 6-19 years) were excellent: after implantation of the BAHA system speech perception increased from approximately 85% with the conventional BCHA to 95-100% with the BAHA. Quality of life was reported to be much better because of the general cosmetic improvement as well as the good acoustic orientation and sound quality with the new hearing devices. We conclude that the interdisciplinary approach provides favorable conditions for rehabilitation in cases of complex malformations of the head and neck.