Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral
permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of
middle-ear implant technologies are opening new perspectives for restoring hearing. Active
middle-ear implants can be used in children and adolescents with
hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in
sensorineural hearing loss active
middle-ear implants are now successfully used in patients with conductive and
mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and
PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral
mixed hearing loss and multiple
dyslalia associated with Franceschetti syndrome (
mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The
middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.