title: Bone-anchored hearing aid (Baha) in patients with Treacher Collins syndrome: tips and pitfalls.
abstract: OBJECTIVE
  Treacher Collins syndrome, also known as mandibulofacial dysostosis, is an autosomal dominant disorder of the cranio-facial morphogenesis affecting 1 of 50,000 live newborns. Most children with this disease present with bilateral, severe conductive hearing loss due to bilateral aural atresia. Auditory rehabilitation of these children can be effectively carried out with bone-anchored hearing aids (Baha). The aim of this retrospective study is to review the "Bambino Gesù" Children's Hospital's experience with Baha in Treacher Collins patients, highlighting the tips and pitfalls of Baha surgery in this particular population.
  
  
  METHODS
  The clinical charts were reviewed of all children with a Treacher Collins syndrome diagnosis receiving a Baha in the "Bambino Gesù" Children's Hospital from January 1995 to January 2010. Data were collected concerning patients' anagraphics and medical history, comorbidities, surgical technique, complications and functional outcome.
  
  
  RESULTS
  23 Treacher Collins children were included. A two-stage surgery was adopted in 51% (n=12) cases, while a one-stage approach was chosen in 49% (n=11). 2 children underwent 1st stage surgery as they were younger than 5. There were neither cases of osseointegration failure, nor cases of traumatic implant loss. Overall, the local complication rate was not different in the one-stage and in the two-stage group. The functional gain was significantly better with the Baha than with the conventional bone-conduction hearing aids (p<0.0002). There was no difference in terms of functional outcome between the 7 patients receiving Baha at an age younger than 5 years and the rest (p=0.23).
  
  
  CONCLUSIONS
  Baha can provide effective auditory rehabilitation for children with Treacher Collins syndrome, as long as it is performed in a tertiary care center where a multidisciplinary approach to the frequent comorbidities is possible. The main challenge of Baha surgery in this population is the poor or irregular thickness of the patient's calvarial bone, which often makes it necessary to drill additional holes, to place the fixture in contact with the dura or to use materials for bone augmentation.
authors.affiliation: "Bambino Gesù" Children's Hospital, Rome, Vatican City State.
authors.forename: Pasquale
authors.initials: P
authors.lastname: Marsella
authors.forename: Alessandro
authors.initials: A
authors.lastname: Scorpecci
authors.forename: Concettina
authors.initials: C
authors.lastname: Pacifico
authors.forename: Luigi
authors.initials: L
authors.lastname: Tieri
mesh_terms: D000293:Adolescent; D001844:Bone Conduction; D002648:Child; D002675:Child, Preschool; D005260:Female; D006310:Hearing Aids / Q000009:adverse effects; D006314:Hearing Loss, Conductive / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D008342:Mandibulofacial Dysostosis / Q000150:complications / Q000601:surgery*; D011183:Postoperative Complications; D012189:Retrospective Studies; D053441:Suture Anchors / Q000009:adverse effects; D016896:Treatment Outcome