title: Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome.
abstract: OBJECTIVE
  The aim of this study was to assess the main ear malformations, hearing loss and auditory rehabilitation in children with Treacher Collins syndrome.
  
  
  METHODS
  We performed a retrospective study of 9 children with Treacher Collins syndrome treated in a central hospital between January 2003 and January 2013.
  
  
  RESULTS
  This study showed a high incidence of malformations of the outer and middle ear, such as microtia, atresia or stenosis of the external auditory canal, hypoplastic middle ear cavity, dysmorphic or missing ossicular chain. Most patients had bilateral hearing loss of moderate or high degree. In the individuals studied, there was functional improvement in patients with bone-anchored hearing aids in relation to conventional hearing aids by bone conduction.
  
  
  CONCLUSIONS
  Treacher Collins syndrome is characterized by bilateral malformations of the outer and middle ear. Hearing rehabilitation in these children is of utmost importance, and bone-anchored hearing aids is the method of choice.
authors.affiliation: Department of Otolaryngology and Neck-Facial Surgery, Hospital de Santo António-Hospital Centre of Porto, Portugal. Electronic address: franciscorosa97@gmail.com.
authors.forename: Francisco
authors.initials: F
authors.lastname: Rosa
authors.affiliation: Department of Otolaryngology and Neck-Facial Surgery, Hospital de Santo António-Hospital Centre of Porto, Portugal.
authors.forename: Miguel Bebiano
authors.initials: MB
authors.lastname: Coutinho
authors.affiliation: Department of Otolaryngology and Neck-Facial Surgery, Hospital de Santo António-Hospital Centre of Porto, Portugal.
authors.forename: João Pinto
authors.initials: JP
authors.lastname: Ferreira
authors.affiliation: Department of Otolaryngology and Neck-Facial Surgery, Hospital de Santo António-Hospital Centre of Porto, Portugal.
authors.forename: Cecilia Almeida
authors.initials: CA
authors.lastname: Sousa
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004431:Ear, External / Q000002:abnormalities*; D004432:Ear, Middle / Q000002:abnormalities*; D004867:Equipment Design; D005260:Female; D006310:Hearing Aids; D006312:Hearing Loss, Bilateral / Q000235:genetics* / Q000534:rehabilitation; D006314:Hearing Loss, Conductive / Q000235:genetics* / Q000534:rehabilitation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008342:Mandibulofacial Dysostosis / Q000150:complications*; D019683:Penetrance; D012189:Retrospective Studies; D055815:Young Adult
keywords: Audífono anclado en hueso; Disostosis mandibulofacial; Ear malformations; Hearing loss, Bone-anchored hearing aids; Hearing rehabilitation; Hipoacusia; Malformaciones del oído; Mandibulofacial dysostosis; Rehabilitación auditiva; Síndrome de Treacher-Collins; Treacher Collins syndrome