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Vol. 67. Issue 3.
Pages 142-147 (May - June 2016)
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Vol. 67. Issue 3.
Pages 142-147 (May - June 2016)
Original article
DOI: 10.1016/j.otoeng.2016.05.001
Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome
Malformaciones del oído, hipoacusia y rehabilitación auditiva en los niños con el síndrome de Treacher-Collins
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Francisco Rosa
Corresponding author
franciscorosa97@gmail.com

Corresponding author.
, Miguel Bebiano Coutinho, João Pinto Ferreira, Cecilia Almeida Sousa
Department of Otolaryngology and Neck-Facial Surgery, Hospital de Santo António-Hospital Centre of Porto, Portugal
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Tables (2)
Table 1. Anagraphic, surgical and audiometric data of the patients included into the study.
Table 2. Relationship between ear malformations and hearing loss.
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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.

Keywords:
Treacher Collins syndrome
Mandibulofacial dysostosis
Ear malformations
Hearing loss, Bone-anchored hearing aids
Hearing rehabilitation
Resumen
Objetivo

Evaluar las principales malformaciones del oído, hipoacusia y la rehabilitación auditiva en los niños con síndrome de Treacher-Collins.

Métodos

Estudio retrospectivo de 9 niños con síndrome de Treacher-Collins tratados en un hospital central, entre enero de 2003 y enero de 2013.

Resultados

Este estudio demostró una alta incidencia de malformaciones del oído externo y medio, como microtia, atresia o estenosis del conducto auditivo externo, la cavidad timpánica hipoplásica, la cadena de huesecillos dismórfica, o ausente. La mayoría de los pacientes tenían una hipoacusia de conducción grado moderado o alto. En los sujetos estudiados, se observó una mejoría funcional en los pacientes con audífono anclado en hueso en relación con los audífonos convencionales de conducción ósea.

Conclusiones

El síndrome de Treacher-Collins se caracteriza por malformaciones bilaterales del oído externo y medio. La rehabilitación auditiva en estos niños es muy importante, y el audífono anclado en hueso es el método de elección.

Palabras clave:
Síndrome de Treacher-Collins
Disostosis mandibulofacial
Hipoacusia
Audífono anclado en hueso
Rehabilitación auditiva
Malformaciones del oído

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