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Published on: February 29, 2020
Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome
Francisco Rosa1, Miguel Bebiano Coutinho1, João Pinto Ferreira1
1Department of Otolaryngology and Neck-Facial Surgery, Hospital de Santo António-Hospital Centre of Porto, Portugal.
Insights
Children with Treacher Collins syndrome often have bilateral ear malformations and hearing loss. Bone-anchored hearing aids offer significant functional improvement for auditory rehabilitation in these cases.
Area of Science:
- Otolaryngology
- Genetics
- Pediatric Medicine
Background:
- Treacher Collins syndrome is a rare genetic disorder characterized by craniofacial abnormalities.
- Ear malformations, including outer and middle ear anomalies, are common in Treacher Collins syndrome.
- Hearing loss is a significant complication, impacting speech and language development.
Purpose of the Study:
- To evaluate the spectrum of ear malformations in children with Treacher Collins syndrome.
- To assess the degree and type of hearing loss associated with the syndrome.
- To determine the effectiveness of auditory rehabilitation strategies, particularly bone-anchored hearing aids.
Main Methods:
- Retrospective analysis of 9 pediatric patients with Treacher Collins syndrome.
- Data collected from January 2003 to January 2013.
- Evaluation of audiological assessments and rehabilitation outcomes.
Main Results:
- High incidence of outer and middle ear malformations, including microtia, atresia, and ossicular abnormalities.
- Predominantly bilateral, moderate to severe hearing loss observed in most patients.
- Bone-anchored hearing aids demonstrated superior functional improvement compared to conventional bone conduction aids.
Conclusions:
- Treacher Collins syndrome frequently presents with bilateral outer and middle ear malformations.
- Effective hearing rehabilitation is crucial for affected children.
- Bone-anchored hearing aids are the preferred method for auditory rehabilitation in this population.
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.
