Cornelia De Lange Syndrome and Cochlear Implantation
Introduction: Literature regarding the different degrees of hearing loss in patients with Cornelia de Lange syndrome (CDLS) reports that half of the affected patients exhibit severe to profound sensorineural hearing loss. We present the first pre-school child with CDLS who underwent cochlear implant...
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| Format: | Article |
| Language: | English |
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Mashhad University of Medical Sciences
2018-11-01
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| Series: | Iranian Journal of Otorhinolaryngology |
| Subjects: | |
| Online Access: | http://ijorl.mums.ac.ir/article_11767_731f2d23917492221c98aff08faed8f3.pdf |
| _version_ | 1818178370596241408 |
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| author | George Psillas Stefanos Triaridis Vasiliki Chatzigiannakidou Jiannis Constantinidis |
| author_facet | George Psillas Stefanos Triaridis Vasiliki Chatzigiannakidou Jiannis Constantinidis |
| author_sort | George Psillas |
| collection | DOAJ |
| description | Introduction: Literature regarding the different degrees of hearing loss in patients with Cornelia de Lange syndrome (CDLS) reports that half of the affected patients exhibit severe to profound sensorineural hearing loss. We present the first pre-school child with CDLS who underwent cochlear implantation for congenital profound sensorineural hearing loss. Case Report: A 3-year-old boy with CDLS underwent unilateral cochlear implantation for bilateral profound sensorineural hearing loss. He had characteristic facial features, bushy eyebrows and synophrys, limb anomalies, growth and mental retardation. Based on the results of postoperative speech perception and production tests, his gain in language skills and expressive vocabulary was modest. However, a cochlear implantation had a significant effect on auditory development, in terms of making him aware of sound localization and the different types of environmental sound. Conclusion: Criteria for cochlear implantation are expanding and now include children with disabilities in addition to deafness, such as those with CDLS. Profoundly hearing-impaired children affected by borderline mental retardation should be considered as potential candidates for cochlear implantation. |
| first_indexed | 2024-12-11T20:46:54Z |
| format | Article |
| id | doaj.art-9d6967aaa55c4d4581457c3362483c8c |
| institution | Directory Open Access Journal |
| issn | 2251-7251 2251-726X |
| language | English |
| last_indexed | 2024-12-11T20:46:54Z |
| publishDate | 2018-11-01 |
| publisher | Mashhad University of Medical Sciences |
| record_format | Article |
| series | Iranian Journal of Otorhinolaryngology |
| spelling | doaj.art-9d6967aaa55c4d4581457c3362483c8c2022-12-22T00:51:20ZengMashhad University of Medical SciencesIranian Journal of Otorhinolaryngology2251-72512251-726X2018-11-0130636937310.22038/ijorl.2018.25075.181311767Cornelia De Lange Syndrome and Cochlear ImplantationGeorge Psillas0Stefanos Triaridis1Vasiliki Chatzigiannakidou2Jiannis Constantinidis3Ist Academic ENT Department, AHEPA Hospital, Aristotle University of Thessaloniki1st Academic ENT Department, AHEPA Hospital, Aristotle University of Thessaloniki1st Academic ENT Department, AHEPA Hospital, Aristotle University of Thessaloniki1st Academic ENT Department, AHEPA Hospital, Aristotle University of ThessalonikiIntroduction: Literature regarding the different degrees of hearing loss in patients with Cornelia de Lange syndrome (CDLS) reports that half of the affected patients exhibit severe to profound sensorineural hearing loss. We present the first pre-school child with CDLS who underwent cochlear implantation for congenital profound sensorineural hearing loss. Case Report: A 3-year-old boy with CDLS underwent unilateral cochlear implantation for bilateral profound sensorineural hearing loss. He had characteristic facial features, bushy eyebrows and synophrys, limb anomalies, growth and mental retardation. Based on the results of postoperative speech perception and production tests, his gain in language skills and expressive vocabulary was modest. However, a cochlear implantation had a significant effect on auditory development, in terms of making him aware of sound localization and the different types of environmental sound. Conclusion: Criteria for cochlear implantation are expanding and now include children with disabilities in addition to deafness, such as those with CDLS. Profoundly hearing-impaired children affected by borderline mental retardation should be considered as potential candidates for cochlear implantation.http://ijorl.mums.ac.ir/article_11767_731f2d23917492221c98aff08faed8f3.pdfCochlear ImplantationDe Lange Syndromehearing lossChildPreschool |
| spellingShingle | George Psillas Stefanos Triaridis Vasiliki Chatzigiannakidou Jiannis Constantinidis Cornelia De Lange Syndrome and Cochlear Implantation Iranian Journal of Otorhinolaryngology Cochlear Implantation De Lange Syndrome hearing loss Child Preschool |
| title | Cornelia De Lange Syndrome and Cochlear Implantation |
| title_full | Cornelia De Lange Syndrome and Cochlear Implantation |
| title_fullStr | Cornelia De Lange Syndrome and Cochlear Implantation |
| title_full_unstemmed | Cornelia De Lange Syndrome and Cochlear Implantation |
| title_short | Cornelia De Lange Syndrome and Cochlear Implantation |
| title_sort | cornelia de lange syndrome and cochlear implantation |
| topic | Cochlear Implantation De Lange Syndrome hearing loss Child Preschool |
| url | http://ijorl.mums.ac.ir/article_11767_731f2d23917492221c98aff08faed8f3.pdf |
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