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- Title
Cochlear implantation in congenital middle and inner ear malformations in children.
- Authors
Porowski, M.; Skarżyński, H.; Skarżyński, P. H.; Mrówka, M.; Pastuszak, A.
- Abstract
Introduction: Congenital structural inner ear malformations in syndromic patients are very rare and also difficult to manage. To list some of these syndromes: Treacher Collins, Goldenhar, Apert, Crouzon. Some syndromes, like branchiooculo- facial are so extremely rare, that we could see only one or two such cases in our practice. Aim: The aim of the study was to show surgical technique used for cochlear implantation in patients with some of these rare syndromes and discuss the results obtained after surgical treatment. Methods: Patients with BOF, Goldenhar, EEC, and Goltz-Gorlin syndromes were qualified for CI treatment because of the bilateral sensorineural hearing loss and no effects with hearing aids. There were several problems during the operations: small mastoid, hypoplasia of the cochlea, congenital deformation of the ossicles, temporal bone hypoplasia and facial nerve displacement. We used Med-El implant systems in three patients and Nucleus system in one patient with Goldenhar syndrome. The technique of cochlear implantation was facial recess. Results: In all these cases we achieved satisfactory hearing levels although the results are worse than in children without structural deformities of the inner ear. Also, the results depend on malformation type and concomitant medical condition. No early or late surgical complications were observed. Conclusions: We point that even in cases with complicated anatomy and congenital malformations there is possibility to improve hearing using implantable devices. It is of crucial importance to focus on hearing improvement to maintain appropriate hearing levels and to give chance for those children to be active society members.
- Subjects
POLAND; MIDDLE ear abnormalities; COCHLEAR implants; INNER ear; CONFERENCES &; conventions; TREATMENT effectiveness; CHILDREN
- Publication
Journal of Hearing Science, 2022, Vol 12, Issue 1, p159
- ISSN
2083-389X
- Publication type
Article