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X-linked Malformati...
X-linked Malformation and Cochlear Implantation
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- Smeds, H. (författare)
- Karolinska Institutet,Karolinska universitetssjukhuset,Karolinska University Hospital
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- Wales, J. (författare)
- Karolinska universitetssjukhuset,Karolinska University Hospital,Karolinska Institutet
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- Asp, Filip (författare)
- Karolinska Institutet
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- Lofkvist, U. (författare)
- Karolinska Institutet,Universitetet i Oslo,University of Oslo
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- Falahat, B. (författare)
- Karolinska universitetssjukhuset,Karolinska University Hospital
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- Anderlid, B. M. (författare)
- Karolinska Institutet
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- Anmyr, L. (författare)
- Karolinska Institutet,Karolinska universitetssjukhuset,Karolinska University Hospital
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- Karltorp, E. (författare)
- Karolinska Institutet,Karolinska universitetssjukhuset,Karolinska University Hospital
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(creator_code:org_t)
- Ovid Technologies (Wolters Kluwer Health), 2017
- 2017
- Engelska.
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Ingår i: Otology and Neurotology. - : Ovid Technologies (Wolters Kluwer Health). - 1531-7129 .- 1537-4505. ; 38:1, s. 38-46
- Relaterad länk:
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http://dx.doi.org/10...
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https://doi.org/10.1...
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https://research.cha...
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Abstract
Ämnesord
Stäng
- Objective: To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. Study Design: Retrospective patient review in combination with a multidisciplinary follow-up. Patients: Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015. Nine of the children had a mutation affecting the gene POU3F4 on Xq21. Main Outcome Measures: Surgical events, intraoperative measures and electrical stimulation levels, hearing and spoken language abilities. Results: In all, 15 cochlear implantations were performed. In three cases the electrode was found to be in the internal auditory canal on intraoperative x-ray and repositioned successfully. One child had a postoperative rhinorrhea confirmed to be cerebrospinal fluid but this resolved on conservative treatment. No severe complications occurred. Postoperative electrical stimulation levels were higher in 9 of 10 children, as compared with typically reported average levels in patients with a normal cochlea. Eight patients developed spoken language to various degrees while two were still at precommunication level. However, speech recognition scores were lower than average pediatric cases. Conclusion: Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Odontologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Dentistry (hsv//eng)
Nyckelord
- Cochlear implantat
- children
- mutations
- X-
- Neurosciences & Neurology
- sound localization
- Pediatric otology
- Otorhinolaryngology
- POU3F4
- deafness
- X-linked deafness
- Congenital cochlear anomalies
Publikations- och innehållstyp
- art (ämneskategori)
- ref (ämneskategori)
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