X-linked Malformation and Cochlear Implantation
Artikel i vetenskaplig tidskrift, 2017

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.



sound localization



Pediatric otology


X-linked deafness


Neurosciences & Neurology

Congenital cochlear anomalies

Cochlear implantat


H. Smeds

Karolinska Institutet

Karolinska universitetssjukhuset

J. Wales

Karolinska Institutet

Karolinska universitetssjukhuset

Filip Asp

Chalmers, Signaler och system

U. Lofkvist

Universitetet i Oslo

Karolinska Institutet

B. Falahat

Karolinska universitetssjukhuset

B. M. Anderlid

Karolinska Institutet

L. Anmyr

Karolinska Institutet

Karolinska universitetssjukhuset

E. Karltorp

Karolinska universitetssjukhuset

Karolinska Institutet

Otology and Neurotology

1531-7129 (ISSN)

Vol. 38 1 38-46





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