X-linked Malformation and Cochlear Implantation.
Otol Neurotol
; 38(1): 38-46, 2017 01.
Article
em En
| MEDLINE
| ID: mdl-27779564
ABSTRACT
OBJECTIVE:
To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. STUDYDESIGN:
Retrospective patient review in combination with a multidisciplinary follow-up.SETTING:
Tertiary referral hospital and cochlear implant program. 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. INTERVENTION Cochlear implantation. MAIN OUTCOMEMEASURES:
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.
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Base de dados:
MEDLINE
Assunto principal:
Implante Coclear
/
Fatores do Domínio POU
/
Perda Auditiva Neurossensorial
Tipo de estudo:
Observational_studies
/
Risk_factors_studies
Limite:
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article