Change search
ReferencesLink to record
Permanent link

Direct link
Causes of hearing impairment in the Norwegian paediatric cochlear implant program
Department of Otolaryngology, Faculty Division Rikshospitalet, University of Oslo, Norway; Department of Otolaryngology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Show others and affiliations
2010 (English)In: International Journal of Audiology, ISSN 1499-2027, E-ISSN 1708-8186, Vol. 49, no 8, 596-605 p.Article in journal (Refereed) Published
Abstract [en]

Severe to profound hearing impairment (HI) is estimated to affect around 1/2000 young children. Advances in genetics have made it possible to identify several genes related to HI. This information can cast light upon prognostic factors regarding the outcome in cochlear implantation, and provide information both for scientific and genetic counselling purposes. From 1992 to 2005, 273 children from 254 families (probands) were offered cochlear implants in Norway. An evaluation of the causes of HI, especially regarding the genes GJB2, GJB6, SLC26A4, KCNQ1, KCNE1, and the mutation A1555G in mitochondrial DNA was performed in 85% of the families. The number of probands with unknown cause of HI was thus reduced from 120 to 68 (43% reduction). Ninety-eight (46%) of the probands had an identified genetic etiology of their HI. A relatively high prevalence of Jervell and Lange-Nielsen syndrome was found. The main causes of severe and profound HI were similar to those found in other European countries. GJB2 mutations are a common cause of prelingual HI in Norwegian cochlear implanted children.

Place, publisher, year, edition, pages
Informa Healthcare, 2010. Vol. 49, no 8, 596-605 p.
Keyword [en]
Connexin 26, Genetics, Severe and profound hearing loss, Cochlear implantation
National Category
URN: urn:nbn:se:umu:diva-60395DOI: 10.3109/14992021003743269ISI: 000280512600008PubMedID: 20553101OAI: diva2:559750
Available from: 2012-10-10 Created: 2012-10-10 Last updated: 2015-09-11Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Laurent, Claude
In the same journal
International Journal of Audiology

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 41 hits
ReferencesLink to record
Permanent link

Direct link