Abstract
Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.
| Original language | English |
|---|---|
| Pages (from-to) | 1107-1112 |
| Number of pages | 6 |
| Journal | International Journal of Pediatric Otorhinolaryngology |
| Volume | 74 |
| Issue number | 10 |
| DOIs | |
| State | Published - Oct 1 2010 |
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Keywords
- Cochlear implantation
- Genetic deafness
- GJB2
- GJB3
- Hearing loss
- Jervell and Lange-Nielson
- Usher syndrome
- Wardenburg syndrome
ASJC Scopus subject areas
- Otorhinolaryngology
- Pediatrics, Perinatology, and Child Health
Cite this
Cochlear implantation in common forms of genetic deafness. / Vivero, Richard J.; Fan, Kenneth; Angeli, Simon I; Balkany, Thomas J.; Liu, Xue Z.
In: International Journal of Pediatric Otorhinolaryngology, Vol. 74, No. 10, 01.10.2010, p. 1107-1112.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Cochlear implantation in common forms of genetic deafness
AU - Vivero, Richard J.
AU - Fan, Kenneth
AU - Angeli, Simon I
AU - Balkany, Thomas J.
AU - Liu, Xue Z
PY - 2010/10/1
Y1 - 2010/10/1
N2 - Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.
AB - Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.
KW - Cochlear implantation
KW - Genetic deafness
KW - GJB2
KW - GJB3
KW - Hearing loss
KW - Jervell and Lange-Nielson
KW - Usher syndrome
KW - Wardenburg syndrome
UR - http://www.scopus.com/inward/record.url?scp=77956474070&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77956474070&partnerID=8YFLogxK
U2 - 10.1016/j.ijporl.2010.06.010
DO - 10.1016/j.ijporl.2010.06.010
M3 - Article
C2 - 20655117
AN - SCOPUS:77956474070
VL - 74
SP - 1107
EP - 1112
JO - International Journal of Pediatric Otorhinolaryngology
JF - International Journal of Pediatric Otorhinolaryngology
SN - 0165-5876
IS - 10
ER -