TY - JOUR
T1 - Outcomes and Time to Emergence of Auditory Skills after Cochlear Implantation of Children with Charge Syndrome
AU - Young, Nancy M.
AU - Tournis, Elizabeth
AU - Sandy, Jenelle
AU - Hoff, Stephen R.
AU - Ryan, Maura
PY - 2017/9/1
Y1 - 2017/9/1
N2 - Objective: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). Study Design: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. Setting: Tertiary care children's hospital. Patients: Twelve children, seven males and five females. Mean age implant=3.5 years (1.7-8.2 yr); mean duration follow-up=4.7 years (1.5-10.1 yr). Intervention: Cochlear implantation. Main Outcome Measures: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. Results: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7dB SAT preoperative and 27.1dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. Conclusion: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.
AB - Objective: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). Study Design: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. Setting: Tertiary care children's hospital. Patients: Twelve children, seven males and five females. Mean age implant=3.5 years (1.7-8.2 yr); mean duration follow-up=4.7 years (1.5-10.1 yr). Intervention: Cochlear implantation. Main Outcome Measures: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. Results: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7dB SAT preoperative and 27.1dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. Conclusion: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.
KW - CHARGE syndrome
KW - Cochlear implant
KW - Cochlear malformation
KW - Cochlear nerve deficiency
KW - Expressive communication mode
KW - Speech perception
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U2 - 10.1097/MAO.0000000000001488
DO - 10.1097/MAO.0000000000001488
M3 - Article
C2 - 28657954
AN - SCOPUS:85021449609
VL - 38
SP - 1085
EP - 1091
JO - American Journal of Otology
JF - American Journal of Otology
SN - 1531-7129
IS - 8
ER -