Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome.

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 51.7 dB SAT preoperative and 27.1 dB 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.