Early-stage use of hearing aids preserves auditory cortical structure in children with sensorineural hearing loss.

Hearing is critical to spoken language, cognitive, and social development. Little is known about how early auditory experiences impact the brain structure of children with bilateral sensorineural hearing loss. This study examined the influence of hearing aid use and residual hearing on the auditory cortex of children with severe to profound congenital sensorineural hearing loss. We evaluated cortical preservation in 103 young pediatric cochlear implant candidates (55 females and 48 males) by comparing their multivoxel pattern similarity of auditory cortical structure with that of 78 age-matched children with typical hearing. The results demonstrated that early-stage hearing aid use preserved the auditory cortex of children with bilateral congenital sensorineural hearing loss. Children with less residual hearing experienced a more pronounced advantage from hearing aid use. However, this beneficial effect gradually diminished after 17 months of hearing aid use. These findings support timely fitting of hearing aids in conjunction with early implantation to take advantage of neural preservation to maximize auditory and spoken language development.

Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder.

OBJECTIVE: To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD). STUDY DESIGN: Retrospective case review and parent survey. SETTING: Tertiary care children's hospital. PATIENTS: Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI = 3.5 years (0.8-11.8), mean age at diagnosis of ASD = 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up = 10.5 years (1.4-21.6). Parents of 7 children returned a survey. INTERVENTION: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change. RESULTS: Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use. Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking. CONCLUSIONS: Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.

Safety and Effectiveness of Cochlear Implantation of Young Children, Including Those With Complicating Conditions.

OBJECTIVE: Determine safety and effectiveness of cochlear implantation of children under age 37 months, including below age 12 months. STUDY DESIGN: Retrospective review. SETTING: Tertiary care children's medical center. PATIENTS: 219 children implanted before age 37 mos; 39 implanted below age 12 mos and 180 ages 12-36 mos. Mean age CI = 20.9 mos overall; 9.4 mos (5.9-11.8) and 23.4 mos (12.1-36.8) for the two age groups, respectively. All but two ≤12 mos (94.9%) received bilateral implants as did 70.5% of older group. Mean follow-up = 5.8 yrs; age last follow-up = 7.5 yrs, with no difference between groups. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Surgical and anesthesia complications, measurable open-set speech discrimination, primary communication mode(s). RESULTS: Few surgical complications occurred, with no difference by age group. No major anesthetic morbidity occurred, with no critical events requiring intervention in the younger group while 4 older children experienced desaturations or bradycardia/hypotension. Children implanted under 12 mos developed open-set earlier (3.3 yrs vs 4.3 yrs, p ≤ 0.001) and were more likely to develop oral-only communication (88.2% vs 48.8%, p ≤ 0.001). A significant decline in rate of oral-only communication was present if implanted over 24 months, especially when comparing children with and without additional conditions associated with language delay (8.3% and 35%, respectively). CONCLUSIONS: Implantation of children under 37 months of age can be done safely, including those below age 12 mos. Implantation below 12 mos is positively associated with earlier open-set ability and oral-only communication. Children implanted after age 24 months were much less likely to use oral communication exclusively, especially those with complex medical history or additional conditions associated with language delay.

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.

Pediatric cochlear implantation of children with eighth nerve deficiency.

OBJECTIVE: To evaluate the auditory outcomes of children implanted in an ear with eighth nerve hypoplasia or aplasia and to determine whether preoperative characteristics are predictive of auditory performance achieved. STUDY DESIGN: retrospective case review. SETTING: tertiary care medical center. PATIENTS: ten children implanted in an ear with eighth nerve hypoplasia or aplasia, as determined by high resolution magnetic resonance imaging. MAIN OUTCOME MEASURES: Neural response test measurements, detection and speech awareness thresholds, Meaningful Auditory Integration Scale scores, as well as speech perception level achieved. RESULTS: Post-implantation, three children demonstrated little to no detection of sound, three had improved detection and awareness of environmental sounds, one developed closed-set speech perception and spoken language, and three developed open-set speech perception and spoken language. No imaging findings appeared related to outcomes. Significantly better implant detection thresholds and Meaningful Auditory Integration Scale scores were found in children who had preoperative aided auditory detection (p's ≤ 0.02-0.05). CONCLUSION: Some children with eighth nerve hypoplasia or aplasia may derive significant benefit from a cochlear implant. In our study high resolution magnetic resonance imaging was more sensitive than high resolution computer tomography in detecting neural deficiency. However, no imaging findings were predictive of auditory performance level achieved post-implantation.