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.
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.
Importance: Children with bilateral sensorineural hearing loss (SNHL) benefit from binaural amplification provided by hearing aids (HA) to develop auditory skills, language and social abilities. However, few studies have examined the effect of amplification on brain structure in children with SNHL. This study evaluates a group of children with bilateral SNHL who were cochlear implant candidates.Objective/Design/Setting: To investigate whether the extent of HA use before cochlear implantation (CI) modulates neuroanatomical structural changes in the brain. A cross-sectional, population-based study was conducted.Participants: One hundred and three children with bilateral SNHL who were candidates for CI. Seventy-eight children with normal hearing were selected from the NIH MRI Study of Normal Brain Development.Exposures: The length of HA use was obtained. Moreover, the unaided speech-frequency pure-tone average (PTA) was measured to represent residual hearing.Main Outcomes and Measures: Multivoxel pattern similarity analysis was used to examine the similarity of gray matter (GM) density in the local spatial morphological pattern of the auditory cortex for children with SNHL in comparison with age expectations (children with normal hearing).Results: One hundred and three children with hearing loss were included (55 females and 48 males). The mean (SD) age was 15.84 (11.53) months. Children’s GM preservation in the left Heschl’s gyrus was nonlinearly affected by their experience of HA use (β = -0.380, p = 0.03). During the first year of HA use, children who had longer experience of HA use showed higher GM preservation, while further use of HA attenuated their GM preservation. Such effect was moderated by the degree of HL (β = -0.347, p = 0.03). Children with worse residual hearing benefited more from HA use compared to those with better residual hearing. Conclusions and Relevance: Amplification may prevent auditory structural changes which result from a lack of auditory input in children with HL, especially those whose loss is in the profound range. Although HA does not provide adequate audibility of spoken language for children who are cochlear implant candidates, pre-CI use of HA serves to preserve the auditory cortex during the first year of HA use. After the first year, this effect disappears, which reinforces the need for early cochlear implantation as the main treatment modality for hearing loss.
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 ¼ 3.5 years (0.8-11.8), mean age at diagnosis of ASD ¼ 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up ¼ 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.
Prediction of improvement in speech perception after cochlear implantation (CI) is clinically important to optimize pediatric habilitation. Conventional methods using non-neural measures do not permit accurate prediction on the individual child level. In this study, we investigate whether white matter patterns detected by diffusion tensor imaging (DTI) magnetic resonance imaging (MRI) predict speech perception improvement. Pre-surgical DTI of CI candidates was compared to matched normal-hearing (NH) children to determine cortical regions affected by hearing impairment. Speech Recognition Index in Quiet was measured at baseline and 6 months post implantation to compute improvement in speech perception. Machine learning prediction of speech perception improvement (high or low) was performed using non-imaging and DTI white matter characteristics of whole, affected and unaffected brain. Affected and unaffected white matter regions were determined by comparing DTI multi-voxel pattern similarity maps of white matter integrity indices between CI and NH. Abnormal white matter patterns were found throughout brain of CI candidates. Prediction of 6-month post-CI improvement accuracy, sensitivity and specificity using unaffected regions (0.86, 0.91, 0.80, respectively) and whole brain white matter (0.85, 0.91, 0.80, respectively) yielded similar results, and were more predictive than regions affected by hearing impairment (0.72, 0.74, 0.70, respectively) or non-imaging features (0.67, 0.55, 0.78, respectively). Findings support that presurgical neural white matter pathways, especially in the association auditory and cognitive regions not affected by auditory deprivation, play a critical role in speech development after CI and are more predictive of outcome than traditional non-neural variables such as age at implant.
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