Although deaf children with cochlear implants (CIs) are able to develop good language skills, the large variability in outcomes remains a significant concern. The first aim of this study was to evaluate language skills in children with CIs to establish benchmarks. The second aim was to make an estimation of the optimal age at implantation to provide maximal opportunities for the child to achieve good language skills afterward. The third aim was to gain more insight into the causes of variability to set recommendations for optimizing the rehabilitation process of prelingually deaf children with CIs. Receptive and expressive language development of 288 children who received CIs by age five was analyzed in a retrospective multicenter study. Outcome measures were language quotients (LQs) on the Reynell Developmental Language Scales and Schlichting Expressive Language Test at 1, 2, and 3 years after implantation. Independent predictive variables were nine child-related, environmental, and auditory factors. A series of multiple regression analyses determined the amount of variance in expressive and receptive language outcomes attributable to each predictor when controlling for the other variables. Simple linear regressions with age at first fitting and independent samples t tests demonstrated that children implanted before the age of two performed significantly better on all tests than children who were implanted at an older age. The mean LQ was 0.78 with an SD of 0.18. A child with an LQ lower than 0.60 (= 0.78-0.18) within 3 years after implantation was labeled as a weak performer compared with other deaf children implanted before the age of two. Contralateral stimulation with a second CI or a hearing aid and the absence of additional disabilities were related to better language outcomes. The effect of environmental factors, comprising multilingualism, parental involvement, and communication mode increased over time. Three years after implantation, the total multiple regression model accounted for 52% of the variance in receptive language scores and 58% of the variance in expressive language scores. On the basis of language test scores of this large group of children, an LQ of 0.60 or lower was considered a risk criterion for problematic language development compared with other deaf children using CIs. Children attaining LQs below 0.60 should be monitored more closely and perhaps their rehabilitation programs should be reconsidered. Improved language outcomes were related to implantation under the age of two, contralateral stimulation, monolingualism, sufficient involvement of the parents, and oral communication by the parents. The presence of an additional learning disability had a negative influence on language development. Understanding these causes of variation can help clinicians and parents to create the best possible circumstances for children with CIs to acquire language.

Hypothesis: An objective cochlear framework, for evaluation of the cochlear anatomy and description of the position of an implanted cochlear implant electrode, would allow the direct comparison of measures performed within the various subdisciplines involved in cochlear implant research. Background: Research on the human cochlear anatomy in relation to tonotopy and cochlear implantation is conducted by specialists from numerous disciplines such as histologists, surgeons, physicists, engineers, audiologists, and radiologists. To allow accurate comparisons between and combinations of previous and forthcoming scientific and clinical studies, cochlear structures and electrode positions must be specified in a consistent manner. Methods: Researchers with backgrounds in the various fields of inner ear research as well as representatives of the different manufacturers of cochlear implants (Advanced Bionics Corp., Med-El, Cochlear Corp.) were involved in consensus meetings held in Dallas, March 2005, and Asilomar, August 2005. Existing coordinate systems were evaluated, and requisites for an objective cochlear framework were discussed. Results: The consensus panel agreed upon a 3-dimensional, cylindrical coordinate system of the cochlea using the "Cochlear View" as a basis and choosing a z axis through the modiolus. The zero reference angle was chosen at the center of the round window, which has a close relationship to the basal end of the Organ of Corti. Conclusion: Consensus was reached on an objective cochlear framework, allowing the outcomes of studies from different fields of research to be compared directly.

Research from JAMA — Newborn Hearing Screening vs Later Hearing Screening and Developmental Outcomes in Children With Permanent Childhood Hearing Impairment

To investigate the preservation of residual hearing in subjects who received the Nucleus Hybrid L24 cochlear implant. To investigate the performance benefits up to one year post-implantation in terms of speech recognition, sound quality, and quality of life. Prospective, with sequential enrolment and within-subject comparisons. Post-operative performance using a Freedom Hybrid sound processor was compared with that of pre-operative hearing aids. Sixty-six adult hearing-impaired subjects with bilateral severe-to-profound high frequency hearing loss. Group median increase in air-conduction thresholds in the implanted ear for test frequencies 125-1000 Hz was < 15 dB across the population; both immediately and one year post-operatively. Eighty-eight percent of subjects used the Hybrid processor at one year post-op. Sixty-five percent of subjects had significant gain in speech recognition in quiet, and 73% in noise (≥ 20 percentage points/2 dB SNR). Mean SSQ subscale scores were significantly improved (+ 1.2, + 1.3, + 1.8 points, p < 0.001), as was mean HUI3 score (+ 0.117, p < 0.01). Combining residual hearing with CI gave 22-26 %age points mean benefit in speech recognition scores over CI alone (p < 0.01). Useful residual hearing was conserved in 88% of subjects. Speech perception was significantly improved over preoperative hearing aids, as was sound quality and quality of life.

It is still largely unknown how receiving a cochlear implant affects the emotion understanding in deaf children. We examined indices for emotion understanding and their associations with communication skills in children aged 2.5–5 years, both hearing children (n = 52) and deaf children with a cochlear implant (n = 57). 2 aspects of emotion understanding were examined: (a) emotion recognition in facial expressions and (b) emotion attribution in a situational context. On all emotion-understanding tasks, children with a cochlear implant were less proficient than children with normal hearing. In children with normal hearing, performance and language skills were positively associated. In children with cochlear implants, language was positively associated only with tasks in which a verbal demand was made on children. These findings indicate that hearing loss in children, despite a cochlear implant, affects all aspects of emotion understanding measured in this study, including their nonverbal emotion-understanding skills.