Deaf children with cochlear implants before the age of 1 year: comparison of preverbal communication with normally hearing children

OBJECTIVES: To compare preverbal behaviors of deaf children implanted under 1 year of age with age-matched hearing children. METHODS: The study assessed 20 children; 10 deaf children implanted under 1 year of age and 10 normally hearing children of the same age. Preverbal skills were measured before, 6 months, and 1 year after implantation, using Tait Video Analysis that is able to predict later speech outcomes in young implanted children. RESULTS: Regarding vocal turns, the normally hearing group outperformed the implanted group although the latter children became quite vocal, nearly 60% of their turns being taken in this way. The mean vocal autonomy in implanted children, 1 year after implantation, was very close to the respective of hearing children (38.5 versus 43.5). Regarding the non-looking vocal turns, by the 12-month interval, hearing children had somewhat higher scores than implanted children, but the difference was not significant and the increase in implanted children was much higher (40-fold increase versus 4-fold increase). However, implanted children were more likely to use silent communication than hearing children, although gestural turns were decreasing with time. CONCLUSIONS: The small numbers in this study, although two of the largest European cochlear implant centers were combined to recruit such young implantees, led us to be cautious in interpreting the results. However, it seems that in deaf implanted children under 1 year of age, some preverbal communication behaviors are developing to an extent (although at a somewhat lower level) not significantly different from those of age-matched normally hearing children.     

Age at implantation and development of vocal and auditory preverbal skills in implanted deaf children

BACKGROUND: Preverbal vocal and auditory skills are essential precursors of spoken language development and they have been shown previously to predict later speech perception and production outcomes in young implanted deaf children. OBJECTIVES: To assess the effect of age at implantation on the development of vocal and auditory preverbal skills in implanted children. METHODS: The study assessed 99 children, 33 in each of three groups (those implanted between 1 and 2 years; 2 and 3 years; and 3 and 4 years). Preverbal skills were measured in three areas: turn taking, autonomy and auditory awareness of spoken language, using the Tait video analysis method. RESULTS: The youngest implanted group made an exceptional progress outperforming in all measures the two other groups (p<0.01), 6 and 12 months post-implantation, whereas there was no such difference before implantation. In the youngest group there was also significantly greater use of an auditory/oral style of communication: 85% of the group by 12 months post-implantation compared with 30% and 18% of the two older groups. CONCLUSIONS: Vocal and auditory preverbal skills develop much more rapidly in children implanted between 1 and 2 years in comparison with older implanted children and reach a significantly higher level by 6 and 12 months post-implantation. In addition, younger implanted children are significantly more likely by 12 months post-implantation to adopt an auditory/oral mode of communication. These findings favour cochlear implantation as early as between 1 and 2 years, provided that correct diagnosis and adequate hearing-aid trial have been achieved.     

Prelingual auditory-perceptual skills as indicators of initial oral language development in deaf children with cochlear implants

ObjectivesTo assess when prelingually deaf children with a cochlear implant (CI) achieve the First Milestone of Oral Language, to study the progression of their prelingual auditory skills in the first year after CI and to investigate a possible correlation between such skills and the timing of initial oral language development.MethodsThe sample included 44 prelingually deaf children (23 M and 21 F) from the same tertiary care institution, who received unilateral or bilateral cochlear implants. Achievement of the First Milestone of Oral Language (FMOL) was defined as speech comprehension of at least 50 words and speech production of a minimum of 10 words, as established by administration of a validated Italian test for the assessment of initial language competence in infants. Prelingual auditory-perceptual skills were assessed over time by means of a test battery consisting of: the Infant Toddler Meaningful Integration Scale (IT-MAIS); the Infant Listening Progress Profile (ILiP) and the Categories of Auditory Performance (CAP).ResultsOn average, the 44 children received their CI at 24 ± 9 months and experienced FMOL after 8 ± 4 months of continuous CI use. The IT-MAIS, ILiP and CAP scores increased significantly over time, the greatest improvement occurring between baseline and six months of CI use. On multivariate regression analysis, age at diagnosis and age at CI did not appear to bear correlation with FMOL timing; instead, the only variables contributing to its variance were IT-MAIS and ILiP scores after six months of CI use, accounting for 43% and 55%, respectively.ConclusionPrelingual auditory skills of implanted children assessed via a test battery six months after CI treatment, can act as indicators of the timing of initial oral language development. Accordingly, the period from CI switch-on to six months can be considered as a window of opportunity for appropriate intervention in children failing to show the expected progression of their auditory skills and who would have higher risk of delayed oral language development.     

Evolution of communication abilities after cochlear implantation in prelingually deaf children

Objective

This study tries to evaluate different factors on communication ability outcomes in cochlear implanted children.

Methods

Communication abilities are studied using the validated APCEI-scale based on five components of the language: cochlear implant acceptance, perceptive language performance, comprehension of the oral orders, expressive language and speech intelligibility.APCEI-scores were calculated every 6 months for the first 2 years, then yearly.The studied variables were: gender, social origin, preoperative residual hearing, age, aetiology of hearing loss, and associated disabilities.

Results

Communication ability scores increased with high socioeconomic level, presence of residual hearing, younger patients when no residual hearing, connexin mutation related deafness, and absence of associated disabilities. No significant difference has been noted between both sexes.

Conclusion

Many different factors influence the evolution of communication abilities of cochlear implanted children.Investigating the cause of hearing loss, presence of associated disabilities and residual hearing before surgery may help to predict outcome and plan appropriate care to those children with negative predictive factors.     

Age at implantation and auditory memory in cochlear implanted children

Abstract

Early cochlear implantation, before the age of 3 years, provides the best outcome regarding listening, speech, cognition an memory due to maximal central nervous system plasticity. Intensive postoperative training improves not only auditory performance and language, but affects auditory memory as well. The aim of this study was to discover if the age at implantation affects auditory memory function in cochlear implanted children. A total of 50 cochlear implanted children aged 4 to 8 years were enrolled in this study: early implanted (1-3y) n = 27 and late implanted (4-6y) n = 23. Two types of memory tests were used: Immediate Verbal Memory Test and Forward and Backward Digit Span Test. Early implanted children performed better on both verbal and numeric tasks of auditory memory. The difference was statistically significant, especially on the complex tasks. Early cochlear implantation, before the age of 3 years, significantly improve auditory memory and contribute to better cognitive and education outcomes.     

The use and reliability of Tait video analysis in assessing preverbal language skills in profoundly deaf and normally hearing children under 12 months of age

BACKGROUND: Assessment measures in evaluating preverbal skills and their progress in very young deaf children are lacking. However, their importance is highlighted by the recent trend of implanting children under 1 year of age. Tait video analysis is a technique for assessing preverbal communication behaviours in very young children with hearing impairment and has been found to be strongly related to speech discrimination and intelligibility outcomes post-implantation. AIM: To assess feasibility and inter-user reliability of Tait video analysis in assessing preverbal communication skills in children under 1 year of age. MATERIAL AND METHODS: Ten children (five profoundly deaf and five normally hearing) under 1 year of age were assessed by Tait video analysis. Three observers analysed the samples independently, according to the established protocol. RESULTS: There was complete agreement on 305 judgements and 8 discrepancies between observers over all the measures. Four of the discrepancies occurred in the samples of deaf children and four in the normally hearing samples. Statistical analysis revealed that the correlation coefficients between the different observers were extremely high ranging from 0.94 to 1 (perfect agreement). All of them were found to be statistically significant (p<0.01). CONCLUSION: The very high rate of inter-observer reliability suggests that the video recordings of children under 12 months can be scored consistently, and Tait video analysis is therefore a valid method of monitoring the development of vocal and auditory preverbal skills in very young deaf children, either following cochlear implantation or using acoustic hearing aids.     

Bilateral implantation in children with cochleovestibular nerve hypoplasia

To report on the outcomes of sequential bilateral cochlear implantation (CI) in children with inner ear malformation. The study design is a retrospective case study. The setting is a tertiary reference center. Two children presenting a profound bilateral congenital hearing loss with bilateral hypoplasia of the cochleovestibular nerves and hypoplasic external semicircular canal had a cochlear implant at respectively 16 months and 33 months. A second implant was proposed at respectively 17 and 20 months after the first implant. The main outcome measures are audiometry, perceptive results in closed and open set words (CSW and OSW) and oral production at follow-up. The first cochlear implant gave respectively mean thresholds at 60 dB and 70 dB. Bilateral CI showed mean threshold at respectively 40 dB and 55 dB. In case 1, perceptive assessment was 83% and 70% in respectively CSW and OSW with oral production and comprehension of sentences after 1 year follow-up. In case 2, the perceptive assessment showed no perceptive or linguistic evolution at 6 months follow-up. In cochleovestibular nerve hypoplasia, bilateral implantation could be discussed in cases of limited result after unilateral implant.     

Level of emotion comprehension in children with mid to long term cochlear implant use: How basic and more complex emotion recognition relates to language and age at implantation

ObjectivesThe current study was designed with three main aims: To document the level of emotional comprehension skills, from basic to more complex ones, reached by a wide sample of cochlear implant (CI) deaf children with at least 36 months of device use; To investigate subjective and audiological factors that can affect their emotional development; To identify, if present, a “critical age”, in which early intervention might positively affect adequate emotional competence development.DesignThis is an observational cohort study. Children with congenital severe/profound deafness were selected based on: aged by 4–11 years, minimum of 36 months of CI use, Italian as the primary language in the family; normal cognitive level and absence of associated disorders or socio-economic difficulties. Audiological characteristics and language development were assessed throughout standardized tests, to measure speech perception in quiet, lexical comprehension and production. The development of emotions' understanding was assessed using the Test of Emotion Comprehension (TEC) of Pons and Harris, a hierarchical developmental model, where emotion comprehension is organized in 3 Stages (external, mental and reflective). Statistical analysis was accomplished via the Spearman Rank Correlation Coefficient, to study the relationship between the personal and audiological characteristics; a multivariate linear regression analysis was carried out to find which variables were better associated with the standardized TEC values; a chi-squared test with Yate's continuity correction and Mann-Whitney U test were used to account for differences between continuous variables and proportions.Results72 children (40 females, 32 males) with a mean age of 8.1 years were included. At TEC score, 57 children showed normal range performances (79.17% of recipients) and 15 fell below average (20.83% of recipients). The 16.63% of older subjects (range of age 8–12 years) didn't master the Stage 3 (reflective), which is normally acquired by 8 years of age and failed 2 or all the 3 items of this component. Subjects implanted within 18 months of age had better emotion comprehension skills. TEC results were also positively correlated with an early diagnosis, a longer implant use, better auditory skills and higher scores on lexical and morphosintactic tests. On the contrary, it was negatively correlated with the presence of siblings and the order of birth. The gender, the side and the severity of deafness, type of implant and strategy were not correlated.ConclusionsEarly implanted children have more chance to develop adequate emotion comprehension, especially when the complex aspects are included, due to the very strong link between listening and language skills and emotional development. Furthermore, longer CI auditory experience along with early intervention allows an adequate communication development which positively influences the acquisition of such competencies.     

Cochlear implantation in children with cerebral palsy. A preliminary report

Objectives

The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy.

Methods

This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months.

Results

At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf person's speech, and one child had connected speech intelligible to all listeners.

Conclusions

Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.     

Neuroelectrical imaging study of music perception by children with unilateral and bilateral cochlear implants

Abstract

Objective

To investigate by means of non-invasive neuroelectrical imaging the differences in the perceived pleasantness of music between children with cochlear implants (CI) and normal-hearing (NH) children.

Methods

5 NH children and 5 children who received a sequential bilateral CI were assessed by means of High-Resolution EEG with Source Reconstruction as they watched a musical cartoon. Implanted children were tested before and after the second implant. For each subject the scalp Power Spectral Density was calculated in order to investigate the EEG alpha asymmetry.

Results

The scalp topographic distribution of the EEG power spectrum in the alpha band was different in children using one CI as compared to NH children (see figure). With two CIs the cortical activation pattern changed significantly, becoming more similar to the one observed in NH children.

Conclusions

The findings support the hypothesis that bilateral CI users have a closer-to-normal perception of the pleasantness of music than unilaterally implanted children.     

Balance in children following cochlear implantation

Objectives: To assess the vestibular function of children who had unilateral and bilateral cochlear implants compared with a control group of otherwise healthy children who had not been implanted.

Study design: Observational case–control study.

Methods: Posturography was carried out in the form of a Modified Clinical Test of Sensory Interaction on Balance using a Wii Balance Board and the Vestio App on an iPod Touch. Thirty children in total were tested, 10 children in each cohort. Results in the form of root mean square calculations were available for each child.

Results: Results showed a significant difference in the vestibular function of implanted children and the non-implanted control group (P?P?Conclusions: Our study demonstrates posturography as an inexpensive, easily operated tool that can be used to assess paediatric vestibular function. It showed a significant difference between the control group and the implanted groups. Further work prompted by this study will include interval post-operative testing to more accurately assess the effect that implantation has on vestibular function.     

Impact to the head increases cochlear implant reimplantation rate in children

OBJECTIVE: To describe the outcome of cochlear implantation in children and to discuss the cause and management of cochlear reimplantation. STUDY DESIGN: Retrospective chart review. METHODS: The medical records of 110 patients younger than 18 years of age, who underwent cochlear implantation at the Department of ORL, Head and Neck Surgery, of the University of Kiel, Germany, were reviewed for demographics, complications, and history of revision surgery. The patients had previously had implantation with either Nucleus (including the Contour) devices or MED-EL devices. RESULTS: Length of use before cochlear explanation ranged from 4 days to 3.9 years. Reimplantation was caused by traumatic device failure, wrong electrode insertion and infection of implanted area. Results indicated a reimplantation rate of 5.4% in children compared to 0.8% in adults, mostly resulting from the greater risk of children receiving an impact to the head. Postoperative performance data showed no decrease in scores taken before failure. CONCLUSIONS: Though young children who are developing their motor skills are probably at greater risk of a cochlear reimplantation resulting from device failure following head trauma, surgical revision with reimplantation can be performed safely and without decrement to performance.     

Bilateral cochlear implantation in children with Noonan syndrome

Noonan syndrome is a mostly autosomal dominant inherited disorder, which can be accompanied by hearing disorders or deafness, coagulation disorders, combined heart defects and developmental disorders. We are reporting on two children with an established Noonan syndrome with a severe bilateral hearing loss of respectively 95 and 100 dB and proper findings in the CT/MRI of the petrous bone. After complete otologic and radiologic diagnostics, both children underwent bilateral cochlear implantation successfully. According to the authors’ knowledge, this is the first time that cochlear implant therapy is discussed in patients with Noonan syndrome.     

Predictors of pediatric cochlear implantation outcomes in South Africa

ObjectiveTo identify and describe predictors of pediatric cochlear implantation outcomes in a South African population.MethodsA retrospective study of 301 pediatric cochlear implant (CI) recipients from five CI programs was conducted and cross-sectional outcome data were added at the time of data collection. Twenty potential prognostic factors were identified from the retrospective dataset, including demographical, CI, risk and family factors. Multiple regression analyses were performed to identify predictor variables that influence outcomes in terms of auditory performance (CAP scores), speech production (SIR scores), communication mode and educational placement.ResultsAlthough implanted children within this sample did not have equal opportunity to access a second implant, bilateral implantation was strongly predictive of better auditory performance and speech production scores, an oral mode of communication and mainstream education. NICU admittance/prematurity were associated with poorer auditory performance and speech production scores, together with a higher probability for non-oral communication and non-mainstream education. The presence of one or more additional developmental condition was predictive of poorer outcomes in terms of speech production and educational placement, while a delay between diagnosis and implantation of more than one year was also related to non-mainstream education. Ethnicities other than Caucasian were predictive of poorer auditory performance scores and a lower probability for mainstream education.ConclusionAn extensive range of prognostic indicators were identified for pediatric CI outcomes in South Africa. These predictive factors of better and poorer outcomes should guide pediatric CI services to promote optimal outcomes and assist professionals in providing evidence-based informational counseling.     

Atypical failure after cochlear implantation in children

We report a case of intermittent dysfunction in a 10-years-old boy, implanted with MedEL^® cochlear implant. Few weeks after the surgery the boy described short and intermittent episodes of implant dysfunction with rapid return to a normal function. No evidence for any electric or neural dysfunction was found. After few weeks, a clinical link was discovered to episodes of sneeze or nose blowing. Clinical and surgical implications are discussed.     

Bilateral cochlear implantation in Friedreich's ataxia: A case study

Abstract

With an incidence of 1:29 000 among Caucasians, Friedreich's ataxia (FRDA) is the most common inherited ataxia, leading to both sensory and motor degeneration. Despite many FRDA patients exhibiting normal or near normal sound detection thresholds, many individuals show abnormal neural conduction along their central auditory pathways. Electrophysiological testing can show abnormal or absent cochlear nerve and auditory brainstem recordings in the presence of normal pre-neural cochlear function (otoacoustic emissions or cochlear microphonics). This pattern of normal pre-neural cochlear function and disrupted neural conduction has been termed auditory neuropathy spectrum disorder (ANSD). Studies of FRDA patients with ANSD have shown that they exhibit severe deficits in temporal processing, impaired frequency discrimination, and deficits in speech perception. Rehabilitation of these auditory percept deficits remains difficult, as hearing aids may amplify sounds without adding clarity to the temporally disrupted or distorted signal that FRDA patients with ANSD may receive. There is limited data on the best intervention for patients with FRDA with ANSD, although personal radio aids (FM systems) have been shown to be beneficial. We report a case, where cochlear implantation has led to a dramatic improvement in speech perception in an individual with FRDA and ANSD. The majority of the literature on ANSD treatment has focused on paediatric patients with the ‘dyssynchrony’ type of ANSD, rather than the true neuropathy type underlying the hearing loss in FRDA patients.     

Comparison of outcomes in children with hearing aids and cochlear implants

Abstract

Objectives

The purpose of this study was to document the performance of a group of children with moderately severe to severe hearing loss who use hearing aids on a range of speech recognition, speech–language, and literacy measures and to compare these results to children with severe to profound hearing loss, who have learned language through cochlear implants.

Methods

This study involved 41 children with bilateral sensorineural hearing impairment, aged 6–18 years. Twenty children had moderately severe/severe hearing loss and used hearing aids, and 21 had severe to profound hearing loss and used cochlear implants. Communication and academic skills were assessed using speech recognition tests and standardized measures of speech production, language, phonology, and literacy.

Results

The two groups did not differ in their open-set speech recognition abilities or speech production skills. However, children with hearing aids obtained higher scores than their peers with cochlear implants in the domains of receptive vocabulary, language, phonological memory, and reading comprehension. The findings also indicate that children with moderately severe or severe hearing loss can develop spoken language skills that are within the range expected for normal hearing children.

Conclusions

School-aged children with moderately severe and severe hearing loss performed better in several domains than their peers with profound hearing loss who received cochlear implants between age 2 and 5 years. Further research is required to evaluate the benefits of hearing aids and cochlear implants in children with hearing loss who are diagnosed and receive intervention within the first year of life.     

Compliance with cochlear implantation in children subsequently diagnosed with autism spectrum disorder

Objective: To assess the compliance with cochlear implantation (CI) in children subsequently diagnosed with autism spectrum disorder (ASD).

Methods: This was a retrospective case review and survey performed at a tertiary referral centre. Children meeting the criteria for CI who were implanted between 1989 and 2015 and who subsequently received a diagnosis of ASD were included. The primary outcome measure was to assess compliance with CI in children subsequently diagnosed with ASD. Secondary outcome measures included assessment of pre-CI risk factors that may have identified children at higher risk of a subsequent diagnosis of ASD, as well as the benefit obtained by these children following CI.

Results: 1050 children were implanted between 1989 and 2015. Of these, 22 children were diagnosed with ASD after receiving their CI. The average age at implantation was 2.6 years (median 3, range 1–8 years). The average age for diagnosis of ASD was 5 years, approximately 2 years (median 22 months, range 2–85 months) following CI. Of these, 16/22 (712.7%) regularly use their CI. 6/22 (27.2%) children became non-users of their implant. Some degree of verbal communication was used by 13/22 (59%) of our studied group.

Conclusion: There is a range of level of disabilities in ASD, with some relatively minor social communication difficulties through to severe language, cognitive, and behavioural difficulties. Compliance with CI is variable and appears to correlate with the severity of the ASD. Preoperative counselling should include information about the possible impact of later diagnosed disabilities such as ASD on performance.     

Outcome for bilateral cochlear implantation in patients with congenital Cytomegalovirus infection

ObjectivesTo analyze the impact of bilateral cochlear implantation (CI) on perceptual and linguistic development in hearing-impaired children with congenital Cytomegalovirus (CMV) infection.Patients and methodA retrospective study was performed for the period 1991-2016 in a pediatric CI reference center. Closed Set Word (CSW) recognition scores, Categories of Auditory Performance (CAP) and linguistic level on the MT Lenormand scale (MTL) were compared between bilateral (Bi) and unilateral (Uni) groups 12, 24 and 36 months after first CI (CI-1).Results84 patients with congenital CMV infection who underwent CI were included, in 2 groups: sequential or simultaneous bilateral CI (Bi) (N = 20), and unilateral CI (Uni) (N = 64). Twelve, 24 and 36 months after CI-1, CSW scores were 35.56%, 64.52% and 82.93% in Uni and 60.3%, 85% (P = 0.0084*), and 100% (P = 0.00085*) in Bi. CAP scores 12, 24 and 36 months after CI-1 were 2.57, 3.85 and 4.3 in Uni and 3.91 (P = 0.0068*), 5.00 (p = 0.029*) and 5.50 (P = 0.051*) in Bi. MTL linguistic level scores at 12, 24 and 36 months were 0.72, 1.25 and 1.65 in Uni, and 1.72, 3 (P = 0.033) and 3.11 (P = 0.045) in Bi. These significantly better scores in Bi at 24 and 36 months after CI-1 were also found on analysis of subgroups with no associated neurologic disorder (P = 0.046* and P = 0.032*), no associated psychiatric pathology (P = 0.0055* and P = 0.0073*), and no other associated disorder (P = 0.0018* and P = 0.035*), and for all subgroups together (P = 0.0036 and P = 0.037).ConclusionBilateral CI is a faster way than unilateral CI for patients with congenital CMV infection to achieve structured fluent oral language. 50% of the series showed cerebral abnormalities on MRI, without difference between groups. This was not in itself predictive of poor progression of oral communication, unless associated with major neurologic disorder. Some children made little or no use of their CI in the medium term.     

Practitioners' perspectives on the functioning of school-age children with cochlear implants

Abstract

Objective

The availability of cochlear implants has increased the number of children with profound deafness educated in classrooms alongside peers with normal hearing. The purpose of this research was to better understand the functioning of these children from the perspective of their service providers.

Methods

Semi-structured focus group interviews were conducted with 28 practitioners to elicit their perceptions of children's abilities in oral communication, academic, and social functioning. Data were coded inductively and examined through content analysis.

Results

The central theme was that cochlear implantation has improved school functioning for children both in hearing and related spoken language abilities and beyond hearing in academic and social development. While these benefits were a consistent theme, a wide range of performance was identified across all areas of functioning. In particular, areas of concern included full participation in classroom activities and social interaction with peers.

Conclusions

The findings provide insights into functioning for children with cochlear implants from the perspective of those who interact with them in everyday settings. These findings underscore the challenges for children in achieving full participation despite improvements in communication skills. Practitioners identified areas where intervention is required to facilitate the inclusion of children in school programs.