Quality Standard for Rehabilitation of Young Deaf Children Receiving Cochlear Implants.

Background and objectives: More and more children with severe-to-profound hearing loss are receiving cochlear implants (CIs) at an early age to improve their hearing and listening abilities, speech recognition, speech intelligibility, and other aspects of spoken language development. Despite this, the rehabilitation outcomes can be very heterogeneous in this population, not only because of issues related to surgery and fitting or the specific characteristics of the child with his/her additional disabilities but also because of huge differences in the quality of the support and rehabilitation offered by the therapist and the family. These quality standards for the rehabilitation of young deaf children receiving CIs are developed within the European KA202 Erasmus+ project "VOICE"-vocational education and training for speech and language therapists and parents for the rehabilitation of children with CIs, Ref. No.: 2020-1-RO01-KA202-080059. Material and methods: To develop these quality standards, we used the input from the face-to-face interviews of 11 local rehabilitation experts in CIs from the four partner countries of the project and the outcomes of the bibliographic analysis of 848 publications retrieved from six databases: Pub Med, Psych Info, CINAHL, Scopus, Eric, and Cochrane. Based on all this information, we created a first set of 32 quality standards over four domains: general, fitting, rehabilitation, and for professionals. Further on, the Delphi method was used by 18 international rehabilitation experts to discuss and agree on these standards. Results: The results from the literature analysis and the interviews show us that more than 90% of the consulted international experts agreed on 29 quality standards. They focus on different aspects of rehabilitation: the multidisciplinary team, their expertise and knowledge, important rehabilitation topics to focus on, and programming issues related to rehabilitation. Conclusions: These quality standards aim to optimize the activity of speech rehabilitation specialists so that they reach the optimal level of expertise. Also presented is the necessary equipment for the IC team to carry out the rehabilitation sessions in good conditions. This set of quality standards can be useful to ensure the appropriate postoperative care of these children. As a result, the rehabilitation process will be more relaxed, and therapists will have the opportunity to focus more on the specific needs of each child, with the provision of quality services, which will result in better results. This theme is particularly complex and dependent on multifactorial aspects of medicine, education, speech therapy, social work, and psychology that are very intricate and interdependent.

Robotic cochlear implantation in post-meningitis ossified cochlea.

AIM: To report the experience of an image-guided and navigation-based robot arm as an assistive surgical tool for cochlear implantation in a case with a labyrinthitis ossificans. PATIENT: A 55-years-old man with a history of childhood meningitis whose hearing deteriorated progressively to bilateral profound sensorineural hearing loss. INTERVENTION: Robotic Assisted Cochlear Implant Surgery (RACIS) with a straight flexible lateral wall electrode. PRIMARY OUTCOME MEASURES: Electrode cochlear insertion depth with RACIS with facial recess approach and autonomous inner ear access with full electrode insertion of a flexible straight cochlear implant array. CONCLUSIONS: Intra cochlear ossifications pose a challenge for entering the cochlea and full-length insertion of a cochlear implant. RACIS has shown that computations of radiological images combined with navigation-assisted robot arm drilling can provide efficient access to the inner ear.

Strategies for the implementation of the living guidelines for cochlear implantation in adults.

Introduction: Clinical guidelines for cochlear implants (CI) exist in several countries, however, they lack consistency and often do not encompass the full user journey. This study aims to explore the barriers and facilitators for implementing global Living Guidelines for cochlear implantation in adults with severe, profound or moderate sloping to profound sensorineural hearing loss (SPSNHL) as well as identify guideline implementation (GI) tools that may support uptake. Methods: A convenience sample of Task Force members were recruited for semi-structured interviews. Interview transcripts were thematically analysed to group country-specific barriers, facilitators and GI tools into three levels: health care provider (HCP), consumer and structural. Once identified, barriers and facilitators were classified into four themes related to awareness, economic, guideline or other. Results: Interviews were conducted with 38 Task Force members, representing 20 countries. Lack of CI and hearing loss awareness was a major barrier at the HCP (85% of countries), consumer (80%) and structural (20%) levels. Economic and guideline barriers followed at the HCP (35%; 25%), consumer (45%; 0%) and structural (55%; 30%) levels, respectively. Facilitators focused on raising awareness of hearing loss and CIs as well as guideline related initiates at the HCP (80%; 70%), consumer (70%; 10%) and structural (25%; 70%) levels. GI tools including education, economic evaluations, quick reference resources and social media can help improve awareness and uptake. Conclusion: Awareness is the primary barrier to implementing Living Guidelines globally for adults with SPSNHL. Endorsement from key professional bodies and using the best available evidence can enhance uptake.

Translation and validation of the revised version of the LittlEARS® early speech production questionnaire (LEESPQ®), in Dutch-speaking children with normal hearing.

INTRODUCTION: With the introduction of newborn hearing screening, children with different degrees of hearing loss can receive hearing aids and cochlear implants in the early months after birth, which is also the case in the Netherlands and in Flanders, the Dutch speaking part of Belgium. MATERIALS AND METHODS: This has intensified the need for a validated questionnaire in the Dutch language to assess the speech development of children under the age of two. The LittlEARS Early Speech Production Questionnaire® (LEESPQ®), which was originally developed and validated in German language, was translated and validated into the Dutch language. Questionnaires of 355 normal hearing children were analysed. RESULTS: Total score was highly correlated with age (r = .775) and a normative curve was created. Internal consistency was reached with a high value of α = 0.870, which indicates that the questionnaire almost exclusively assesses speech production ability. The Dutch version of the LEESPQ®, is reliable, consistent and independent of gender or lingual status. As such, the Dutch LEESPQ®, may be a useful tool for language monitoring for children from birth to 18 months of age. CONCLUSION: The Dutch LittlEARS Early Speech Production Questionnaire®, was found to be a reliable and valid tool to assess and monitor the early speech and language production skills in children up to 18 months of age.

Prevalence of cochlear implants in Europe: trend between 2010 and 2016.

Objective: To collect figures on the numbers of children and adults receiving cochlear implants across Europe, compare the figures for 2016 with those for 2010, and identify any trends. Materials and methods: In 2018 EURO-CIU invited their 23 member countries to conduct a survey collecting data on the number of CI recipients in 2016 and 2017. Data were received from 15 countries, representing more than 100 000 CI recipients in Europe. Results: For paediatric CI, there was an increase in nearly all European countries (except Denmark, the UK and Luxembourg) between 2010 and 2016. We found an annual figure of one CI per 1000 newborns common in most countries where reimbursement of paediatric CI's is available. Conversely the adult data reveals no increase between 2010 and 2016 and the data is less homogeneous than the paediatric data with huge differences across countries. Conclusion: There is little agreement on data on numbers of CI across Europe, which makes it difficult to plan public health policy, funding or services. In all European countries included in this study (except Germany) there needs to be work on raising more awareness of adult hearing loss and adult cochlear implantation to improve access.

Translation and validation of the Listen Inventory for Education Revised into Dutch.

INTRODUCTION: In Belgium the majority of children with CI's are being educated in mainstream schools. In mainstream schools difficult listening situations occur (e.g. due to background noise) which may result in educational risks for children with CI's. A tool that identifies potential listening difficulties, the English Listen inventory for Education Revised (LIFE-R), was translated and validated into Dutch for elementary and secondary schools (LIFE-NL, LIFE2-NL respectively). METHODS: Two forward-backward translations were performed followed by a linguistic evaluation and validation by a multidisciplinary committee. The LIFE-NL was further validated on content by pre-testing the questionnaire in 5 students with hearing loss (8-13 years). After minor cross-cultural adaptations normative data were assembled from 187 normal-hearing (NH) students enrolled in mainstream secondary education (1st to 4th grade). The normative data were further analysed based on grade and school type. Additionally, the internal consistency was evaluated by calculating Cronbach's alpha for 3 different scales of the LIFE2-NL: the LIFE total (situation 1-15), LIFE class (situation 1-10: listening situations in classroom) and LIFE social (situation 11-15: social listening situations in school). RESULTS: NH students scored on average 72.0 (SD = 19.9%) on the LIFE2-NL, indicating they experience some difficulties in secondary mainstream schools. The most difficult listening situations were those where fellow students are noisy or when students have to listen in large classrooms. NH students scored significantly higher on the LIFE class compared to the LIFE social (84.1 ±â€¯14.7% vs. 68.1 ±â€¯19.0%, p < .000). Moreover the LIFE social tend to decrease from the 3rd grade on. The different subscales of the LIFE2-NL showed high internal consistency (Cronbach's alpha of 0.86, 0.89 and 0.75 for LIFE total, LIFE class and LIFE social respectively). CONCLUSION: The LIFE-NL and LIFE2-NL are valid Dutch translations of the original LIFE-R and are fully comprehensible for students with hearing loss. The normative data of the LIFE2-NL provide a representative framework for interpreting the results of mainstreamed students with hearing loss in secondary schools.

Verbal cognition in deaf children using cochlear implants: effect of unilateral and bilateral stimulation.

The aim of this study is to assess the role of bilateral/bimodal device use in auditory speech perception in complex listening situations and long-term verbal cognition in deaf children using cochlear implants (CIs). Two groups of children are compared (unilateral and bilateral device users) concerning vocabulary, speech perception at conversational level and in complex listening situations, and verbal cognition. In this retrospective study, we collected data of 37 deaf children with normal learning potential of whom 16 were unilateral CI users and 21 were bilateral device users (9 with a bimodal fitting and 12 with bilateral CIs). We came to the conclusion that deaf children who use bilateral devices have the opportunity to develop good speech perception skills in complex listening conditions. These abilities enable at least some of the children to develop age-equivalent verbal cognition skills.

Behavioral problems in school-aged hearing-impaired children: the influence of sociodemographic, linguistic, and medical factors.

The purpose of this study was to examine several behavioral problems in school-aged hearing-impaired children with hearing aids or cochlear implants, compared to normally hearing children. Additionally, we wanted to investigate which sociodemographic, linguistic, and medical factors contributed to the level of behavioral problems, to pinpoint where targeted interventions can take place. This large, retrospective study included a sample of 261 school-aged children (mean age = 11.8 years, SD = 1.6), that consisted of three age- and gender-matched subgroups: 75 with hearing aids, 57 with cochlear implants, and 129 normally hearing controls. Self- and parent-reports concerning reactive and proactive aggression, delinquency, and symptoms of psychopathy, attention deficit hyperactivity disorder, oppositional defiant disorder, and conduct disorder were used. In addition, several language and intelligence tests were administered. Hearing-impaired children showed significantly more proactive aggression, symptoms of psychopathy, attention deficit hyperactivity disorder, oppositional defiant disorder, and conduct disorder than their normally hearing peers. More behavioral problems were associated with special schools for the deaf, sign (-supported) language, hearing aids (in contrast to cochlear implants), higher age, male gender, lower socioeconomic status, lower intelligence, and delayed language development. Hearing-impaired children face multiple problems regarding their behavior. The outcomes implicate that professionals should be aware of the higher risk of developing behavioral problems, in order to screen, detect, and treat in time. Furthermore, the associated risk and protective factors emphasize that clinicians must always consider the heterogeneity of the group of hearing-impaired children, in order to help and support the individual patient.

Narrative spoken language skills in severely hearing impaired school-aged children with cochlear implants.

Cochlear implants have a significant positive effect on spoken language development in severely hearing impaired children. Previous work in this population has focused mostly on the emergence of early-developing language skills, such as vocabulary. The current study aims at comparing narratives, which are more complex and later-developing spoken language skills, of a contemporary group of profoundly deaf school-aged children using cochlear implants (n=66, median age=8 years 3 months) with matched normal hearing peers. Results show that children with cochlear implants demonstrate good results on quantity and coherence of the utterances, but problematic outcomes on quality, content and efficiency of retold stories. However, for a subgroup (n=20, median age=8 years 1 month) of deaf children without additional disabilities who receive cochlear implantation before the age of 2 years, use two implants, and are raised with one spoken language, age-adequate spoken narrative skills at school-age are feasible. This is the first study to set the goals regarding spoken narrative skills for deaf children using cochlear implants.

Expressive vocabulary, morphology, syntax and narrative skills in profoundly deaf children after early cochlear implantation.

Practical experience and research reveal generic spoken language benefits after cochlear implantation. However, systematic research on specific language domains and error analyses are required to probe sub-skills. Moreover, the effect of predictive factors on distinct language domains is unknown. In this study, outcomes of 70 school-aged children with cochlear implants were compared with hearing peers. Approximately half of the children with cochlear implants achieved age-adequate language levels. Results did not reveal systematic strong or weak language domains. Error analyses showed difficulties with morphological and syntactic rules and inefficient narrative skills. Children without additional disabilities who received early intervention were raised with one spoken language, and used a second cochlear implant or contralateral hearing aid were more likely to present good language skills.

Accessibility to cochlear implants in Belgium: state of the art on selection, reimbursement, habilitation, and outcomes in children and adults.

Belgium, and especially the northern region called Flanders, has been a center of expertise in cochlear implants and early hearing screening for many years. Some of their surgeons and engineers were pioneers in the development of cochlear implants and in 1998 Flanders was the first region in Europe to implement a universal hearing screening program for all neonates. The Belgian National Institute for Health and Disability Insurance has reimbursed cochlear implants in children and adults since 1994 and bilateral implantation in children under the age of 12 years since February 2010. These deaf children, screened and implanted early, achieve higher auditory, speech and language outcomes and increasing numbers are going to regular schools using fewer interpreters. In 2010, 93% of severe-to-profound deaf preschool children in Flanders had received cochlear implants and 25% had bilateral implants. Although on average twice as many adults as children are implanted a year in Belgium, we have less research data available from this adult population. Also very little is published about the growth curves and minimal rehabilitation requirements (intensity, duration etc.) after implantation for both children and adults. So, there still remain many challenges for the future.

Emotion understanding in deaf children with a cochlear implant.

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.

The listening cube: a three dimensional auditory training program.

OBJECTIVES: Here we present the Listening Cube, an auditory training program for children and adults receiving cochlear implants, developed during the clinical practice at the KIDS Royal Institute for the Deaf in Belgium. We provide information on the content of the program as well as guidance as to how to use it. METHODS: The Listening Cube is a three-dimensional auditory training model that takes the following into consideration: the sequence of auditory listening skills to be trained, the variety of materials to be used, and the range of listening environments to be considered. During auditory therapy, it is important to develop training protocols and materials to provide rapid improvement over a relatively short time period. Moreover, effectiveness and the general real-life applicability of these protocols to various users should be determined. RESULTS: Because this publication is not a research article, but comes out of good daily practice, we cannot state the main results of this study. We can only say that this auditory training model is very successful. Since the first report was published in the Dutch language in 2003, more than 200 therapists in Belgium and the Netherlands followed a training course elected to implement the Listening Cube in their daily practice with children and adults with a hearing loss, especially in those wearing cochlear implants. CONCLUSION: The Listening Cube is a tool to aid in planning therapeutic sessions created to meet individual needs, which is often challenging. The three dimensions of the cube are levels of perception, practice material, and practice conditions. These dimensions can serve as a visual reminder of the task analysis and of other considerations that play a role in structuring therapy sessions.

Anxiety in children with hearing aids or cochlear implants compared to normally hearing controls.

OBJECTIVES/HYPOTHESIS: The objectives of this study were to examine the levels of anxiety in hearing-impaired children with hearing aids or cochlear implants compared to normally hearing children, and to identify individual variables that were associated with differences in the level of anxiety. STUDY DESIGN: Large retrospective cohort study. METHODS: Self-reports and parent-reports concerning general anxiety, social anxiety, and generalized anxiety disorder were used. The study group (mean age, 11.8 years) consisted of three age-matched subgroups: 32 children with cochlear implants, 51 children with conventional hearing aids, and 127 children without hearing loss. RESULTS: Levels of anxiety in children with cochlear implants and normally hearing children were similar. Early implantation was associated with lower levels of general and social anxiety. Remarkably, children with conventional hearing aids had higher levels of social anxiety, and their parents also reported more generalized anxiety disorder. CONCLUSIONS: The outcomes demonstrate that in their level of anxiety, children with cochlear implants might be more comparable to normally hearing children than to children with hearing aids. This positive finding can be the consequence of audiological factors or other aspects of the cochlear implant rehabilitation program.

The Listening Cube: A Three Dimensional Auditory Training Program

OBJECTIVES: Here we present the Listening Cube, an auditory training program for children and adults receiving cochlear implants, developed during the clinical practice at the KIDS Royal Institute for the Deaf in Belgium. We provide information on the content of the program as well as guidance as to how to use it. METHODS: The Listening Cube is a three-dimensional auditory training model that takes the following into consideration: the sequence of auditory listening skills to be trained, the variety of materials to be used, and the range of listening environments to be considered. During auditory therapy, it is important to develop training protocols and materials to provide rapid improvement over a relatively short time period. Moreover, effectiveness and the general real-life applicability of these protocols to various users should be determined. RESULTS: Because this publication is not a research article, but comes out of good daily practice, we cannot state the main results of this study. We can only say that this auditory training model is very successful. Since the first report was published in the Dutch language in 2003, more than 200 therapists in Belgium and the Netherlands followed a training course elected to implement the Listening Cube in their daily practice with children and adults with a hearing loss, especially in those wearing cochlear implants. CONCLUSION: The Listening Cube is a tool to aid in planning therapeutic sessions created to meet individual needs, which is often challenging. The three dimensions of the cube are levels of perception, practice material, and practice conditions. These dimensions can serve as a visual reminder of the task analysis and of other considerations that play a role in structuring therapy sessions.

A longitudinal study on auditory perception and speech intelligibility in deaf children implanted younger than 18 months in comparison to those implanted at later ages.

OBJECTIVE: The objective of this study was to measure longitudinally the progress in the auditory performance and speech intelligibility of young deaf children implanted under the age of 18 months in comparison to those implanted after 18 months and to create normative data on these children implanted under 18 months. STUDY DESIGN: : This is a retrospective longitudinal study based on data available at our centre 'KIDS-Hasselt-Belgium'. PATIENTS: 52 deaf children who received a cochlear implant under the age of 18 months. METHODS: LiP (Listening Progress profile) and CAP (Categories of Auditory Performance) were administered to measure auditory perception and SIR (Speech Intelligibility Rating) was used to measure speech intelligibility. Over a 4 year period, six assessments were made to monitor the auditory and speech development of the children: a pre-test before implantation and five post-tests at 6, 12, 24, 36 and 48 months after implantation. RESULTS: Deaf children receiving cochlear implants before the age of 18 months show more rapid progress in auditory performance and speech intelligibility in comparison to children implanted at an older age. Their auditory performance and speech intelligibility also reach a higher level 4 years after implantation compared with children implanted later in life. DISCUSSION AND CONCLUSION: This study shows encouraging results in the cochlear implantation of children under the age of 18 months. As demonstrated, using the LiP, CAP and SIR measures, children who receive a cochlear implant before the age of 18 months show, that at 3 to 4 years after implantation, significantly greater improvement is made in auditory perception and speech intelligibility than their later implanted peers, although there is a considerable variety in the outcomes.

Education and rehabilitation of deaf children with cochlear implants: a multidisciplinary task.

In the last decade the population of deaf children has changed dramatically in a lot of countries, especially in those where universal neonatal hearing screening, early multi-disciplinary support, digital hearing aids and cochlear implants are available. Most of these children can acquire intelligible spoken language and choose spoken language as their main means of communication and for access to education, because they go to mainstream schools in larger proportions, and fewer to schools for the deaf. Mainstream placement does not eliminate the need for services, which will vary depending upon the child's age, language modality, and other child specific factors. But there is also evidence that the outcomes after paediatric implantation are very heterogeneous. All this is a big change for the educational services. We must ensure that their staff have the skills to meet these challenges: to be flexible, continually updated with the technology and changing expectations (ongoing professional training), to provide an environment which will utilise the hearing while meeting the linguistic and curricular needs of the children, to meet the psycho-social needs of this group as they grow through adolescence, and to work with other professionals. Now, and looking ahead, the challenge for deaf education is also to embrace the diversity of this population and to appropriately address the specific needs of each child.

Three-year postimplantation auditory outcomes in children with sequential bilateral cochlear implantation.

OBJECTIVES: We report on the auditory abilities and speech performance in quiet and noise of 35 children with sequential bilateral cochlear implantation after 3 years of bilateral implant use. METHODS: Testing was done in bilateral and both unilateral listening conditions. The assessments took place before the second implantation and at several time intervals after fitting. As different auditory tests were used, the children were categorized by their age at the second implantation: younger or older than 6 years. RESULTS: The pure tone averages for the bilateral condition were significantly better than those for either unilateral condition after 12 months of bilateral implant use and remained so from that test interval onward. The speech recognition outcomes in quiet and noise also improved significantly for almost all children after 36 months, although a linear regression analysis showed a beneficial effect of younger age at first implantation on the speech-in-noise results. CONCLUSIONS: Bilateral cochlear implantation offered advantages to all children in comparison with the first implant--even the children who received the second implant after the age of 6 years. Compared to the younger children, the older children needed a longer adjustment period to gain bilateral benefit. However, they obtained similar results after 2 years of bilateral implant use.