Children then, adults now: long-term outcomes-performance at 15, 20, and 25 years of cochlear implant use.

Motivation: Severe to profound sensorineural hearing loss interferes with a child's development at the cognitive, linguistic, academic, and social levels. Since the beginning of the pediatric auditory rehabilitation program through cochlear implantation in the Ear, Nose, and Throat (ENT) Service of the Coimbra Hospital and University Center (CHUC), Portugal, its mentors defended the early diagnosis of hearing loss followed by timely intervention, and this was considered the starting point to optimize (re)habilitation through this method. Three decades or so later, recently we conducted this study to evaluate the performance of patients implanted in the initial phase of the cochlear implantation program. Objectives: The study aimed to evaluate the performance of individuals with severe to profound congenital hearing loss who underwent pediatric cochlear implantation and have used the cochlear implant for at least 25 years, to analyze the beneficial effect of early intervention in improving performance results. Methods: The study sample is composed of 31 individuals with severe to profound congenital hearing loss and no other comorbidities, divided into two groups (Group 1: age at implantation was under 3 years; Group 2: age at implantation was over 3 years). All 31 subjects were evaluated at 15, 20, and 25 years of cochlear implant (CI) use with a comprehensive set of tests. In addition, data were collected regarding the academic level of each participant. The results of both groups were compared to find out if there is an effect of age at implantation on auditory performance, and if there is an improvement in the performance with CI over time (15, 20, and 25 years of use). Results: The results show that there is a positive effect, with statistical significance, of early implantation on auditory performance, and telephone use. In both groups, there is an increase in performance over time, but it tends to stabilize after 20 years of CI use. Discussion and conclusion: The results obtained in this work support the importance of early intervention in patients with severe to profound hearing loss who are cochlear implant users and show that CI is an effective and reliable method in the treatment of these patients, contributing to their improved socio-educational integration, and that the benefits last over time.

Desenvolvimento e validação de conteúdo de um Programa de Intervenção em Processamento Auditivo para crianças em idade escolar / Auditory Processing Intervention Program for school-aged children - development and content validation

RESUMO Objetivo O estudo teve como objetivos o desenvolvimento e validação de conteúdo de um Programa de Intervenção em Processamento Auditivo, destinado a crianças em idade escolar, com perturbação do processamento auditivo, falantes do português europeu. Método A primeira etapa consistiu no desenvolvimento do programa e respetivo manual de instruções, que inclui objetivos, atividades, procedimentos, materiais, reforços, instruções e estímulos verbais utilizados, para as competências auditivas de discriminação auditiva, atenção auditiva, memória auditiva, fechamento, figura-fundo; separação binaural, integração binaural e fusão binaural; a segunda etapa consistiu na validação de conteúdo, por dois painéis de peritos que analisaram o programa, através de um questionário. A validade de conteúdo foi calculada usando o índice de validade de conteúdo. Resultados A avaliação do programa revela uma excelente validade de conteúdo. Alguns itens foram modificados depois da análise dos comentários e sugestões dos peritos (ex: instruções, desníveis de intensidade, personagem principal). Conclusão O estudo permitiu o desenvolvimento e validação de um programa de intervenção em processamento auditivo, com estímulos verbais, selecionados de acordo com critérios linguísticos rigorosos. Futuramente, está prevista a realização de estudos de aceitabilidade e eficácia do programa junto da população alvo.

ABSTRACT Purpose The study aimed at the development and content validation of an Auditory Processing Intervention Program for school-aged European Portuguese speaking children with Auditory Processing Disorder. Methods The first step was the program's development and its instructions manual, which includes objectives, activities, procedures, materials, reinforcement, instructions, and verbal stimuli used, for the following auditory skills: auditory discrimination, auditory attention; auditory memory; auditory closure; figure-ground; auditory separation; auditory integration; binaural fusion; content validation was performed next, with two expert panels analyzing the program, through the use of a questionnaire. Content validity was calculated using the content validity index. Results Program evaluation shows an excellent content validity. Some items were modified after analyzing the experts' comments and suggestions (e.g. instructions, intensity differences, main character). Conclusion This work allowed the development and content validation of an auditory processing intervention program, with verbal stimuli, selected according to strict linguistic criteria. In the future, the acceptability and efficacy of this program with the target population should be analyzed.

Auditory Processing Intervention Program for school-aged children - development and content validation. / Desenvolvimento e validação de conteúdo de um Programa de Intervenção em Processamento Auditivo para crianças em idade escolar.

PURPOSE: The study aimed at the development and content validation of an Auditory Processing Intervention Program for school-aged European Portuguese speaking children with Auditory Processing Disorder. METHODS: The first step was the program's development and its instructions manual, which includes objectives, activities, procedures, materials, reinforcement, instructions, and verbal stimuli used, for the following auditory skills: auditory discrimination, auditory attention; auditory memory; auditory closure; figure-ground; auditory separation; auditory integration; binaural fusion; content validation was performed next, with two expert panels analyzing the program, through the use of a questionnaire. Content validity was calculated using the content validity index. RESULTS: Program evaluation shows an excellent content validity. Some items were modified after analyzing the experts' comments and suggestions (e.g. instructions, intensity differences, main character). CONCLUSION: This work allowed the development and content validation of an auditory processing intervention program, with verbal stimuli, selected according to strict linguistic criteria. In the future, the acceptability and efficacy of this program with the target population should be analyzed.

OBJETIVO: O estudo teve como objetivos o desenvolvimento e validação de conteúdo de um Programa de Intervenção em Processamento Auditivo, destinado a crianças em idade escolar, com perturbação do processamento auditivo, falantes do português europeu. MÉTODO: A primeira etapa consistiu no desenvolvimento do programa e respetivo manual de instruções, que inclui objetivos, atividades, procedimentos, materiais, reforços, instruções e estímulos verbais utilizados, para as competências auditivas de discriminação auditiva, atenção auditiva, memória auditiva, fechamento, figura-fundo; separação binaural, integração binaural e fusão binaural; a segunda etapa consistiu na validação de conteúdo, por dois painéis de peritos que analisaram o programa, através de um questionário. A validade de conteúdo foi calculada usando o índice de validade de conteúdo. RESULTADOS: A avaliação do programa revela uma excelente validade de conteúdo. Alguns itens foram modificados depois da análise dos comentários e sugestões dos peritos (ex: instruções, desníveis de intensidade, personagem principal). CONCLUSÃO: O estudo permitiu o desenvolvimento e validação de um programa de intervenção em processamento auditivo, com estímulos verbais, selecionados de acordo com critérios linguísticos rigorosos. Futuramente, está prevista a realização de estudos de aceitabilidade e eficácia do programa junto da população alvo.

Auditory Processing Disorder Test Battery in European Portuguese-Development and Normative Data for Pediatric Population.

There is an increasing need for state-of-the-art Central Auditory Processing assessment for Portuguese native speakers, applicable as early as possible. As a contribution to answering this need, this paper presents a new battery for Central Auditory Processing assessment for European Portuguese applicable to children aged 5 and above, named BAPA-PE, providing information regarding test selection and development. The battery consists of six behavioral tests: Staggered Spondaic Words (SSW) for European Portuguese, Filtered Speech, Speech in Noise, Detection Interval in Noise, Duration, and Frequency Pattern. The normative data for children aged 5 to 12 are also reported. A sample was obtained of 217 subjects without ear pathology and with typical development. Each age group was composed of at least 30 children. All children were evaluated using pure tone audiometry, speech audiometry, impedance, and otoacoustic emissions. Normative scores are reported for each of the six auditory processing tests. The assessment is applicable to young children (aged 5 and 6). The statistical analyses showed significant effects in scores of Age for all tests and of Ear for several tests. The main result from the work presented, the Auditory Processing Assessment Battery-European Portuguese (BAPA-PE), is available for clinical use with normative data. This battery is a new tool for behaviorism assessment of European Portuguese speakers with suspected central auditory pathology and for monitoring the results of auditory training.

Facial nerve stimulation after cochlear implantation: Our experience in 448 adult patients.

Facial nerve stimulation (FNS) after cochlear implant activation is a well-known side effect, with an incidence rate raging between 1% and 14.9%. Some causes of deafness have been associated with a higher incidence of this entity, however, there is still no consensus regarding its pathophysiological mechanisms. Although FNS can be solved with changes in speech processor programming, in some cases this can lead to a decrease in performance. The aim of this work was to review the epidemiologic, clinical aspects, and performance results in a group of FNS after cochlear implantation. It was conducted a retrospective chart review of 448 adult patients, all implanted between 1985 and 2016. Speech perception tests results were statistically analysed, using non-parametric tests. We registered a group of 13 patients with FNS, contributing to a prevalence of 2.9%. The causes of hearing loss in this group varied between otosclerosis, Menière's disease, head trauma, and idiopathic cause. Six cases were managed by changing the programming strategy and the other seven required the deactivation of the affected electrodes. Statistical evaluation showed no statistically significant difference between the performance results of the groups with and without FNS. In this series, the overall incidence of FNS was consistent with the literature. Our study supports the current idea that FNS can frequently be eliminated by changing programming strategies or deactivating the involved electrodes, without affecting the implant's performance.

A European Perspective on Auditory Processing Disorder-Current Knowledge and Future Research Focus.

Current notions of "hearing impairment," as reflected in clinical audiological practice, do not acknowledge the needs of individuals who have normal hearing pure tone sensitivity but who experience auditory processing difficulties in everyday life that are indexed by reduced performance in other more sophisticated audiometric tests such as speech audiometry in noise or complex non-speech sound perception. This disorder, defined as "Auditory Processing Disorder" (APD) or "Central Auditory Processing Disorder" is classified in the current tenth version of the International Classification of diseases as H93.25 and in the forthcoming beta eleventh version. APDs may have detrimental effects on the affected individual, with low esteem, anxiety, and depression, and symptoms may remain into adulthood. These disorders may interfere with learning per se and with communication, social, emotional, and academic-work aspects of life. The objective of the present paper is to define a baseline European APD consensus formulated by experienced clinicians and researchers in this specific field of human auditory science. A secondary aim is to identify issues that future research needs to address in order to further clarify the nature of APD and thus assist in optimum diagnosis and evidence-based management. This European consensus presents the main symptoms, conditions, and specific medical history elements that should lead to auditory processing evaluation. Consensus on definition of the disorder, optimum diagnostic pathway, and appropriate management are highlighted alongside a perspective on future research focus.

Auditory rehabilitation after cochlear implantation in adults with hearing impairment after head trauma.

INTRODUCTION: In this paper, the authors analyze the auditory rehabilitation after cochlear implantation in adults with hearing impairment after head trauma, comparing their performance with that of other cochlear implant (CI) adult users who have post-lingual hearing impairment with other etiologies. METHODS: The participants were divided into two groups: group 1 (N = 14) composed of CI adult users who have acquired severe to profound hearing loss after head trauma; group 2 (N = 231) composed of CI adult users who have severe to profound hearing loss from other etiologies. Performance was assessed using the following tests: tonal audiometry, speech audiometry, consonantal phonemes identification test, 100 words test, 100 words through the telephone test, monosyllables test, numbers test, sentences test, and sentences through the telephone test. RESULTS: Average results from group 1 were lower when compared with those of group 2 in all the tests used. No statistically significant difference was found for most tests. Statistically significant difference was found for consonantal phonemes identification test, 100 words through the telephone test, monosyllables test (when analyzed regarding the phonemes correctly repeated), and sentences through the telephone test. DISCUSSION: The performance of the group of CI adult users who have acquired hearing impairment after head trauma was globally lower than that observed on the group of hearing impairment with other etiologies. However, the difference was not statistically significant for most tests. Despite this difference in performance, the results from the group of CI adult users who have acquired hearing impairment after head trauma show the effectiveness of auditory rehabilitation through cochlear implantation in these situations.

Two portuguese cochlear implanted dizygotic twins: a case report.

Individual's hearing performance after cochlear implant (CI) is variable and depends on different factors such as etiology of deafness, age at implantation, and social/family hearing environment. Here we report the case of dizygotic twins, boy and girl, presenting with neurosensorial profound deafness prior CI (age of implantation = 3.5 years old). Both parents have severe/profound deafness, since childhood, and use sign language as primary mode of communication. Clinical and genetic characterization was performed, as well as the assessment of the auditory and oral (re)habilitation after CI, applying a battery of audiological, speech, and language tests. The twin girl and the father were homozygous for the c.35delG mutation in the GJB2 gene, while the twin boy and the mother were compound heterozygotes, both monoallelic for c.35delG and for the deletion del(GJB6-D13S1830) in the GJB6 gene. The remaining hearing impaired relatives were c.35delG homozygotes. The genetic cause of deafness was thus identified in this family. Some noteworthy differences were observed regarding twins' auditory and oral performance after CI. Subsequent follow-up of these children allowed us to conclude that those differences were most likely due to the different environment in which the twins have been living than to their different GJB2/GJB6 genotypes.

Cochlear implant rehabilitation outcomes in Waardenburg syndrome children.

OBJECTIVES: The purpose of this study was to review the outcomes of children with documented Waardenburg syndrome implanted in the ENT Department of Centro Hospitalar de Coimbra, concerning postoperative speech perception and production, in comparison to the rest of non-syndromic implanted children. METHODS: A retrospective chart review was performed for children congenitally deaf who had undergone cochlear implantation with multichannel implants, diagnosed as having Waardenburg syndrome, between 1992 and 2011. Postoperative performance outcomes were assessed and confronted with results obtained by children with non-syndromic congenital deafness also implanted in our department. Open-set auditory perception skills were evaluated by using European Portuguese speech discrimination tests (vowels test, monosyllabic word test, number word test and words in sentence test). Meaningful auditory integration scales (MAIS) and categories of auditory performance (CAP) were also measured. Speech production was further assessed and included results on meaningful use of speech Scale (MUSS) and speech intelligibility rating (SIR). RESULTS: To date, 6 implanted children were clinically identified as having WS type I, and one met the diagnosis of type II. All WS children received multichannel cochlear implants, with a mean age at implantation of 30.6±9.7months (ranging from 19 to 42months). Postoperative outcomes in WS children were similar to other nonsyndromic children. In addition, in number word and vowels discrimination test WS group showed slightly better performances, as well as in MUSS and MAIS assessment. CONCLUSIONS: Our study has shown that cochlear implantation should be considered a rehabilitative option for Waardenburg syndrome children with profound deafness, enabling the development and improvement of speech perception and production abilities in this group of patients, reinforcing their candidacy for this audio-oral rehabilitation method.

DFNB1-associated deafness in Portuguese cochlear implant users: prevalence and impact on oral outcome.

OBJECTIVES: Hearing loss is a condition that interferes with the development of the child at a cognitive and language level. Therefore, early diagnosis of deafness is important for (re)habilitation, namely through the use of cochlear implant (CI). The present study aimed at screening CI Portuguese individuals for the presence of mutations in the genes GJB2 and GJB6 (DFNB1 locus), and searching a possible correlation between the genotype and the oral habilitation outcome following implantation. METHODS: Our sample included 117 CI individuals implanted longer than 5 years. Sequencing of GJB2 entire coding region was first performed. The presence of deletions del(GJB6-D13S1830) and del(GJB6-D13S1854) was subsequently tested by multiplex PCR. To assess the oral outcome of these individuals, a global score is calculated through a formula that integrates the results of a battery of speech and audiological tests routinely used in ORL services. This global oral performance score was used to test whether individuals with DFNB1-associated deafness perform significantly better than individuals without DFNB1-associated deafness. RESULTS: In 35% of the cases, deafness was clearly associated to DFNB1. The most common mutated allele was c.35delG (85%). Other variants have also been found, namely p.Gly130Ala, p.Asn206Ser, p.Val37Ile, p.Glu47X, p.Arg184Trp, p.Trp24X and the two common GJB6 deletions, del(GJB6-D13S1854) and del(GJB6-D13S1830), the last one identified for the first time in our population. Regarding the oral outcome, after testing the homogeneity of the two groups it could be observed that, in mean, the individuals with DFNB1-associated deafness perform significantly better (p=0.012) than the individuals without DFNB1-associated deafness. DISCUSSION AND CONCLUSION: This first screening of DFNB1 genes in the Portuguese CI population provides clear evidence of the high proportion of DFNB1-associated deafness amongst the Portuguese implanted individuals. DFNB1 status is significantly associated to higher oral performance scores, with DFNB1 individuals performing, on average, 6% better than the individuals without DFNB1-associated deafness.