Communication about hereditary cancer risk to offspring: A systematic review of children's perspective.

OBJECTIVE: The present review describes how children experience hereditary cancer risk communication within the family. METHODS: Searches for studies between 1990 and 2020 on PubMed and EBSCO were undertaken, and 15 studies met the inclusion criteria, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The findings informed: (1) how, when and what is discussed about hereditary cancer risk in the family; (2) how does family communication about hereditary cancer risk impact children on psychosocial and behavioral outcomes; (3) what are the child's preferences regarding hereditary cancer risk communication within the family. RESULTS: Disclosure is done mostly by both parents, or mothers only, which is in accordance with the children's preferences. Children value open communication about cancer risk with their parents, although they report experiences of fear, surprise, feeling unhappy, and concern about the increased risk of cancer. Regardless of the method of disclosure, children may be particularly sensitive to their parent's emotional state at the time of disclosure, and they learn from their parents' experiences the potential implications of cancer risk. Children also report that it would be helpful to learn more about genetic cancer syndromes via written materials, and/or meet a genetic counselor. CONCLUSIONS: Children rely on their parents as the primary models of the hereditary cancer experience. Therefore, parents play a central role in the psychological adjustment of children. Findings point to the relevance of family-centered care in hereditary cancer risk that targets not only the mutation carrier individually but also their children and partners.

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.

Patient and Public Involvement in Youth Mental Health Research: Protocol for a Systematic Review of Practices and Impact.

Various health settings have advocated for involving patients and members of the public (PPI) in research as a means to increase quality and relevance of the produced knowledge. However, youth PPI has been an understudied area. This protocol paper describes a new project that aims to summarize what is known about PPI with young people in mental health research. In line with the Preferred Reporting Items for Systematic reviews and Meta-Analyses Statement guidelines we will identify and appraise suitable articles and extract and synthesize relevant information including at least two reviewers at each stage of the process. Results will be presented in two systematic reviews that will describe (a) how youth PPI has been conducted (Review1) and (b) what impact youth PPI had on the subsequent research and on stakeholders (Review2). To our knowledge, this is the first set of reviews that uses a critical appraisal tool, which is co-developed with children and young people. Findings from this project will provide valuable insights and set out the key steps to adopting adequate PPI methods when involving children and young people in mental health research.

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.

Pixantrone, a new anticancer drug with the same old cardiac problems? An in vitro study with differentiated and non-differentiated H9c2 cells.

Pixantrone (PIX) is an anticancer drug approved for the treatment of multiple relapsed or refractory aggressive B-cell non-Hodgkin's lymphoma. It is an aza-anthracenedione synthesized to have the same anticancer activity as its predecessors, anthracyclines (e.g. doxorubicin) and anthracenediones (e.g. mitoxantrone), with lower cardiotoxicity. However, published data regarding its possible cardiotoxicity are scarce. Therefore, this work aimed to assess the potential cytotoxicity of PIX, at clinically relevant concentrations (0.1; 1; and 10 µM) in both non-differentiated and 7-day differentiated H9c2 cells. Cells were exposed to PIX for 48 h and cytotoxicity was evaluated through phase contrast microscopy, Hoescht staining and the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide (MTT) reduction and neutral red (NR) uptake assays. Cytotoxicity was observed in differentiated and non-differentiated H9c2 cells, with detached cells and round cells evidenced by phase contrast microscopy, mainly at the highest concentration tested (10 µM). In the Hoechst staining, PIX 10 µM showed a marked decrease in the number of cells when compared to control but with no signs of nuclear condensation. Furthermore, significant concentration-dependent mitochondrial dysfunction was observed through the MTT reduction assay. The NR assay showed similar results to those obtained in the MTT reduction assay in both differentiated and non-differentiated H9c2 cells. The differentiation state of the cells was not crucial to PIX effects, although PIX toxicity was slightly higher in differentiated H9c2 cells. To the best of our knowledge, this was the first in vitro study performed with PIX in H9c2 cells and it discloses worrying cytotoxicity at clinically relevant concentrations.

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.

[European Portuguese EARS test battery adaptation]. / Adaptação da bateria de testes EARS ao Português Europeu.

INTRODUCTION: The use of adequate assessment tools in health care is crucial for the management of care. The lack of specific tools in Portugal for assessing the performance of children who use cochlear implants motivated the translation and adaptation of the EARS (Evaluation of Auditory Responses to Speech) test battery into European Portuguese. This test battery is today one of the most commonly used by (re)habilitation teams of deaf children who use cochlear implants worldwide. The goal to be achieved with the validation of EARS was to provide (re)habilitation teams an instrument that enables: (i) monitoring the progress of individual (re)habilitation, (ii) managing a (re)habilitation program according to objective results, comparable between different (re)habilitation teams, (iii) obtaining data that can be compared with the results of international teams, and (iv) improving engagement and motivation of the family and other professionals from local teams. MATERIAL AND METHODS: For the test battery translation and adaptation process, the adopted procedures were the following: (i) translation of the English version into European Portuguese by a professional translator, (ii) revision of the translation performed by an expert panel, including doctors, speech-language pathologists and audiologists, (iii) adaptation of the test stimuli by the team's speechlanguage pathologist, and (iv) further review by the expert panel. RESULTS: For each of the tests that belong to the EARS battery, the introduced adaptations and adjustments are presented, combining the characteristics and objectives of the original tests with the linguistic and cultural specificities of the Portuguese population. DISCUSSION: The difficulties that have been encountered during the translation and adaptation process and the adopted solutions are discussed. Comparisons are made with other versions of the EARS battery. CONCLUSION: We defend that the translation and the adaptation process followed for the EARS test battery into European Portuguese was correctly conducted, respecting the characteristics of the original instruments and adapting the test stimuli to the linguistic and cultural reality of the Portuguese population, thus meeting the goals that have been set.

Introdução: A utilização de instrumentos de avaliação em saúde adequados é fundamental na gestão da prestação de cuidados. A escassez, em Portugal, de instrumentos específicos para a avaliação do desempenho de crianças utilizadoras de implantes cocleares motivou o trabalho de tradução e de adaptação da bateria de testes EARS (Evaluation of Auditory Responses to Speech) para o português europeu. Esta bateria de testes é hoje um dos instrumentos mais comummente utilizados por equipas de (re)habilitação de crianças surdas com implantes cocleares em todo o mundo. O objetivo a atingir com a validação do EARS foi fornecer às equipas de (re)habilitação um instrumento que permita: (i) monitorizar a evolução individual da reabilitação; (ii) gerir um programa de (re)habilitação de acordo com resultados objetivos, comparáveis entre diferentes equipas de (re)habilitação; (iii) obter dados comparáveis comequipas internacionais; e (iv) melhorar a adesão e a motivação da família e restantes profissionais no ambulatório.Material e Métodos: No processo de tradução e de adaptação da bateria de testes, os procedimentos adotados foram os seguintes: (i) tradução da versão inglesa para português europeu por um tradutor profissional; (ii) revisão dessa tradução realizada por um painel de especialistas constituído por otorrinolaringologistas, terapeutas da fala e técnicos de audiologia; (iii) adaptação dos estímulos de teste pela equipa de terapeutas da fala; e (iv) nova revisão por parte do painel de especialistas.Resultados: São apresentados, para cada um dos instrumentos que compõem a bateria EARS, as adaptações introduzidas, conciliando as características e os objetivos originais dos instrumentos com as particularidades linguísticas e culturais da população portuguesa.Discussão: São discutidas as dificuldades encontradas durante o processo de tradução e de adaptação e as soluções adotadas. São feitas comparações com outras versões da bateria EARS.Conclusão: Considera-se que o processo de tradução e adaptação da bateria de testes EARS para o português europeu foi realizado de forma apropriada, respeitando as características dos instrumentos originais e adequando os estímulos de teste à realidade linguística e cultural da população portuguesa, cumprindo assim os objetivos propostos.

Protocolo de avaliação da amusia: exemplo português / Evaluation protocol for amusia: portuguese sample

A amusia é uma disfunção que compromete o processamento musical. Parte desse processamento é feito a nível do cortéx auditivo primário. O estudo dessa afecção permite-nos avaliar também as vias auditivas centrais. OBJETIVO: Explorar os testes de avaliação diagnóstica da amusia. MÉTODO: Os autores propõem um protocolo de avaliação para doentes com suspeita de amusia (após lesão cerebral ou por queixas de má percepção musical), paralelamente com a avaliação do processamento auditivo central, já implementada no serviço. A base dos testes de avaliação assenta na Montreal Battery of Evaluation of Amusia. Desta ampla bateria de testes, foram selecionados alguns dos exemplos musicais para avaliação de diferentes vertentes da área musical, nomeadamente memória e percepção musical, capacidade de reconhecimento e discriminação musical. Em termos de memória, foi criado um teste de avaliação da memória tardia, adaptado à cultura portuguesa. Estudo prospectivo. RESULTADOS e CONCLUSÕES: Embora ainda em fase experimental e com possibilidades de ajustes na avaliação realizada, considera-se que esta avaliação, aliada ao estudo do processamento auditivo central, permitirá compreender algumas lesões e disfunções centrais, congênitas ou adquiridas, que limitam a percepção auditiva.

Amusia is a disorder that affects the processing of music. Part of this processing happens in the primary auditory cortex. The study of this condition allows us to evaluate the central auditory pathways. OBJECTIVE: To explore the diagnostic evaluation tests of amusia. METHOD: The authors propose an evaluation protocol for patients with suspected amusia (after brain injury or complaints of poor musical perception), in parallel with the assessment of central auditory processing, already implemented in the department. The Montreal Evaluation of Battery of amusia was the basis for the selection of the tests. From this comprehensive battery of tests we selected some of the musical examples to evaluate different musical aspects, including memory and perception of music, ability concerning musical recognition and discrimination. In terms of memory there is a test for assessing delayed memory, adapted to the Portuguese culture. Prospective study. RESULTS AND CONCLUSIONS: Although still experimental, with the possibility of adjustments in the assessment, we believe that this assessment, combined with the study of central auditory processing, will allow us to understand some central lesions, congenital or acquired hearing perception limitations.

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.

Evaluation protocol for amusia: Portuguese sample.

UNLABELLED: Amusia is a disorder that affects the processing of music. Part of this processing happens in the primary auditory cortex. The study of this condition allows us to evaluate the central auditory pathways. OBJECTIVE: To explore the diagnostic evaluation tests of amusia. METHOD: The authors propose an evaluation protocol for patients with suspected amusia (after brain injury or complaints of poor musical perception), in parallel with the assessment of central auditory processing, already implemented in the department. The Montreal Evaluation of Battery of amusia was the basis for the selection of the tests. From this comprehensive battery of tests we selected some of the musical examples to evaluate different musical aspects, including memory and perception of music, ability concerning musical recognition and discrimination. In terms of memory there is a test for assessing delayed memory, adapted to the Portuguese culture. Prospective study. RESULTS AND CONCLUSIONS: Although still experimental, with the possibility of adjustments in the assessment, we believe that this assessment, combined with the study of central auditory processing, will allow us to understand some central lesions, congenital or acquired hearing perception limitations.

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.