As malformações congênitas de orelha causam dificuldades auditivas, interferindo no desenvolvimento das habilidades de fala e linguagem e, consequentemente, prejudicando o desenvolvimento social, emocional, cognitivo e acadêmico da criança. Para minimizar estas dificuldades, o aparelho de amplificação sonora individual (AASI) por condução óssea é indicado. Com a possibilidade de adaptação do sistema de frequência modulada (FM) pelo SUS, de acordo com a portaria n° 1.274 do Ministério da Saúde, de 25 de junho de 2013, o objetivo deste trabalho foi avaliar o benefício deste dispositivo em crianças e adolescentes com malformação de orelha externa e/ou média, usuários de AASI por condução óssea, sob a ótica dos próprios usuários, seus pais e professores. Participaram da pesquisa vinte e quatro crianças e adolescentes, doze pais ou responsáveis e doze professores. Foram utilizados os instrumentos: Listas de Sentenças em Português, para avaliar a percepção de fala no ruído e Classroom Participation Questionnaire (CPQ), para a auto avaliação da participação em sala de aula (usuários); Questionário Avaliação do Sistema FM, para avaliar o desempenho em diferentes situações auditivas (pais) e Screening Instrument For Targeting Educational Risk in Secondary Students (SIFTER), para a avaliação do desempenho acadêmico (professor). Todas as avaliações foram realizadas nas condições com e sem o sistema de FM e para a comparação dos resultados foi utilizado o teste t pareado. Em todos os procedimentos estatísticos foi adotado nível de significância de 5% (p<0,05). Foi observada diferença significante com o uso do sistema de FM para todos os aspectos avaliados, evidenciando o benefício da adaptação deste dispositivo para esta população. / Congenital ear malformations cause hearing impairment, interfering with speech and language development, and, consequently, impairing the social, emotional, cognitive and academic development of the child. Bone conduction hearing aids are indicated to minimize these difficulties. With the possibility of fitting the FM System by the Brazilian Health Public System, according to the ordinance no. 1,274 from the Ministry of Health, dated June 25, 2013, this study aimed to evaluate the benefit of the FM System in children and adolescents with external ear and/or middle ear malformation, users of bone conduction hearing aids, through the perspective of the users, their parents and teachers. Twenty-four children and adolescents, twelve parents or guardians, and twelve teachers participated in this study. The instruments used were: Sentence Lists in Brazilian Portuguese, to evaluate speech perception in noise, and Classroom Participation Questionnaire (CPQ), for self-assessment of classroom participation (users); FM System Evaluation Questionnaire, to evaluate performance in different auditory situations (parents); and Screening Instrument for Targeting Educational Risk in Secondary Students (SIFTER), to evaluate academic performance (teachers). All evaluations were performed with and without the FM System, and the paired t-test was used to compare the results. A significance level of 5% was adopted (p<0.05) in all statistical tests. A significant difference was observed with the use of the FM system in all aspects evaluated, demonstrating the benefit of fitting this device in this population.

As malformações congênitas de orelha causam dificuldades auditivas, interferindo no desenvolvimento das habilidades de fala e linguagem e, consequentemente, prejudicando o desenvolvimento social, emocional, cognitivo e acadêmico da criança. Para minimizar estas dificuldades, o aparelho de amplificação sonora individual (AASI) por condução óssea é indicado. Com a possibilidade de adaptação do sistema de frequência modulada (FM) pelo SUS, de acordo com a portaria n° 1.274 do Ministério da Saúde, de 25 de junho de 2013, o objetivo deste trabalho foi avaliar o benefício deste dispositivo em crianças e adolescentes com malformação de orelha externa e/ou média, usuários de AASI por condução óssea, sob a ótica dos próprios usuários, seus pais e professores. Participaram da pesquisa vinte e quatro crianças e adolescentes, doze pais ou responsáveis e doze professores. Foram utilizados os instrumentos: Listas de Sentenças em Português, para avaliar a percepção de fala no ruído e Classroom Participation Questionnaire (CPQ), para a auto avaliação da participação em sala de aula (usuários); Questionário Avaliação do Sistema FM, para avaliar o desempenho em diferentes situações auditivas (pais) e Screening Instrument For Targeting Educational Risk in Secondary Students (SIFTER), para a avaliação do desempenho acadêmico (professor). Todas as avaliações foram realizadas nas condições com e sem o sistema de FM e para a comparação dos resultados foi utilizado o teste t pareado. Em todos os procedimentos estatísticos foi adotado nível de significância de 5% (p<0,05). Foi observada diferença significante com o uso do sistema de FM para todos os aspectos avaliados, evidenciando o benefício da adaptação deste dispositivo para esta população. / Congenital ear malformations cause hearing impairment, interfering with speech and language development, and, consequently, impairing the social, emotional, cognitive and academic development of the child. Bone conduction hearing aids are indicated to minimize these difficulties. With the possibility of fitting the FM System by the Brazilian Health Public System, according to the ordinance no. 1,274 from the Ministry of Health, dated June 25, 2013, this study aimed to evaluate the benefit of the FM System in children and adolescents with external ear and/or middle ear malformation, users of bone conduction hearing aids, through the perspective of the users, their parents and teachers. Twenty-four children and adolescents, twelve parents or guardians, and twelve teachers participated in this study. The instruments used were: Sentence Lists in Brazilian Portuguese, to evaluate speech perception in noise, and Classroom Participation Questionnaire (CPQ), for self-assessment of classroom participation (users); FM System Evaluation Questionnaire, to evaluate performance in different auditory situations (parents); and Screening Instrument for Targeting Educational Risk in Secondary Students (SIFTER), to evaluate academic performance (teachers). All evaluations were performed with and without the FM System, and the paired t-test was used to compare the results. A significance level of 5% was adopted (p<0.05) in all statistical tests. A significant difference was observed with the use of the FM system in all aspects evaluated, demonstrating the benefit of fitting this device in this population.

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La Microsomie Hémifaciale (MHF) est un spectre de maladie qui entraîne le développement hypoplasique unilatéral des structures faciales dérivées du premier et deuxième arc pharyngé. Bien que ce soit l'une des pathologies crâniofaciales congénitales des plus courantes, son algorithme de traitement idéal reste à débattre, principalement en raison de la nature complexe des patients et de leur grande variété phénotypique. Plus spécifiquement, la reconstruction de microtie représente un défi chirurgical chez les patients atteints de MHF, principalement dû à leur anatomie hypoplasique unilatérale. Malheureusement, notre revue de littérature a démontré que les études abordant cette question sont rare/de faible qualité, avec seulement 11 études fournissant 22 recommandations de grade C (n = 5) ou D (n = 17). Plus précisément, le positionnement approprié du pavillon de l’oreille reconstruite demeure une des tâches particulièrement difficiles. Étant donné que la transposition des mesures faciales du côté non affecté (comme cela est classiquement fait pour la reconstruction de la microtie) ne serait pas symétrique et que de futures chirurgies faciales correctives (qui peuvent modifier l’apparence faciale) sont généralement effectuées, la détermination de l'emplacement idéal du pavillon reconstruit n’est pas facile. Par conséquent, une nouvelle méthode de placement symétrique du pavillon reconstruit, basée sur le crâne plutôt que sur le visage, est proposée. Nous avons mené une étude rétrospective basée sur des mesures craniométriques provenant de tomodensitométries 3-D de 36 patients atteints de MHF, représentant 44 scans. Tout d'abord, l'intégrité de la voûte crânienne postérieure chez les patients atteints d'HFM a été prouvée (p > 0.001). En outre, les relations craniométriques entre le pavillon et l'endocrâne/la voûte crânienne postérieure étaient très précises chez les témoins sains (p > 0.001), et ont été validées pour tous les groupes d'âge (p > 0.001) dans notre cohorte MHF. Les "mesures L" proposées sont des mesures linéaires entre le pavillon sain et la voûte crânienne postérieure, qui peuvent être utilisées pour prédire avec précision l'emplacement idéal du pavillon reconstruit du côté malade. Celles-ci représentent un complément simple et peu coûteux dans la planification préopératoire de la reconstruction de microtie pour les patients atteints de MHF. / Hemifacial Microsomia (HFM) is a disease spectrum causing the unilateral hypoplastic development of facial structures derived from the first and second pharyngeal arches. Although it is one of the most common congenital craniofacial disorders, its optional treatment algorithm remains unclear, mainly due to the complex nature of these patients and the wide variety of phenotypes. More specifically, microtia reconstruction is a highly challenging surgery among HFM patients, given their unilateral hypoplastic anatomy. Unfortunately, our review of the literature demonstrated it is quite sparse/of low-quality regarding studies addressing such, with only 11 studies providing 22 main recommendations of either grade C (n = 5) or D (n = 17). More specifically, appropriate construct positioning is a particularly difficult task in microtia reconstruction for HFM patients. Given that transposing facial measurements from the unaffected side (as is classically done for microtia reconstruction) would not be symmetric and that future corrective skeletal surgeries (that may alter the facies) are usually performed, determining the construct’s ideal location is difficult. Therefore, a novel method for symmetric construct placement, based on the cranium rather than the face, was hypothesized. We conducted a retrospective, 3-D CT based craniometric study of 36 HFM patients accounting for 44 scans. First, the integrity of the posterior cranial vault among HFM patients was determined (p > 0.001). Further, craniometric relationships between the auricle and endocranium/the posterior cranium were highly precise in healthy controls (p > 0.001), and were proved to hold true across all age groups (p > 0.001) in our HFM cohort. The proposed “L measures” are linear measurements between the healthy auricle and the posterior cranium, that can be used to accurately predict ideal construct location on the diseased side. These represent a simple and costless adjunct in the pre-operative planning of microtia reconstruction for HFM patients.