Abstract Objective: To compare the incidence and the histopathological aspect of spontaneous and two induced Mongolian gerbils’ models of cholesteatoma: External Auditory Canal (EAC) obliteration model and the Auditory Tube (AT) cauterization model. Methods: Fifty-four ears of 27 animals were divided into EAC obliteration, AT cauterization, and control groups and histologically assessed for cholesteatoma incidence and classification at intervals of 2, 4, 8, and 16 weeks. Results: Cholesteatoma was diagnosed in 30 of the 53 ears evaluated with a significantly higher incidence in groups that received some type of intervention (p < 0.0001). It was not possible to histologically distinguish cholesteatomas of the same stage between the study groups. Conclusion: Although we observed a significant increase in cholesteatoma incidence with the two methods used when compared to the control group, all developed cholesteatomas were apparently identical from a histological point of view.

Abstract Objectives: Analyze the prevalence of retractions in different areas of the Tympanic Membrane (TM), the correlations between the involvement of the Pars Tensa (PT) and Pars Flaccida (PF), and the air-bone gaps. Methods: A cross-sectional study. Patients with moderate and/or severe TM retraction of 2200 consecutive patients with chronic otitis media between August 2000 and January 2019 were included. Ears with previous surgery were excluded. Ears were classified as isolated PF and PT retractions and association of both. The degrees of severity and presence of effusion were evaluated. The data were analyzed using the SPSS Statistics software program. Results: 661 ears were included. The prevalence of isolated atical retractions was 24.9%, of isolated posterior quadrants was 10.6%, and of association of quadrants was 64%. There was no correlation between the retractions in the different areas of the TM (posterior and attic quadrants: r = 0.13; p = 0.041; anterior and posterior quadrants: r = 0.23; p = 0.013, anterior and attic quadrants: r = 0.06; p = 0.043). Effusion was present in 30.7% of the ears. ABG median was lower in ears with PF retraction (6.25 dB HL) than PT retraction, isolated (15 dB HL) or not (13.75 dB HL; p < 0.05); 72% of the ears had an ABG ≤ 20dB HL. For severity of the retraction of PF, the ABG was similar across groups. For the PT, there was a global difference in the medians of ABG in terms of the degree of severity, with a moderate correlation. Conclusion: The prevalence of moderate and severe retractions was 24.5%; 64% of the ears had an association of affected regions. There was no correlation between the retraction in the different areas of the TM. We found a significant correlation between the severity of retraction and the worsening of ABG threshold, only for PT. Evidence level: 4.

Abstract Introduction Sensory deficits of the upper airways are common in people with Parkinson disease. Compounded by considerable deterioration of the swallowing function, these deficits may contribute to the elevated rates of morbidity and mortality among this population. Objective To examine the sensitivity of the vocal tracts of people with Parkinson disease using nasal videoendoscopy and to compare the results with paired controls. Methods The present was a prospective, observational, case-control study. Sensitivity assessments were conducted in a sample of 24 people divided into 2 groups: one group of 12 patients with Parkinson’s disease, and a control group with 12 healthy subjects. The study group also underwent a fiberoptic endoscopic evaluation of swallowing and answered the Swallowing Disturbance Questionnaire (SQD) to detect dysphagia. Results There was a significant difference (p < 0.05) in the region of the arytenoid cartilages, showing that sensitivity was better preserved among the control group, and that sensitivity deficits were present in the study group. The qualitative results showed sensorial impairment in the study group than in the control group regarding the base of the tongue, the vestibular folds, and the vocal folds. The study group showed self-awareness regarding the deficits in the swallowing function, but there was no statistically significant association between swallowing function deficits and deterioration of sensorial function among them. Conclusions Sensorial deficits were present in the study group when compared to the healthy subjects, mainly in the qualitative evaluation.

Abstract Introduction Cholesteatoma is a disease with significant clinical impact but is incompletely understood. The challenge of performing studies with long-term follow-up in humans is a factor that has restricted the advance of knowledge in this field. Thus, the use of animal models is highly pertinent, and the Mongolian gerbil model has emerged as one of the most useful. Objective The present study aims to evaluate, through serial otoendoscopies, the development and characteristics of pars flaccida retraction pocket and cholesteatoma in Mongolian gerbils after obliteration of the eustachian tube and compare it with the control group. Methods Forty Mongolian gerbils were divided into two groups of 20 animals each. In the intervention group, the animals were followed with serial otoendoscopies after eustachian tube obliteration. In the control group, the animals were only followed through serial otoendoscopies. Results At the end of the 16-week follow-up, cholesteatoma was present in 13 of 38 (34.2%) ears in the intervention group, and in 7 of 34 (20.6%) in the control group (p = 0.197). When we considered cholesteatoma and its potential precursor, pars flaccida retraction pocket, in a combined way, we verified it in 23 of 38 (60.8%) in the intervention group and in 11 of 34 (32.3%) in the control group (p = 0.016). Conclusions Over the 16 weeks of follow-up, serial otoendoscopies enabled us to evaluate the formation and development of pars flaccida retraction pockets and cholesteatomas in Mongolian gerbils and proved to be an excellent diagnostic tool.

Resumo Introdução: O câncer de língua é um dos subtipos mais comuns do câncer de cabeça e pescoço. Os efeitos agressivos do tratamento causam impactos estéticos, psicossociais e funcionais, principalmente a disfagia, os quais afetam a qualidade devida do paciente. A reabilitação, essencial para a máxima recuperação funcional, auxilia o paciente a lidar com as novas estruturas e tem impacto positivo na qualidade de vida. Objetivo: Verificar o impacto da fonoterapia na Qualidade de Vida relacionada à deglutição de pacientes tratados por câncer de língua. Método: Ensaio clínico randomizado paralelo, realizado em um hospital público de Porto Alegre, RS, Brasil. Antes e após a intervenção, os participantes foram avaliados por meio de um questionário de Qualidade de Vida (o Índice de Desvantagem da Deglutição), classificados quanto a gravidade da disfagia (por meio do exame de Videoendoscopia da Deglutição) e quanto ao nível da escala funcional de ingestão por via oral. O grupo experimental foi submetido a quatro sessões semanais de fonoterapia no período de um mês, enquanto o grupo controle recebeu o acompanhamento habitual da instituição. Resultados: Trinta indivíduos tratados para câncer de língua foram divididos em um grupo estudo e um grupo controle. Houve uma diminuição significativa, de aproximadamente 40 pontos (p <0,001), nos escores de Qualidade de Vida após a intervenção no grupo experimental. Verificou-se correlação significativa entre a melhora da Qualidade de Vida, a redução da gravidade da disfagia e o aumento dos níveis da escala funcional de ingestão por via oral (p <0,001). Conclusão: Após a fonoterapia, pacientes tratados por câncer de língua apresentaram melhora nos escores de Qualidade de Vida relacionada à deglutição e na gravidade da disfagia.

Abstract Introduction: Tongue cancer is one of the most common subtypes of head and neck cancer. The aggressive effects of treatment cause aesthetic, psychosocial and functional deficits, especially dysphagia, which affects patient quality of life. Rehabilitation, which is essential for functional maximum recovery, helps patients deal with new and altered structures and has a positive impact on quality of life. Objective: To verify the impact of speech therapy on swallowing quality of life in tongue cancer patients after treatment. Methods: This parallel randomized clinical trial was conducted at a public hospital in Porto Alegre, RS, Brazil. Before and after the intervention, a quality of life questionnaire (the Deglutition Handicap Index) was employed, dysphagia severity was assessed with fiberoptic endoscopic evaluation of swallowing, and the Functional Oral Intake Scale carried out. The experimental group underwent four-week sessions of speech therapy over one month, while the control group received the institution’s usual follow-up. Results: Thirty individuals treated for tongue cancer were divided into a study and a control group. Deglutition Handicap Index scores decreased significantly (approximately 40 points) (p < 0.001) after the intervention in the study group. There was a significant correlation between improved quality of life, reduced dysphagia severity and increased in Functional Oral Intake Scale scores (p <0.001). Conclusion: After speech therapy, quality of life scores related to deglutition and dysphagia severity improved in patients treated for tongue cancer.

Abstract Introduction The treatment of cholesteatoma is generally surgical, and the major obstacle is the high prevalence of recidivism. The endoscopic ear surgery technique is proposed to minimize this problem. Objectives To utilize endoscopes to visualize and manipulate cholesteatoma residues after microscopic removal Methods Cross-sectional study. Thirty-two patients with cholesteatoma underwent microscopic wall-up mastoidectomy combined with the endoscopic approach. The subjects were assessed for the presence and location of covert disease. Results Of the 32 cases, 17 (53.12%) had residual cholesteatoma in the endoscopic phase. Minimal disease was found, usually fragments of the cholesteatoma matrix. Pars tensa cholesteatomas had more covert disease than pars flaccida cholesteatomas (62.50% vs 43.75%). Posterior recesses (47.05%) and tegmen tympani (41.17%) were the locations with more covert disease (p< 0.05). Conclusion Cholesteatomas of the pars tensa presented more residual disease and were significantly more common in the posterior recesses and tegmen tympani.

Abstract Objective: To evaluate the impact of the Universal Neonatal Hearing Screening (UNHS) on the age at diagnosis, beginning of treatment, and first cochlear implant surgery. Methods: A retrospective cohort study with children up to 12 years old with bilateral hearing loss were divided into two groups: patients who underwent UNHS and the ones who didn't. The groups were compared according to their age at the beginning of the evaluation at a specialized center, at the beginning of the intervention, and, for the ones who had indication, at the cochlear implant surgery. The group who underwent UNHS was divided between the ones who passed the screening test and the ones who didn’t. They were compared according to their ages at the same moments as the first two groups. Results: 135 patients were included. The median age at the first appointment in a specialized center was 1.42 (0.50 and 2.50) years, at the beginning of treatment 2.00 (1.00 and 3.52) years, and the cochlear implant surgery 2.83 (1.83 and 4.66) years. Children who underwent UNHS were younger than those who didn't, at the three evaluated moments (p < 0.001). In a subanalysis, children who passed the UNHS but were later diagnosed with hearing loss reached the first appointment with a specialist and started treatment older than those who failed the tests. Conclusion: Performing UNHS interfered with the timing of deafness diagnosis and treatment. However, children who passed the screening but were later diagnosed with hearing loss were the category with the most important delay.

Abstract Introduction Hearing loss etiology depends on the population studied as well as on the ethnicity and the socio-economic condition of the analyzed region. Etiological diagnosis contributes to the improvement of preventive measures and to the early identification of this deficiency. Objective To identify the etiological factors of hearing loss and its prevalence in a tertiary hospital in southern Brazil, to verify the frequency of mutations in GJB2 and GJB6 genes, and to correlate the degree of hearing loss with the etiological factors of deafness. Methods This prevalence study involved 140 children with bilateral sensorineural or mixed hearing loss. Medical history, physical examination, audiometry, and evoked auditory brainstem response were conducted. Imaging and genetic examinations were also performed. Results Etiologies and their prevalence were as follows: (a) indeterminate causes, 31.4%; (b) conditions related to neonatal period, 22.1%; (c) genetic, 22.1%; (d) auditory neuropathy, 10%; (e) other factors (cortical malformation, intracranial hemorrhage, and internal ear malformations), 7.9% and (f) congenital infections, 6.4%. Within the genetic cases, ten homozygous and seven heterozygotes of the 35delG mutation were identified, besides two cases of rare variants of GJB2: p.Try172* and p.Arg184Pro. One case with homozygosis of del(GJB6-D13S1830) was found. Regarding severity of hearing loss, in 78.6% of the cases the degree of hearing loss was profound and there were no significant differences when comparing between etiologies. Conclusion The number of indeterminate etiologies is still high and congenital CMV infection may be a possible cause of undiagnosed etiology for hearing loss. The predominance of etiologies related to neonatal conditions and infectious causes are characteristic of developing countries. The most prevalent mutation was 35delG, the main GJB2 gene, probably because of the European influence in the genotype of our population.

Resumo Introdução A etiologia da perda auditiva depende da população estudada, da etnia e da condição socioeconômica da região analisada. O diagnóstico etiológico contribui para o aprimoramento das medidas preventivas e para a identificação precoce dessa deficiência. Objetivos Identificar os fatores etiológicos da perda auditiva e sua prevalência em um hospital terciário do sul do Brasil, verificar a frequência de mutações nos genes GJB2 e GJB6 e correlacionar o grau da perda auditiva com os fatores etiológicos da deficiência auditiva. Método Este estudo de prevalência avaliou 140 crianças com perda auditiva neurossensorial bilateral ou mista. Foram submetidos a anamnese com histórico médico, exame físico, audiometria e potencial evocado auditivo de tronco encefálico. Exames de imagem e genéticos também foram feitos. Resultados As etiologias e sua prevalência foram as seguintes: (a) causas indeterminadas, 31,4%; (b) condições relacionadas ao período neonatal, 22,1%; (c) genética, 22,1%; (d) neuropatia auditiva, 10%; (e) outros fatores (malformação cortical, hemorragia intracraniana e malformações da orelha interna), 7,9% e (f) infecções congênitas, 6,4%. Entre os casos genéticos, foram identificados dez casos homozigotos e sete heterozigotos da mutação 35delG, além de dois casos de variantes raras do GJB2: p.Try172* e p.Arg184Pro. Foi encontrado um caso homozigoto da mutação del (GJB6‐D13S1830). Em relação à gravidade da perda auditiva, em 78,6% dos casos o grau da perda auditiva foi profundo e não houve diferenças significantes na comparação entre as etiologias. Conclusão O número de etiologias indeterminadas ainda é elevado e a infecção congênita por CMV pode ser uma possível causa de etiologia não diagnosticada para perda auditiva. A predominância das etiologias relacionadas às condições neonatais e às causas infecciosas são características de países em desenvolvimento. A mutação mais prevalente foi a 35delG e o principal gene foi o GJB2, provavelmente devido à influência europeia no genótipo de nossa população.

RESUMO Objetivo Analisar o uso e os motivos para o não uso do Sistema de Frequência Modulada (FM) por crianças e adolescentes com perda auditiva e usuários de aparelhos de amplificação sonora individual (AASI) e/ou com implante coclear (IC). Métodos Foi aplicado um questionário em forma de entrevista, por meio de contato telefônico. As questões foram retiradas do questionário FM Listening Evaluation for Children, traduzido e adaptado para a língua portuguesa por Jacob et al. (2010) e denominado Avaliação do Sistema FM, bem como outras três perguntas elaboradas pelos pesquisadores. Resultados Foram entrevistados 87 pais/responsáveis, verificando-se que o sistema FM era utilizadopor menos da metade da amostra. Quanto à mediana de uso de horas diárias do sistema, observou-se diferença nos pacientes com FM no IC, que usavam o dispositivo por maior número de horas diárias, do que os usuários de AASI. Igualmente apresentou diferença estatística o principal motivo para onão uso, relacionado ao fato de os pacientes terem recebido novos AASI e/ou IC incompatíveis com a tecnologia obtida anteriormente. Ainda, os entrevistados gostariam que lhes fossem fornecidas melhores orientações. Conclusão a maior parte dos pacientes não utiliza o sistema FM, sendo o principal motivo o uso de novos AASI e/ou IC. Os pacientes com IC usam o FM de forma mais efetiva (diariamente).

ABSTRACT Purpose To analyze de use and reasons for not using of FM systems by children and adolescents with hearing loss and users of hearing aids (HA) and or cochlear implant (CI). Methods A questionnaire applied in the form of an a interview through telephone contact was used. The questions were removed from the questionnaire “FM Listening Evaluation for children”, translated and adapted for Portuguese language by Jacob et al. (2010) and named “Avaliação do Sistema FM”, and three other questions prepared by the researchers. Results 87 parents/guardians were interviewed, and it was verified that less than half of the sample used the FM system. As for the median use of the FM systems daily hours, there was a statistical difference in patients with FM in CI, Who used the device for a greater number of daily hours than hearing AID users. The main reason for non-use also presented statistical difference to the fact that patients have received new hearing aids and/or CI incompatible with previously obtained. Also, respondents would like the guidance provided improved. Conclusion Most patients did not use the FM system, the main reason being the usage of new hearing aids and/or CI. Patients with CI use FM more effectively (daily).

Resumo Introdução: As evidências de prováveis efeitos de otite média com efusão precoce no proces-samento auditivo central, ressaltam a necessidade de se considerar tais efeitos também em sujeitos com otite média crônica. Objetivo: Investigar e analisar o impacto da otite média crônica não colesteatomatosa no processamento auditivo central em adolescentes. Método: Estudo para o qual foram recrutados 68 adolescentes, 34 com diagnóstico de otite média crônica não colesteatomatosa (grupo de estudo) e 34 sem história otológica (grupo controle). A avaliação dos indivíduos consistiu de: anamnese, audiometria do limiar auditivo para tons puros, audiometria vocal e bateria de testes comportamentais para avaliação do processamento auditivo central. Resultados: Foi encontrada uma diferença estatisticamente significante entre as médias observadas nos grupos de estudo e controle em todos os testes. Foi encontrada uma associação entre o grupo controle e os subgrupos do grupo de estudo com alterações unilaterais em todos os testes. Houve associação entre os resultados dos grupos controle e de estudo para a renda familiar, com maior impacto nos indivíduos com menor renda. Conclusões: A otite média crônica não colesteatomatosa afeta o processamento auditivo central em adolescentes, a audição monoaural de baixa redundância é o mecanismo auditivo mais afetado. Alterações condutivas unilaterais causam mais danos do que as bilaterais e a menor renda familiar parece conduzir a mais alterações no processamento auditivo central de indivíduos com otite média crônica não colesteatomatosa.

Abstract Introduction: Evidences of possible effects of early age otitis media with effusion in the central auditory processing, emphasize the need to consider such effects also in subjects with chronic otitis media. Aim: To investigate and analyze the impact of non-cholesteatomatous chronic otitis media on central auditory processing in teenagers. Methods: This is a study in which 68 teenagers were recruited, 34 with a diagnosis of non-cholesteatomatous chronic otitis media (study group) and 34 without otological disease history (control group). The evaluation of the subjects consisted of: anamnesis, pure-tone threshold audiometry, speech audiometry and a behavioral test battery for assessment of central auditory processing. Results: A statistically significant difference was found between the means observed in the study and control groups in all tests performed. An association was found between the control group and subgroups of the study group with unilateral alterations in all tests. An association was shown between the results for the control group and study group for family income, with a greater impact on subjects with a lower income. Conclusions: Non-cholesteatomatous chronic otitis media affects the central auditory processing in teenagers suffering from the disorder, and monaural low-redundancy hearing is the most affected auditory mechanism. Unilateral conductive changes cause more damage than bilateral ones, and lower family income seems to lead to more changes to the central auditory processing of subjects with non-cholesteatomatous chronic otitis media.

Abstract Introduction The Mismatch Negativity (MMN) auditory evoked potential evaluation is a promising procedure to assess objectively the ability of auditory discrimination. Objective To characterize the latency and amplitude values of MMN in children with normal auditory thresholds and without auditory complaints. Methods Children between 5 and 11 years old participated in the present study. All participants underwent acoustic immittance measurements and tonal and vocal audiometry. The MMN was recorded with the MASBE ATC Plus system (Contronic, Pelotas, RS, Brazil). The electrodes were fixed in Fz (active electrode), Fpz (ground electrode) and in M2 and M1 (references electrodes). The intensity used was 80 dBHL, the frequent stimulus was 1,000 Hz and the rare stimulus was 2,000 Hz. The stimuli were presented in both ears separately. Results For the female group, the mean latencies and amplitude of MMN were 177.3 ms and 5.01 μV in the right ear (RE) and 182.4ms and 5.39 μV in the left ear (LE). In the male group, themean latencies were 194.4 ms in the RE and 183.6ms in the LE, with an amplitude of 5.11 μV in the RE and 5.83 μV in the LE. There was no statistically significant difference between ears (p = 0.867 - latency and p = 0.178 - amplitude), age (p > 0.20) and the gender of the participants (p > 0.05). Conclusion Using the described protocol, the mean latency value of MMN was 184.0 ms for RE and 182.9 ms for LE, and the amplitude was 5.05 μV and 5.56 μV for the left and right ears, respective.

Resumo Introdução: A fístula labiríntica é uma das complicações mais comuns associadas ao colesteatoma. Representa uma perda erosiva do osso endocondral que recobre o labirinto. As razões para a ocorrência da fístula labiríntica induzida pelo colesteatoma ainda são mal compreendidas. Objetivo: Avaliar pacientes com colesteatoma, a fim de identificar possíveis fatores de risco ou achados clínicos associados à fístula labiríntica. Os objetivos secundários foram determinar a prevalência de fístula labiríntica no estudo de coorte, analisar o papel da tomografia computadorizada e descrever os resultados auditivos após a cirurgia. Método: Este foi um estudo de coorte retrospectivo. Foram incluídos pacientes com colesteatoma adquirido de orelha média em pelo menos um lado sem cirurgia prévia que haviam sido submetidos à audiometria e tomografia computadorizada de orelha ou cirurgia em nossa instituição. Os resultados auditivos após a cirurgia foram analisados de acordo com a classificação de fístula labiríntica e da técnica empregada. Resultados: Analisamos um total de 333 pacientes, dos quais 9 (2,7%) apresentavam fístula labiríntica no canal semicircular lateral. Em 8 pacientes, a fístula foi identificada na tomografia computadorizada e confirmada durante a cirurgia. Em pacientes com colesteatomas epitimpânicos posteriores e de via dupla, a prevalência foi de 5,0%; e nos casos com padrão de crescimento de colesteatoma remanescente, a prevalência foi de 0,6% (p = 0,16). Além disso, a taxa de prevalência de fístula labiríntica entre pacientes com e sem vertigem foi de 2,1. Dos pacientes sem perda auditiva neurossensorial antes da cirurgia, 80,0% permaneceram com os mesmos limiares de condução óssea, enquanto 20,0% progrediram para perda auditiva profunda. Dos pacientes com perda auditiva neurossensorial antes da cirurgia, 33,33% permaneceram com a mesma deficiência auditiva, enquanto 33,33% apresentaram melhora da média de dos limiares de condução óssea aos tons puros. Conclusão: A fístula labiríntica deve ser descartada antes do procedimento cirúrgico, particularmente nos casos de colesteatomas epitimpânicos posteriores e de dupla via. A tomografia computadorizada é uma boa modalidade diagnóstica para a fístula do canal semicircular lateral. A perda auditiva neurossensorial pode ocorrer pós-cirurgicamente, mesmo em pacientes previamente não afetados, a despeito da técnica empregada.

Abstract Introduction: Labyrinthine fistula is one of the most common complications associated with cholesteatoma. It represents an erosive loss of the endochondral bone overlying the labyrinth. Reasons for cholesteatoma-induced labyrinthine fistula are still poorly understood. Objective: Evaluate patients with cholesteatoma, in order to identify possible risk factors or clinical findings associated with labyrinthine fistula. Secondary objectives were to determine the prevalence of labyrinthine fistula in the study cohort, to analyze the role of computed tomography and to describe the hearing results after surgery. Methods: This retrospective cohort study included patients with an acquired middle ear cholesteatoma in at least one ear with no prior surgery, who underwent audiometry and tomographic examination of the ears or surgery at our institution. Hearing results after surgery were analyzed according to the labyrinthine fistula classification and the employed technique. Results: We analyzed a total of 333 patients, of which 9 (2.7%) had labyrinthine fistula in the lateral semicircular canal. In 8 patients, the fistula was first identified on image studies and confirmed at surgery. In patients with posterior epitympanic and two-route cholesteatomas, the prevalence was 5.0%; and in cases with remaining cholesteatoma growth patterns, the prevalence was 0.6% (p = 0.16). In addition, the prevalence ratio for labyrinthine fistula between patients with and without vertigo was 2.1. Of patients without sensorineural hearing loss before surgery, 80.0% remained with the same bone conduction thresholds, whereas 20.0% progressed to profound hearing loss. Of patients with sensorineural hearing loss before surgery, 33.33% remained with the same hearing impairment, whereas 33.33% showed improvement of the bone conduction thresholds' Pure Tone Average. Conclusion: Labyrinthine fistula must be ruled out prior to ear surgery, particularly in cases of posterior epitympanic or two-route cholesteatoma. Computed tomography is a good diagnostic modality for lateral semicircular canal fistula. Sensorineural hearing loss can occur post-surgically, even in previously unaffected patients despite the technique employed.

RESUMO Objetivos Analisar as publicações científicas sobre audição em indivíduos com osteogênese imperfeita. Estratégias de pesquisa Trata-se de revisão sistemática de literatura. Foram selecionados os descritores Hearing OR Hearing Loss AND Osteogenesis Imperfecta. Duas revisoras consultaram as bases de dados Cochrane Library, PubMed, LILACS, Scopus e Embase. Critérios de seleção utilizaram-se os Descritores em Ciências da Saúde, não sendo delimitado período de publicação dos estudos. Foram critérios de inclusão estudos de coorte, seguimento, transversais, casos controle e em idioma inglês. Foram critérios de exclusão artigos editoriais, relatos/séries de casos, resumos de eventos e estudos conduzidos em animais. Os estudos selecionados foram analisados pela Iniciativa STROBE e pelo Sistema GRADE. Resultados Dos 652 estudos obtidos, foram selecionados 16. A perda auditiva do tipo condutiva foi o tipo mais comum na população com osteogênese imperfeita (OI) e seu início ocorre por volta da segunda década de vida. A definição e a classificação para aferição de perda auditiva, nas publicações com a população com osteogênese imperfeita, apresentaram discordância entre os estudos. Conclusão Esta revisão respondeu às perguntas a que se propôs, constatando que as alterações audiológicas encontradas em pacientes com OI são do tipo condutivo, neurossensorial e misto. Alterações condutivas são mais comuns em pacientes mais jovens e alterações neurossensoriais, em pacientes mais velhos. A Iniciativa STROBE apontou itens descritos de forma parcial e o Sistema GRADE concluiu que os estudos apresentaram alguma falha metodológica.

ABSTRACT Purpose To analyze scientific publications on the hearing issue of patients with osteogenesis imperfecta. Reserch strategy This study is a systematic literature review. The following descriptors were selected “Hearing” OR “Hearing Loss” AND “Osteogenesis Imperfecta”. Two reviewers searched the Cochrane Library, Pubmed, Latin American and Caribbean Health Sciences Literature, Scopus and Embase databases. Selection criteria Descriptors in Health Sciences were used and the publication period of studies was not limited. Inclusion criteria were cohort, follow-up, cross-sectional, and control studies in English language. Exclusion criteria were editorial articles, case reports, case summaries, and animal studies. Selected studies were analyzed by the STROBE Initiative and the GRADE System. Results Of the 652 studies, 16 were selected. Conductive hearing loss is the most common type in the osteogenesis imperfecta (OI) population and its onset is around the second decade of life. Definition and classification for hearing loss measurement in publications with the osteogenesis imperfecta population present disparities among studies. Conclusion This review met the objectives proposed, concluding that audiological alterations found in OI patients are of conductive, sensorineural and mixed types; conductive alterations are more common in younger patients and sensorineural alterations in older ones. The STROBE initiative partially pointed out described items and the GRADE system concluded that studies present some methodological failure.

Resumo A privação auditiva associada à otite média precoce com efusão tem sido considerada um fator de risco para o processamento auditivo central (CAP), bem como para o desenvolvimento de diversas funções cognitivas. Objetivo: Estudar as funções neuropsicológicas da atenção, memória de trabalho e função executiva em adolescentes com e sem otite média crônica não-colesteatomatosa (NCCOM) e analisar suas inter-relações com a avaliação comportamental da PAC. Métodos: Sessenta e oito adolescentes foram recrutados, 34 foram diagnosticados com NCCOM (grupo de estudo - GE) e 34 não tinham história otológica (grupo controle - GC). Utilizou-se o Instrumento de Avaliação Neuropsicológica Breve de Neupsilin. O CAP foi avaliado por: Diferença de Nível de Mascaramento, Identificação de Frase Sintética, Teste de Detecção de Intervalo Aleatório, Teste de Sequência de Padrão de Duração e Teste de Dígitos Dicóticos. Resultados: Os resultados de Neupsilin mostraram menores escores no grupo de estudo quando comparados ao grupo controle nos seguintes testes: repetição de sequência de dígitos, ordenação de dígitos ascendentes, extensão de sentenças auditivas e fluência verbal fonêmica. Foi encontrada uma associação entre os testes do processamento auditivo central e os subtestes de Neupsilin. Conclusão: Os efeitos do NCCOM na atenção, memória e função executiva relacionados ao transtorno do processamento auditivo central em adolescentes parecem ser potencializados pela gravidade da doença.

Abstract Auditory deprivation associated with early otitis media with effusion has been considered a risk factor for central auditory processing (CAP), as well as for the development of a number of cognitive functions. Objective: To study the neuropsychological functions of attention, working memory and executive function in adolescents with and without non-cholesteatomatous chronic otitis media (NCCOM) and analyze their interrelationships with the behavioral evaluation of CAP. Methods: Sixty-eight adolescents were recruited, 34 were diagnosed with NCCOM (study group - SG), and 34 had no otological history (control group - CG). The Neupsilin Brief Neuropsychological Assessment Instrument was used. CAP was assessed by: Masking Level Difference, Synthetic Sentence Identification, Random Gap Detection Test, Duration Pattern Sequence Test and Dichotic Digits Test. Results: The results of Neupsilin showed lower scores in the study group when compared to the control group on the following tests: digit sequence repetition, ascending digit ordering, auditory sentence span, and phonemic verbal fluency. An association was found between central auditory processing tests and Neupsilin subtests. Conclusion: The effects of NCCOM on attention, memory and executive function related to central auditory processing disorder in adolescents seem to be enhanced by the severity of the disease.

ABSTRACT Introduction: Cleft lip and palate is the most frequent malformation of the head region and affects more than 10 million people worldwide. This study aims at evaluating the selfesteem in patients with cleft lip and palate and comparing that with the self-esteem of non-affected individuals during followup at the Department of Craniomaxillofacial Plastic Surgery of the Hospital de Clínicas of Porto Alegre. Methods: This is a cross-sectional, contemporary study with 160 participants, comprising 80 patients with cleft lip and palate who have already undergone surgical procedures for correcting the condition and 80 non-affected students and employees of the publicschool system as a control group. We used a questionnaire to characterize the group and the Rosenberg self-esteem scale for data collection. Results: There was a significant difference between groups in terms of marital status, schooling, and school retention. Patients with a cleft lip and palate had lower levels of self-esteem than non-affected individuals. Among them, the individuals with bilateral clefts or complete clefts; female gender; economic strata of D/E; low level of schooling; families reconstituted during childhood; and with unsatisfactory results concerning communication, dentition, and lip scar also showed lower levels of self-esteem. Conclusion: There was a significant relationship between cleft lip and palate and low self-esteem.

RESUMO Introdução: A fissura labiopalatina é a malformação mais frequente da região da cabeça e afeta mais de 10 milhões de pessoas no mundo. O objetivo do estudo foi avaliar a autoestima em pacientes portadores de fissura labiopalatina em acompanhamento no Serviço de Cirurgia Plástica Craniomaxilofacial do Hospital de Clínicas de Porto Alegre, comparando-os com indivíduos não fissurados. Métodos: Estudo transversal contemporâneo, com 160 participantes, sendo 80 pacientes com fissura labiopalatina já submetidos a procedimentos cirúrgicos relacionados à afecção e, como grupo controle, 80 alunos e funcionários da rede pública de ensino. Um questionário para caracterizar o grupo e a escala de autoestima de Rosenberg foram utilizados para a coleta de dados. Resultados: Houve diferença significativa entre os grupos quanto ao estado civil, escolaridade e repetência escolar. Os pacientes com fissura labiopalatina apresentam níveis de autoestima menores em relação a indivíduos não afetados. Dentre eles, os subgrupos dos indivíduos com fissura bilateral, fissura completa, do gênero feminino, classe econômica D/E, baixa escolaridade, situação familiar reconstituída na infância e com resultados não satisfatórios em relação à comunicação, dentição e cicatriz de lábio também mostraram níveis de autoestima menores. Conclusão: Houve relação significativa entre fissura labiopalatina e baixa autoestima.