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Introduction Turner syndrome (TS) affects ∼ 1 in 2,500 live births. The presence of hearing alterations is one of the comorbidities found in this syndrome. Objective The present study aimed to evaluate the central auditory abilities in TS and to associate the alterations found with the cytogenetic pattern of the syndrome. Methods We included children and adults aged 9 to 39 years old, diagnosed with TS, with numerical or structural alterations of sex chromosomes in their karyotype. A battery of behavioral tests of central auditory processing (CAP) was performed, including a test within the modalities: monoaural low-redundancy, dichotic listening, binaural interaction, and temporal processing (resolution and ordering). We studied auditory skills in the total sample and in the sample stratified by age, divided into groups: G1 (9 to 13 years old), G2 (14 to 19 years old), and G3 (20 to 31 years old). For the association of the cytogenetic pattern, the division was T1 (chromosome monosomy X), and T2 (other TS cytogenetic patterns). Statistical analysis presented data expressed as median and interquartile range for numerical data and as frequency and percentage for categorical data. Results We found alterations in four auditory skills in the three age groups, but there was a statistically significant difference between the age groups only in the Gaps in Noise Test (GIN) ( p -value = 0.009). Regarding karyotype, a greater number of alterations in the T1 cytogenetic pattern (chromosome monosomy X) was observed in four auditory skills, but without a statistically significant difference. Conclusion The alterations found point to an impairment in CAP in TS.

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Abstract Introduction Turner syndrome (TS) affects ~ 1 in 2,500 live births. The presence of hearing alterations is one of the comorbidities found in this syndrome. Objective The present study aimed to evaluate the central auditory abilities in TS and to associate the alterations found with the cytogenetic pattern of the syndrome. Methods We included children and adults aged 9 to 39 years old, diagnosed with TS, with numerical or structural alterations of sex chromosomes in their karyotype. A battery of behavioral tests of central auditory processing (CAP) was performed, including a test within the modalities: monoaural low-redundancy, dichotic listening, binaural interaction, and temporal processing (resolution and ordering). We studied auditory skills in the total sample and in the sample stratified by age, divided into groups: G1 (9 to 13 years old), G2 (14 to 19 years old), and G3 (20 to 31 years old). For the association of the cytogenetic pattern, the division was T1 (chromosome monosomy X), and T2 (other TS cytogenetic patterns). Statistical analysis presented data expressed as median and interquartile range for numerical data and as frequency and percentage for categorical data. Results We found alterations in four auditory skills in the three age groups, but there was a statistically significant difference between the age groups only in the Gaps in Noise Test (GIN) (p-value = 0.009). Regarding karyotype, a greater number of alterations in the T1 cytogenetic pattern (chromosome monosomy X) was observed in four auditory skills, but without a statistically significant difference. Conclusion The alterations found point to an impairment in CAP in TS.

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The Masking Level Difference (MLD) test is one of the main instruments for investigating binaural interaction. Studies with children aged 7-12 years still disagree about the influence of age on test performance and present discordant reference values. This study aimed to verify the effect of age on the performance of children aged 7-12 years in the MLD test and to establish reference values and cutoff criteria for this age group. Fifty-nine children with normal hearing were organized in three groups according to their age: 7-8 (n = 20), 9-10 (n = 20), and 11-12 (n = 19) years. The participants completed the MLD test by Auditec®. The Kruskal-Wallis statistical test was used to compare groups. Reference values were obtained by calculating mean, standard deviation, median, mode, and percentiles, while the cutoff criterion was obtained by subtracting two standard deviations from the mean. No statistically significant differences were observed between the groups regarding the MLD test measures. The mean MLD was 10.51 ± 1.84 dB and the cutoff point was set at 7 dB. Thus, reference values for the MLD test were established for children aged 7-12 years, who presented no effect of age on test performance.

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PURPOSE: To verify the test-retest reliability of the Masking Level Difference in normal hearing female university students. METHODS: Prospective descriptive study with 78 young female adults without hearing complaints, submitted to the compact disc version of the Masking Level Difference by Auditec of Saint Louis. The threshold was determined by the difference between signal-to-noise ratios at hearing thresholds found in the antiphasic and homophasic conditions. The test was applied by the same examiner in two moments (test and retest) with an interval of seven to 14 days between them. Inferential statistical analysis included comparison of test and retest situations using Student's t test for paired samples, calculation of the intraclass correlation coefficient and calculation of 95% confidence intervals for signal-to-noise ratios at hearing thresholds found in the antiphasic and homophasic conditions and for masking level difference. RESULTS: The average signal-to-noise ratio at hearing threshold ​​in the homophasic condition was -12.59 dB and -12.46 dB in the Test and Retest situations, respectively, and -21.54 dB and -21.08 dB in the antiphasic condition. The average value ​​in the final Masking Level Difference result was 8.95 dB in the Test and 8.74 dB in the Retest. Intraclass correlation coefficient values ​​obtained were 0.436, 0.625 and 0.577 for homophasic, antiphasic and Masking Level Difference conditions, respectively. CONCLUSION: The Masking Level Difference showed moderate test-retest reliability in normal hearing adults female university students.

OBJETIVO: Verificar a confiabilidade teste-reteste do Masking Level Difference em estudantes universitárias normo-ouvintes. MÉTODO: Estudo prospectivo descritivo com 78 adultos jovens do gênero feminino sem queixas auditivas, submetidas à versão, em compact disc, do Masking Level Difference da Auditec of Saint Louis. O Masking Level Difference foi determinado por meio da diferença entre as relações sinal-ruído nos limiares auditivos encontrados nas condições antifásica e homofásica. O teste foi aplicado pelo mesmo examinador em dois momentos (teste e reteste) com intervalo de sete a 14 dias entre eles. A análise estatística inferencial incluiu comparação das situações teste e reteste por meio do teste t de Student para amostras pareadas, cálculo do coeficiente de correlação intraclasse e dos intervalos de confiança de 95% para as relações sinal/ruído nos limiares auditivos nas condições antifásica e homofásica e para o cálculo do Masking Level Difference. RESULTADOS: A média da relação sinal-ruído no limiar auditivo na condição homofásica foi -12,59 dB e -12,46 dB nas situações teste e reteste, respectivamente, e -21,54 dB e -21,08 dB na condição antifásica. A média do Masking Level Difference foi 8,95 dB no teste e 8,74 dB no reteste. Os coeficientes de correlação intraclasse obtidos foram 0,436, 0,625 e 0,577 para as condições homofásica, antifásica e Masking Level Difference, respectivamente. CONCLUSÃO: O teste Masking Level Difference mostrou grau moderado de confiabilidade teste-reteste em estudantes universitárias normo-ouvintes.

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ABSTRACT Purpose: to identify the most used tests and the changed hearing skills in the central auditory processing of individuals with sensorineural hearing loss. Methods: an integrative literature review conducted between March and August 2021 in PubMed/MEDLINE, LILACS, SciELO, IBECS, and Index Psi Scientific Journals. The review included studies addressing central hearing skills with central auditory processing behavioral tests in subjects with mild to moderate sensorineural hearing loss. Two independent reviewers made the study selection, data extraction, and qualitative synthesis. The selection aimed at verifying whether the studies answered the research question. Literature Review: 39 studies were found for full-text reading; after applying the eligibility criteria, 13 studies were included in the review. Participants diagnosed with sensorineural hearing loss predominantly had hearing changes in verbal tests, in comparison with normal standards for individuals without hearing loss. Thus, the most changed skills are the ones that depend on speech sound discrimination. Conclusion: the Staggered Spondaic Word Test predominated among the most used behavioral tests, while the most researched central auditory skills were binaural integration and binaural separation.

RESUMO Objetivo: identificar os testes mais utilizados e as habilidades auditivas alteradas no processamento auditivo central, em indivíduos com perda auditiva neurossensorial. Métodos: revisão de literatura integrativa, realizada de março a agosto de 2021 nas bases de dados: PubMed/Medline, Lilacs, Scielo, IBECS e Index Psicologia Periódicos técnico-científicos. Foram incluídos estudos que realizaram a pesquisa das habilidades auditivas centrais, através do teste comportamental do processamento auditivo central, em sujeitos portadores de perda auditiva neurossensorial, de grau leve a moderado. A seleção, extração e síntese qualitativa foram realizadas por dois revisores independentes. A seleção teve como objetivo verificar se os estudos contemplavam a pergunta do estudo. Revisão da Literatura: foram encontrados 39 estudos para leitura do texto na íntegra e, ao aplicar os critérios de elegibilidade, foram incluídos 13 estudos. Observou-se que os participantes diagnosticados com perda auditiva neurossensorial apresentam predomínio de alteração auditiva em testes verbais quando comparados aos padrões de normalidade estabelecidos para indivíduos sem perda. Sendo assim, as habilidades mais alteradas são as que dependem da discriminação dos sons da fala. Conclusão: entre os testes comportamentais mais utilizados, predominou o teste SSW. Quanto as habilidades auditivas centrais mais pesquisadas foram integração binaural e separação binaural.

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ABSTRACT Purpose: to analyze the results of neonatal hearing screening examinations in newborns with and without microcephaly, exposed to the Zika virus, without other risk indicators for hearing loss, and verify the association between screening results, sample characteristics, and the gestational trimester when exposure took place. Methods: a descriptive cross-sectional study. Subjects included in the study had no risk indicator for hearing loss other than microcephaly, and presented, along with their mothers, positive RT-PCR results, respectively at birth and during pregnancy. The transient evoked otoacoustic emission and brainstem auditory evoked potential examinations were applied by the researcher between March 2016 and December 2017. Newborns failed the screening when they failed at least one retest in at least one ear. The data were descriptively analyzed, using the Fisher exact test; p-values equal to or lower than 0.05 were considered significant. Results: out of the 45 subjects, 30 (66.7%) were females, 6.7% were likely to have sensorineural hearing loss, with or without auditory neuropathy spectrum disorder - which was possibly present in only one ear of one of these three subjects. Failure in the screening was statistically significant in subjects with at least one of the congenital Zika syndrome characteristics and subjects with subcortical calcification and brain cortex thinning, macular chorioretinal atrophy with focal pigmentary mottling, and hypertonia with symptoms of extrapyramidal involvement. The gestational trimester of exposure was associated with screening results. Conclusion: the responses in screening point to the possibility of hearing loss in newborns with and without microcephaly, whereas the presence of microcephaly was not significant to examination failures. Exposure in the first gestational trimester indicated a possible relationship with screening failures.

RESUMO Objetivo: analisar os resultados dos exames de triagem auditiva neonatal de recém-nascidos sem e com microcefalia expostos ao vírus Zika, que não apresentaram outros indicadores de risco para deficiência auditiva e verificar a associação entre o resultado da triagem, as características da amostra e o trimestre gestacional em que ocorreu a exposição. Métodos: estudo descritivo de corte transversal. Fizeram parte do estudo sujeitos sem indicadores de risco para deficiência auditiva, com exceção da microcefalia, cujas mães apresentaram na gestação o exame RT-PCR positivo e sujeitos que tiveram o RT-PCR positivo, ao nascimento. Os exames de Emissões Otoacústicas Transientes e Potencial Evocado Auditivo de Tronco Encefálico foram aplicados, pela pesquisadora, entre março de 2016 e dezembro de 2017. O recém-nato foi considerado reprovado na triagem quando apresentou falha em pelo menos um reteste, em ao menos uma orelha. Os dados foram analisados de forma descritiva e utilizando-se o teste estatístico exato de Fisher, p valor menor ou igual a 0,05 foi considerado significante. Resultados: dos 45 sujeitos, 30 (66,7%) eram do sexo feminino, 6,7% apresentaram a probabilidade de alteração do tipo sensorioneural, com ou sem espectro da neuropatia auditiva e somente um dentre esses três sujeitos apresentou uma das orelhas com chance do espectro da neuropatia auditiva, isoladamente. A falha na triagem foi estatisticamente significativa, nos sujeitos que apresentavam pelo menos uma característica da Síndrome da Zika Congênita e nos sujeitos com calcificação subcortical com afilamento do córtex cerebral, atrofia coriorretiniana macular com moteado pigmentar focal e hipertonia com sintomas de envolvimento extrapiramidal. Houve associação do trimestre gestacional da exposição com o resultado de triagem. Conclusão: as respostas verificadas pela triagem apontam para a possibilidade de alteração auditiva em recém-nascidos sem e com microcefalia, onde a presença da microcefalia não foi significante para falha nos exames. A exposição no primeiro trimestre gestacional indicou uma possível relação com falhas na triagem.

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RESUMO Objetivo investigar o efeito inibitório da via auditiva eferente na síndrome de Turner e relacionar com o perfil citogenético. Método estudo descritivo transversal com grupo de comparação. Amostra: Grupo estudo formado por 40 pacientes com síndrome de Turner (17,6 anos); e Grupo controle constituído por 54 indivíduos (18,9 anos), do sexo feminino sem síndrome. Os indivíduos selecionados foram submetidos à pesquisa do efeito inibitório da via auditiva eferente. Resultados A média do Efeito inibitório da via auditiva eferente no grupo estudo na orelha direita foi 0,4 dB e no grupo comparação foi de 1,9 dB, entretanto na orelha esquerda a média do efeito inibitório da via auditiva eferente foi 1,4 dB no grupo estudo e 0,8 dB no grupo comparação. O efeito inibitório da via auditiva eferente foi presente em 14 indivíduos com monossomia e em 15 com outras alterações citogenéticas. Conclusão No grupo estudo o valor do efeito inibitório da via auditiva eferente foi significantemente maior na orelha esquerda e significativamente menor que o grupo controle na direita. Não houve diferença significativa no efeito inibitório da via auditiva eferente entre os tipos de cariótipo.

ABSTRACT Purpose The goal of this study is to investigate the efferent auditory pathways inhibition in Turner's syndrome and to relate it to the cytogenetic profile. Methods This is a cross-sectional study with a comparison group. A sample with 94 participants divided into two groups: The study group was a sample of 40 patients diagnosed with Turner's syndrome (17.6 years of age). The control group was composed of 54 volunteers (18.9 years of age), female, without syndrome. The selected individuals were submitted to efferent auditory pathways inhibition research. Results The mean of the inhibitory effect of the efferent auditory pathway in the study group in the right ear was 0.4 dB and in the comparison group it was 1.9 dB, however in the left ear the mean of the inhibitory effect of the efferent auditory pathway was 1.4 dB in the study group and 0.8 dB in the comparison group. The inhibitory effect of the efferent auditory pathway was present in 14 individuals with monosomy and in 15 with other cytogenetic alterations. Conclusions In the study group, the efferent auditory pathways inhibition value was significantly higher in the left ear and significantly lower than the control group in the right ear. There was no significant difference in efferent auditory pathways inhibition of right ear and left ear between the karyotype types.

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RESUMO Objetivo Verificar a confiabilidade teste-reteste do Masking Level Difference em estudantes universitárias normo-ouvintes. Método Estudo prospectivo descritivo com 78 adultos jovens do gênero feminino sem queixas auditivas, submetidas à versão, em compact disc, do Masking Level Difference da Auditec of Saint Louis. O Masking Level Difference foi determinado por meio da diferença entre as relações sinal-ruído nos limiares auditivos encontrados nas condições antifásica e homofásica. O teste foi aplicado pelo mesmo examinador em dois momentos (teste e reteste) com intervalo de sete a 14 dias entre eles. A análise estatística inferencial incluiu comparação das situações teste e reteste por meio do teste t de Student para amostras pareadas, cálculo do coeficiente de correlação intraclasse e dos intervalos de confiança de 95% para as relações sinal/ruído nos limiares auditivos nas condições antifásica e homofásica e para o cálculo do Masking Level Difference. Resultados A média da relação sinal-ruído no limiar auditivo na condição homofásica foi -12,59 dB e -12,46 dB nas situações teste e reteste, respectivamente, e -21,54 dB e -21,08 dB na condição antifásica. A média do Masking Level Difference foi 8,95 dB no teste e 8,74 dB no reteste. Os coeficientes de correlação intraclasse obtidos foram 0,436, 0,625 e 0,577 para as condições homofásica, antifásica e Masking Level Difference, respectivamente. Conclusão O teste Masking Level Difference mostrou grau moderado de confiabilidade teste-reteste em estudantes universitárias normo-ouvintes.

ABSTRACT Purpose To verify the test-retest reliability of the Masking Level Difference in normal hearing female university students. Methods Prospective descriptive study with 78 young female adults without hearing complaints, submitted to the compact disc version of the Masking Level Difference by Auditec of Saint Louis. The threshold was determined by the difference between signal-to-noise ratios at hearing thresholds found in the antiphasic and homophasic conditions. The test was applied by the same examiner in two moments (test and retest) with an interval of seven to 14 days between them. Inferential statistical analysis included comparison of test and retest situations using Student's t test for paired samples, calculation of the intraclass correlation coefficient and calculation of 95% confidence intervals for signal-to-noise ratios at hearing thresholds found in the antiphasic and homophasic conditions and for masking level difference. Results The average signal-to-noise ratio at hearing threshold ​​in the homophasic condition was -12.59 dB and -12.46 dB in the Test and Retest situations, respectively, and -21.54 dB and -21.08 dB in the antiphasic condition. The average value ​​in the final Masking Level Difference result was 8.95 dB in the Test and 8.74 dB in the Retest. Intraclass correlation coefficient values ​​obtained were 0.436, 0.625 and 0.577 for homophasic, antiphasic and Masking Level Difference conditions, respectively. Conclusion The Masking Level Difference showed moderate test-retest reliability in normal hearing adults female university students.

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PURPOSE: The goal of this study is to investigate the efferent auditory pathways inhibition in Turner's syndrome and to relate it to the cytogenetic profile. METHODS: This is a cross-sectional study with a comparison group. A sample with 94 participants divided into two groups: The study group was a sample of 40 patients diagnosed with Turner's syndrome (17.6 years of age). The control group was composed of 54 volunteers (18.9 years of age), female, without syndrome. The selected individuals were submitted to efferent auditory pathways inhibition research. RESULTS: The mean of the inhibitory effect of the efferent auditory pathway in the study group in the right ear was 0.4 dB and in the comparison group it was 1.9 dB, however in the left ear the mean of the inhibitory effect of the efferent auditory pathway was 1.4 dB in the study group and 0.8 dB in the comparison group. The inhibitory effect of the efferent auditory pathway was present in 14 individuals with monosomy and in 15 with other cytogenetic alterations. CONCLUSIONS: In the study group, the efferent auditory pathways inhibition value was significantly higher in the left ear and significantly lower than the control group in the right ear. There was no significant difference in efferent auditory pathways inhibition of right ear and left ear between the karyotype types.

OBJETIVO: investigar o efeito inibitório da via auditiva eferente na síndrome de Turner e relacionar com o perfil citogenético. MÉTODO: estudo descritivo transversal com grupo de comparação. Amostra: Grupo estudo formado por 40 pacientes com síndrome de Turner (17,6 anos); e Grupo controle constituído por 54 indivíduos (18,9 anos), do sexo feminino sem síndrome. Os indivíduos selecionados foram submetidos à pesquisa do efeito inibitório da via auditiva eferente. RESULTADOS: A média do Efeito inibitório da via auditiva eferente no grupo estudo na orelha direita foi 0,4 dB e no grupo comparação foi de 1,9 dB, entretanto na orelha esquerda a média do efeito inibitório da via auditiva eferente foi 1,4 dB no grupo estudo e 0,8 dB no grupo comparação. O efeito inibitório da via auditiva eferente foi presente em 14 indivíduos com monossomia e em 15 com outras alterações citogenéticas. CONCLUSÃO: No grupo estudo o valor do efeito inibitório da via auditiva eferente foi significantemente maior na orelha esquerda e significativamente menor que o grupo controle na direita. Não houve diferença significativa no efeito inibitório da via auditiva eferente entre os tipos de cariótipo.

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Abstract Introduction: Turner syndrome is a frequent genetic disorder that affects female individuals and covers a large phenotypic variability. Scientific literature suggests an association between hearing loss and Turner syndrome, but it remains a controversial topic. Objective: To associate the cytogenetic alteration with the audiometric profile of individuals with Turner syndrome. Methods: Cross-sectional study, with a hospital-based, convenience sample. Patients diagnosed with Turner syndrome were included and those with difficulty understanding the audiometry and/or other associated syndromes were excluded. The participants were studied with pure tone audiometry. Results: Of the 65 patients included, 36.9% had X chromosome monosomy and 63.0% had other alterations. Regarding the audiometry, 64.6% had normal thresholds and 35.3% had hearing impairment. Of these, 30.4% had hybrid hearing loss, 26.0% alteration at 6 and/or 8 kHz, 17.3% had conductive hearing loss, 13.0% sensorineural loss and 13.0% had mixed hearing loss. We observed that the mild degree was the most frequent one. There was no statistically significant association between the cytogenetic type of Turner syndrome and the presence or absence of hearing loss, or with the type and degree of hearing loss. Conclusion: The cytogenetic alteration in Turner syndrome was not associated with the audiometric profile, which showed variability regarding the type and degree of hearing loss.

Resumo Introdução: A síndrome de Turner é uma alteração frequente e genética que acomete indivíduos do sexo feminino e abrange grande variabilidade fenotípica. A literatura científica sugere uma relação entre perda auditiva e síndrome de Turner, porém ainda é um tema controverso. Objetivo: Relacionar a alteração citogenética com o perfil audiométrico de indivíduos com síndrome de Turner. Método: Estudo transversal, com amostra de conveniência, de base hospitalar. Foram incluídas pacientes com diagnóstico de síndrome de Turner e excluídas as com dificuldade para compreender a audiometria e/ou outras síndromes associadas. As participantes foram submetidas à audiometria tonal. Resultados: Das 65 pacientes incluídas, 36,9% apresentaram monossomia do cromossomo X e 63,0%, outras alterações. Com relação à audiometria, 64,6% apresentaram limiares dentro da normalidade e 35,3% alteração auditiva. Dessas, 30,4% apresentaram perda auditiva híbrida, 26,0% alteração em 6 e/ou 8 KHz; 17,3% perda auditiva condutiva, 13,0% perda neurossensorial e 13,0% perda auditiva mista. Observamos que o grau leve foi o mais frequente. Não foi observada associação estatiscamente significativa entre o tipo citogenético da síndrome de Turner e a presença ou não perda auditiva, ou com o tipo e grau de perda auditiva. Conclusão: A alteração citogenética na síndrome de Turner não teve associação com o perfil audiométrico, o qual apresentou variabilidade quanto ao tipo e grau da perda auditiva.

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