Cognitive screening in HTLV-1-infected people using a self-perceived memory score and auditory P300.

The HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is the most common neurological manifestation associated with human T-cell lymphotropic virus type-1 (HTLV-1) infection. Although cognitive impairment has been highlighted in the spectrum of HTLV-1 neurological manifestations, it may go unnoticed in those who do not spontaneously report it. We aimed at evaluating the applicability of a self-perceived memory score (SMS) and the cognitive event-related potential (P300) for early detection of cognitive impairment in HTLV-1-infected people. The SMS was measured by a 0-10 point numeric scale combined with a sad-happy face rating scale. The higher the number, the better was the SMS. The P300 was obtained through an oddball paradigm with a mental counting task. The participants were 15 (21.4%) individuals with HAM/TSP, 20 (28.6%) HTLV-1 asymptomatic carriers, and 35 (50%) seronegative controls. We found that SMS (p < 0.001) and P300 latency (p < 0.001) got progressively worse from the seronegative controls to the asymptomatic carriers and then to the HAM/TSP. The results that indicated cognitive impairment were SMS < 7.2 points and P300 latency > 369.0 ms. The HAM/TSP group showed the highest prevalence of altered P300 (80%) and SMS (87%). Interestingly, the asymptomatic group also presented significantly higher prevalence of altered SMS (60%) and P300 (35%) when compared to controls (< 10%). The frequency of cognitive impairment was 16 times higher in the HTLV-1 asymptomatic group and 69 times higher in the HAM/TSP group when compared to controls. The use of SMS in the medical consultation was a useful and easy-to-apply method to screen HTLV-1-infected subjects for everyday memory complaints.

Decreased Reemerging Auditory Brainstem Responses Under Ipsilateral Broadband Masking as a Marker of Noise-Induced Cochlear Synaptopathy.

OBJECTIVES: In mammals, a 2-hr exposure to an octave-band noise (OBN) at 100 to 108 dB SPL induces loss of synaptic ribbons between inner hair cells and auditory nerve fibers with high thresholds of response (hiT neurons), that encode high-intensity sounds. Here, we tackle the challenge of diagnosing this synaptopathy by a noninvasive functional audiological test, ultimately in humans, despite the expected absence of auditory-threshold elevation and of clear electrophysiological abnormality, hiT neuron contributions being hidden by those of more sensitive and robust neurons. DESIGN: The noise-induced synaptopathy was replicated in mice (at 94, 97, and 100 dB SPL; n = 7, 7, and 8, respectively, against 8 unexposed controls), without long-lasting auditory-threshold elevation despite a twofold decrease in ribbon-synapse number for the 100-dB OBN exposure. Auditory brainstem responses (ABRs) were collected using a simultaneous broadband noise masker just able to erase the ABR response to a 60-dB tone burst. Tone burst intensity was then increased up to 100 dB SPL for eliciting reemerging ABRs (R-ABRs), dependent on hiT neurons as more sensitive neurons are masked. RESULTS: In most ears exposed to 97-dB-SPL and all ears exposed to 100-dB-SPL OBN, contrary to controls, R-ABRs from the overexposed region have vanished, whereas standard ABR distributions widely overlap. CONCLUSIONS: R-ABRs afford an individual noninvasive marker of normal-auditory-threshold cochlear synaptopathy. A simple modification of standard ABRs would allow hidden auditory synaptopathy to be searched in a patient. ABBREVIATIONS: ABR: auditory brainstem response; dB SPL: decibel sound pressure level; DPOAE: distortion-product otoacoustic emission; hiT neuron: high-threshold neuron; IHC: inner hair cell; loT neuron: low-threshold neuron; OBN: octave-band noise; OHC: outer hair cell; PBS: phosphate buffer saline; R-ABR: reemerging ABR.

Immunological signature of the different clinical stages of the HTLV-1 infection: establishing serum biomarkers for HTLV-1-associated disease morbidity.

This study aimed at establishing the immunological signature and an algorithm for clinical management of the different clinical stages of the HTLV-1-infection based on serum biomarkers. A panel of serum biomarkers was evaluated by four sets of innovative/non-conventional data analysis approaches in samples from 87 HTLV-1 patients: asymptomatic carriers (AC), putative HTLV-1 associated myelopathy/tropical spastic paraparesis (pHAM/TSP) and HAM/TSP. The analysis of cumulative curves and molecular signatures pointed out that HAM/TSP presented a pro-inflammatory profile mediated by CXCL10/LTB-4/IL-6/TNF-α/IFN-γ, counterbalanced by IL-4/IL-10. The analysis of biomarker networks showed that AC presented a strongly intertwined pro-inflammatory/regulatory net with IL-4/IL-10 playing a central role, while HAM/TSP exhibited overall immune response toward a predominant pro-inflammatory profile. At last, the classification and regression trees proposed for clinical practice allowed for the construction of an algorithm to discriminate AC, pHAM and HAM/TSP patients with the elected biomarkers: IFN-γ, TNF-α, IL-10, IL-6, IL-4 and CysLT. These findings reveal a complex interaction among chemokine/leukotriene/cytokine in HTLV-1 infection and suggest the use of the selected but combined biomarkers for the follow-up/diagnosis of disease morbidity of HTLV-1-infected individuals.

Elderly people with human T-cell leukemia virus type 1-associated myelopathy present an early impairment in cognitive skills.

BACKGROUND: Cerebral changes occur in individuals with human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy (HAM) and seem to predominate in subcortical areas. Little is known about the cognitive decline in the elderly living with HTLV-1. OBJECTIVE: To evaluate the cognitive aging of individuals infected with HTLV-1 aged ≥ 50 years. METHODS: This is a cross-sectional study of former blood donors infected with HTLV-1 who have been followed in the cohort of the Interdisciplinary Research Group on HTLV-1 since 1997. The groups of study consisted of 79 HTLV-1 infected individuals aged ≥ 50 years, with 41 of them presenting symptomatic HAM and 38 being asymptomatic carriers, and 59 seronegative individuals (controls) aged ≥ 60 years. All were submitted to the P300 electrophysiological test and neuropsychological tests. RESULTS: Individuals with HAM presented delayed P300 latency in relation to the other groups, and this latency delay increased progressively with aging. The performance of this group in the neuropsychological tests was also the worst. The HTLV-1- asymptomatic group performance was similar to that of the control group. CONCLUSIONS: Individuals with HAM presented cognitive decline that progressed with aging and, although HTLV-1-asymptomatic carriers appear to present cognitive aging similar to that of healthy elderly people, concern about a subclinical cognitive impairment is warranted in this population.

ANTECEDENTES: Alterações cerebrais ocorrem em indivíduos com mielopatia associada ao vírus da leucemia de células T humanas tipo 1 (HTLV-1) (HAM) e parecem predominar em áreas subcorticais. Pouco se sabe sobre o declínio cognitivo em idosos vivendo com HTLV-1. OBJETIVO: Avaliar o envelhecimento cognitivo de indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos. MéTODOS: Trata-se de um estudo transversal com ex-doadores de sangue infectados pelo HTLV-1 acompanhados na coorte do Grupo Interdisciplinar de Pesquisa em HTLV-1 há 20 anos. Os grupos de estudo foram compostos por 79 indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos, sendo que 41 apresentavam HAM e 38 eram portadores assintomáticos, e 59 indivíduos soronegativos (controles) com idade ≥ 60 anos. Todos foram submetidos ao teste eletrofisiológico P300 e testes neuropsicológicos. RESULTADOS: Indivíduos com HAM apresentaram atraso na latência do P300 em relação aos demais grupos, e esse atraso de latência aumentou progressivamente com o envelhecimento. O desempenho desse grupo nos testes neuropsicológicos também foi o pior. O desempenho do grupo HTLV-1- assintomático foi semelhante ao do grupo controle. CONCLUSãO: Indivíduos com HAM apresentaram declínio cognitivo que progrediu com o envelhecimento e, embora os portadores assintomáticos do HTLV-1 pareçam apresentar envelhecimento cognitivo semelhante ao dos idosos saudáveis, justifica-se a preocupação com um comprometimento cognitivo subclínico nessa população.

Reliability of Contralateral Suppression in Evoked Distortion Product Otoacoustic Emissions.

Introduction Distortion product otoacoustic emissions (DPOAE) and their suppression may be considered useful in monitoring cochlear function and the efferent auditory pathway inhibitory effect. Nonetheless, the establishment of reliable parameters of response variations is of great importance. Objectives To verify the replicability of test and retest in the research of the inhibitory effect of the efferent pathway using contralateral suppressing stimulus during DPOAE recording for clinical applicability. Methods Cross-sectional study with 48 volunteers, aged 18 to 30 years, with normal audiometric thresholds. The procedures included were audiometric and immittance measures to overrule any conductive or sensorineural conditions and DPOAE recordings without and with contralateral suppression with a 60 dBHL white noise. Distortion product otoacoustic emissions amplitudes were analyzed and compared in both conditions with Wilcoxon test, and the Spearman correlation test was used to assess test-retest reliability. Results The comparative analysis showed differences between amplitudes in test and retest conditions only in 1,500 Hz for DPOAE measures with all other tested frequencies showing no differences, and no difference was observed in all recorded frequencies in the test and retest comparison for DPOAE suppression. The degree of correlation between test and retest of DPOAE amplitude was good at 6,000 Hz and strong (r > 0.880) at the other frequencies. For DPOAE with suppression, all frequencies presented strong correlation between test and retest: 1,500 Hz (r = 0.880), 2,000 Hz (r = 0.882), 3,000 Hz (r = 0.940), and 6,000 Hz (r = 0.957). Conclusions The study found good replicability in contralateral suppression of DPOAE with potential clinical applicability, and we recommend conducting the test from 2000Hz to higher frequencies for more reliable results.

Reading fluency during the COVID-19 pandemic: a longitudinal and cross-sectional analysis.

BACKGROUND: International studies performed during the periods of social isolation highlighted the potential loss of student's learning skills. The present study fills a gap in Brazilian research on this topic and focuses on the development of reading fluency. OBJECTIVE: To investigate the development of the reading fluency of students in the early years of elementary school during e-learning as a result of the social distancing measures put into effect due to the coronavirus disease 2019 (COVID-19) pandemic. METHODS: Students from grades 2 to 5 were recorded. The number of words read per minute and of those read correctly per minute were analyzed. Descriptive statistical analysis was performed, using analysis of variance (ANOVA) for repeated measures with Bonferroni correction in the longitudinal study, and the t-test in the cross-sectional study. RESULTS: In the cross-sectional study, 162 students participated. Only the comparison between the 2nd grade classes of 2020 and 2021 showed a statistically significant difference. In the prepandemic classes, the students had better results in reading accuracy than the students assessed during the pandemic. The longitudinal study included 75 students, who improved in fluency rate and accuracy as expected between March and December 2020. In March 2021, the results showed a drop, which may be related to school closures during the Brazilian summer vacation. CONCLUSIONS: The present research demonstrates the results of Brazilian students in terms of the development of reading fluency during the pandemic. There was an expressive development in grades 2 and 3, with stability in the following grades. The 2nd grade class of 2021 suffered a major impact due to the pandemic.

ANTECEDENTES: Estudos internacionais realizados durante os períodos de isolamento social destacaram a potencial perda das habilidades de aprendizagem dos alunos. O presente estudo preenche uma lacuna nas pesquisas brasileiras sobre este tema, e se centra no desenvolvimento da fluência de leitura. OBJETIVO: Investigar o desenvolvimento da fluência de leitura de alunos nos anos iniciais do Ensino Fundamental durante as aulas remotas ministradas em decorrência das medidas de distanciamento social postas em prática por conta da pandemia de doença do coronavírus 2019 (coronavirus disease 2019, COVID-19, em inglês). MéTODOS: Estudantes do 2o ao 5o anos foram gravados. Foram analisadoso número de palavras lidas por minuto e o de palavras lidas corretamente por minuto. Realizou-se análise estatística descritiva por meio de análise de variância (analysis of variance, ANOVA, em inglês) para medidas repetidas com correção de Bonferroni no estudo longitudinal, e teste T no estudo transversal. RESULTADOS: Ao todo, 162 estudantes participaram do estudo transversal. Apenas a comparação entre as turmas de 2o ano de 2020 e 2021 apresentou diferença estatisticamente significativa. Nas aulas pré-pandemia, as turmas apresentaram resultados melhores em termos de precisão de leitura do que as turmas avaliadas durante a pandemia. O estudo longitudinal incluiu 75 alunos; entre março e dezembro de 2020, eles melhoraram sua taxa de fluência e acurácia, conforme esperado. Em março de 2021, os resultados mostraram uma queda, o que pode estar relacionado ao fechamento das escolas durante as férias. CONCLUSõES: Esta pesquisa demonstra os resultados do desenvolvimento da leitura de uma amostra de estudantes brasileiros durante a pandemia. Houve uma evolução expressiva no 2° e 3° anos, com estabilidade nos anos seguintes. A turma de 2° ano de 2021 sofreu um grande impacto devido à pandemia.

Impact of HIV co-infection on immunological biomarker profile of HTLV-1 infected patients.

The impact of HIV co-infection on the plasma immunological biomarker profile of HTLV-1 infected patients was evaluated. The plasma levels of leukotrienes and chemokines/cytokines were quantified by ELISA and Cytometric Bead Array. A total of 138 volunteers were enrolled and divided into two subgroups ("HTLV-1(+)HIV(-)" and "HTLV-1(+)(HIV(+)"), which were categorized according to the HTLV-1-associated neurological disease (AS, pHAM and HAM). Reference controls were BD and HIV mono-infected patients. HAM(+) exhibited higher CD4+ T-cell counts as compared to HIV+ mono-infected patients and lower HTLV-1 proviral load as compared to mono-infected HAM(-) patients. AS(+) exhibited higher levels of CysLT, CXCL8/IL-8 and lower levels of CCL5/RANTES as compared to AS(-). Increased levels of IL-6 and TNF with reduced levels of CXCL10/IP10 and CCL5/RANTES were observed in co-infected pHAM(+) as compared to mono-infected pHAM(-). HAM(+) patients revealed an increase in CXCL8/IL-8, CCL2/MCP-1, CXCL-10/IP-10, TNF and a decrease in IL-2 as compared to HAM(-) subgroup.

Case Report: Cognitive Impairment without Clinical Spinal Disease May Be the First Sign of HTLV-1 Neurological Alteration.

Human t-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy (HAM) is a progressive neurological disease whose diagnosis is defined by clinical manifestations and seropositivity for HTLV-1 infection. Cognitive impairment (CI) is considered to occur after spinal impairment. A 51-year-old HTLV-1-infected man classified as an asymptomatic carrier presented difficulties in listening comprehension and executive memory. He was assessed for central auditory processing (CAP), cognition (event-related auditory evoked potential [P300]), and otoneurological functions (galvanic vestibular-evoked myogenic potential [gVEMP]). Altered responses were found in CAP, P300, and gVEMP, but the neurological examination and cognitive screening were normal. After a 2-year follow-up, we disclosed a positive Babinski sign, a mild CI, worsened P300, and gVEMP latencies, and the patient reported progressive lumbar pain and difficulty running. He was, then, reclassified as HAM. The first examination, in 2016, had already shown abnormal results in P300 and gVEMP despite the HTLV-1-asymptomatic carrier status. Therefore, tests that provide subclinical measures of neurological disease progression can be useful tools for an early diagnosis and intervention in HTLV-1 patients. Electrophysiological results had worsened as well as the clinical status and the cognitive function and the progression from asymptomatic status to an HTLV-1-associated neurological disease occurred within 2 years. Thus, HTLV-1-infected individuals with complaints of CI, hearing, or otoneurological manifestations should be submitted to neuropsychological and electrophysiological tests, allowing them to be properly cared in case of HAM progression.

Vestibular Evoked Myogenic Potential on Ocular, Cervical, and Soleus Muscles to Assess the Extent of Neurological Impairment in HTLV-1 Infection.

Introduction: Vestibular Evoked Myogenic Potential (VEMP) can be used to test central vestibular pathways from the midbrain to the lumbar spine, according to the muscle tested. Purpose: to compare the spinal cord alteration in individuals with HTLV-1-associated myelopathy (HAM) and with HTLV-1-asymptomatic infection using the VEMP recorded from different muscles. Methods: VEMP was recorded in 90 individuals of whom 30 had HAM, 30 were HTLV-1 asymptomatic carriers, and 30 negative controls. VEMP was recorded in the oculomotor muscle (oVEMP), testing the vestibulo-ocular reflex, and in the cervical muscle (cVEMP) and soleus muscle (sVEMP), testing the vestibulospinal reflex, respectively, in the cervical and in the lumbar spinal level. The type of stimulation was auditory for oVEMP and cVEMP, and galvanic for sVEMP. The compared variables were the latencies of the electrophysiological waves. Results: HTLV-1-asymptomatic group was similar to the controls regarding oVEMP (p = 0.461), but different regarding cVEMP (p < 0.001) and sVEMP (p < 0.001). HAM group has presented the worst latencies and was different from the HTLV-1-asymptomatic group in the VEMP of all the tested muscles (p < 0.001). The concomitant occurrence of VEMP alterations in the three recorded muscles of the same individual was found in 2 (6.7%) asymptomatic carriers and in 20 (66.7%) patients with HAM (p = 0.001). The analysis of VEMP alteration per group and per muscle has showed that, in HTLV-1-asymptomatic group, oVEMP was altered in 3 (10.0%) individuals, cVEMP in 10 (33.3%) and sVEMP in 13 (43.3%). In HAM group, oVEMP was altered in 23 (76.6%) individuals, cVEMP in 27 (90%), and sVEMP in 30 (100%). Conclusion: HTLV-1-neurological damage has followed an ascendant progression beginning at the lumbar spine in the stage of a clinically asymptomatic infection, whereas HAM has affected not only the spine, but also the midbrain.

Ocular vestibular evoked myogenic potential (VEMP) reveals mesencephalic HTLV-1-associated neurological disease.

PURPOSE: Vestibular Myogenic Evoked Potential (VEMP) evaluates vestibulo-ocular and vestibulo-collic reflexes involved in the function of the otolithic organs and their afferent pathways. We compared the results of cervical and ocular VEMP in HTLV-1 associated myelopathy (HAM) and HTLV-1-asymptomatic infection. PARTICIPANTS AND METHODS: This cross-sectional study included 52 HTLV-1-infected individuals (26 HAM and 26 asymptomatic carriers) and 26 seronegative controls. The groups were similar regarding age and gender. Participants underwent simultaneous ocular and cervical VEMP. The stimulus to generate VEMP was a low-frequency tone burst sound tone burst, with an intensity of 120 decibels normalized hearing level, bandpass filter from 10 to 1,500 Hertz (Hz), with 100 stimuli at 500 Hz and 50 milliseconds recording time. The latencies of the electrophysiological waves P13 and N23 for cervical VEMP and N10 and P15 waves for ocular VEMP were compared among the groups. The absence or delay of the electrophysiological waves were considered abnormal results. RESULTS: Ocular VEMP was similar among the groups for N10 (p = 0.375) and different for P15 (p≤0.001). Cervical VEMP was different for P13 (p = 0.001) and N23 (p = 0.003). About ocular VEMP, in the HTLV-1-asymptomatic group, normal waves were found in 23(88.5%) individuals; in HAM group, normal waves were found in 7(26.9%). About cervical VEMP, 18(69.2%) asymptomatic carriers presented normal waves and only 3(11.5%) patients with HAM presented normal waves. Abnormalities in both VEMPs were found in 1(3.8%) asymptomatic carrier and in 16(61.5%) patients with HAM. CONCLUSION: Neurological impairment in HAM was not restricted to the spinal cord. The mesencephalic connections, tested by ocular VEMP, have been also altered. Damage of the oculomotor system, responsible for eye stabilization during head and body movements, may explain why dizziness is such a frequent complaint in HAM.

Intrarater and interrater agreement and reliability of vestibular evoked myogenic potential triggered by galvanic vestibular stimulation (galvanic-VEMP) for HTLV-1 associated myelopathy testing.

BACKGROUND: The vestibular evoked myogenic potential triggered by galvanic vestibular stimulation (galvanic-VEMP) has been used to assess the function of the vestibulospinal motor tract and is a candidate biomarker to predict and monitor the human T-cell lymphotropic virus type 1 (HTLV-1) associated myelopathy (HAM). This study determined the agreement and reliability of this exam. METHODS: Galvanic-VEMP was performed in 96 participants, of which 24 patients presented HAM, 27 HTLV-1-asymptomatic carriers, and 45 HTLV-1-negative asymptomatic controls. Galvanic vestibular stimulation was achieved by passing a binaural and bipolar current at a 2 milliamperes (mA) intensity for 400 milliseconds (ms) between the mastoid processes. Galvanic-VEMP electromyographic wave responses of short latency (SL) and medium latency (ML) were recorded from the gastrocnemius muscle. Intrarater (test-retest) and interrater (two independent examiners) agreement and reliability were assessed by standard error of measurement (SEM), coefficient of repeatability (CR), intraclass correlation coefficient (ICC), and Kappa coefficient. RESULTS: In the total sample (n = 96), SL and ML medians were 56 ms (IQR 52-66) and 120 ms (IQR 107-130), respectively. The intrarater repeatability measures for SL and ML were, respectively: SEM of 6 and 8 ms; CR of 16 and 22 ms; ICC of 0.80 (p<0.001) and 0.91 (p<0.001); and a Kappa coefficient of 0.53 (p<0.001) and 0.82 (p<0.001). The interrater reproducibility measures for SL and ML were, respectively: SEM of 3 and 10 ms; CR of 8 and 27 ms; ICC of 0.95 (p<0.001) and 0.86 (p<0.001); and a Kappa coefficient of 0.77 (p<0.001) and 0.88 (p<0.001). CONCLUSION: Galvanic-VEMP is a reliable and reproducible method to define the integrity of the vestibulospinal tract. Longitudinal studies will clarify its validity in the clinical context, aimed at achieving an early diagnosis and the monitoring of HAM.

Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model.

Damage to cochlear outer hair cells (OHCs) usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or "hidden" synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18-30 stages when high-frequency auditory thresholds remained near-normal. Nonetheless, total loss of high-frequency distortion product otoacoustic emissions pointed to nonfunctioning basal OHCs. This "discordant profile" came with a huge low-frequency shift of masking tuning curves that plot the level of interfering sound necessary to mask the response to a probe tone, against interfering frequency. Histology revealed intense OHC hair bundle abnormalities in the basal cochlea uncharacteristically associated with OHC survival and preserved coupling with the tectorial membrane. This pattern dismisses the superficial diagnosis of "hidden" neuropathy while underpinning a disorganization of cochlear frequency mapping with optimistic high-frequency auditory thresholds perhaps because responses to high frequencies are apically shifted. The audiometric advantage of frequency transposition is offset by enhanced masking by low-frequency sounds, a finding essential for guiding rehabilitation.

Elderly people with human T-cell leukemia virus type 1-associated myelopathy present an early impairment in cognitive skills / Idosos com mielopatia associada ao vírus da leucemia de células T humanas tipo 1apresentam comprometimento precoce em habilidades cognitivas

Abstract Background Cerebral changes occur in individuals with human T-cell leukemia virus type 1 (HTLV-1 )-associated myelopathy (HAM) and seem to predominate in subcortical areas. Little is known about the cognitive decline in the elderly living with HTLV-1. Objective To evaluate the cognitive aging of individuals infected with HTLV-1 aged ≥ 50 years. Methods This is a cross-sectional study of former blood donors infected with HTLV-1 who have been followed in the cohort of the Interdisciplinary Research Group on HTLV-1 since 1997. The groups of study consisted of 79 HTLV-1 infected individuals aged ≥ 50 years, with 41 of them presenting symptomatic HAM and 38 being asymptomatic carriers, and 59 seronegative individuals (controls) aged ≥ 60 years. All were submitted to the P300 electrophysiological test and neuropsychological tests. Results Individuals with HAM presented delayed P300 latency in relation to the other groups, and this latency delay increased progressively with aging. The performance of this group in the neuropsychological tests was also the worst. The HTLV-1- asymptomatic group performance was similar to that of the control group. Conclusions Individuals with HAM presented cognitive decline that progressed with aging and, although HTLV-1-asymptomatic carriers appear to present cognitive aging similar to that of healthy elderly people, concern about a subclinical cognitive impairment is warranted in this population.

Resumo Antecedentes Alterações cerebrais ocorrem em indivíduos com mielopatia associada ao vírus da leucemia de células T humanas tipo 1 (HTLV-1) (HAM) e parecem predominar em áreas subcorticais. Pouco se sabe sobre o declínio cognitivo em idosos vivendo com HTLV-1. Objetivo Avaliar o envelhecimento cognitivo de indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos. Métodos Trata-se de um estudo transversal com ex-doadores de sangue infectados pelo HTLV-1 acompanhados na coorte do Grupo Interdisciplinar de Pesquisa em HTLV-1 há 20 anos. Os grupos de estudo foram compostos por 79 indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos, sendo que 41 apresentavam HAM e 38 eram portadores assintomáticos, e 59 indivíduos soronegativos (controles) com idade ≥ 60 anos. Todos foram submetidos ao teste eletrofisiológico P300 e testes neuropsicológicos. Resultados Indivíduos com HAM apresentaram atraso na latência do P300 em relação aos demais grupos, e esse atraso de latência aumentou progressivamente com o envelhecimento. O desempenho desse grupo nos testes neuropsicológicos também foi o pior. O desempenho do grupo HTLV-1- assintomático foi semelhante ao do grupo controle. Conclusão Indivíduos com HAM apresentaram declínio cognitivo que progrediu com o envelhecimento e, embora os portadores assintomáticos do HTLV-1 pareçam apresentar envelhecimento cognitivo semelhante ao dos idosos saudáveis, justificase a preocupação com um comprometimento cognitivo subclínico nessa população.

Galvanic vestibular stimulation to improve postural instability, voluntary attention, and quality of life in Parkinson's disease patients / Estimulação vestibular galvânica na melhora da atenção voluntária e da qualidade de vida em pacientes com doença de Parkinson e desequilíbrio corporal

ABSTRACT Galvanic vestibular stimulation (GVS) influences body balance and has proved to be useful to improve patients' mood, quality of life, and cognitive skills. This study aimed to present three cases of patients with Parkinson's disease and postural instability who had been submitted to GVS to improve their balance, by assessing the impact of this intervention on their cognition, mood, and quality of life. Patients were assessed before and after GVS sessions concerning P300 latency and scores on the 15-item Geriatric Depression Scale (GDS-15) and the 39-item quality-of-life Parkinson's Disease Questionnaire (PDQ-39). The three patients' P300 latency improved, possibly indicating improved attention. Their PDQ-39 score also improved, possibly indicating a positive impact on their quality of life. Their GDS-15 score did not change before and after the intervention. None of the patients had any intervention side effects. This three-case experimental pilot study has shown that GVS is a safe method, possibly useful to improve attention and, therefore, the quality of life of patients presented with Parkinson's disease.

RESUMO A Estimulação Vestibular Galvânica (EVG) atua no equilíbrio corporal e tem se mostrado útil na melhora do humor, da qualidade de vida e de habilidades cognitivas. O objetivo deste estudo foi apresentar três casos de pacientes com doença de Parkinson e instabilidade postural que foram submetidos à EVG para melhorar o equilíbrio e avaliar o impacto dessa intervenção na cognição, no humor e na qualidade de vida. Os pacientes foram avaliados antes e após as sessões de EVG quanto a latência do potencial evocado P300, pontuação na escala de depressão geriátrica de 15 itens (EDG-15) e pelo questionário de qualidade de vida na doença de Parkinson de 39 itens (PDQ-39). Os três pacientes apresentaram melhora na latência do P300, indicando possível melhora na atenção. Apresentaram melhora na pontuação do PDQ-39, indicando possível impacto positivo na qualidade de vida. A pontuação na EDG-15 não modificou antes e após a intervenção. Nenhum paciente apresentou efeitos colaterais decorrentes da intervenção. Com base neste estudo piloto experimental de três casos, a EVG mostrou-se um método seguro e possivelmente útil para melhorar a atenção e, consequentemente, a qualidade de vida de pacientes com doença de Parkinson.

Vestibular-evoked myogenic potential triggered by galvanic vestibular stimulation may reveal subclinical alterations in human T-cell lymphotropic virus type 1-associated myelopathy.

BACKGROUND: Vestibular-evoked myogenic potential triggered by galvanic vestibular stimulation (galvanic-VEMP) evaluates the motor spinal cord and identifies subclinical myelopathies. We used galvanic-VEMP to compare spinal cord function in individuals infected with human T-cell lymphotropic virus type 1 (HTLV-1) from asymptomatic status to HTLV-1-associated myelopathy (HAM). METHODOLOGY/PRINCIPAL FINDINGS: This cross-sectional study with 122 individuals included 26 HTLV-1-asymptomatic carriers, 26 individuals with possible HAM, 25 individuals with HAM, and 45 HTLV-1-seronegative individuals (controls). The groups were similar regarding gender, age, and height. Galvanic stimuli (duration: 400 ms; intensity: 2 mA) were applied bilaterally to the mastoid processes and VEMP was recorded from the gastrocnemius muscle. The electromyographic parameters investigated were the latency and amplitude of the short-latency (SL) and medium-latency (ML) responses. While SL and ML amplitudes were similar between groups, SL and ML latencies were delayed in the HTLV-1 groups compared to the control group (p<0.001). Using neurological examination as the gold standard, ROC curve showed an area under the curve of 0.83 (p<0.001) for SL and 0.86 (p<0.001) for ML to detect spinal cord injury. Sensibility and specificity were, respectively, 76% and 86% for SL and 79% and 85% for ML. Galvanic-VEMP disclosed alterations that were progressive in HTLV-1-neurological disease, ranging from SL delayed latency in HTLV-1-asymptomatic carriers, SL and ML delayed latency in possible HAM group, to absence of VEMP response in HAM group. CONCLUSIONS/SIGNIFICANCE: The worse the galvanic-VEMP response, the more severe the myelopathy. Galvanic-VEMP alteration followed a pattern of alteration and may be a prognostic marker of progression from HTLV-1-asymptomatic carrier to HAM.

Perception of hearing loss: use of the subjective faces scale to screen hearing among the elderly. / Percepção da perda auditiva: utilização da escala subjetiva de faces para triagem auditiva em idosos.

Presbycusis is a disorder present among the elderly. However, it is under-diagnosed, making it important to develop and enhance simple screening tools. OBJECTIVE: The subjective faces scale has been proposed as a method to assess auditory self-perception among the elderly, and its correlation with audiological tests. METHODS: We looked at elderly patients referred to the audiology service of a reference center for the care of the elderly in a public university hospital between February and November 2013. Patients were submitted to meatoscopy, tonal and vocal audiometry and the whisper test. They also answered the subjective faces scale. A total of 164 elderly individuals participated, and the average age was 77. RESULTS: We found a good correlation between the subjective faces scale and audiometry thresholds (r = 0.66). Our results show that the faces and hearing loss correlate, with face 1 corresponding to normal hearing, face 2 to mild hearing loss, and face 3 to Grade I moderate hearing loss. When evaluating the psychometric qualities of the subjective faces scale, we found that faces 2 or 3 have good sensitivity and specificity, with the area under the ROC curve being 0.81. CONCLUSION: The subjective faces scale seems to be a good, low-cost and easy to use supplementary tool for auditory screening in geriatric services.

A presbiacusia é uma alteração prevalente na população idosa, porém subdiagnosticada, desta forma, é importante aprimorar instrumentos de triagem simples. A escala subjetiva de faces foi proposta como forma de avaliar a autopercepção auditiva do idoso e sua correlação com exames audiológicos. Foram avaliados todos os pacientes encaminhados para o serviço de audiologia de um centro de referência de atenção à saúde do idoso no período de fevereiro a novembro de 2013. Os pacientes foram examinados por meatoscopia, audiometria tonal e vocal e responderam a escala subjetiva de faces e o teste do sussurro. Participaram 164 idosos com média de idade de 77 anos. Encontrou-se boa correlação entre a escala subjetiva de faces e o limiar audiométrico (r = 0,66). Houve correspondência entre as faces e o grau da perda auditiva, sendo a face 1 correspondente a audição normal, face 2 a perda auditiva leve e face 3 a perda auditiva moderada grau I. Ao avaliar as qualidades psicométricas da escala subjetiva de faces, verificou-se que as faces 2 e 3 apresentam bons índices de sensibilidade e especificidade, com área sob a curva ROC de 0,81. A escala subjetiva de faces parece ser um bom instrumento complementar de triagem auditiva em serviços gerontológicos, de fácil aplicação e baixo custo.

Use of hearing devices and fluency in Brazilian Sign Language and oral language in deaf students / Uso de dispositivos auxiliares da audição e fluência na Libras e na língua oral de estudantes surdos

ABSTRACT Purpose: to assess the relationship between deaf students' fluency in Brazilian Sign Language (BSL) and oral language and hearing aid use. Method: the sample comprised 112 professional BSL translators, interpreters, and instructors. They answered a questionnaire on hearing characteristics and BSL and oral language fluency of students accompanied by them, who attended municipal schools in a Brazilian city. Association analysis between oral language fluency, BSL fluency, the degree of hearing loss, and device use was made with the chi-square test or Fisher's Exact test. The significance level was set at p<0.05. Results: the professionals' responses referred to 88% (n = 126) of all deaf students enrolled in municipal schools. The students' mean age was 13 years; 72 (57%) were males, 98 (78%) had severe or profound hearing loss, 57 (45%) used electronic hearing devices, 83 (66%) were fluent only in BSL, 12 (10%) were fluent only in oral language, and 18 (14%) were fluent in both BSL and oral language. Hearing device use was statistically associated with oral fluency (p < 0.001). Of all students fluent in oral language (n = 30), 18 (60%) were also fluent in BSL (p < 0.001). Conclusion: BSL was the communication modality most used by students, including those who also used oral language and electronic hearing devices. This may indicate a change in the social perception of deaf people, their language, and their culture.

RESUMO Objetivo: avaliar a relação entre fluência na Libras, fluência na língua oral e o uso de dispositivos auxiliares para audição de estudantes surdos. Métodos: participaram 112 profissionais tradutores, intérpretes ou instrutores de Libras que informaram as características auditivas, fluência na Libras e língua oral dos estudantes acompanhados por eles, matriculados na rede municipal de uma cidade brasileira. A análise da associação entre fluência na Libras, fluência na língua oral, grau da perda auditiva e uso de dispositivo foi realizada por meio do teste qui-quadrado ou teste exato de Fisher, consideraram-se significantes valores de p<0,05. Resultados: as respostas dos profissionais referiram-se a 88% (n=126) do total de estudantes surdos matriculados na rede municipal. A média de idade dos estudantes foi de 13 anos, sendo 72 (57%) meninos, 98 (78%) com perda auditiva severa ou profunda, 57 (45%) usuários de dispositivos eletrônicos auxiliares para audição, 83 (66%) fluentes somente na Libras, 12 (10%) fluentes apenas na língua oral e 18 (14%) fluentes na Libras e língua oral. Estudantes que utilizavam dispositivo auxiliar para audição apresentaram melhor fluência oral (p<0,001). Dos estudantes que apresentavam fluência oral (n=30), 18 (60%) eram fluentes em Libras (p<0,001). Conclusão: a Libras foi a modalidade comunicativa utilizada majoritariamente pelos estudantes surdos, mesmo por aqueles que utilizavam também a língua oral e dispositivos eletrônicos, o que pode indicar mudança de percepção social em relação ao surdo, à sua língua e cultura.

Screening of hearing in elderly people: assessment of accuracy and reproducibility of the whispered voice test. / Triagem auditiva em idosos: avaliação da acurácia e reprodutibilidade do teste do sussurro.

Given the high prevalence of presbycusis and its detrimental effect on quality of life, screening tests can be useful tools for detecting hearing loss in primary care settings. This study therefore aimed to determine the accuracy and reproducibility of the whispered voice test as a screening method for detecting hearing impairment in older people. This cross-sectional study was carried out with 210 older adults aged between 60 and 97 years who underwent the whispered voice test employing ten different phrases and using audiometry as a reference test. Sensitivity, specificity and positive and negative predictive values were calculated and accuracy was measured by calculating the area under the ROC curve. The test was repeated on 20% of the ears by a second examiner to assess inter-examiner reproducibility (IER). The words and phrases that showed the highest area under the curve (AUC) and IER values were: "shoe" (AUC = 0.918; IER = 0.877), "window" (AUC = 0.917; IER = 0.869), "it looks like it's going to rain" (AUC = 0.911; IER = 0.810), and "the bus is late" (AUC = 0.900; IER = 0.810), demonstrating that the whispered voice test is a useful screening tool for detecting hearing loss among older people. It is proposed that these words and phrases should be incorporated into the whispered voice test protocol.

Devido à alta prevalência da presbiacusia e as suas consequências, métodos de triagem auditiva são necessários no âmbito da atenção primária. Diante disso, o objetivo do estudo foi verificar a reprodutibilidade e acurácia do teste do sussurro como metodologia de triagem auditiva em idosos. Trata-se de estudo transversal com medidas de acurácia que incluiu 210 idosos, entre 60 e 97 anos, submetidos ao teste do sussurro com dez expressões diferentes e ao exame de referência audiometria tonal limiar. Calculou-se a sensibilidade, a especificidade, o valor preditivo positivo, o valor preditivo negativo e a acurácia do teste, avaliada por meio da área sob a curva Receiver Operating Characteristic (ROC). O teste foi repetido em 20% das orelhas por um segundo examinador com a finalidade de avaliar a reprodutibilidade interexaminador (RIE). As expressões que apresentaram melhor área sob a curva ROC (AUC) e RIE foram: "sapato" (AUC = 0,918; RIE = 0,877), "janela" (AUC = 0,917; RIE = 0,869), "parece que vai chover" (AUC = 0,911; RIE = 0,810) e o "ônibus está atrasado" (AUC = 0,900; RIE = 0,810). Estas são, pois, as expressões propostas para fazerem parte do protocolo do teste do sussurro que mostrou-se como uma ferramenta útil para triagem auditiva em idosos.

Reliability of Contralateral Suppression in Evoked Distortion Product Otoacoustic Emissions

Abstract Introduction Distortion product otoacoustic emissions (DPOAE) and their suppression may be considered useful in monitoring cochlear function and the efferent auditory pathway inhibitory effect. Nonetheless, the establishment of reliable parameters of response variations is of great importance. Objectives To verify the replicability of test and retest in the research of the inhibitory effect of the efferent pathway using contralateral suppressing stimulus during DPOAE recording for clinical applicability. Methods Cross-sectional study with 48 volunteers, aged 18 to 30 years, with normal audiometric thresholds. The procedures included were audiometric and immittance measures to overrule any conductive or sensorineural conditions and DPOAE recordings without and with contralateral suppression with a 60 dBHL white noise. Distortion product otoacoustic emissions amplitudes were analyzed and compared in both conditions with Wilcoxon test, and the Spearman correlation test was used to assess test-retest reliability. Results The comparative analysis showed differences between amplitudes in test and retest conditions only in 1,500 Hz for DPOAE measures with all other tested frequencies showing no differences, and no difference was observed in all recorded frequencies in the test and retest comparison for DPOAE suppression. The degree of correlation between test and retest of DPOAE amplitude was good at 6,000 Hz and strong (r > 0.880) at the other frequencies. For DPOAE with suppression, all frequencies presented strong correlation between test and retest: 1,500 Hz (r = 0.880), 2,000 Hz (r = 0.882), 3,000 Hz (r = 0.940), and 6,000 Hz (r = 0.957). Conclusions The study found good replicability in contralateral suppression of DPOAE with potential clinical applicability, and we recommend conducting the test from 2000Hz to higher frequencies for more reliable results.

Validação da Escala de Autopercepção de Habilidades do Processamento Auditivo Central (EAPAC) para adultos / Validation of the Central Auditory Processing Skill Self-Perception Scale (CAPSSPS) for adults

RESUMO Objetivo validar um questionário autorreferido para avaliação do processamento auditivo central para adultos. Métodos o instrumento foi testado e validado com 123 estudantes universitários de 18 a 59 anos, sem alteração auditiva e sem histórico de tratamento para transtorno do processamento auditivo central. Os participantes realizaram os testes Gaps in Noise e Fala com Ruído. Por meio da análise estatística, foi definido o ponto de corte para alterações, a sensibilidade e a especificidade. Resultados o instrumento foi elaborado contendo 21 questões relacionadas ao histórico de saúde, aos hábitos de vida, às queixas auditivas e de aprendizagem. Após a análise fatorial, as questões relacionadas ao hábito de vida e condições de saúde foram retiradas por apresentarem carga fatorial baixa. Assim, a versão final da escala foi composta por 13 questões. Os constructos de primeira ordem e o indicador diagnóstico apresentaram níveis de confiabilidade exigidos. Foram definidos os pontos de corte 6 e 5 que indicassem alteração nos testes Gaps in Noise e Fala com Ruído branco, respectivamente. Conclusão a escala apresentou resultados válidos, confiáveis e consistentes e foi capaz de realizar inferências sobre o processamento auditivo.

ABSTRACT Purpose To validate a self-report questionnaire to assess the central auditory processing in adults. Methods The instrument was tested and validated with 123 university students aged 18 to 59 years, without hearing changes or history of treatment for central auditory processing disorder. The participants were submitted to the Gaps-in-Noise and speech-in-white-noise tests. The cutoff scores for changes, sensitivity, and specificity were defined with statistical analysis. Results The instrument was developed with 21 questions related to health history, life habits, and hearing and learning complaints. After factor analysis, the questions related to life habits and health conditions were removed because they had a low factor loading. Thus, the final version of the scale comprised 13 questions. The first-order constructs and the diagnostic indicator achieved the required levels of reliability. The cutoff scores to indicate abnormal results in the Gaps-in- Noise and speech-in-white-noise tests were defined respectively as 6 and 5. Conclusion The scale obtained valid, reliable, and consistent results and enabled professionals to make inferences about auditory processing.