Benign rolandic epilepsy of childhood and central auditory processing disorder: A noncasual neurophysiological association.

PURPOSE: The purpose of this study was to demonstrate the association between benign rolandic epilepsy of childhood (BREC) and central auditory processing disorders (CAPDs) and to test the hypothesis that an early onset of BREC could be associated with more cases of CAPD. METHOD: This study has a retrospective cross-sectional design conducted from January 2006 to January 2016 including 93 patients with BREC and without intellectual disability, dyslexia, and attention-deficit hyperactivity disorders. All patients were evaluated for central auditory processing (CAP), and its presence or absence was compared with age of seizure onset. RESULTS: In all patients, audiometric test results were normal, and in 43 cases (46.2%), CAPD was detected. There was no significant statistical difference in the mean age of seizure onset. CONCLUSION: A significant proportion of children with BREC will have a diagnosis of CAPD at school age.

Phenotypic and genotypic aspects of Townes-Brock syndrome: case report of patient in southern Brazil with a new SALL1 hotspot region nonsense mutation.

BACKGROUND: Townes-Brocks syndrome (TBS) is a rare autosomal dominant condition characterized by renal, anal, limb, and auditory abnormalities. TBS diagnosis can be challenging in settings where genetic analysis is not readily available. TBS traits overlap with those of Goldenhar and VACTERL syndromes. CASE PRESENTATION: Here, we present the case of a 5-year-old Brazilian boy born with an anorectal abnormality, limb and external ears malformations, genitourinary anomalies, and a congenital heart defect. Genetic analysis revealed a SALL1 nonsense mutation. The case is discussed in the context of the current literature. CONCLUSIONS: Because of the variability in TBS clinical presentation, genetic analysis is key to the differential diagnosis of TBS relative to phenotypically similar syndromes.

The Importance of Labyrinthine Examination in the Prognosis and Therapy for Balance in Spinocerebellar Ataxia.

INTRODUCTION: Spinocerebellar ataxias (SCAs) are a heterogeneous group of neurodegenerative diseases that are characterized by the presence of progressive cerebellar ataxia. OBJECTIVE: Identify vestibular disorders and demonstrate the importance of labyrinthine examination in the prognosis and therapy for balance in patients with SCAs. MATERIALS AND METHODS: The study had a retrospective cross-sectional design and evaluated 57 patients, mean age of 41.6 years and standard deviation of 13 years. Patients underwent the following procedures: anamnesis, ENT examination and vestibular exam using electronystagmography (ENG). RESULTS: The most frequent complaints were gait imbalance (71.9%), dysarthria (49.1%), dizziness (43.8%) and dysphagia (36.8%). 84.2% of the tests showed alterations. The most common tests with alterations were the caloric test (78.9%), slow saccades (61.4%) and the rotating chair test (49.1%). CONCLUSION: The clinical history of the patient and oculomotor alterations in the labyrinthine examination provide sufficient information for the proper use of virtual rehabilitation protocols in the treatment of imbalance, making it the most effective therapy method. It was evident that changes in ENG are related to the severity of the SCA or the clinical stage of the disease. The labyrinthine examination proved to be an important concomitant tool to clinical and genetic study.

Risk of autism spectrum disorder in children with infantile epileptic spasms syndrome: a retrospective study in a single center in Brazil.

OBJECTIVE: This study aimed to investigate the prevalence of autism spectrum disorder and its possible correlations with clinical characteristics in patients with infantile epileptic spasms syndrome in a single center in Brazil. METHODS: This retrospective cross-sectional study examined 53 children with the diagnosis of infantile epileptic spasms syndrome prior to an autism spectrum disorder assessment. Participants were divided into two groups based on the presence or absence of autism spectrum disorder. Available variables (sex, medications, median age at onset of infantile epileptic spasms syndrome, and presence of comorbidities) were compared using Mann-Whitney U or chi-square tests. RESULTS: Among the included patients, 12 (23 %) were diagnosed with autism spectrum disorder, corresponding to a relative risk of 0.29 (95 % confidence interval 0.174-0.492). The age at the first seizure ranged from 3 to 15 months, with a mean of 6.65 months. This age significantly differed between participants with autism spectrum disorder (10.58 months) and those without (5.43 months), p<0.001. CONCLUSION: Children with infantile epileptic spasms syndrome have a higher risk of being diagnosed with autism spectrum disorder. Later age of onset and period of spasm occurrence might be predisposing risk factors.

Vestibular dysfunction in adolescents and young adults after kidney transplant.

INTRODUCTION: Chronic kidney disease is a slowly progressive disease that causes irreversible loss of renal function and is considered a public health problem worldwide. OBJECTIVE: To evaluate the vestibular behavior in patients with chronic kidney disease undergoing renal transplantation. METHODS: A retrospective cross-sectional study was performed. Thirty patients were evaluated, 33.3% female and 66.7% male (mean age 16.9 (± 3.6) years old). Patients underwent the following procedures: anamnesis, ENT (ear, nose, and throat) evaluation and vestibular evaluation. RESULTS: The patients reported dizziness when they were on dialysis. 50% the patients showed an abnormality in the vestibular test, which occurred in the caloric test. The abnormality was more prevalent in the peripheral vestibular system and there was a predominance of deficit peripheral vestibular disorders. CONCLUSION: The dizziness was the most significant symptom for the vestibular test in correlation with neurotological symptoms. Alteration in the vestibular exam occurred in the caloric test, there was a prevalence of alterations for the peripheral vestibular system with a predominance of deficit vestibular dysfunction. We emphasize the need to show professionals involved in patients with chronic kidney disease, those undergoing renal transplant and dialitic treatment the importance of prevention and early identification of otoneurological involvement.

Effects of sleep deprivation on central auditory processing.

BACKGROUND: Sleep deprivation is extremely common in contemporary society, and is considered to be a frequent cause of behavioral disorders, mood, alertness, and cognitive performance. Although the impacts of sleep deprivation have been studied extensively in various experimental paradigms, very few studies have addressed the impact of sleep deprivation on central auditory processing (CAP). Therefore, we examined the impact of sleep deprivation on CAP, for which there is sparse information. In the present study, thirty healthy adult volunteers (17 females and 13 males, aged 30.75±7.14 years) were subjected to a pure tone audiometry test, a speech recognition threshold test, a speech recognition task, the Staggered Spondaic Word Test (SSWT), and the Random Gap Detection Test (RGDT). Baseline (BSL) performance was compared to performance after 24 hours of being sleep deprived (24hSD) using the Student's t test. RESULTS: Mean RGDT score was elevated in the 24hSD condition (8.0±2.9 ms) relative to the BSL condition for the whole cohort (6.4±2.8 ms; p=0.0005), for males (p=0.0066), and for females (p=0.0208). Sleep deprivation reduced SSWT scores for the whole cohort in both ears [(right: BSL, 98.4%±1.8% vs. SD, 94.2%±6.3%. p=0.0005)(left: BSL, 96.7%±3.1% vs. SD, 92.1%±6.1%, p<0.0001)]. These effects were evident within both gender subgroups [(right: males, p=0.0080; females, p=0.0143)(left: males, p=0.0076; females: p=0.0010). CONCLUSION: Sleep deprivation impairs RGDT and SSWT performance. These findings confirm that sleep deprivation has central effects that may impair performance in other areas of life.

Otoneurological findings in spinocerebellar ataxia.

OBJECTIVE: Describe findings observed in ENG of patients with spinocerebellar ataxias. METHOD: Forty-three patients were studied, and the following procedures were carried out: anamnesis, otorhinolaryngological and vestibular evaluation (ENG). RESULTS: The clinical findings in the entire group of patients were: gait disturbances (83.72%), speech difficulties (48.83%), dizziness (41.86%) and dysphagia (39.53%). Vestibular examination disclosed abnormal caloric exam (83.71%) and saccadic movements (69.76%) with the highest rates of abnormality. The overall presence of alterations in vestibular tests was (90.70%), and the most frequent finding was central vestibular disorder in (74.42%) of patients. CONCLUSION: The study showed that alterations in ENG are related to the severity of SCAs or clinical stage of the disease. We emphasize the importance of studying the vestibular system concomitantly to clinical and genetic follow up.

The Use of Exergames in the Neurorehabilitation of People with Parkinson Disease: The Impact on Daily Life.

Introduction Parkinson disease (PD) is a progressive degeneration characterized by motor disorders, such as tremor, bradykinesia, stiffness and postural instability. Objective To evaluate the independence, confidence and balance in the development of daily activities in patients with PD before and after rehabilitation. Methods A descriptive, retrospective cross-sectional study was carried out with 16 patients (mean 57.6 ± 18.7 years), submitted to anamnesis, otolaryngological evaluation and vestibular assessment. The Vestibular Disorders Activities of Daily Living (VADL) and the Activities-Specific Balance Confidence (ABC) scales were applied before and after rehabilitation with virtual reality. Results a) The instrumental subscale of the questionnaire showed statistically significant result ( p = 0.022; 95% CI 1.21; 2.21) between the first and second assessments; b) The correlation between the questionnaires showed statistically significant result in the ambulation subscale ( p = 0.011; 95% CI -0.85; -0.17) first and ( p = 0.002, 95% CI -0.88; -0.31) second assessments, and the functional subscale was only verified in the second assessment ( p = 0.011, 95% CI -0.85; -0.17); and c) The patients presented clinical improvement in the final assessment after rehabilitation with significant result for the tightrope walk ( p = 0.034, 95% CI -12.5; -0.3) and ski slalom games ( p = 0.005, 95% CI -34.8; -6.6). Conclusions Our results showed that the VADL and ABC questionnaires, applied before and after rehabilitation, were important tools to measure the independence, confidence and balance while developing daily activities. The VADL and ABC questionnaires may effectively contribute to quantify the effect of the applied therapeutics and, consequently, its impact on the quality of life of patients with PD.

Pure-Tone Hearing Thresholds and Brainstem Auditory Evoked Potentials in Sporadic Ataxia.

Introduction Spinocerebellar ataxia (SCA) is part of a genetic and clinical heterogeneous group of neurodegenerative diseases characterized by progressive cerebellar ataxia. Objective To describe the results of audiological and electrophysiological hearing evaluations in patients with sporadic ataxia (SA). Methods A retrospective cross-sectional study was carried out with 11 patients submitted to the following procedures: anamnesis, otorhinolaryngological evaluation, tonal and vocal audiometry, acoustic immittance and brainstem auditory evoked potential (BAEP) tests. Results The patients presented with a prevalence of gait imbalance, of dysarthria, and of dysphagia; in the audiometric and BAEPs, four patients presented with alterations; in the acoustic immittance test, five patients presented with alterations, predominantly bilateral. Conclusion The most evident alterations in the audiological evaluation were the prevalence of the descending audiometric configuration between the frequencies of 2 and 4 kHz and the absence of the acoustic reflex between the frequencies of 3 and 4 kHz bilaterally. In the electrophysiological evaluation, the patients presented changes with a prevalence of increased I, III and V wave latencies and the interval in the interpeak I-III, I-V and III-V. In the present study, it was observed that auditory complaints did not have a significant prevalence in this type of ataxia, which does not occur in some types of autosomal recessive and dominant ataxia.

Epileptic seizures and EEG features in juvenile systemic lupus erythematosus.

INTRODUCTION: Juvenile systemic lupus erythematosus is more incident in female affecting different systems including the central nervous system. The aim of this study was to check the incidence of seizures and electroencephalographic features in these patients. METHOD: It was analyzed all patients with juvenile systemic lupus erythematosus referred to the Pequeno Príncipe Hospital in Curitiba, PR, Brazil, in the year of 2007. The patients were submitted to EEG and subdivided into two groups according to the presence or absence of epileptic seizures. Mann-Whitney statistical test was used. RESULTS: Forty-nine cases were included, there were 73.45% female, with an age between 3 and 28 years (micro=17.00 years; s=5.01 years). Seizures (13/26.50%) were the most frequent manifestation followed by headache (13/26.50%) and ischemic stroke (6/12.25%). Cerebral vasculites were the most frequent alteration in neuroimage. The abnormalities of EEG were characterized by asymmetry of the electric cerebral activity, diffuse disorganized background activity, focal epileptiform discharges in the right central-temporal region, generalized paroxysmal of 3 Hz spike-waves, and bursts of theta-delta slowness activity in the right parietal-occiptal region. The statistic analysis showed no significantly difference between age of onset of symptoms and the risk of seizures (p 0.675) as well as between time of the disease and the risk of seizures (p 0.436). CONCLUSION: Neurologic manifestations, in special epileptic seizures, are frequent in systemic lupus erythematosus. Age of onset of symptoms and the time of disease did not increase the risk of epileptic seizures in this disease.

De Morsier syndrome associated with periventricular nodular heterotopia: case report.

INTRODUCTION: Septo-optic dysplasia (De Morsier syndrome) is defined as the association between optic nerve hypoplasia, midline central nervous system malformations and pituitary dysfunction. CASE REPORT: Third child born to nonconsanguineous parents, female, adequate pre-natal medical care, cesarean term delivery due to breech presentation, Apgar score 3 at the first minute and 8 at 5 minutes, symptomatic hypoglycemia at 18 hours. Neurological follow-up identified a delay in acquisition of motor and language developmental milestones. Epileptic generalized seizures began at 12 months and were controlled with phenobarbital. EEG was normal. MRI revealed agenesis of the pituitary stalk, hypoplasia of the optic chiasm and periventricular nodular heterotopia. Ophthalmologic evaluation showed bilateral optic disk hypoplasia. Endocrine function laboratory tests revealed primary hypothyroidism and hyperprolactinemia. CONCLUSION: The relevance of this case report relies on its uniqueness, since periventricular heterotopia had not been described in association with septo-optic dysplasia until 2006.

Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system.

OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%): seizures (17 / 36.2%), intractable headache (7 / 14.9%), mood disorders (5 / 10.6%), cerebrovascular disease (4 / 8.5%), acute confusional state (3 / 6.4%), aseptic meningitis (3 / 6.4%), psychosis (3 / 6.4%), chorea (3 / 6.4%), Guillain-Barré syndrome (2 / 4.3%) and cranial neuropathy (1 / 2.1%). Morbidity indexes (SLEDAI and SLICC) were higher among patients with neuropsychiatric manifestations (p<0.05). CONCLUSION: Neuropsychiatric syndromes are frequent, and add significant morbidity to juvenile systemic lupus erythematosus.

PERINATAL FEATURES OF CHILDREN WITH AUTISM SPECTRUM DISORDER. / CARACTERÍSTICAS PERINATAIS DE CRIANÇAS COM TRANSTORNO DO ESPECTRO AUTISTA.

OBJECTIVE: To analyze perinatal features of children with autism spectrum disorder (ASD). METHODS: Retrospective review of the medical records of 75 children with ASD, between January 2008 and January 2015. Inclusion criteria were diagnosis of ASD based on DSM-5 criteria, and the informed consent form signed by the person who is legally responsible. The exclusion criterion was missing on the medical record. The variables analyzed were maternal age, prematurity (gestational age under 37 weeks), low birth weight (<2,500 g), and perinatal asphyxia (5th minute Apgar score <7). Data were analyzed using the difference between proportions test, being significant p<0.05. RESULTS: Seventy-five patients were included. Maternal age ranged from 21.4 to 38.6 years (29.8±4.1 years). Premature birth occurred in 14 (18.7%) patients, perinatal asphyxia in 6 (8.0%), and low birth weight in 32 (42.6%) patients. The prevalence of prematurity, low birth weight, and perinatal asphyxia among the children in our study was higher than the general prevalence of these conditions among all live births in our country, region, and state, which are, respectively, 11.5, 2.3, and 8.5% in Brazil; 11.0, 2.2, and 8.5% in Southern Brazil; and 10.5, 2.0, and 8.4% in the state of Paraná. CONCLUSIONS: Our findings show a higher prevalence of prematurity, low birth weight, and perinatal asphyxia among children with ASD. Some limitations are the retrospective study design, and the small sample size. Large prospective studies are needed to clarify the possible association between perinatal complications and ASD.

OBJETIVO: Analisar características perinatais de crianças com transtorno do espectro autista (TEA). MÉTODOS: Revisão retrospectiva dos prontuários medicos de 75 crianças com TEA, entre janeiro de 2008 e janeiro de 2015. Os critérios de inclusão foram o diagnóstico de TEA baseado no DSM-5 e o termo de consentimento assinado pelo responsável legal. O critério de exclusão foi ausência de todos os dados no prontuário médico. As variáveis analisadas foram: idade materna, prematuridade (idade gestacional menor que 37 semanas), baixo peso ao nascer (<2.500 g) e asfixia perinatal (Apgar menor que 7 no quinto minuto). Os dados foram avaliados por meio do teste de diferença entre as proporções (nível de significância de p<0,05). RESULTADOS: Setenta e cinco pacientes foram incluídos no estudo. A idade materna variou de 21,4 a 38,6 anos (29,8±4,1 anos). O parto prematuro ocorreu em 14 (18,7%) pacientes, asfixia perinatal em 6 (8,0%) e baixo peso ao nascer em 32 (42,6%). As prevalências de prematuridade, asfixia perinatal e baixo peso ao nascer entre as crianças com TEA neste estudo foram maiores do que as prevalências gerais dessas condições entre todos os nascidos vivos em nosso país, região e estado, as quais são, respectivamente, 11,5, 2,3 e 8,5% no Brasil, 11,0; 2,2 e 8,5% na região Sul e 10,5, 2,0 e 8,4% no estado do Paraná. CONCLUSÕES: Nossos achados mostraram maior prevalência de prematuridade, baixo peso ao nascer e asfixia perinatal em crianças com TEA. Algumas limitações são o desenho retrospectivo do estudo e a amostra de pequeno tamanho. Grandes estudos prospectivos são necessários para esclarecer a possível associação entre intercorrências perinatais e TEA.

Lissencephaly, abnormal genitalia and refractory epilepsy: case report of XLAG syndrome.

INTRODUCTION: X-linked lissencephaly with ambiguous genitalia (XLAG) is a recently described genetic disorder caused by mutation in the aristaless-related homeobox (ARX) gene (Xp22.13). Patients present with lissencephaly, agenesis of the corpus callosum, refractory epilepsy of neonatal onset, acquired microcephaly and male genotype with ambiguous genitalia. CASE REPORT: Second child born to healthy nonconsanguineous parents, presented with seizures within the first hour of life that remained refractory to phenobarbital, phenytoin and midazolam. Examination identified microcephaly, axial hypotonia, pyramidal signs and ambiguous genitalia. EEG showed disorganized background activity and seizures starting at the right midtemporal, central and occipital regions. MRI showed diffuse pachygyria, moderate thickening of the cortex, enlarged ventricles, agenesis of the corpus callosum and septum pellucidum. Karyotype showed a 46,XY genotype. Additional findings were hypercalciuria, vesicoureteral reflux, patent ductus arteriosus and chronic diarrhea.

The Use of Exergames in the Neurorehabilitation of People with Parkinson Disease: The Impact on Daily Life

Abstract Introduction Parkinson disease (PD) is a progressive degeneration characterized by motor disorders, such as tremor, bradykinesia, stiffness and postural instability. Objective To evaluate the independence, confidence and balance in the development of daily activities in patients with PD before and after rehabilitation. Methods A descriptive, retrospective cross-sectional study was carried out with 16 patients (mean 57.6 ± 18.7 years), submitted to anamnesis, otolaryngological evaluation and vestibular assessment. The Vestibular Disorders Activities of Daily Living (VADL) and the Activities-Specific Balance Confidence (ABC) scales were applied before and after rehabilitation with virtual reality. Results a) The instrumental subscale of the questionnaire showed statistically significant result (p = 0.022; 95 % CI 1.21; 2.21) between the first and second assessments; b) The correlation between the questionnaires showed statistically significant result in the ambulation subscale (p = 0.011; 95 % CI -0.85; -0.17) first and (p = 0.002, 95 % CI -0.88; -0.31) second assessments, and the functional subscale was only verified in the second assessment (p = 0.011, 95 % CI -0.85; -0.17); and c) The patients presented clinical improvement in the final assessment after rehabilitation with significant result for the tightrope walk (p = 0.034, 95 % CI -12.5; -0.3) and ski slalom games (p = 0.005, 95 % CI -34.8; -6.6). Conclusions Our results showed that the VADL and ABC questionnaires, applied before and after rehabilitation, were important tools to measure the independence, confidence and balance while developing daily activities. The VADL and ABC questionnaires may effectively contribute to quantify the effect of the applied therapeutics and, consequently, its impact on the quality of life of patients with PD.

Pure-Tone Hearing Thresholds and Brainstem Auditory Evoked Potentials in Sporadic Ataxia

Abstract Introduction Spinocerebellar ataxia (SCA) is part of a genetic and clinical heteroge- neous group of neurodegenerative diseases characterized by progressive cerebellar ataxia. Objective To describe the results of audiological and electrophysiological hearing evaluations in patients with sporadic ataxia (SA). Methods A retrospective cross-sectional study was carried out with 11 patients submitted to the following procedures: anamnesis, otorhinolaryngological evaluation, tonal and vocal audiometry, acoustic immittance and brainstem auditory evoked potential (BAEP) tests. Results The patients presented with a prevalence of gait imbalance, of dysarthria, and of dysphagia; in the audiometric and BAEPs, four patients presented with alterations; in the acoustic immittance test, five patients presented with alterations, predominantly bilateral. Conclusion The most evident alterations in the audiological evaluation were the prevalence of the descending audiometric configuration between the frequencies of 2 and 4 kHz and the absence of the acoustic reflex between the frequencies of 3 and 4 kHz bilaterally. In the electrophysiological evaluation, the patients presented changes with a prevalence of increased I, III and V wave latencies and the interval in the interpeak I-III, I-V and III-V. In the present study, it was observed that auditory complaints did not have a significant prevalence in this type of ataxia, which does not occur in some types of autosomal recessive and dominant ataxia.

Central auditory processing in patients with spinocerebellar ataxia.

BACKGROUND: Autosomal dominant spinocerebellar ataxias (SCAs) are a group of rare and heterogeneous neurodegenerative diseases characterized by the presence of progressive cerebellar ataxia. Although the symptomatology of SCAs is well known, information regarding central auditory functioning in these patients is lacking. Therefore, we assessed the central auditory processing disorders (CAPD) in patients with different subtypes of SCA. METHODS: In a retrospective cross-sectional study, we subjected 43 patients with SCAs to otorhinolaryngological, audiological, Brainstem Auditory Evoked Potential (BAEP) and acoustic immittance evaluations as well as CAPD tests, namely the Standard Spondaic Word (SSW) and the Random Gap Detection Test (RGDT). RESULTS: Most patients (83.7%) reported an imbalance when walking; many reported difficulty speaking (48.8%), dizziness (41.8%), and dysphagia (39.5%). In the audiometric test, 14/43 patients (32.5%) presented alterations, including 4/12 patients with SCA3 (33.3%), 1/8 patients with SCA2 (12.5%), 1/1 patient with SCA4 (100%), 1/1 patient with SCA6 (100%), 1/1 patient with SCA7 (100%), 3/6 patients with SCA10 (50%), and 3/14 patients with an undetermined type of SCA (21.4%). In the BAEP test, 20/43 patients (46.5%) presented alterations (11.6% na orelha esquerda e 34.9% bilateralmente), including 7/12 patients with SCA3 (58.3%), 5/8 patients with SCA2 (62.5%), 1/1 patient with SCA4 (100%), 1/1 patient with SCA6 (100%), 1/1 patient with SCA7 (100%), 4/6 patients with SCA10 (66.7%), and 2/14 patients with an undetermined type of SCA (14.2%). In the SSW, 22/40 patients (55%) presented alterations (2.5% in the right ear, 15% in the left ear, and 37.5% bilaterally), including 6/10 patients (60%) with SCA3, 3/8 (37.5%) with SCA2, 1/1 (100%) with SCA4, 1/1 (100%) with SCA6, 1/1 (100%) with SCA7, 4/5 (80%) with SCA10, and 8/14 (57.1%) with an undetermined type SCA. For the RGDT, 30/40 patients (75%) presented alterations, including 8/10 (80%) with SCA3, 6/8 (75%) with SCA2, 1/1 (100%) with SCA4, 1/1 (100%) with SCA6, 1/1 (100%) with SCA7, 4/5 (80%) with SCA10, and 9/14 (64.3%) with an undetermined type of SCA. In immittance testing, 19/43 patients (44.1%) presented alterations, including 6/12 (50%) with SCA3, 4/8 (50%) with SCA2, 1/1 (100%) with SCA4, 1/1 (100%) with SCA6, 1/1 (100%) with SCA7, 2/6 (33.3%) with SCA10, and 4/14 (28.6%) with an undetermined type of SCA. CONCLUSIONS: A majority of patients exhibited SSW test deficits, with a predominance of bilateralism, and three-fourths had impaired RGDT performance, pointing to difficulties with binaural integration and temporal resolution. Assessment of CAPD is important for therapeutic follow ups in patients with SCA.

[Acute coma in children: etiology, morbidity and mortality]. / Etiologia e a morbi-letalidade do coma agudo em crianças.

OBJECTIVE: An analysis was conducted on the etiology and the morbi-mortality of pediatric patients in acute coma, hospitalized at the Intensive Care Unit of Hospital Infantil Pequeno Príncipe (UTI-HIPP). METHOD: One hundred and four control sheets of children hospitalized at the UTI-HIPP and diagnosed as being in acute coma were analyzed. The Glasgow coma scale duly modified for children was used, with a score count equal to or lower than 8 points. The observation period was from March/98 to January/2001. All the supplementary exams as well as the neurological conditions of the patients when discharged were analyzed. RESULTS: The study comprised 104 children whose ages varied from 2 months to 13 years, with 57 (54.8%) of them being males. Hospital - stay time varied from 1 to 114 days, plus 3 cases in a persistent vegetative condition. As regards to etiology, 31 (29.8%) of the cases were due to meningo-encephalitis, 24 (23.1%) to an epileptic condition, 19 (18.3%) were toxic-metabolic, 16 (15.4%) to intra-cranial hypertension, 7 (6.7%) to shock/anoxia, 4 (3.8%) to an indeterminate etiology and 3(2.9%) were miscellaneous. Insofar as the clinical evolution of the children is concerned, 23 (22.1%) died, 32 (30.8%) evolved without any sequelae, 39 (37.5%) were discharged with neurological sequelae and for 10 (9.6%), no information is available. CONCLUSION: According to this study, one third of the children has died, one third presented neurological sequelae, and one third presented no further complications.

Vestibular findings in military band musicians.

Introduction Exposure to music is the subject of many studies because it is related to an individual's professional and social activities. Objectives Evaluate the vestibular behavior in military band musicians. Methods A retrospective cross-sectional study was performed. Nineteen musicians with ages ranging from 21 to 46 years were evaluated (average = 33.7 years and standard deviation = 7.2 years). They underwent anamnesis and vestibular and otolaryngologic evaluation through vectoelectronystagmography. Results The most evident otoneurologic symptoms in the anamnesis were tinnitus (84.2%), hearing difficulties (47.3%), dizziness (36.8%), headache (26.3%), intolerance to intense sounds (21.0%), and earache (15.7%). Seven musicians (37.0%) showed vestibular abnormality, which occurred in the caloric test. The abnormality was more prevalent in the peripheral vestibular system, and there was a predominance of irritative peripheral vestibular disorders. Conclusion The alteration in vestibular exam occurred in the caloric test (37.0%). There were changes in the prevalence of peripheral vestibular system with a predominance of irritative vestibular dysfunction. Dizziness was the most significant symptom for the vestibular test in correlation with neurotologic symptoms. The present study made it possible to verify the importance of the labyrinthine test, which demonstrates that this population should be better studied because the systematic exposure to high sound pressure levels may cause major vestibular alterations.

Otoneurologic findings in a fishermen population of the state of santa catarina: preliminary study.

Introduction Fishing, one of the oldest productive activities, is an important sector of the national and world economy. Aim To evaluate the vestibular behavior in a population of fishermen. Methods In a retrospective and cross-sectional study, 13 fishermen (mean 45.0), between 33 and 62 years of age, were submitted to anamnesis, otorhinolaryngological evaluation, and vestibular exam through the vector electronystagmography. Results The most evident otoneurologic symptoms were hearing loss (76.9%), tinnitus (61.7%), dizziness (46.1%), and headache (46.1%). The most evident clinical symptoms were fatigue (46.1%), depression (23.0%), anxiety (15.3%), insomnia (7.7%), and agitation during sleep (7.7%). There were alterations in the vestibular exam in 5 fishermen (38.5%) discovered in the caloric test. There was a prevalence of alteration in the peripheral vestibular system. There was a major frequency of the peripheral vestibular irritative syndrome. Conclusion The otoneurologic complaints were frequent in the population studied to verify the importance of allowing labyrinth exams and the need for adopting preventive measures relating to noise exposure as well as carbon monoxide exposure, because they can cause and/or enhance various manifestations of labyrinthine vestibular impairment that can affect the quality of life of these workers.