Atypical Presentation of Medullary Syndrome in a Young Woman

Wallenburg syndrome occurs due to damage to lateral segment of the medulla. Medial medullarysyndrome occurs due to damage to upper portion of the medulla. I report a case of a 30 years old woman diagnosed with medullary syndrome[both medial & lateral features] in absence of CT scan findings sent to the department of Physiology for electrophysiological tests like nerve conduction studies, blink reflex, brainstem auditory evoked potential (BERA) & visual evoked potential (VEP). She had loss of sensations on the ipsilateral half of face (right), hemisensory loss on contralateral trunk & extremities, headache, contralateral hemiparesis (left), ipsilateral lingual paresis with atrophy, fibrillations with contralateral positive Babinski’s sign. The electrophysiological tests showed decrease in conduction velocity of right facial nerve, left tibial & peroneal nerves with decreased amplitude. The nerve conduction studies of median nerve (both motor & sensory) were normal. In blink reflex, latency of R2 ipsilateral & R2 contralateral of supraorbital nerves were increased on right side. There were increasedlatencies of waves II, III, IV & V of BERA & increased P100 latencies in VEP.The results of the electrophysiological tests of the patient showed that she had features of both medial & lateral medullary syndrome suggesting a lesion of both upper & middle medulla. The CT scan in this case was normal but conduction of MRI & CT guided angiography of posterior cerebral & vertebral arteries could have further localized the lesion causing this mixed symptomology

Effects of intrauterine latent iron deficiency on auditory neural maturation in full-term newborns / Efeitos da deficiência de ferro latente intrauterino na maturação neural auditiva em recém-nascidos a termo

Abstract Objective: This study analyzed the relationship between latent iron deficiency evaluated by ferritin, and the myelination of the central nervous system evaluated through the brainstem evoked response audiometry test. Method: A total of 109 full-term newborns, born without anemia and risk factor for hearing deficiency, were enrolled. After delivery, umbilical cord blood was collected to determine ferritin and hematocrit levels. The brainstem evoked response audiometry test was carried out in the first 28 days of life. Analysis was performed between the control group (n = 71) with ferritin greater than 75 ng/mL, and the latent iron deficiency group (n = 38) with ferritin between 11 and 75 ng/mL. Results were presented as mean ± standard deviation. Statistical analysis was performed using GraphPad prism7 and SPSS with a significance level of 5%. Results: A significant higher V-wave (p = 0.02) and interpeak intervals I-III (p = 0.014), I-V (p = 0.0003), and III-V (p = 0.0002) latencies were found in the latent iron deficiency group, as well as a significant inversely proportional correlation between ferritin and the same wave and intervals (p = 0.003, p = 0.0013, p = 0.0002, p = 0.009, respectively). Multiple correlation analysis showed a significant correlation of latent iron deficiency with all interpeak intervals, even taking into account newborn gestational age. Conclusion: Iron deficiency anemia is a prevalent pathology; this study showed auditory delayed maturation associated to intrauterine iron deficiency, even in its latent form. This reinforces the importance of adopting effective measures, on a global scale, to prevent and treat this pathology in different life periods, especially in the most vulnerable population.

Resumo Objetivo Este estudo analisou a relação entre deficiência de ferro latente avaliada pela ferritina e a mielinização do sistema nervoso central avaliada pelo teste de Potenciais Evocados Auditivos de Tronco Encefálico. Método Foram incluídos no estudo 109 recém-nascidos a termo, nascidos sem anemia e fator de risco para deficiência auditiva. Após o parto, o sangue do cordão umbilical foi coletado para determinar os níveis de ferritina e hematócrito. O teste de Potenciais Evocados Auditivos de Tronco Encefálico foi realizado nos primeiros 28 dias de vida. A análise foi realizada entre o grupo controle (n = 71) com ferritina acima de 75 ng/mL e o grupo com deficiência de ferro latente (n = 38) com ferritina entre 11 e 75 ng/mL. Os resultados foram apresentados como média ± desvio-padrão. A análise estatística foi realizada utilizando o software GraphPad prism7 e SPSS com nível de significância de 5%. Resultados Latências significativamente maiores da onda V (p = 0,02) e dos intervalos interpicos I-III (p = 0,014), I-V (p = 0,0003) e III-V (p = 0,0002) foram encontradas no grupo de deficiência de ferro latente, assim como uma correlação significativa inversamente proporcional entre a ferritina e a mesma onda e intervalos (p = 0,003, p = 0,0013, p = 0,0002, p = 0,009, respectivamente). A análise de correlação múltipla mostrou uma correlação significativa da deficiência de ferro latente com todos os intervalos interpicos, mesmo se levarmos em consideração a idade gestacional do recém-nascido. Conclusão A anemia ferropriva é uma patologia prevalente e este estudo demonstrou maturação auditiva tardia associada à deficiência intrauterina de ferro, mesmo em sua forma latente. Isso reforça a importância da adoção de medidas efetivas, em escala global, para prevenir e tratar essa patologia em diferentes períodos da vida, principalmente na população mais vulnerável.

A Comparative Study on Brain Stem Evoked Response Audiometry among Pre-school Children with Normal and Delayed Development of Speech Attending Bankura Sammilani Medical College

Introduction: The main consequence of hearing loss,especially in children, is the impact caused by sensorydeprivation in the development of auditory and languageskills and learning. Any degree of hearing loss can resultin significant damage, as it interferes with perception andunderstanding of speech sounds.This proposed descriptivecross sectional study tries to compare BERA parametersbetween normal and delayed speech/language impairmentchildren. Study also examines possible abnormalities inBERA in children with speech and language impairment.Material and Methods: One descriptive study withcross-sectional design was conducted in neurophysiologylaboratory in the Department of Physiology, BankuraSammilani Medical College and Hospital for one year.About106 pre-school children (1 to below 6years) of eithersex were selected from those referred from Paediatric andENT Department with complaint of delayed speech who hadbeen advised BERA test. About 105 children without havingdelayed speech development were chosen randomly.Results: Descriptive analysis was done of BERA parametersamong all subjects. Mean and standard deviation of both maleand female were calculated separately. Independent ‘t’ testwas done between the BERA parameters of normal childrenand children diagnosed with speech impairment. The testshowed significant changes (p value <0.05) in waves I, IIIlatency, I-III, I-V, III-V inter peak in study subject.Conclusion: The brainstem speech evoked auditory responsescan serve as an efficient tool in identifying underlying auditoryprocessing difficulties in children with learning disability andcan help in early intervention.

Role Of Bera In Evaluation Of Hearing Loss In Pediatric Age Population

Introduction:Hearing impairment in pediatric population specially in early age could affect their learning abilities, skills and communication. Early diagnosis is utmost important in these cases to prevent this disability permanently. Aims:In this study, we evaluate the role of BERA in early diagnosis of hearing impairment specially in high risk pediatric population.Materials and methods:A total 65 high risk cases below 12 years of age are included in the study and BERA was obtained and data obtained and analysed.Results:In our study, 40.9 % cases showed hearing impairment on BERA investigation. Severe to modied hearing loss was found in 2 4 cases (88.9 %).Conclusion:In our study, BERA is very simple, useful, reliable, screening tool specially in high risk and un-cooperative pediatric population for early diagnose and management of hearing loss.

Finding out incidence of deafness among neonates at a tertiary care centre of western Rajasthan, India using otoacoustic emission

Background: Newborn hearing screening is conducted to identify suspected hearing loss and not to confirm the presence/absence of hearing loss or define features of the loss. Speech and hearing are interrelated, i.e., a problem with one could mean a problem with the other as speech and language is acquired normally through auditory system.Methods: A descriptive study conducted in the Department of Paediatrics, Dr. S. N. Medical College, Jodhpur, from June 2016 to December 2017. 5000 neonates were screened using otoacoustic emissions (OAE) in 2 stages at birth during 3rd to 7th day and 15-30 days respectively, followed by BERA at 3 months of age.Results: 1.4 infants per thousand infants had hearing loss. Presence of high-risk factors was seen to be associated be associated with hearing loss more than normal infants on screening with distortion product otoacoustic emissions (DPOAE) tests. However, on testing with BERA no such association was seen.Conclusions: 1.4 per 1000 infants had hearing loss. This study has shown that two stage distortion product otoacoustic emissions (DPOAE) hearing screening followed by british educational research association '(BERA) to confirm the hearing deficit, can be successfully implemented as new born hearing screening method in a hospital set-up, for early detection of hearing impaired, on a large scale, to achieve the high-quality standard of screening programs in a resource limited and developing nation like India.

Comparación de resultados del BERA eléctrico pre y post implante coclear / Comparison of results of electric BERA pre and post coclear implantation / Comparação de resultados do BERA eléctrico pré-post implantecoclear

Existen determinados pacientes en los cuales la presencia o función del nervio coclear es dudosa, por ejemplo, en los casos de malformaciones, deficiencia del nervio coclear (CND) o NF 2 (neurofibromatosis Tipo 2). El E-BERA (Potencial Evocado de Tronco Cerebral Eléctrico) es una herramienta que permite estimular eléctricamente el nervio auditivo mediante un electrodo en promontorio. Las respuestas son registradas y evaluadas por una electrofisiología en búsqueda de la onda V. En este trabajo nos propusimos validar la utilidad del E-BERA y comparar los resultados obtenidos mediante E-BERA preoperatorio con el estimulador de corriente constante del Prof. Paul R. Kileny (Michigan, EE.UU.) y los obtenidos intraoperatoriamente con el implante coclear colocado. Se midieron E-BERA preoperatorio y con el implante coclear a 10 pacientes con hipoacusia sensorioneural profunda (sin otra condición asociada), bajo anestesia general y monitoreo cardiológico. En todos los pacientes el E-BERA fue positivo. Los resultados indicaron que no hubo diferencias significativas entre la configuración, la morfología y la latencia de las ondas V obtenidas con ambos estudios de potenciales evocados.

There are certain patients in whom the presence or function of the cochlear nerve is doubtful, for example, in cases of malformations or NF2. The Evoked Potential of Electric Brainstem is a tool that allows us to electrically stimulate the auditory nerve by means of a promontory electrode. The responses were recorded and evaluated by an electrophysiologist in search of the V wave. In this paper we proposed to explain the utility of E-BERA and compare the results obtained by E-BERA with the constant current stimulator of Prof. Paul R. Kileny (Michigan, USA) and those obtained intraoperatively with the cochlear implant placed. We measured E-BERA and BERA with the cochlear implant in 10 patients with apparent CND (cochlear nerve deficiency) or NF2, under general anesthesia and cardiologic monitoring. In all patients E-BERA was positive. The results indicated that there were no significant differences between the configuration, morphology and latency of the V waves obtained with both studies of evoked potentials.

Há determinados pacientes em que a presença ou a função do nervo coclear é questionável, por exemplo, em casos de mal formada ou NF2. O Potencial Evocado de Tronco Encefálico Electric é uma ferramenta que nos permite estimular eletricamente o nervo auditivo através de um eletrodo no promontório. As respostas são registradas e avaliadas por uma electrophysiologist em busca da onda V. Neste trabalho nos propusemos a explicar a utilidade do E-BERA e comparar os resultados obtidos por E-BERA com estimulador de corrente constante Prof. Paul R. Kileny (Michigan, EUA) e obtido no intra-operatório com o implante coclear colocado. E-BERA e foram medidos com o implante coclear 10 pacientes com aparente CND (deficiência de nervo coclear) ou NF2, sob anestesia geral e monitorieo cardíaca. Em todos os pacientes o E-BERA foi positiva. Os resultados indicaram que não houve diferenças significativas entre a configuração, morfologia e latência da onda V obtida com ambos os estudos do potencial evocado.

Using Bera To Detect Persistence of Auditory Injury In High Risk Infants.

Objective: This cross-sectional comparative study tried to assess the hearing status of the high risk infants by Brainstem Evoked Response Audiometry (BERA) and compare with that of the normal infants. Material & Methods: BERA was done on 127 infants of 6 to18 months age of which 87 were high risk. All were given monaural acoustic stimulation using Cz-M1/M2 Montage. Waves I, III and V were analysed for absolute & interpeak latencies (in ms) & also for amplitudes (in µv) & their ratio. All the parameters were compared at 70 dB stimulus at p<0.05 significance. Results were analysed by Statistical Package for Social Sciences (SPSS) software, version 14.0. Results: There was no significant difference of mean age and sex between the two group. In the study froup, mean values of all the Absolute and Inter-Peak Latencies of both ears were significantly higher and mean Amplitudes of waves I and V of both ears were significantly smaller than that of the Control group. Conclusion: The study found evidence of persistent injury to the various parts of the auditory pathway even as the high risk infants grew up.

Some normal Results of Brainstem Evoked Response / Шинэ санаа Шинэ нээлт

Brainstem Evoked Response Audiometry test is a method to diagnose and differentiate the type, degree and the location of cochlear and retroocochlear hearing loss for infants early. Test methods to determine cochlear and retroocochlear hearing loss was introduced relatively late in the clinical practice of our country. Even though, we started to conduct BERA tests since 2007, there hasn’t beenany research conducted for determining the normal results for children aged 0-5. Aim: The study aims to determine some average Results of BERA tests for Mongolian children aged 0-5 with normal hearing by age groups using Octavus-BERA apparatus.The research has been conducted using cross sectional method of analytic research from 2009-2013.For the study, 110 children from children aged 0-5 who were diagnosed to have normal hearing from audiological department of Otorhinolaryngological hospital and 16 children with sensorineural (cochlear) hearing loss have been selected randomly. When the latency of waves for children aged 0-5 with normal hearing were measured and the average results for 70 dB were obtained in the BERA test: wave I was identified to be 1.64±0.15 – 2.08±0.5msec. Wave III was 3.68±0.18 - 4.47±0.73 msec, Wave V was 5.37±0.23 - 6.76±0.65 sec. When the latency between waves were measured and the average results were obtained in the BERA test: Between waves I-III, it was 2.05±0.20 – 2.39±0.53 msec. Between waves III-V was 1.69±0.10 -2.34±0.70 msec and between waves I-V was 3.70±0.29 - 4.65±0.56 msec. The latency of wave I for children with sensorineural hearing loss was 2.32±0.76 msec, 4.62±0.59msec for wave III and 6.55±0.65 msec for wave V, which was elongated with a statistical probability (p=0.00-0.05). Latency between waves was 2.39±0.45 msec between wave I-III, 1.83±0.16 msec between waves III-V and 3.97±0.88 msec between waves I-V, which doesn’t have statistically significant difference from the normal results.As the children’s age increases, the latency of the waves will shorten and becomes relatively stable from 25-30 months which is similar to adults. While the latency between waves I-III had no difference with statistical probability for children with all ages, latencies for waves III-V and I-V shortens as children’s age increases.Latency for children with sensorineural hearing loss was elongated with statistical probability (p0.05). This Result has critical importance in diagnosing cochlear deafness.

Neonatal Hearing Screening – Experience from a Tertiary Care Hospital in Southern India.

Objective: To implement a neonatal hearing screening program using automated auditory brainstem response audiometry in a tertiary care set-up and assess the prevalence of neonatal hearing loss. Design: Descriptive study. Setting: Tertiary care hospital in Southern India. Participants: 9448 babies born in the hospital over a period of 11 months. Intervention: The neonates were subjected to a two stage sequential screening using the BERAphone. Neonates suspected of hearing loss underwent confirmatory testing using auditory steady state response audiometry. In addition, serological testing for TORCH infections, and connexin 26 gene was done. Main outcome measures: Feasibility of the screening program, prevalence of neonatal hearing loss and risk factors found in association with neonatal hearing loss. Results: 164 babies were identified as suspected for hearing loss, but of which, only 58 visited the audiovestibular clinic. Among 45 babies who had confirmatory testing, 39 were confirmed to have hearing loss and were rehabilitated appropriately. 30 babies had one or more risk factors; 6 had evidence of TORCH infection and 1 had connexin 26 gene mutation. Conclusion: Neonatal hearing screening using BERAphone is a feasible service. The estimated prevalence of confirmed hearing loss was comparable to that in literature. Overcoming the large numbers of loss to follow-up proves to be a challenge in the implementation of such a program.

Reversibility of Brainstem Evoked Response Audiometry Abnormalities at 3 Months in Term Newborns with Hyperbilirubinemia.

Background: High bilirubin level is toxic to developing brain and auditory system but the current debate surrounds the toxicity of bilirubin in healthy term infants. Methods: Longitudinal observational study to find BERA abnormalities in term newborns with isolated hyperbilirubinemia of 20 mg/dL and more and to follow up babies at 3 months to find out about the reversibility in BERA abnormalities noted at birth. Results: BERA abnormalities were present in 17.64% of babies with isolated hyperbilirubinemia at discharge. There was a reversibility of BERA abnormalities in 61.61% during follow up. Conclusion: BERA abnormalities are reversible in term neonates with hyperbilirubinemia.

Normalidade de variáveis: métodos de verificação e comparação de alguns testes não-paramétricos por simulação / Normality of variables: diagnosis methods and comparison of some nonparametric tests by simulation

Introdução: os principais testes estatísticos têm como suposição a normalidade dos dados, que deve ser verificada antes da realização das análises principais. Objetivo: revisar as técnicas de verificação da normalidade dos dados e comparar alguns testes de aderência à normalidade para diferentes distribuições de origem e tamanho amostral. Metodologia: através da simulação de cinco distribuições (Normal, t-student, Qui-Quadrado, Gama e Exponencial) e seis tamanhos amostrais (10, 30, 50, 100, 500 e 1000) foram simulados 5000 amostras de cada par distribuição-tamanho amostral e realizados os testes Qui-quadrado, Kolmogorov-Smirnov, Lilliefors, Shapiro-Wilk, Shapiro-Francia, Cramer-von Mises, Anderson-Darling e Jarque-Bera. Resultados: os resultados obtidos mostram uma clara superioridade dos testes Shapiro-Francia e Shapiro-Wilk, com percentuais de acerto de 72,41% e 72,15%, respectivamente. Entre os piores resultados encontramos o Kolmogorov-Smirnov e Qui-Quadrado, com percentual de acerto de 44,78% e 61,58%, respectivamente. Conclusões: Para amostras pequenas recomenda-se que sejam utilizados procedimentos não paramétricos diretamente para a análise, em função da baixa performance dos testes de aderência à normalidade, dado o baixo percentual de acertos. Para amostras maiores, recomenda-se o uso dos testes Shapiro-Francia ou Shapiro-Wilk.

Introduction: The main statistical tests have the normality assumption that must be verified before performing the main analyzes. Objective: To review the techniques of testing for normality of data and compare some adherence tests for different true distributions and sample size. Methodology: Through simulation of five distributions (Normal, t-Student, Chi-Square, Gamma and Exponential) and six sample sizes (10, 30, 50, 100, 500 and 1000) were simulated 5000 samples of each pair sample size-distribution and applied the Chi-square, Kolmogorov-Smirnov, Lilliefors, Shapiro-Wilk, Shapiro-Francia, Cramer-von Mises, Anderson-Darling and Jarque-Bera tests. Results: The results show a clear superiority of the Shapiro-Francia and Shapiro-Wilk tests, with percentages of accuracy of 72.41% and 72.15% respectively. Among the worst results we find the Kolmogorov-Smirnov and Chi-Square, with percentage of accuracy of 44.78% and 61.58% respectively. Conclusions: For small samples it is recommended to use non-parametric procedures directly for the analyzes, due to the low performance of the tests of adherence to normality, given the low percentage of accuracy. For larger samples, we recommend the use of the Shapiro-Francia and Shapiro-Wilk tests.

Auditory evaluation of the microcephalic children with brain stem evoked response audiometry (BERA).

Microcephaly implies a reduced occipito-frontal circumference (< 2 Standard Deviation of normal) and therefore a small brain size, which is usually associated with different neurodeficit. Intactness of the auditory pathway in microcephalic as well as normal children was assessed by Brain stem Evoked Response Audiometry (BERA) to locate the exact site of lesion resulting in the auditory impairment, so that appropriate early rehabilitative measures can be taken. The study revealed that absolute peak latency of wave V, inter peak latencies of III–V and I–V were significantly higher (P- value <0.05 in each case) in microcephalics than the normal children. Auditory impairment in microcephaly is a common neurodeficit that can be authentically assessed by BERA. The hearing impairment in microcephalics is mostly due to insufficiency of central components of auditory pathway at the level of brainstem, function of peripheral structures being almost within normal limit.