Study on gene therapy for DPOAE and ABR threshold changes in adult Otof-/- mice / 临床耳鼻咽喉头颈外科杂志

Objective:This study aims to analyze the threshold changes in distortion product otoacoustic emissions（DPOAE） and auditory brainstem response（ABR） in adult Otof-/- mice before and after gene therapy, evaluating its effectiveness and exploring methods for assessing hearing recovery post-treatment. Methods:At the age of 4 weeks, adult Otof-/- mice received an inner ear injection of a therapeutic agent containing intein-mediated recombination of the OTOF gene, delivered via dual AAV vectors through the round window membrane（RWM）. Immunofluorescence staining assessed the proportion of inner ear hair cells with restored otoferlin expression and the number of synapses.Statistical analysis was performed to compare the DPOAE and ABR thresholds before and after the treatment. Results:AAV-PHP. eB demonstrates high transduction efficiency in inner ear hair cells. The therapeutic regimen corrected hearing loss in adult Otof-/- mice without impacting auditory function in wild-type mice. The changes in DPOAE and ABR thresholds after gene therapy are significantly correlated at 16 kHz. Post-treatment,a slight increase in DPOAE was observeds,followed by a recovery trend at 2 months post-treatment. Conclusion:Gene therapy significantly restored hearing in adult Otof-/- mice, though the surgical delivery may cause transient hearing damage. Precise and gentle surgical techniques are essential to maximize gene therapy's efficacy.

Wave V threshold among children with delayed speech and language, using brainstem evoked response audiometry – A cross-sectional study

Background: Brainstem Evoked Response Audiometry (BERA) is an objective test for audiometric assessment of children. As deprived linguistic skill is often associated with auditory impairment in initial years of life; early diagnosis and interventions are much needed. Aim and Objective: To detect Wave V, in the lowest intensity, as it is a reliable parameter to assess BERA threshold for hearing among different age group of children. Materials and Methods: This cross-sectional study included 250 children (n = 250) with impaired speech and 120 normal children. The case population was divided into four groups according to age – Group 1 (<1 year); Group 2 (1–3 years); Group3 (3–6 years); and Group 4 (6–12 years). Wave V threshold was determined by BERA (measure brainstem auditory evoked potential). Cases were also divided into two categories depending on unilateral or bilateral involvement of ear. Mean threshold of each group was compared with corresponding control group of similar age. The test of significance used was Mann–Whitney U-test. Intragroup comparison was done by Kruskal–Wallis test. Results: In Group 1, 91.7%; Group 2, 100%; Group 3; 88.6%, and Group 4; 85% of children were suffering from bilateral hearing loss. In all the groups, mean threshold was statistically significantly increased in comparison with control group. In intragroup comparison, there was no statistically significant difference of threshold among all the groups of cases in both of the ears. Conclusions: This study identified increased wave V threshold in children with impaired speech of all four groups. Thereby, it indicates the necessity of BERA as screening method.

Limiar Diferencial de mascaramento e potencial evocado auditivo de tronco encefálico em adultos normo-ouvintes com zumbido / Masking Level Difference and Auditory Brainstem ResponseIn Normal Hearing Adults With Tinnitus / Umbral de enmascaramiento diferencial y potencial evocado auditivo del tronco encefálico en adultos con audición normal y tinnitus

Introdução: O zumbido é uma ilusão auditiva consciente, uma sensação sonora não relacionada com uma fonte externa de estimulação. Objetivos: Caracterizar a Acufenometria, Limiar Diferencial de Mascaramento, o questionário de qualidade de vida Inventário de Desvantagem do Zumbido e Potencial Auditivo de Tronco Encefálico em adultos normo-ouvintes com zumbido, com a finalidade de comparar seus achados. Método: Vinte indivíduos do sexo feminino e masculino, entre 20 e 60 anos de idade, normo-ouvintes com queixa de zumbido, foram submetidos ao Acufenometria, Limiar Diferencial de Mascaramento, Inventário de Desvantagem do Zumbido e Potencial Evocado Auditivo de Tronco Encefálico. Resultados: AAcufenometriarevelou que o pitch médio foi de 4,3 KHz à orelha direita e 4,6 KHz à orelha esquerda. O loudness médio foi de 21,7 dBNS à orelha direita e 23,5 dBNS à orelha esquerda. O Limiar Diferencial de Mascaramento médio mostrou-se alterado. O Inventário de Desvantagem do Zumbido médio correspondeu à classificação de grau leve. O Potencial Evocado Auditivo de Tronco Encefálico apresentou parâmetros dentro da normalidade bilateralmente. Conclusão: Constatou-se que adultos normo-ouvintes com queixa de zumbido apresentam zumbido de pitch agudo bilateral com discreto impacto na qualidade de vida, condução adequada das vias auditivas até o tronco encefálico e comprometimento na identificação de sons na presença de ruído, demonstrando que o zumbido pode ter repercussões nas habilidades auditivas centrais. (AU)

Introduction: Tinnitus is a conscious auditory illusion, a sound perception unrelated to any external stimulus source. Objectives: To characterize the Acuphenometry, Masking Level Difference, the quality of life questionnaire Tinnitus Handicap Inventory and Auditory Brainstem Response in normal hearing adults with tinnitus, with the purpose of comparing the findings. Method: Twenty female and male individuals, between 20 and 60 years of age, normal hearing with complaints of tinnitus, underwent Acuphenometry, Masking Level Difference, Tinnitus Handicap Inventory and Auditory Brainstem Response. Results: The Acuphenometry showed the average pitch was 4.3 KHz to the right ear and 4.6 KHz to the left ear. The average loudness was 21.7 dBSL to the right ear and 23.5 dBs to the left ear. The average Masking Level Difference was altered. The average Tinnitus Handicap Inventory corresponded to the classification of mild grade. Auditory Brainstem Response showed parameters within normal range bilaterally. Conclusion: It was found that normal hearing adults with tinnitus complaints have bilateral acute pitch tinnitus with a slight impact on quality of life, appropriate conduction of auditory pathways to the brainstem and impaired identification of sounds in the presence of noise, demonstrating that tinnitus can have repercussions on central auditory skills. (AU)

Introducción: El tinnitus es una ilusión auditiva consciente, una sensación de sonido no relacionada con una fuente externa de estimulación. Objetivos: Caracterizar la coincidencia de tono y volumen, el umbral de enmascaramiento diferencial, el inventario de minusvalía para acúfenos y el potencial auditivo del tronco encefálico en adultos normoyentes con acúfenos, con el fin de comparar sus hallazgos. Método:Veinte sujetos masculinos y femeninos, con edades entre 20 y 60 años, audición normal con tinnitus, fueron sometidos a acúfenos, Umbral de Enmascaramiento Diferencial, Inventario de Desventajas de Tinnitus y Potenciales Evocados Auditivos del Tronco Encefalico. Resultados: La combinación de tono y volumen reveló que el tono promedio era de 4,3 KHz en el oído derecho y de 4,6 KHz en el oído izquierdo. Mientras que el volumen medio fue de 21,7 dBNS para el oído derecho y de 23,5 dBNS para el oído izquierdo. Se modificó el umbral diferencial de enmascaramiento promedio. El Inventario de Desventajas de Tinnitus promedio correspondió a la clasificación de grado leve. El Potenciales Evocados Auditivos del Tronco Encefalico presentó parámetros dentro del rango normal bilateralmente. Conclusión:Se encontró que los adultos normooyentes con quejas de tinnitus presentan tinnitus de tono alto bilateral con leve impacto en la calidad de vida, conducción adecuada de las vías auditivas al tronco encefálico y deterioro en la identificación de sonidos en presencia de ruido, demostrando que Tinnitus puede tener repercusiones en las habilidades auditivas centrales. (AU)

Potenciais evocados auditivos de tronco encefálico em indivíduos com doença de Parkinson: revisão de literatura / Brainstem auditory evoked potentials in individuals with Parkinson's disease: literature review

Objetivo: investigar a integridade da via auditiva por meio do Potencial Evocado Auditivo de Tronco Encefálico (PEATE) em indivíduos com Doença de Parkinson (DP) por meio de uma revisão integrativa da literatura. Métodos: foram utilizadas as bases de dados Embase, Google acadêmico, Scielo, bem como o portal eletrônico completo da Biblioteca Virtual da Saúde, com os seguintes descritores: DP (Parkinson Disease ou Parkinsonian Disorders) e PEATE (Evoked Potentials, Auditory, Brain Stem ou Evoked Potentials, Auditory). Foram selecionados artigos nacionais e internacionais, sem limitação de ano ou idioma. Os estudos foram descritos e analisados qualitativamente e quantitativamente quanto à latência absoluta e à amplitude das ondas I, III e V, e quanto aos interpicos I-III, III-V e I-V. Resultados: após exclusão dos títulos repetidos, 420 artigos foram encontrados, sendo que 17 atenderam aos critérios de inclusão. Nos resultados do PEATE, estudos descreveram o aumento da latência de todas as ondas, principalmente das ondas III e V e, também, aumento dos interpicos I-V e III-V. Conclusão: indivíduos com DP apresentam neurodegeneração das vias auditivas centrais com diminuição na velocidade de transmissão neural do estímulo acústico.

Purpose: investigate the integrity of the auditory pathway through the Brainstem Auditory Evoked Potential (BAEP) in individuals with Parkinson's Disease (PD) through an integrative literature review. Methods: Embase, Academic Google, Scielo databases were used, as well as the complete electronic portal of the Virtual Health Library, with the following descriptors: PD (Parkinson Disease or Parkinsonian Disorders) and BAEP (Evoked Potentials, Auditory, Brain Stem or Evoked Potentials, Auditory). National and international articles were selected, without limitation of year or language. The studies were described and analyzed qualitatively and quantitatively regarding the absolute latency and amplitude of waves I, III and V, and regarding the interpeaks I-III, III-V and I-V. Results: After excluding repeated titles, 420 articles were found, however only 17 met the inclusion criteria. In the ABR results, studies described the increase in latency of all waves, especially waves III and V, and also an increase in interpeaks I-V and III-V. Conclusion: individuals with PD present neurodegeneration of the central auditory pathways with a decrease in the neural transmission speed of the acoustic stimulus.

Endoscopic botulinum toxin injection combined with balloon dilatation for treatment of cricopharyngeal achalasia in patient with brainstem stroke / 中南大学学报(医学版)

OBJECTIVES@#At present, there are many reports about the treatment of cricopharyngeal achalasia by injecting botulinum toxin type A (BTX-A) into cricopharyngeal muscle guided by ultrasound, electromyography or CT in China, but there is no report about injecting BTX-A into cricopharyngeal muscle guided by endoscope. This study aims to evaluate the efficacy of endoscopic BTX-A injection combined with balloon dilatation in the treatment of cricopharyngeal achalasia after brainstem stroke, and to provide a better method for the treatment of dysphagia after brainstem stroke.@*METHODS@#From June to December 2022, 30 patients with cricopharyngeal achalasia due to brainstem stroke were selected from the Department of Rehabilitation Medicine, the First Hospital of Changsha. They were randomly assigned into a control group and a combined group, 15 patients in each group. Patients in both groups were treated with routine rehabilitation therapy, while patients in the control group were treated with balloon dilatation, and patients in the combined group were treated with balloon dilatation and BTX-A injection. Before treatment and after 2 weeks of treatment, the patients were examined by video fluoroscopic swallowing study, Penetration-aspiration Scale (PAS), Dysphagia Outcome Severity Scale (DOSS), and Functional Oral Intake Scale (FOIS) were used to assess the swallowing function.@*RESULTS@#In the combined group, 1 patient withdrew from the treatment because of personal reasons. Two weeks after treatment, the scores of DOSS, PAS, and FOIS in both groups were better than those before treatment (all P<0.01), and the combined group was better than the control group (all P<0.001). The effective rate was 85.7% in the combined group and 66.7% in the control group, with no significant difference between the 2 groups (P>0.05).@*CONCLUSIONS@#BTX-A injection combined with balloon dilatation is more effective than balloon dilatation alone in improving swallowing function and is worthy of clinical application.

Effects of transcranial magnetic stimulation on the swallowing and brain-stem auditory evoked potentials of dysphagic stroke survivors / 中华物理医学与康复杂志

Objective:To observe any effects of contralateral repeated transcranial magnetic stimulation (rTMS) of the swallowing motor cortex on the swallowing and brainstem auditory evoked potentials (BAEPs) of stroke survivors with dysphagia.Methods:A total of 83 stroke survivors with dysphagia were randomly divided into an ipsilesional stimulation group ( n=22), a contralesional stimulation group ( n=21), a bilateral stimulation group ( n=20), and a control group ( n=20). In addition to their conventional dysphagia training, those in the three stimulation groups received 3Hz rTMS while the control group was given fake stimulation. The treatment was administered daily for 20 minutes, 6 days a week, for 5 consecutive weeks. Before and after the treatment, swallowing function was assessed videofluoroscopically and using the Dysphagia Outcome and Severity Scale (DOSS). The oral and pharyngeal stages of swallowing were evaluated using the videofluoroscopic dysphagia scale (VDS). Brain stem conduction was assessed using BAEPs. Results:After treatment the average DOSS scores of all 4 groups were significantly better than before the treatment. The average DOSS scores of the contralesional and bilateral sti-mulation groups were then significantly better than those of the other two groups. The sub-item and total VDS scores of all 4 groups had decreased significantly, but the average score of the bilateral stimulation group was significantly lower than the control group′s average. Ipsilesional stimulation significantly improved the VDS sub-item scores for the triggering of pharyngeal swallowing, laryngeal elevation, and pharyngeal transit time compared with the control group. In the contralesional stimulation group the average total score and the VDS sub-item scores for apraxia, premature bolus loss, oral transit times, the triggering of pharyngeal swallowing, vallecular residue, laryngeal elevation, coating on the pharyngeal wall, and pharyngeal transit time were significantly lower than those of the control group, on average. After the treatment the latencies of BAEP waves I, III and V and the I-III, III-V and I-V interpeak intervals had decreased significantly in all four groups, but the average latencies and intervals of the bilateral and contralesional groups were significantly shorter than those of the control group. The latencies and intervals of the bilateral stimulation group were then significantly shorter than those in the ipsilesional stimulation group on average. The average latency of wave V in the bilateral stimulation group (6.53±0.73ms) was significantly shorter than that in the contralesional stimulation group after the treatment.Conclusion:Bilateral rTMS over the swallowing motor cortex combined with conventional dysphagia training can significantly improve the swallowing of dysphagic stroke survivors.

A comparison of the human voice (“Baah”) test and the automated auditory brainstem response in detecting neonates with hearing loss in a community setting

Objective@#To assess the usage of the “Baah” Test compared to the AABR (Automated Auditory Brainstem Response) in detecting hearing loss of neonates in the community setting. @*Methods@#This is a retrospective cross-sectional study. The targeted sample population are infants less than a month old who underwent screening at a testing facility in Malolos, Bulacan spanning the years 2011 and 2012. @*Results@#A total of 201 infants were included in the study, with a mean age of 10.77 days with a standard deviation of 7.79. The ratio of males to females was almost equal at 1:1.01. For infants who passed hearing screening on at least one ear, 96% (193 infants) correlated with the results of “Baah” testing. For those with bilateral refer results on AABR, 4 out of the 6 correlated with the “Baah” Test. @*Conclusion@#There is potential in using the “Baah” Test as a tool for hearing loss assessment of infants in situations wherein the usual hearing screening tests are inaccessible. It makes use of little resources, and though it does have its limitations in assessing for unilateral hearing loss (as the test cannot test ears in isolation), it would be able to identify infants likely to have bilateral hearing loss.

Assessment of Wave V latency and Wave I-V Interpeak latency in preterm babies by Brainstem Evoked Response Audiometry

Background: Early screening of hearing impairment optimizes communication, social, academic, and vocational outcomes for each child with hearing loss measurement of the auditory brain stem response which is considered the most sensitive method of assessing the auditory activity of neonates. Aims and Objectives: This study aims to compare wave V latency and interpeak I-V latency by brainstem auditory evoked response in preterm babies (32 weeks–36 weeks) with age-specific normal response and intergroup comparison (Group 1–32 weeks, Group 2–34 weeks, and Group 3–36 weeks) for the identification of hearing impairment if any. Materials and Methods: The present study was done on 50 preterm newborn, and after satisfying inclusion and exclusion criteria, preterm babies were subdivided into three groups on the basis of gestational age (Group A: 32 weeks [n = 12], Group B: 34 weeks [n = 18], and Group C: 36 weeks [n = 20]). Babies were subjected to brainstem evoked response audiometry (BERA) test on RMS EMG EP MARK-II machine in the neurophysiology unit of the Department of Physiology, Gandhi Medical College, Bhopal. Interpretation of the data as compared to normal values was done. Results: A trend toward decrease in absolute peak latencies of wave V with advancing gestational age was observed indicating progressive maturation, but the values recorded in all the three groups were longer as compared to the normal term values suggesting impairment in the maturation process. Wave I-V interpeak latencies values showed appreciable prolongation in all groups as compared to normal term values. Conclusion: The present study has shown that the preterm babies had altered BERA findings signifying hearing impairment.

The effect of increase in blood glucose level on hearing loss

Abstract Objective: Previous studies have shown that hearing function is also vulnerable to the effects of diabetes mellitus which can be shown by brainstem auditory evoked potential and distortion product otoacoustic emission recordings. This study aimed to investigate the changes of brainstem auditory evoked potential and distortion product otoacoustic emission in hyperglycemia and whether there is a relationship between reactive oxygen substances production and hearing deterioration in the rat model. Methods: 25 streptozotocin induced diabetic rats were divided into three groups: control, high blood glucose, and diabetes mellitus. Brainstem auditory evoked potential and distortion product otoacoustic emission were recorded, and thiobarbituric acid reactive substances levels were measured in the brainstem tissue. Results: At 8 kHz, the latencies of I, II, III, IV, and V brainstem auditory evoked potential waves in high blood glucose and diabetes mellitus groups were elongated, at 16 kHz, only these wave latencies of the diabetes mellitus group were prolonged compared with the control group. A significant decrease was also found in distortion product otoacoustic emission amplitudes at 4, 6, 8, and 10 kHz in the high blood glucose and diabetes mellitus groups compared to the control group. There was a significant increase in thiobarbituric acid reactive substances values due to the increase in blood glucose levels in the high blood glucose and diabetes mellitus groups compared to the control group. Conclusion: These results suggested that high blood glucose levels may cause hearing impairment not only in the diabetic state but also in the period of hyperglycemia before the onset of manifest diabetes mellitus and reactive oxygen substances may play an important role in the pathophysiology of diabetes mellitus. We suggest that regulating high glucose levels even before the onset of manifest diabetes mellitus may prevent hazardous effects on hearing function. Level of evidence: Level 3.

Hearing and language development in children with brainstem implants: a systematic review

Abstract Objective: To present scientific evidence, based on a systematic review of the literature, on the benefit of brainstem implants in auditory rehabilitation and language development in children. Methods: A systematic search was used to identify studies that contain information about the benefit of brainstem implants in the auditory rehabilitation and language development of children. The review was conducted based on a structured literature search, following the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) checklist. The search was carried out in the PubMed, Web of Science and Scopus databases, using the combination "Auditory brainstem implants" AND "Pediatric", without restriction of language, period, and location. The quality assessment of the articles was performed using the Study Quality Assessment Tools. Results: Regarding hearing, children with brainstem implants showed sound detection, access to most speech sounds, basic auditory perception skills, recognition of ambient sounds, recognition of some frequently used words and phrases, in addition to some closed-set word discrimination capability. Expressive and comprehensive language were identified in children using auditory brainstem implants, increasing significantly in the short and long terms in most cases; however, in some of the children, such skills remained stable. Conclusion: The auditory brainstem implant can be considered an effective alternative for children with cochlear malformation and/or auditory nerve deficiency and for those who cannot benefit from cochlear implant surgery.

The Relationship between Contralateral Suppression of Transient Evoked Otoacoustic Emission and Unmasking of Speech Evoked Auditory Brainstem Response

Abstract Introduction Several studies have shown that efferent pathways of the auditory system improve perception of speech-in-noise. But, the majority of investigations assessing the role of efferent pathways on speech perception have used contralateral suppression of otoacoustic emissions as a measure of efferent activity. By studying the effect of efferent activity on the speech-evoked auditory brainstem response (ABR), some more light could be shed on the effect of efferent pathways on the encoding of speech in the auditory pathway. Objectives To investigate the relationship between contralateral suppression of transient evoked otoacoustic emission (CSTEOAE) and unmasking of speech ABR. Methods A total of 23 young adults participated in the study. The CSTEOAE was measured using linear clicks at 60 dB peSPL and white noise at 60 dB sound pressure level (SPL). The speech ABR was recorded using the syllable /da/ at 80 dB SPL in quiet, ipsilateral noise, and binaural noise conditions. In the ipsilateral noise condition, white noise was presented to the test ear at 60 dB SPL, and, in the binaural noise condition, two separate white noises were presented to both ears. Results The F0 amplitude of speech ABR was higher in quiet condition; however, the mean amplitude of F0 was not significantly different across conditions. Correlation analysis showed a significant positive correlation between the CSTEOAE and the magnitude of unmasking of F0 amplitude of speech ABR. Conclusions The findings of the present study suggests that the efferent pathways are involved in speech-in-noise processing.

Comparative study of brainstem auditory evoked responses in females having premenstrual symptoms with normal population

Background: Premenstrual symptoms (PMS) have largely been ignored, because ages so present study are done in females specifically having PMS comparing with females with no PMS. Very little work has been done in past in this field, because females revealing menstrual history are a big taboo in our society. Aim and Objective: The aim of this study was to study brainstem auditory evoked potential (BAEP) in females having premenstrual symptoms and comparing it with females having no premenstrual symptoms. Materials and Methods: BAEP was conducted on 60 females age group of 18–40 years in two groups in Research laboratory, Department of Physiology, Lala Lajpat Rai Memorial Medical College in collaboration with SVBP Hospital, Meerut for 6 months. The subjects were divided into two groups, Group A having premenstrual symptoms and Group B having no premenstrual symptoms. The test was conducted in later part of luteal phase 23rd–25th day of menstrual cycle. The absolute latencies (ALs) and interpeak latencies (IPLs) were the parameters measured for the analysis of BAEP. Results: IPL, that is, interpeak latencies I–III and III–V in age group 18–25 years, showed statistically significant decrease in luteal phase in females having premenstrual syndrome (PMS) as compared to normal cycling females. Conclusion: Premenstrual symptoms females are showing few significant changes in auditory responses at mid brain level as recorded by BAEP study which can be due to the modulatory effect of female gonadal hormones progesterone and estrogen on neurotransmitters (serotonin, gamma-aminobutyric acid, and glutamate) in central nervous system.

Muerte súbita inesperada en epilepsia. Revisión sobre la implicación del tallo encefálico / Sudden unexpected death in epilepsy. Review about the brainstem involvement

RESUMEN La muerte súbita inesperada en epilepsia, se define como la muerte repentina -presenciada o no- de personas con epilepsia, no traumática ni por ahogamiento, con o sin evidencias de crisis, y en quienes el examen postmorten no revela una causa estructural o toxicológica de muerte. El objetivo de esta revisión es describir las evidencias más recientes, publicadas en la literatura, sobre la participación crucial del tallo encefálico en la fisiopatología de la muerte súbita inesperada en epilepsia. Se realizó una búsqueda bibliográfica en la base de datos computarizada PubMed. Los estudios en modelos animales han demostrado que los mecanismos de la muerte súbita inesperada en epilepsia involucran un primer evento mediado por una crisis, seguido por la despolarización cortical, que se propaga al tallo encefálico y que resulta en una disfunción autonómica causante de apnea central, edema pulmonar o arritmia cardiaca. Los estudios en humanos se han apoyado en las imágenes de resonancia magnética para evaluar el papel de diferentes áreas del tallo encefálico en la muerte súbita inesperada en epilepsia. Las evidencias acumuladas por la literatura, tanto en estudios con animales como humanos, evidencian el papel fundamental desempeñado por las estructuras del tallo encefálico en la fisiopatología de la muerte súbita inesperada en epilepsia.

ABSTRACT Sudden unexpected death in epilepsy is defined as the sudden death-whether witnessed or not-of people with epilepsy, not traumatic or due to drowning, with or without evidence of seizures, and in whom postmortem examination does not reveal a structural or toxicological cause of death. The aim of this review is to describe the most recent evidence published in the literature, on the crucial involvement of the brain stem in the pathophysiology of sudden unexpected death in epilepsy. A bibliographic search was conducted in PubMed computerized database. Studies in animal models have shown that the mechanisms of sudden unexpected death in epilepsy involve a first seizure-mediated event, followed by cortical depolarization, which spreads to the brainstem and results in autonomic dysfunction causing central apnea, pulmonary edema or cardiac arrhythmia. Studies in humans have relied on magnetic resonance imaging to assess the role of the brainstem in sudden unexpected death in epilepsy. The evidence accumulated in the literature, both in animal and in human studies, shows the role played by brainstem structures in the pathophysiology of sudden unexpected death in epilepsy.

Frequency-Following Response and Auditory Behavior in Children with Prenatal Exposure to the Zika Virus

Abstract Introduction Prenatal exposure to the Zika virus can impair neurodevelopment and cause auditory damage. Objective To analyze the frequency-following response (FFR) and the auditory behavior (with the LittlEars® questionnaire) of children with and without prenatal exposure to Zika virus infection. Methods A total of 30 children participated in the present study, divided into 3 groups: 10 children with microcephaly and prenatal exposure to the Zika virus; 10 normocephalic children with prenatal exposure to the Zika virus; and 10 children with no evidence of prenatal exposure to the virus. The FFR test was performed with the /da/ syllable. The LittlEars® questionnaire was used with parents/guardians. Results For the FFR measurements, there was no difference between the groups. The children with exposure to the Zika virus presented a final score in the questionnaire below what is expected from children with normal hearing. A significant difference was observed for the final, semantic, and expressive scores between the group with microcephaly and the other groups. A strong negative correlation was seen between the LittlEars® questionnaire final score and the FFR measurements for the group with microcephaly when compared with the other groups. Conclusion Children exposed to the Zika virus, with and without microcephaly, presented FFR patterns similar to what was seen in children with no evidence of virus exposure. However, they showed signs of immature auditory behavior, suggesting auditory development delay.

Eficacia del «Programa de tamizaje auditivo neonatal universal¼ de la Caja Costarricense del Seguro Social en la detección de sordera en niñas y niños, entre 2016 y 2018 / Effectiveness of «Universal Newborn Hearing Screening program¼ of Caja Costarricense del Seguro Social, in detecting deafness in children, from 2016 to 2018

Resumen Introducción: el tamizaje auditivo es un método de detección y su propósito es intervenir de forma temprana para reducir las consecuencias negativas de una eventual afección auditiva en el desarrollo integral del infante. Costa Rica ha implementado en el sistema de salud público un programa de tamizaje auditivo neonatal universal, donde se examina todo niño o niña antes del primer mes de vida. Aunque se han percibido múltiples beneficios, es importante demostrar con evidencia científica si cumple sus objetivos. Metodología: se analizó la eficacia del «Programa de tamizaje auditivo neonatal universal» (TANU) de la Caja Costarricense de Seguro Social, a partir de la información registrada de 37 656 infantes evaluados entre los años 2016 y 2018, mediante el contraste empírico con las técnicas descriptivas de distribución de frecuencias de variables, y las pruebas inferenciales chi cuadrado y análisis de varianza (ANOVA). Resultados: en cuanto a la edad al momento del examen, el 71 % de la muestra tenía entre 0 y 3 días. Su desempeño a través del tiempo y la extensión a distintos centros de salud ha sido satisfactorio, así mismo, la cantidad de infantes valorados ha sido hasta del 98 % de la población. Conclusiones: el programa TANU es eficaz en la mayoría de aspectos analizados, destaca la identificación de factores de riesgo y la detección temprana de afecciones auditivas, sin embargo, se proponen mejoras en relación con el manejo de la información.

Abstract Introduction: Hearing screening is a detection method, its objective is to provide an early intervention that allows reducing the negative consequences of an eventual hearing impairment, in the integral development of the infant. Costa Rica has implemented a universal neonatal hearing screening program in the public health system, where the hearing of every child born in the country is examined before the first month of life. Although multiple benefits have been perceived, it is important to demonstrate with scientific evidence if it accomplishes its objectives. Methodology: the effectiveness of the Universal Newborn Hearing Screening Program in Costa Rica was analyzed, based on the information recorded from 37,656 children evaluated since 2016 to 2018, through empirical contrast using both descriptive techniques: frequency distribution of variables, as well as the use of inferential tests: chi square and analysis of variance (ANOVA). Results: 71 % of the sample was between 0 and 3 days old at the moment of being examined. Its performance over time and extension to different health centers has been satisfactory, likewise, the number of infants evaluated has been up to 98 % of the population. Conclusions: the TANU program is effective in most of the aspects analyzed, the identification of risk factors and the early detection of hearing disorders are outstanding, however, improvements are proposed in relation to the handling of information.

Revisão de anatomia e correlações clínicas do tronco encefálico, parte i: bulbo / Review of brain anatomy and clinical correlations, part i: medulla

O Tronco encefálico (TE) é uma estrutura singular do sistema nervoso central, pois nele passam tratos sensoriais ascendentes da medula espinal, tratos sensoriais da cabeça e do pescoço, os tratos descendentes motores originados no prosencéfalo (divisão mais rostral do encéfalo), e as vias ligadas aos centros de movimento dos olhos. Contém ainda os núcleos dos nervos cranianos e está envolvido na regulação do nível de consciência através de projeções ao prosencéfalo oriundas da formação reticular. Todas essas estruturas coexistem em um espaço muito exíguo, o que faz com que o TE seja um local muito sensível às alterações patológicas, sendo que os pacientes apresentam muitos sinais neurológicos mesmo com lesões muito pequenas nesse local. Compreender a anatomia interna do TE é essencial para o diagnóstico neurológico e a prática da medicina clínica. Outros profissionais da saúde também se beneficiam desse conhecimento para melhor manejo dos seus pacientes neurológicos. Essa revisão apresenta detalhes da anatomia macroscópica e microscópica do bulbo, bem como seus correlatos clínicos frente às lesões mais comuns dessa divisão particular do TE, conhecidas como síndromes bulbares.

The brainstem is a unique structure in the central nervous system, since it gives way to ascending sensory tracts from the spinal cord, sensory tracts from the head and neck, motor descending tracts originating from the forebrain, and the pathways connected to the eye movement centers. It also contains the cranial nerve nuclei and is involved in the regulation of consciousness levels through projections to the forebrain originating in the reticular formation. All these structures coexist in a very small space, which makes the brainstem very sensitive to pathological changes, with patients presenting several neurological symptoms even with very small brainstem lesions. Understanding the internal anatomy of the brainstem is essential for neurological diagnosis and the practice of clinical medicine. Other health professionals also benefit from this knowledge to better manage their neurological patients. This review presents detailed information on the macroscopic and microscopic anatomy of the medulla, as well as its clinical correlates in the face of the most common lesions of this particular division of the brainstem, known as medullary syndromes.

Brain stem auditory evoked potential response in neonates after recovery from hyperbilirubinemia: A neurophysiological approach to screening neuronal hearing loss

Objectives: Neonatal hyperbilirubinemia leads to neurological damages including encephalopathy and hearing loss. This study aimed to screen and evaluate the hearing loss in neonates after recovery from hyperbilirubinemia using the Brainstem evoked response audiometry (BERA) test. Materials and Methods: This cross-sectional comparative study was conducted in Physiology Department at Chitwan Medical College, Nepal. It included 20 age and sex-matched neonates recently recovered from hyperbilirubinemia and 20 normal healthy controls. The external acoustic canals of subjects were checked for any blockage or collapse before BERA testing. The BERA recordings were performed after the neonate’s natural sleep following a standard lab protocol explained by Taylor’s Evoked Potential in Clinical Testing. Results: The BERA wave latencies were delayed with a higher number of case group neonates I (IL-75%, IR-80%), III (IIIL-70%, III R-80%), and V (VL- 80%, VR-85%) than those of controls. The percentage of neonates with delayed interwave latencies was comparable between groups. The neonate’s hearing sensitivity assessed using the grades of hearing impairment by WHO revealed slight (threshold of hearing left ear [THL]-25% and threshold of hearing right ear [THR]-30%) and moderate (THL-40% and THR-35%) grades among cases whereas no impairment (THL-60% and THR-55%) in controls. The hearing thresholds were more in cases. Conclusion: The auditory pathway is highly sensitive to elevated serum bilirubin. BERA detects even a minute degree of hearing damage seen after complete treatment of hyperbilirubinemia. Therefore, BERA is a helpful tool in the early screening of hearing impairment in neonates. This improves prognosis by early management so that the neurosensory systems develop to their full extent and one can enjoy a normal social life.

Auditory brainstem response in type 2 diabetes mellitus patients

Background: Diabetes is a chronic metabolic disorder and its complications pose a significant healthcare burden. Basic pathophysiology of diabetic complications is angiopathy leading to neuropathy. Angiopathy of small vessels of cochlea and neuropathy of cochlear nerve may lead to hearing impairment. Aim and Objectives: The present study was taken up with an objective to evaluate the changes in the auditory brainstem evoked potentials in type 2 diabetic patients leading to hearing loss compared to healthy controls. Materials and Methods: A total of 40 subjects in the age group of 40–60 were enrolled into the study and were categorized into 2 groups of 20 each. In the Group 1, age- and sex-matched healthy controls were included and in the Group 2 subjects with type 2 diabetes of more than 5 years duration were included in the study. Any hearing impairment caused by a known disease, drug or injury either traumatic, iatrogenic or noise induced were excluded from the study. They were subjected to auditory brainstem response test Brainstem evoked response audiometry. Absolute latency of wave I-V and interpeak latencies (IPL) I-III, III-V, and I-V was recorded. The data obtained were evaluated using VassarStats. Results: There was no significant difference in the wave latency of wave I and II between the groups; however, the latencies of waves III, IV, and V was higher in the diabetic group compared to controls on both right and left ear stimulation and it was statistically significant. With respect to the IPL comparison, it was observed that IPL I-III, III-V, and I-V were significantly increased in diabetics with both right and left ear stimulation were statistically significant on comparison with the controls. Conclusion: The delayed transmission of the auditory pathway at the level of brainstem and midbrain observed in the study advocates the presence of central neuropathy in patients with type 2 diabetes mellitus.

Comparative assessment of brainstem auditory evoked potentials in apparently healthy medical students with a family history of diabetes mellitus type 2

Background: Neuronal studies on diabetes mellitus (DM) were previously based on peripheral and autonomic nerves. With the advent of Brainstem Auditory Evoked Potential (BAEPs), studies on sensory pathways in the central nervous system become easier and more productive. BAEPs is a non-invasive electrophysiological tool to detect retro-cochlear lesion. Hence, it is helpful to detect early Impairment of the auditory nerve and brainstem function. Aim and Objective: DM Type 2 (T2DM) is a known cause of neuropathy and in earlier course, it involves sensory nerves. T2DM runs in families and it has a genetic predisposition. BAEP is one of the methods to find out problems related to hearing by analyzing latencies of waveforms and inter-peak latencies. Hence, BAEPs in apparently healthy subjects with and without family history of T2DM is assessed. Materials and methods: This is a cross-sectional, casecontrol study. We have taken 110 volunteers from MBBS students of IMS, BHU of 17–23 years of age. Those with co-morbid conditions (eg. diabetes and hypertension), neurodegenerative diseases, neuropathy, schizophrenia, and those on ototoxic and neurotoxic drugs are excluded from the study. After taking consent and conducting a preliminary physical examination, BAEPs are recorded using a proper BAEP recording device. Statistical analysis is done using SPSS 2016 software trial version with Chi-square test. Results: The subjects with positive family history of T2DM in paternal grandfather showed deviation in latencies of BAEPs wave I (P < 0.001), wave III (P = 0.019), wave V (P = 0.033), and inter-peak latency between wave I and wave V (P = 0.019) from the normal values in the left ear. The subjects with positive family history of T2DM in paternal grandmother showed deviation from the normal in case of V/I % in the right ear (P = 0.016). Conclusion: The presence of T2DM in families can affect the wave latencies and inter-peak latencies of BAEPs.

Anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma: a case report / 中华神经科杂志

Paraneoplastic neurological syndromes (PNS) are heterogeneous disorders caused by autoimmune responses of cancer, which can affect any part of the nervous system. Anti-amphiphysin antibody is one of the high-risk PNS antibodies, which is usually associated with small cell lung cancer and breast cancer. However, extrapulmonary neuroendocrine carcinoma is rare in patients with anti-amphiphysin antibody. A case of anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma is reported. The tumor was detected by fluorine 18 fluorodeoxyglucose positron emission tomography and pathologically confirmed by gastroscopic biopsy. The patient′s neurological symptoms were partially improved after treatment of intravenous immunoglobulin and glucocorticoids. However, the disease prognosis is closely related to the accompanying tumor.