Evoked Brainstem Responses Research Articles

Hearing Screening of Infant and Children Suspected of Congenital Hearing Loss at Dustira Cimahi Hospital

Hearing problems in children impact development, especially speech and language. Some risk factors are known to influence the occurrence of congenital deafness. For the last five years, screening with Otoacoustic Emissions (OAE) and Brainstem Evoked Response Audiometry (BERA) examinations has been used at Dustira Hospital to make a diagnosis and intervene as early as possible. This study aims to obtain the prevalence, risk factors, and OAE and BERA examination results at Dustira Cimahi Hospital. A retrospective descriptive study was conducted on children who underwent the OAE and BERA examination by collecting subject data for the period of January 2020– December 2022. The OAE examination uses distortion product otoacoustic emissions, while the BERA examination is based on the International Standard Organization (ISO). Of the 42 patients who were screened for hearing loss, the 3–5-year-old group was the most dominant (40.4%). Most of the patients were male (66%). While the prenatal risk factor was a history of TORCH infection (12%), the perinatal risk factor was low birth weight (14.2%) and the postnatal risk factor was hyperbilirubinemia (19%). The majority of the OAE examination results were refers found at 32 people (76.1%). The results of the BERA click examination were the presence of wave V found in 38 people (90.4%)). Patients with suspected congenital deafness who underwent hearing screening were in the age range of 3–5 years and were all male. The risk factors found were a history of TORCH infection, low birth weight, and hyperbilirubinemia. The majority of the results of the OAE examination was refer and that of the BERA was wave V. Keywords: BERA, children, congenital deafness, OAE

The Assessment of Auditory Function in High-Risk Neonates and Infants Using Brainstem-Evoked Response Audiometry (BERA).

Introduction Hearing impairment in neonates and infants is a critical concern due to its potential to impede language acquisition, cognitive development, and overall quality of life. Brainstem-evoked response audiometry (BERA) stands out as a valuable diagnostic tool. The early detection of hearing impairments is paramount in neonatal care. Hearing loss during infancy can impede speech and language development, social interaction, and academic achievement. High-risk neonates, including those born prematurely or with low birth weight, have a heightened susceptibility to hearing impairment due to various factors such as exposure to ototoxic medications, mechanical ventilation, and complications associated with prematurity. Methods A hospital-based prospective study was conducted in the department of otorhinolaryngology; the study focused on high-risk neonates and infants from the outpatient department and inpatient department. The study was conducted from October 2022 to March 2024. A sample size of 70 patients was taken, including high-risk neonates and infants. Healthy term neonates and healthy infants were excluded from the study. Results In the current study, there were 40 males and 30 females. Among the infants surveyed, prematurity was the most prevalent risk factor, followed by perinatal asphyxia. Low birth weight was observed in 43% of cases, while hyperbilirubinemia and neonatal sepsis were the next. Among the 70 infants assessed, 50% were found to have normal hearing. Mild hearing loss was observed in 23% of cases, while 14% had moderate hearing loss. Severe and profound hearing loss were less common. Conclusion Our study highlighted the importance of early and routine auditory screening usingBERA in high-risk neonates and infants, revealing a significant prevalence of hearing loss linked to various risk factors such as premature babies, low birth weight, hyperbilirubinemia, neonatal intensive care unit stay, perinatal asphyxia, and ototoxic drugs during pregnancy. Prematurity is the most common risk factor.For language development, early diagnosis and intervention were crucial. If babies have profound sensorineural hearing loss, they can go for a cochlear implant.

Chromosome Xp22.3 deletion syndrome with X-linked ichthyosis, Kallmann syndrome, short stature, generalized epilepsy, hearing loss, attention deficit hyperactivity disorder, and intellectual disability – A rare report with review of literature

Chromosome Xp22.3 deletion syndrome is a very rare contiguous gene deletion syndrome with variable phenotype due to the deletion of genes from the distal short arm of the X chromosome (Xp), including the short-stature homeobox (SHOX), anosmin-1 (ANOS1), arylsulfatase (ARSL), neuroligin-4 (NLGN4), and steroid sulfatase (STS) genes. We have reviewed the available literature on the chromosome Xp22.3 deletion syndrome. A 10-year-old boy presented with global developmental delay, generalized epilepsy, decreased hearing, and hyperactivity. He had no significant family history. Examination revealed microcephaly, short stature, and dry and scaly skin lesions on the trunk. He had thick arched eyebrows, a depressed nasal bridge, a long philtrum, high arched palate, retrognathia, brachytelephalangy, brachymetatarsia, and mild scoliosis. Brainstem-evoked response audiometry testing revealed moderate hearing loss. Magnetic resonance imaging showed cerebellar tonsillar ectopia. Clinical exome sequencing revealed a likely pathogenic contiguous deletion (~8.10 Mb) spanning genomic location chrX:g.(_630898)_(8732037_)del encompassing ANOS1, ARSL, NLGN4X, SHOX, and STS genes. We have reviewed the available literature for reported associations of Chromosome Xp22.3 deletion syndrome and report a novel association of X-linked ichthyosis, Kallmann syndrome, global developmental delay, short stature, bilateral hearing loss, generalized epilepsy, attention deficit hyperactivity disorder, and intellectual disability.

COVID-19 Infection During Pregnancy and Hearing Loss in Infants: A Clinical Study.

To find the prevalence of hearing loss in children born to mothers who had COVID-19 infection during pregnancy. This was a prospective observational study conducted on 1960 babies born to mothers who were COVID positive during their pregnancy .All children born to RTPCR positive COVID-19 pregnant women were included in the study and babies who had any other risk factor for neonatal SNHL were excluded from the study. All the babies were subjected to OAE, and if it came pass, the test was repeated 2 weeks later. Those patients with repeat OAE also as refer were subjected to Brainstem Evoked Response Audiometry and findings correlated. The study included 1960 neonates who were subjected to neonatal hearing screening by OAE. There were 1020 males (52.04%) and 940(47.96%) females and 80 cases of consanguinity. Fifty babies were excluded due to comorbidities. In the initial OAE test 380 neonates failed (19.9%) following which BERA was done and ten patients was found to Sensorineural hearing loss. The prevalence of hearing loss in our study on babies born to mothers with COVID-19 infection during pregnancy is 0.005. Although these primary results from our study does not indicate any immediate effect of SARS-COV-2 maternal infection on neonatal hearing. More children need to be tested and followed up over an extended period of time to detect any possible delayed auditory effects. Those patients who were identified to have hearing loss in our study will have to be on long term follow-up as we see long terms effects on cognition, memory, heart health, etc. in COVID survivors. Level 1.

Late-onset of Labyrinthine Dysfunction in Chronic Brucella-induced Encephalitis

Brucellosis, caused by Brucella, dominates in the Middle East. Acute or chronic brucellosis can involve any organ system, and the involvement of the central nervous system leads to encephalitis, called neurobrucellosis (NB). We report a rare case from north India where a variant of Brucella caused NB led to progressive labyrinthine dysfunction; who developed vertigo, disequilibrium, and gradual progressive sensorineural (SN) hearing loss. Audiological tests pure-tone audiometry (PTA), otoacoustic emissions (OAE), stapedial muscle reflexes, auditory evoked brainstem responses (ABR), and vestibular evoked myogenic potentials (VEMPs) were conducted on the patients. PTA showed a bilateral profound SN hearing loss, an absent OAE, and ABR suggestive of cochlear dysfunction. The cervical and ocular VEMP showed delayed n1 and p1 latencies, suggesting superior and inferior nerve branch involvement. Vertigo or disequilibrium and SN hearing loss can be the late-onset manifestations of labyrinthine dysfunction in chronic Brucella-induced encephalitis.

Brainstem auditory evoked potential variability in unmedicated schizophrenic patients

Background: Schizophrenia (SCZ) is one of the most incapacitating and financially disastrous medical conditions and ranks among the most severe and burdensome diseases in the world due to the onset, progression, and frequency of disability. Few researches have been reported that SCZ is linked to a number of neurophysiological abnormalities, including changes in brainstem auditory evoked potentials (BAEPs). Aims and Objectives: The current study aimedto measure brainstem function using BAEPs in patients with SCZ and compare their findings with healthy controls. Materials and Methods: A cross-sectional case–control study was conducted on 51 schizophrenic drug-naive patients selected from the outpatient department of psychiatry, fulfilling the diagnostic criteria of DSM-IV, aged 18-28 years, male and female, with a matching control group of 50 subjects recruited from patients’ relatives. For assessing the severity of SCZ, the positive and negative syndrome scale was utilized. The BAEPs were recorded using a brainstem-evoked response audiometry (BERA) device (NeuroStim-NS 2). Data were analyzed using Independent t-test and Mann–Whitney test based on normality. P-value was considered significant at P < 0.05, P < 0.01 level. Results: A significant difference was observed in patients and control group BAEPs. The absolute latency of waves I and III in the right ear, waves I and V, IPL I-III in the left ear was shown to be significantly different. The results demonstrate that the patient group’s mean latency was higher than the controls. Conclusion: Based on findings, it was concluded that abnormal BAEPs in schizophrenic patients might be attributed to their primary symptom of discontinuity in perceptual and other cerebral functions and slower transmission in the lower brainstem auditory pathway and decreased activity at the brainstem level.

The Effects of Stimulus Repetition Rate on Electrically Evoked Auditory Brainstem Potentials in Postlingually Deafened Adult Cochlear Implant Recipients.

Background: By using outcome prediction scores, it is possible to distinguish between good and poor performers with cochlear implants (CI) after CI implantation. The reasons for poor performance, despite good basic conditions, can be manifold. On the one hand, the postoperative fitting may be inadequate; on the other, neurophysiological disease processes may impair speech understanding with a CI. These disease processes are not yet fully understood. In acoustics, it is known that the auditory brainstem responses (ABR) and their latencies and amplitudes allow differential diagnosis based on reference values for normal-hearing individuals. The aim of this study was to provide reference values for electrically evoked brainstem responses (EABRs) in terms of rate-dependent latencies and amplitudes. Methods: 20 ears of 18 experienced adult CI recipients with a predicted and measured good postoperative word recognition score were recruited from the clinic's patient pool. In the same stimulation mode and intensity we measured latencies and interpeak-latencies of EABRs and electrically evoked compound action potentials (ECAPs). With a defined supra-threshold stimulation intensity above the individual ECAP threshold, we applied stimulation at several rates between 11 and 91 stimuli per second. Results: We found rate dependences for EABR latency t3 and t5 in the order of 0.19 ms and 0.37 ms, respectively, while ECAP was not affected by rate. Correspondingly, the interpeak intervals' rate dependences for t5-t1, t5-t3 and t3-t1 were of the order of 0.37 ms, 0.18 ms and 0.19 ms. Comparing the EABR amplitudes between the stimulation rates 11/s and 81/s, we found that at 81/s the amplitudes were significantly reduced down: to 73% for A3 and 81% for A5. These rate dependences of latency and amplitude in EABR have characteristics comparable to those of acoustic ABR. Conclusions: These data may serve to provide reference values for EABR and ECAP latencies, interpeak intervals and amplitudes with respect to stimulation rate. Altered response patterns of ECAPs and EABRs to normalised stimulation modes could be used in the future to describe and classify neuropathological processes in a better-differentiated way.

Modified universal new-born hearing screening: Practical approach for developing world (Georgean experience).

Analysis of newborn hearing screening (NBS) outcomes and proposing a modified strategy for better performance of NBS in developing world. Descriptive (retrospective and prospective) study. A total of 8412 newborns/neonates were subjected to risk factor assessment followed by a set of subjective (behavioral audiometry: BoA) and objective (OAE: otoacoustic emissions, ABR: auditory brainstem-evoked responses) hearing-screening in different combinations. DPOAE (primary objective tool) was undertaken in 2565 cases. Predictive value of risk factors on deafness was statistically analysed along with affectivity of objective, subjective and questionnaire-based screening tools. Amongst 8412 babies, 45.5% were 'at risk' (major 36.41%, minor 9.17%). The OAE was abnormal in 299 cases (11.6% of 2565 & 3.5% in 8412). The abnormal OAE rate in prospective cases was 3.5% while in retrospective cases that underwent initial screening with BoA was 41%. A significant correlation was seen with consanguinity, high blood pressure, NICU stay>5 days, low birth weight, neonatal jaundice, delayed birth cry, pre-mature status, birth asphyxia and maternal excessive vomiting, while regression models of OAE outcome (sensitivity 99%, accuracy 89%) revealed highest predictive value for the initial 3 factors. BoA-screening revealed a sensitivity of 72.6%, and negative predictive value of 42.4%. Also a perfect correlation was evident between OAE-ABR, OAE-BoA and ABR-BoA. A subjective NBS screening through questionnaire-based-risk-assessment and modified-BoA followed by selective referral for objective assessment is more practical and focussed approach for poor resourced countries that is likely to screen a larger population.

Comparative study of Automated Auditory Brainstem Response (AABR) and Brainstem Evoked Response Audiometry (BERA) for Hearing Loss Detection in High Risk Infants delivered in Dhulikhel Hospital

Background & Objective: Brainstem evoked response audiometry (BERA) is most specific and sensitive test for brain stem dysfunction. It is most important objective method for evaluating peripheral auditory system in neonates, infants, sedated and comatose patients and other person who doesn’t understand the language. Objective of the study was to evaluate correlation BERA with other audiological tests in different types of hearing loss. Material and Methods: This was a hospital based retrospective observational analytic study that used a cross-sectional approach. The sampling used was consecutive sampling until the minimum size fulfilled from 1st September 2021 to 30th September 2022. All high-risk infants aged 0–3 months who suffer from asphyxia, sepsis, LBW, premature, and hyperbilirubinemia, the baby was stable and transportable, the patient’s parents were willing to participate were taken. Results: Out of 46 high risk infants of 0 - 3 months, male:female ratio of 1.7:1 with mean age of 31 days, the most common high risk factor was low birth weight and hyperbilirubinemia i.e 23(50%). 64% of infants were pass in AABR 2nd test in comparison to 71% pass in BERA test. Conclusion: There was no difference between AABR and BERA results for HL detection in high-risk infants at NICU.

Evaluation of the Brain Stem Auditory Evoked Potential Response among New Borns Post-Recovery after Hyper-Bilirubinemia: An Original Research.

Neurological harm from neonatal hyperbilirubinemia includes loss of hearing and encephalopathy. The current research used the "Brainstem Evoked Response Audiometry (BERA)" test to screen for as well as assess hearing loss in newborns who had recovered from hyperbilirubinemia. A cross-sectional comparative investigation was conducted at a tertiary care center. Fifty neonates were included out of which 25 were healthy and 25 received treatment for the increased bilirubin. Prior to BERA testing, the subjects' ears were examined for any obstruction. Following a conventional lab procedure described, the BERA recordings were carried out after the neonate fell asleep on its own. The data collected were compared for the significance using the ANOVA, keeping P < 0.05 as significant. In comparison to the controls, a large proportion of neonates in cases had BERA wave latencies that were delayed (I-R = 80, L = 84; III- R = 76, L = 84; V- R = 84, L = 88 percentages latencies). The percentage of subjects in whom the latencies was noted for the healthy neonates was lesser than the case group (I-R = 8, L = 24; III- R = 8, L = 8; V- R = 4, L = 12 percentages latencies). Comparable numbers of infants in each group had inter-peak latencies that were lengthy. Subjects in the case group showed that the threshold hearing as per the WHO grade was mild (R = 32, L = 36) and moderate (R = 32, L = 28). Elevated serum bilirubin may cause damage to hearing capability. After hyperbilirubinemia has been completely treated, BERA can detect even the slightest degree of hearing damage. BERA is therefore a useful technique for the quick recognition of hearing impairment in newborns. Early treatment helps in the prognosis so that the neurosensory systems can fully mature, and the patient can lead a quality life.

Comparison of the Components of Brainstem Auditory Responses Using Click and Chirp Stimuli in Premature Infants

Background and Objectives: Prematurity means that the baby is born before 37 months of age, which leads to various problems, including hearing defects. Timely detection of hearing loss with the help of tools that facilitate this identification, can have a significant impact on the child’s personal, social, and educational development. The aim of the present study was to achieve better responses using the chirp stimulus along with the click. The chirp stimulus can compensate for the time delay caused by low-frequency stimuli and increases the simultaneous presentation of high and low frequencies in the cochlea. Therefore, the morphology of the waves is improved, and the amplitude of responses increases; thus, the waves can be better identified despite the prematurity of the infants. Methods: Eighy newborns were examined in four groups, which were divided into term and preterm groups based on gestational age (including late preterm, moderate, and very preterm). The intensity of the click and chirp stimuli was fixed at 40 and 80 dB. The amplitude and latency of waves I, III, and V, the inter-peak latencies of I-III, III-V, and I-V, as well as the ratio of the amplitude of wave V/I were among the compared parameters. It should be mentioned in tables paired t-test was used in cases marked with *. Wilcoxon test was used in other cases. Results: The chirp stimulus mainly had less latency than the click stimulus and created a larger amplitude at a high intensity (80 dB). In the intensity close to the threshold, i.e. 40 dB, these findings were slightly different. The more developed the infants, the stronger the responses and the more effective the chirp stimulus in eliciting responses. Conclusion: The chirp stimulus was more effective in obtaining multiple components of brainstem-evoked responses in infants, especially preterm infants, and investigating the status of their auditory system. The use of a higher sample size could lead to an increase in the growth of clinical use and better efficiency of diagnostic protocols and responses.

Changes in the cochlear and retrocochlear parts of the auditory system in 19-39 and 40-60 years old patients with type 1 diabetes mellitus.

Pathophysiological alterations in the cochlea and functional tests of the auditory pathway support that in diabetes both vasculopathy and neural changes could be present. The aim of our research was to study the differential effect of type 1 diabetes mellitus (T1DM) on two different age groups. Audiological investigation was carried out in 42 patients and 25 controls at the same age groups. Investigation of the conductive and sensorineural part of the hearing system by pure tone audiometry, distortion product otoacoustic emission measurement and acoustically evoked brainstem response registration were evaluated. Among the 19-39-year-old people the incidence of hearing impairment was not different in the diabetes and control groups. Among the 40-60-year-old people hearing impairment was more common in the diabetes group (75%) than in the control group (15,4%). Among patients with type 1 diabetes, the mean threshold values were higher in both age groups at all frequencies although significant difference was in 19-39 years old group: 500-4000Hz right ear, 4000Hz left ear, in 40-60 years old group: 4000-8000 Hz both ears. In the 19-39 years old diabetes group only at 8000 Hertz on the left side was a significant (p<0,05) difference in otoacoustic emissions. In the 40-60 years old diabetes group significantly less otoacoustic emissions at 8000 Hz on the right side (p<0,01) and at 4000-6000-8000 Hertz on the left side, (p<0,05, p<0,01, p<0,05 respectively) was present compared to the control group. According to ABR (auditory brainstem response) latencies and wave morphologies, a possible retrocochlear lesion arose in 15% of the 19-39 years old and 25% of the 40-60 years old diabetes group. According to our results, T1DM affects negatively the cochlear function and the neural part of the hearing system. The alterations are more and more detectable with aging.

Physiological and electrophysiological evaluation of the hearing system in low birth weight neonates treated with cholestin: a cohort study

BackgroundInfections in neonates are mainly caused by sepsis and are the most important complications of low birth weight. In the treatment of these infections, it is common to use broad-spectrum antibiotics such as cholestin. Cholestin can cause changes in the latency of brainstem-evoked response waves. The current cohort study tried to investigate the probability of changes caused by cholestin in LBW neonates and benefiting from two common tests to identify the hearing system in neonates after treatment by cholestin and compared them with a control group.MethodsThe study was an exposure-based cohort, during which hearing damage caused by receiving cholestin was evaluated in 104 low birth weight neonates, in two groups (52 exposure and 52 no-exposure to cholestin). OAE and ABR tests were performed at the age of 3 months to identify complications in two groups and their results were compared.ResultsThe absolute latency of waves I, III, and V in the brainstem evoked response test with an intensity of 80 dB Hl showed a significant difference in the exposure group with the control group. The interpeak latency of the waves as well as the effect of the drug on the gender of the infants did not show significant differences with the control group.ConclusionCholestin did not affect the axonal or synaptic transmission time of the auditory nerve to the brainstem. It is possible that the simultaneous presence of risk factors, such as the use of cholestin, low birth weight, noises in the intensive care unit, and other unknown factors, can be effective in the change of the absolute latency of waves.

888 Artificial Neural Network Analysis of Evoked Auditory Brainstem Responses for Prediction of Active Auditory Brainstem Implant (ABI) Electrodes in Patients With Neurofibromatosis Type II

INTRODUCTION: Auditory brainstem implantation (ABI) is used to provide auditory sensations through electric stimulation of the cochlear nucleus and is a key strategy for hearing rehabilitation in patients with neurofibromatosis type II (NF2). The exact position of the 3 by 7 electrode array with respect to the cochlear nucleus is not known, due to complex anatomy following tumor removal, procedures during surgery, potential distortions to the anatomy, and post-operative array migration, and other factors. METHODS: A retrospective study of a prospectively maintained database of ABI users with evoked brainstem responses was carried out at an academic institution. Multi-class classification was conducted using the multilayer perceptron (MLP) feed forward neural network model (FF-NN) to classify and predict the post-operative active electrodes. RESULTS: 1,177 electrodes were stimulated intraoperatively among 85 patients [females, n = 48 (56.6%)]. ABI was implanted to the right and left side in 44 patients (51.7%) and 43 patients (48.3%) respectively. The mean follow-up of ABI was 29.6 months (range, 18-33.4 months). Notably, the heat map showed that electrodes at the center and the medial end (E9-16) of the array showed higher likelihoods of being active, irrespective of right or left side. The network was trained for 500 epochs with a learning rate of 0.3 and achieved a mean classification of about 92.3%. The open accessible software for predicting the monopolar electrode stimulation postoperatively based on bipolar intraoperative stimulation can be found here at: https://hifla.org/abi CONCLUSIONS: An ideal MLP FF-NN structure was achieved and hence may result in understanding more precisely about the ABI stimulation and bringing consistency among the neurosurgeons and audiologists worldwide.

The Effect of Gestational Diabetes Mellitus on Hearing of Neonates in a Tertiary Healthcare Centre.

Congenital hearing loss is hearing loss present in a child at birth or soon after birth. It is a debilitating condition with the potential for lifelong disability. It is thought to be multifactorial in aetiology with both genetic (autosomal and X-linked) and acquired causes (such as maternal infections, drug intake, trauma). Gestational Diabetes Mellitus (GDM) is a relatively common condition found in pregnant females but is a rather understudied risk factor in terms of congenital hearing loss. GDM is easily treatable which makes the hearing loss due to it easily preventable. (1) Determine correlation between Gestational Diabetes Mellitus and congenital hearing loss in neonates. (2) Calculate the prevalence of Gestational Diabetes Mellitus related congenital hearing loss. A two-step screening process was used for hearing evaluation of neonates with normal mothers (non-exposed) and neonates with mothers suffering from GDM (exposed) through Otoacoustic emission (OAE) and Brainstem Evoked Response Audiometry (BERA). (1) The difference of neonates diagnosed with hearing impairment in exposed and non-exposed group was statistically significant with a p-value of 0.024. OR 2.1538 95% CI 0.6120-7.5796, p < 0.05. (2) Prevalence of hearing loss in neonates of GDM mothers: 13.3%. Through rigorous exclusion of the already known risk factors for congenital hearing loss, Gestational Diabetes Mellitus has been isolated as an independent risk factor for neonatal hearing impairment. We hope to identify additional cases of congenital hearing loss early leading to a decrease in disease burden.

Screening for Hearing Impairment in High-Risk Neonates in a Rural Tertiary Care Center

Introduction: Among the many congenital problems, loss of hearing is one of the most common treatable diseases. Hearing loss in early life can have deleterious effects on the child's psychosocial, scholastic, and social-emotional development. Aims: The aim of this study was to estimate the incidence of neonatal hearing loss in high-risk neonates admitted in a rural tertiary care center. To determine the risk factors predictive of hearing impairment in these neonates. Materials and Methods: About 200 high-risk neonates were included in the study. The first screening by otoacoustic emission (OAE) was conducted before the neonate's discharge from the hospital. If it failed, OAE was repeated after 4 weeks or at the first immunization visit. If it again failed, then Brainstem Evoked Response Audiometry (BERA) was conducted within no more than 3 months. Statistical Analysis Used: It was performed using IBM SPSS Statistics (IBM SPSS Version 28.0. Armonk, NY). The Chi-square test was used to calculate the P value. P &lt; 0.05 was considered statistically significant. Results: In the initial screening, 49 out of 200 high-risk neonates had referred OAE. Out of 49, 20 failed in repeat OAE. Out of these 20 neonates, hearing loss was confirmed in 15 neonates (7.5%) by BERA. The risk factors most commonly associated with hearing loss in the present study were neonatal intensive care unit admission &gt;24 h (7%), hyperbilirubinemia (3%), meningitis (3%), and ototoxic drugs (3%). In the neonates who had hearing loss (15), 93.33% were having more than one risk factor. Conclusions: Our study concludes that there is significant hearing impairment in high-risk neonates. Screening for hearing loss and early identification is needed to modulate the period of neural plasticity, preventing them to banish into a world of social isolation, and educational malaise. The health-care facilities in rural regions have limited settings. Hence, at least a targeted screening is recommended for early detection of hearing loss and rehabilitation.

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 (&lt;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.

A cross-sectional analysis to study the effects of mobile use on brainstem auditory response in young healthy male adults

Background: The intensive use of mobile phones has given rise to adverse effects concerning the potential influences of electromagnetic fields on human health. The ear, being the closest, is the direct recipient of electromagnetic radiation, thus making it the most likely affected organ. The present study assessed the effects of mobile usage on brainstem auditory responses in young healthy male adults using brainstem-evoked response audiometry. Aims and Objectives: The present study aimed to evaluate the effects of long-term repeated exposure to mobile phones on the hearing of young healthy male adults by assessing and comparing absolute wave latencies and interpeak latencies (IPLs) of brainstem auditory responses. Materials and Methods: The cross-sectional study was conducted using the RMS EMG SALUS 2C machine in the Physiology Department of SMS Medical College, Jaipur. This was an analytical type of observational study design incorporating a total of 70 young healthy male adults from SMS Medical College, Jaipur, in which Group I was using mobile for &gt;2 h/day for the duration of &gt;1 year and &lt;5 years and Group II was using mobile for &gt;2 h/day for the duration of more than 5 years. Results: The IPL I–III and I–V of the right ear of Group II was significantly prolonged (P &lt; 0.05) compared to IPL I–III and I–V of the right ear of Group I. Conclusion: This study revealed a significant prolongation of IPLs in the right ear of Group II mobile users associated with duration of usage.

Charcot-Marie-Tooth Disease Type 4C and Autosomal Dominant Heterozygous Ichthyosis Vulgaris, with Bilateral Hearing Loss: A Novel Association with Review of Literature.

A 3-year-old boy, firstborn to nonconsanguineous parents, presented with motor development delay and floppiness of bilateral lower limbs since birth. No significant family history presented at time of check-up. He could stand with support, eat with a spoon without spillage, and speak in two-word sentences. There was no history suggestive of cranial nerve impairment. Examination revealed normal head circumference, dry, scaly skin lesions on the trunk, distal weakness with sluggish deep tendon reflexes in bilateral lower limbs, and a high stepping gait. Nerve conduction studies revealed demyelinating polyneuropathy. Brain stem-evoked response audiometry testing revealed auditory neuropathy. Clinical exome sequencing revealed a known pathogenic variant of 3325C > T in the SH3TC2 gene suggestive of Charcot-Marie-Tooth disease type 4C and ichthyosis vulgaris with a novel variant of 2218C > T in the FLG gene. We have reviewed the available literature for reported associations of Charcot-Marie-Tooth disease type 4C and ichthyosis vulgaris. This is probably the first reported association of Charcot-Marie-Tooth disease type 4C and ichthyosis vulgaris with bilateral hearing loss.

Hearing Assessment in Infants: A Retrospective Institutional Study

Introduction Disorders of hearing are one of the most common disabilities in the country, especially in children. Hearing impairment is the most common congenital disease (3.5–9%) considering all degrees of permanent unilateral or bilateral deafness. Hearing disability can further lead to lifestyle difficulties later on in life. Early identification and a comprehensive treatment can help these people lead a better and productive life. Materials and Methods A retrospective study from the hospital records was done of infants admitted in NICU/PICU in a rural setting in, Davangere, Karnataka from January 2017 to January 2021 who had undergone Oto-acoustic emission (OAE) upon discharge, with a Brainstem Evoked Response Audiometry (BERA) test done in cases that failed the initial test after 3 months. Results Out of the 1115 cases that was included 213(19.1%) cases were found to have hearing disability with low birth weight and hyperbilirubinemia counting for most of the cases with 326(29.2%) and 301(27.0%) cases respectively. Conclusion The findings of our study suggest that an early investigation for hearing disability in high-risk infants for early detection and comprehensive management of hearing disorders. It also points towards a further need for advocating a universal screening of neonates for detecting hearing disability when feasible.