Brainstem Evoked Response Audiometry Test Research Articles

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.

Study of sensorineural hearing loss in children with type 1 diabetic mellitus and the corelation with the duration of the disease in a tertiary care center

Background: Annual type 1 diabetes cases in children under the age of 15 are estimated to be around 96,000 worldwide. The pathophysiological explanation for diabetes-related hearing loss is speculative. Usually, hearing loss is bilateral, gradual onset, affecting higher frequencies. It is a well-known entity in type 2 diabetic mellitus. This study aims at knowing the prevalence of hearing loss in children with type 1 diabetic mellitus with respect to duration of diabetic mellitus. Materials and Methods: A hospital-based comparative study was conducted involving 60 diabetic children and 60 healthy (control) children. Brainstem evoked response audiometry test was done in both groups. Reports were compared and prevalence of sensorineural hearing loss in diabetic children and the corelation with the duration of disease were analyzed. Results: The mean age among cases was 11.93±2.9 years and the mean age among controls was 9.08±1.92 years. Among diabetic group, 25 (41.7%) were male, 35 (58.3%) were female. Among non-diabetic group, 33 (55%) were male, 27 (45%) were female. Although none of the diabetic children had hearing loss, the differences in absolute latency waves I, II, III, IV, and V between diabetic group and non-diabetic group in the right ear and waves I and II and III and V between diabetic group and non-diabetic group in the left ear was found to be statistically significant (p<0.05). The difference in interpeak latency values of wave I and wave V between diabetic and non-diabetic group in right ear and between wave I–V and wave III–V in the left ear was found to be statistically strongly significant (p<0.001). This indicates prolonged absolute latency and interpeak latency among diabetics than non-diabetics. Since none of the diabetic children had hearing loss, the corelation of hearing loss with the duration of the disease was unable to obtain. Conclusion: This study stresses on the need for frequent follow-up and hearing evaluation of the type 1 diabetic children for the early detection of hearing loss.

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.

Knowledge Regarding Interpretation of Brain Stem Evoked Response Audiometry (BERA) Report by Speech -Language Pathologists

According to ASHA assessment of hearing loss is part of scope of practice &amp; service delivery area of SLP’s with Brain Stem Evoked Response Audiometry tests being an effective tool. Keeping in view dearth and disparities in the knowledge and professional abilities of SLPs, need of early identification and intervention for hearing impaired population and importance of BERA test and gap in research current study was conceived. Objective: To determine the knowledge regarding interpretation of Brain Stem Evoked Response Audiometry (BERA) report by Speech-Language Pathologists. Methods: This Cross-Sectional study was conducted at Riphah International University, Pakistan from January, 2022 to June, 2022. Study using convenience sampling recruited N=102 speech language pathologists of both genders, aged 25 to 50 years, working in private and public hospitals, special education schools, private clinics, Universities and Rehabilitation Centers of Rawalpindi and Islamabad. A self-developed questionnaire was used for data collection and analysis performed using SPSS Version-20. Results: Knowledge of interpretation of BERA report by Speech and language Therapists/ Pathologist in current study revealed a total mean score of 40.78 ± 7.03, indicating moderate level of knowledge of interpretation of BER. Knowledge of interpretation of BERA is not associated with gender, age and experience, however it revealed significant associated with qualification of SLP. Conclusions: It was concluded from the result of present study that the majority of speech and language Pathologists have moderate level of knowledge regarding interpretation of BERA. Knowledge of interpretation of BERA is associated with qualification of SLP.

Audiological evaluation of the cochlear nerve with brainstem evoked response audiometry in patients with COVID-19.

In this clinical study, it was aimed to prospectively evaluate the cochlear nerve with brainstem evoked response audiometry (BERA) in terms of audiological ailments in patients with COVID-19. Although the relationship of COVID-19 with tinnitus and hearing loss has been investigated since the day this infectious respiratory disease emerged, its relationship with BERA has not been fully demonstrated from a neurological perspective. It was carried out on a group of patients who had COVID-19 in the last 6 months between February and August 2021 in Diyarbakır Gazi Yaşargil Training and Research Hospital. Patients between the ages of 18-50, who applied to the otorhinolaryngology and neurology clinic and had COVID-19 in the last 6 months, were selected. The COVID-19 group of our study consisted of 30 patients, 18 males and 12 females, who had had COVID-19 disease in the last 6 months, and 30 healthy individuals, 16 males and 14 females, as the control group. In patients with COVID-19, the evaluation of the destruction of the cochlear nerve with BERA showed that there was a statistically significant prolongation in I-III and I-V interpeaks at 70, 80 and 90 db nhl. Statistically significant prolongation of especially I-III and I-V Interpeaks in BERA showed that COVID-19 has the potential to cause neuropathy. We believe that the BERA test should be considered in the neurological evaluation of cochlear nerve damage in patients with COVID-19 as a differential diagnosis.

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.

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.

Comparison of Otoacoustic Emission (OAE) and Brainstem Evoked Response Audiometry (BERA) in High Risk Infants and Children under 5Years of Age for Hearing Assessment in Western India: A Modification in Screening Protocol.

There are very few studies from India, which have compared Otoacoustic Emission (OAE) and Brainstem Evoked Response Audiometry (BERA) as a screening modality for detection of hearing loss in children. With the aim of establishing some guidelines regarding the protocols for hearing loss assessment and preventive measures, the present study has been undertaken to compare OAE with BERA done simultaneously, in the diagnosis of paediatric hearing loss, and also to study associated risk factors for hearing loss in children of Rural Central India. Prospective observational study was carried out on 100 children (200ears) in age group of 0-5years. Selection was based on the inclusion and exclusion criteria. In all the 100 children detailed history was taken from the parents and were subjected to distortion product otoacoustic emissions (DPOAE). Irrespective of the pass or refer result children were subjected for BERA test. The interpretation of OAE and BERA test was as follows. Both the results of OAE refer and BERA fail were considered as confirmed HL, OAE pass and BERA fail were considered as children having Auditory Neuropathy (AN), OAE refer and BERA pass were considered as children at risk of permanent hearing loss (HL), OAE pass and BERA pass were considered as children with no evidence of HL. In the present study the male to female ratio was 1.32:1. Of the total 100 children 80% children showed presence of any one or more than one risk factors. In our study, eclampsia [7%] followed by multiparity [6%] and oligohydramnios [5%] were the most common risk factors in prenatal period. Maximum number of infants in AN profile were with Low Apgar score, children exposed to ototoxic medications, non-syndromic cardiac disorders in children [25.8% each]. Maximum number of infants in Confirmed HL profile were with congenital syndromes/ear anomalies [41.86%] followed by other risk factors. In our study, both OAE and BERA test were comparable and statistically significant with p value of 0.0001. OAE has a high specificity and positive predictive value of 93.33% and 97.22% respectively and it has a low sensitivity and negative predictive value of 67.74% and 45.65% respectively. In a developing country like India were universal screening protocols are not followed large number of children may be missed and may present late when it affects child's communication abilities. Hence, we need to modify our screening test and implement high risk screening even in the absence of any hearing or speech complaints.

Hearing Screening of Newborns using Distortion Product Otoacoustic Emissions

Introduction The present study was devised to estimate the prevalence of neonatal hearing loss and document the importance of using DPOAE as a screening tool for identifying hearing loss in newborns. Materials and Methods This hospital based descriptive study was conducted from August 2018 to August 2019. A total of 928 newborn babies were included in the study. These newborn babies were subjected to hearing screening by distortion product otoacoustic emissions (DPOAE) at 24-72 hrs after birth. For pass cases, no further testing was done. For refer cases, repeat testing with DPOAE was done within 15-30 days. Newborns with refer result on repeat DPOAE testing were subjected to Brainstem evoked response audiometry (BERA) within 3 months to confirm hearing loss. Results Nine hundred and twenty eight newborn babies were screened by DPOAE. 851 newborns passed the first DPOAE hearing screening and 77 newborns gave refer result. 21 newborns were lost to follow-up. 56 newborns underwent repeat DPOAE testing and 5 newborns were referred for BERA. Amongst the 5 newborns who underwent BERA testing, one newborn was diagnosed with bilateral profound hearing loss. Hence, the prevalence of hearing loss of 1.08 per thousand newborn babies was estimated in this study. Conclusion Hearing screening of newborns using DPOAE followed by BERA in refer cases to confirm hearing loss is useful for early detection followed by timely intervention and rehabilitation.

Frequency of Sensorineural Hearing Loss among Children with Pyogenic Meningitis

Aim: To find out the frequency of sensorineural hearing loss among children with pyogenic meningitis.&#x0D; Study Design: Cross-sectional.&#x0D; Place and Duration of Study: Study was conducted at Ziauddin university hospital during the period of July 2019 to February 2020.&#x0D; Methodology: About 96 participants were enrolled in the study between the age group of one month up to 12 years. Patients were included on the basis of clinical presentation and laboratory findings of pyogenic meningitis. An expert audiologist performed the brainstem evoked response audiometry test before discharging the patient from the hospital. For data analysis SPSS version-20 was used. All the quantitative variables were calculated as mean with standard deviation while qualitative data were presented as frequency and percentages. To find out association between variables, the Mann Whitney U-test and chi-square test was applied while P-value ≤0.05 was considered as significant.&#x0D; Results: Mean age with standard deviation was 6.8 ±2.3. Majority of the study participants were boys (57%). The frequency of sensorineural hearing loss was 17%. It was more among females than their male counter parts that was 64.7% and 35.3% respectively but no significant association was reported. The younger age group was having higher frequency of sensorineural hearing loss (47.1%), followed by the age group of 6-8 years (29.4%) and the very small number of participants were affected from the age group of 9-12 years (23.5%) but all the age groups were having no significant association with frequency of hearing loss.&#x0D; Conclusion: It can be concluded that sensorineural hearing loss is the most common complication reported among the children with pyogenic meningitis in current setup so there is a need of early evaluation of hearing problems in all patients diagnosed with pyogenic meningitis.

Frequency of sensorineural hearing loss in children with bacterial meningitis.

Objectives: To determine the frequency of sensorineural hearing loss (SNHL) in children with bacterial meningitis. Study Design: Cross Sectional study. Setting: Department of Paediatric Medicine Nishtar Hospital, Multan. Period: October 2019 to March 2020. Material &amp; Methods: The study was conducted after permission from ethical committee. A total of 151 patients admitted with bacterial meningitis were enrolled. Apart from the presenting complaints, necessary information like the age and gender were noted. Examination of the child was done to assess the condition of child. Brainstem evoked response audiometry (BERA) test was performed before discharge of the patient from the hospital. The outcome variable that is SNHL in bacterial meningitis assessed by BERA test was noted in a specifically designed Performa. Results: Out of total of 151 patients, males were 83 (55%) while females 68 (45%). Overall mean age was 11.85+6.12 months. SNHL was found in 26 (17.2%) children. There was no significant effect of age or gender on the frequencies of SNHL. Conclusion: SNHL is a frequently encountered complication in our population of children with bacterial meningitis. SNHL should be carefully sought out and properly diagnosed in all the patients presenting with bacterial meningitis.

Screening Programs for Hearing Assessment in Newborns and Children.

ObjectiveIn this study, we aimed to assess the incidence of hearing loss in the pediatric population through otoacoustic emission (OAE) and brainstem evoked response audiometry (BERA) and to analyze the possible etiological factors responsible for it.Material and methodsA retrospective observational study was conducted in the Otolaryngology (ENT) and Gynecology and Obstetrics Departments at the Jinnah Postgraduate Medical Centre and National Institute of Child Health in Karachi, Pakistan between July 2019 and October 2019. The convenient sampling technique was used to select the patients. The final sample size consisting of newborns and children was 108. Initially, screening procedures were undertaken for newborns to detect permanent or fluctuating, bilateral or unilateral, and sensory or conductive hearing loss, averaging 30-40 dB or more in the frequency region, which indicated potential issues related to speech recognition (approximately 500-4,000 Hz). The screening of newborns involved the use of non-invasive, objective physiologic measures that included OAEs and/or auditory brainstem response (ABR). The children with hearing impairment then underwent BERA; thereafter, further investigations were performed to confirm the defects found on BERA testing.ResultsOf the 108 cases, 96 had normal hearing on OAE screening, and 12 were found to have hearing loss on the OAE test. Further testing was carried out on BERA for 12 cases that had been detected to have hearing loss on OAE, and BERA showed normal hearing for five cases whereas seven were found to have hearing loss. Of the seven patients with hearing loss on the BERA test, five were diagnosed with cochlear deafness, and two had retrocochlear deafness.ConclusionOur present study concludes that in order to avoid any hearing problems in infants, OAE hearing screening and diagnostic BERA screening programs should be carried out in all the hospitals of Pakistan to assess newborn hearing at an early age.

Assessment of Hearing Impairment in NICU Graduates by Otoacoustic Emission and Brainstem Evoked Response Audiometry Tests

Objectives: To find out the incidence of hearing impairment in Neonatal Intensive Care Unit (NICU)-admitted newborns and to correlate between several risk factors. Design: Prospective observational study. Setting: In a tertiary teaching hospital of Odisha between October 2014 and October 2016. Study population: 100 newborns delivered in the institution and admitted in NICU with usual indications and risk factors that underwent otoacoustic emission 1 and 2 (OAE1, OAE2) and Brainstem Evoked Response Audiometry (BERA). Results: 84 babies in OAE1 and 86 babies in OAE2 got “pass” results. In BERA test, 92 babies got normal waveform. 7 (13.7%) of 51 premature babies, 5 (38.5%) of 13 very low birth weight babies, 7 (17.5%) of 40 babies receiving ototoxic medication for &gt;5 days, 7 (31.8%) of 22 babies with hyperbilirubinemia, 6 (42.9%) of 14 babies with sepsis, 5 (20%) of 20 babies with birth asphyxia, 3 (60%) of 5 babies under mechanical ventilation for &gt;5 days, 1 baby with ear abnormality, and 1 (50%) out of 2 babies with congenital infections had hearing impairment and statistically significant association with abnormal OAE and BERA test. Conclusion: There is high incidence of hearing loss in NICU-admitted newborns. OAE and BERA both should be performed for complete evaluation of hearing. Important risk factors are elaborated. Predischarge hearing assessment in NICU is most important.

Effects of intrauterine latent iron deficiency on auditory neural maturation in full‐term newborns

ObjectiveThis 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. MethodA 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 75ng/mL, and the latent iron deficiency group (n=38) with ferritin between 11 and 75ng/mL. Results were presented as mean±standard deviation. Statistical analysis was performed using GraphPad prism7 and SPSS with a significance level of 5%. ResultsA 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. ConclusionIron 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.

A study of brainstem evoked response audiometry in children with severe hearing loss

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Brainstem evoked response audiometry (BERA) is a non-invasive diagnostic tool which can be used to assess the early hearing loss. The objectives of the study were to find out the risk factors for severe hearing loss in children and to evaluate the role of BERA in early diagnosis of severe hearing loss in children.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; The present hospital based cross sectional study was conducted on 105 children suffering from severe hearing loss. Risk factors of hearing loss was assessed in these children and brainstem evoked response audiometry was performed. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Out of 105 children studied risk factors for hearing loss were present in 69 cases (65.71%) in which several cases had multiple risk factors. History of prolonged stay at NICU was present in 23 cases (21.9%). 11 (10.5%) cases had suffered from meningitis while history of cerebral malaria was present in 2 cases (1.9%). History of cerebral palsy was present in 5.7% cases. The family history of hearing loss was present in 15 patients (14.3%). Bilateral severe hearing loss was present in 76 cases (72.4%) while in 13 cases (12.4%) there was bilateral severe to profound hearing loss assessed using BERA test.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Early detection and timely intervention can not only help prevent this silent handicap of deafness but also contribute to social and economic productivity of a community.&lt;/p&gt;

Effects of intrauterine latent iron deficiency on auditory neural maturation in full-term newborns

ObjectiveThis 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. MethodA 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 75ng/mL, and the latent iron deficiency group (n=38) with ferritin between 11 and 75ng/mL. Results were presented as mean±standard deviation. Statistical analysis was performed using GraphPad prism7 and SPSS with a significance level of 5%. ResultsA 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. ConclusionIron 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.

Correlation between specific rubella immunoglobulin G (Ig G) and response evoked brainstem audiometry examination on confirmed congenital rubella syndrome (CRS) patients

Congenital rubella syndrome (CRS) is a health problem that can cause deaf child. Its diagnosis is based on clinical manifestation, specific rubella Immunoglobulin M (IgM) and IgG examination. The aim of this study was to evaluate the correlation between the specific rubella IgG level and the brainstem evoked response audiometry wave V. This was a cross sectional study involving congenital rubella syndrome (CRS) child patients at Dr. Sardjito General Hospital, Yogyakarta from 2011 to December 2016. The inclusion criteria were 1) aged less than 24 months; 2) wave V of brainstem evoked response audiometry examination has been identified. The exclusion criteria were 1) the patient’s parents refused to participate and 2) incomplete data. Based on α: 5%, β: 20% and coefficient correlation estimation 0.04, minimal sample size were 47 subjects. All of subjects was underwent specific rubella IgG examination. Statistical analysis by using Spearman`s correlation test. Wave V at the level 81 db in 32 (68.1%) patients. A significant positive linear correlation between IgG levels (4- 400 IU/mL) and wave V of brainstem evoked response audiometry was reported (p<0.05; r = 0.432). In conclusion, there is a positive correlation between specific rubella IgG level and brainstem evoked response audiometry test results.

Evaluation of SON’OR&amp;lt;sup&amp;gt;&amp;amp;copy;&amp;lt;/sup&amp;gt;, a Medical Device for Provoked Otoacoustic Emissions and Brainstem Evoked Response Audiometry Made in Cameroon

Background: There is a huge gap in the audiological care in Africa by comparison with Western countries. Its main reason is the prohibitive cost of the medical devices used to screen or diagnose patients. A Cameroonian team tackled this problem by developing a medical device (SON’OR©) which integrates a new instrumentation amplifier structure dedicated to perform otoacoustic emissions (OAE) and brainstem evoked response audiometry (BERA). The major technical challenges to perform OAE and BERA are the synchronization and the amplification of signals of very low amplitude. In this work, we emphasize on the general criteria necessary and indispensable to achieve an optimal amplification. The application of a novel instrumentation amplifier structure characterized by its optimized noise factor in the case of BERA and OEA emissions provides simulations and experimental results fully in line with forecasts. The design of SON’OR© is based on general techniques of electronic instrumentation to which we associated the new instrumentation amplifier structure. Objective: To report the clinical evaluation of SON’OR© as a screening and diagnostic tool. Methods: We conducted a cross sectional comparative study in Centre Hospitalier d’Essos in Yaounde. We tested SON’OR© on two sets of subjects, one for OAE with OTODYNAMICS Echoport ILO 292-II as gold standard and the other for BERA with NEUROSOFT NEURO AUDIO© as gold standard. Each patient was tested with both devices and then we studied the inter device differences and calculated the sensitivity, specificity, positive predictive value and negative predictive value for each test. Results: We got 52 subjects for OEA and 51 for BERA testing. Sex ratio was 1 woman for 2 men in both groups. Mean age was 24.86(SD = 10.53) and 26.33 (10.55) for OAE and BERA groups respectively. SON’OR© had good performances, showing sensitivity of 92.85%/95%, specificity of 96.77%/90.47%, positive predictive value of 95.21%/93.44% and negative predictive value of 95.23%/92.68% for OAE/ BERA respectively. Conclusion: SON’OR© has good characteristics as a medical diagnostic tool. Furthermore its stability and performances in poor electrical conditions make it a robust device really suited for resource limited settings.

Hearing assessment in high-risk neonates by brain stem evoked auditory response (BERA) at a teaching hospital in rural Telangana

Introduction: Severe pre-lingual hearing impairment has important consequences for language acquisition, communication, and cognitive, social and emotional development. Indeed evidence is increasing that even moderate hearing loss in very young children can be detrimental. Brainstem evoked response audiometry (BERA), when used and interpreted properly, provide a powerful method of obtaining reliable estimates of auditory sensitivity in infants, young children, and other individuals who cannot or will not provide reliable results on behavioral hearing tests. Materials & Methods: In this study 110 high risk neonates having one or more risk factors, according to the criteria stated by American Academy of Pediatrics, JCIH 2007 were selected from Newborn unit, of MIMS, Ghanpur. The high risk neonates were subjected to BERA testing in a sound proof room in our study, 30 dB is taken as cutoff for normal auditory threshold. Results: Mean birth weight of neonates studied was 2.60±0.67 kg. Mean age of neonates at which first BERA done is 19.32±11.3 days. Hyperbilirubinemia requiring Exchange transfusion and TORCH infection were identified as significant risk factors with p-values 0.028 and 0.011 respectively. Conclusion: Hearing impairment is common in high risk NICU graduates. Bilateral hearing impairment is more common than unilateral. All high risk infants must be screened for hearing impairment prior to discharge and retesting of infants with abnormal BERA after 3 months and subsequently if necessary. Newborn screening is mandatory to identify hearing loss in the prelinguistic period to reduce the burden of handicap in the community