Early Diagnosis Of Hearing Loss Research Articles

The use of hearing tests to assess otitis media with effusion in children with Down syndrome

BackgroundDown syndrome is associated with an increased risk for otitis media with effusion (OME), a childhood condition in which fluid accumulates in the middle ear, potentially leading to hearing loss. The American Academy of Pediatrics Down syndrome guidelines and the American Academy of Otolaryngology – Head and Neck Surgery OME guidelines recommend hearing testing to assess the hearing status of children with Down syndrome diagnosed with OME. MethodsThrough an Institutional Review Board approved retrospective chart review at Children's Mercy, this project assessed how clinical factors affect the frequency in which children with Down syndrome receive hearing testing after diagnosis of OME. The study included data from all children with Down syndrome between 1 and 8 years old diagnosed with OME in the Down syndrome, general pediatrics, and otolaryngology clinics between 2018 and 2020. Demographics and clinical factors, including clinic setting, were collected. ResultsOf the 124 patients identified, 91.1 % were diagnosed with OME in the otolaryngology clinic and 33.1 % received hearing testing. While most diagnoses occurred in the otolaryngology clinic, a higher proportion of hearing testing at the time of diagnosis occurred in the Down syndrome clinic. This could be explained by the fact that the Down syndrome clinic is a multidisciplinary clinic, where yearly visits include hearing screening. Bivariate analysis using chi-square or Fisher's tests showed that clinic setting had a significant association (p-value <0.001) with hearing testing. However, logistic regression depicted all clinical factors had an insignificant effect on hearing testing at 5 % significance. ConclusionWhile results indicate hearing testing is largely not performed to assess OME early in otolaryngology clinics, they may be used to assess intervention efficacy post-diagnosis. Results point to the importance of Down syndrome clinics in early diagnosis of hearing loss leading to timely referrals to otolaryngology clinics which diagnose and manage OME in children with Down syndrome.

Qualitative Analysis of Newborn Hearing Screening Program in Iran.

Early diagnosis of hearing loss and timely interventions are important to minimize the consequences of this condition, especially for children. This research was conducted to analyze the newborn hearing loss screening program in Iran. This qualitative study was conducted using the content analysis method and based on the CIPP model in 2023. The snowball method was used to recruit a sample with maximum diversity. The criteria for selecting people for interviews included having at least three years of experience in the newborn hearing loss screening program and sufficient knowledge in the field. To ensure the reliability of the results, four criteria proposed by Lincoln and Guba were used. Data analysis was conducted by MAXQDA2022 software. In the current research, using content analysis in the form of the CIPP model, based on the viewpoints of the interviewees (40 people), the management requirements of the newborn hearing loss screening program were categorized into the four main categories of context (texture), input, process, and output. Eight subcategories were identified in the context dimension, four subcategories in the input dimension, seven subcategories in the process dimension, and four subcategories in the output dimension. According to the findings of this research, in order to properly implement the newborn hearing loss screening program, there is a need to conduct pilot studies, need assessments, evidence-based programs, and epidemiological studies and to prioritize services and resources. Also, communication between service delivery levels needs to be improved, and attention should be paid to personnel motivation and screening programs.

A study to evaluate the burden of hearing loss and its correlation with risk factors among high-risk infants at a teaching institution, Jaipur.

Hearing loss is a global issue of hearing disability and early detection and rehabilitation of hearing loss are important for the development of speech and language skills in hearing-impaired infants. There are multiple risk factors that aid in hearing loss but some are potential factors that contribute toward hearing loss in infants. The aim of this study was to assess the burden of hearing loss and its correlation with risk factors among high-risk infants at a teaching institution in Jaipur, Rajasthan. This study was carried out after approval of institutional ethics committee on a total of 320 high-risk infants at RUHS College of medical sciences and associated hospitals. Hearing loss was assessed by brainstem evoked response audiometry (BERA). Statistical analysis of data was done by cross-tabulation analysis with Pearson correlation and quantile regression. Out of 320 high-risk infants, 59.69% of infants had normal hearing, 9.09% Unilaterally hearing impaired, 20.31% were bilaterally mild-moderate hearing loss, and 10.94% had severe-profound deafness. The prevalence of important risk factors viz. hyperbilirubinemia, low birth weight, appearance, pulse, grimace, activity, and respiration score, meconium aspiration, respiratory distress, and ventilation greater than five days were 86%, 58.9%, 40%, 36%, 29%, and 22%, respectively. In high-risk infants, hearing loss is a common hearing disorder. Because of this, early diagnosis of hearing loss gives them the best chance of developing functional speech. Brainstem evoked response audiometry is a simple, reliable, and effective technique for the assessment of auditory functions in infants.

THE STUDY OF PATHOLOGICAL FACTORS OF THE DEVELOPMENT HEARING DISORDERS IN NEWBORNS

We analyzed the development of hearing loss factors in newborns. In pediatric cases, the collection of anamnesis is crucial for the early diagnosis of hearing loss. This anamnesis includes questions concerning the perinatal anamnesis of the child, his birth and the first days of life, as well as the postpartum anamnesis before the onset of symptoms, as well as the family history of hearing loss.

Early diagnosis of hearing loss is very important!

Üsküdar University Vocational School of Health Services organized an online event titled "World Hearing Day".

Children then, adults now: long-term outcomes-performance at 15, 20, and 25 years of cochlear implant use.

Severe to profound sensorineural hearing loss interferes with a child's development at the cognitive, linguistic, academic, and social levels. Since the beginning of the pediatric auditory rehabilitation program through cochlear implantation in the Ear, Nose, and Throat (ENT) Service of the Coimbra Hospital and University Center (CHUC), Portugal, its mentors defended the early diagnosis of hearing loss followed by timely intervention, and this was considered the starting point to optimize (re)habilitation through this method. Three decades or so later, recently we conducted this study to evaluate the performance of patients implanted in the initial phase of the cochlear implantation program. The study aimed to evaluate the performance of individuals with severe to profound congenital hearing loss who underwent pediatric cochlear implantation and have used the cochlear implant for at least 25 years, to analyze the beneficial effect of early intervention in improving performance results. The study sample is composed of 31 individuals with severe to profound congenital hearing loss and no other comorbidities, divided into two groups (Group 1: age at implantation was under 3 years; Group 2: age at implantation was over 3 years). All 31 subjects were evaluated at 15, 20, and 25 years of cochlear implant (CI) use with a comprehensive set of tests. In addition, data were collected regarding the academic level of each participant. The results of both groups were compared to find out if there is an effect of age at implantation on auditory performance, and if there is an improvement in the performance with CI over time (15, 20, and 25 years of use). The results show that there is a positive effect, with statistical significance, of early implantation on auditory performance, and telephone use. In both groups, there is an increase in performance over time, but it tends to stabilize after 20 years of CI use. The results obtained in this work support the importance of early intervention in patients with severe to profound hearing loss who are cochlear implant users and show that CI is an effective and reliable method in the treatment of these patients, contributing to their improved socio-educational integration, and that the benefits last over time.

Attitudes of smartphone‐based hearing screening test use among patients with mild cognitive impairment: A qualitative study

AbstractBackgroundWhile the importance of early diagnosis of hearing loss in older adults with cognitive impairment has been increasingly highlighted, smartphone‐based self‐hearing assessments have become widely adopted due to their low cost, easy‐to‐use, and accessibility. The validity and reliability of this method have been accepted for detecting hearing loss. However, few studies were conducted on this vulnerable population. The purpose of this qualitative study was to explore older adults with mild cognitive impairment’s experiences, perceptions, and attitudes toward digital self‐hearing screening tools.MethodSeventeen patients with mild cognitive impairment (MCI) were recruited from memory clinic in Bangkok, Thailand. Focus groups were conducted to understand self‐perception of hearing problems and their experience after using smartphone‐based hearing screening application. Thematic analysis was used to analyze the data.ResultAll focus groups discussed self‐perception of hearing loss, experience of use, as well as feedback for smartphone‐based hearing screening application. The acceptability and attitude of smartphone‐based hearing test was explored. Individual experiences varied depending on personal factors and lifestyle.ConclusionDigital self‐hearing screening tools emerged as potential method for screening hearing impairment. The findings of this study described opinions, feedback, and factors that contributed to the usage of smartphone‐based hearing screening applications. These findings will inform further developments of this tool based on the personalized needs of individuals with cognitive impairment, with the objective of facilitating the early detection and timely intervention for hearing loss in this population.

The Complexity of Hearing Aid Fitting: Children with Congenital Hearing Loss and Middle Ear Dysfunction.

The early diagnosis of hearing loss (HL) and hearing rehabilitation facilitate language and communication development. Some children exhibit mixed HL due to middle ear effusion (MEE) or acute otitis media (AOM). Mixed HL can affect HL evaluation and hearing aid (HA) fitting. The present study retrospectively evaluated the prevalence of MEE/AOM among children with congenital sensorineural HL (SNHL) who were fitted with HAs and its effect on the HA fitting. Thirty-six HA fittings carried out between 2017 and 2020 at one rehabilitation center were examined. Medical and audiological information was retrieved for children between 6 and 32 months old. The number of appointments and HA fitting times were recorded. Twenty-eight children were included in the study. Eighteen children, in addition to SNHL, had a conductive component resulting from MEE/AOM. The children with these pathologies required significantly more HA fitting sessions and hearing tests, fewer real ear to coupler difference (RECD) measurements and longer HA fitting periods. The findings indicate that a large number of children fitted with HAs have an additional conductive component that makes the fitting process longer. Since early rehabilitation is necessary for language development, otolaryngologists should be aware of the adverse effects of MEE/AOE on the HA fitting process. It is important to inform parents that when there is a conductive component, the HA fitting process may take longer and that treatment by an otolaryngologist is vital. This study stresses the importance of multidisciplinary cooperation for optimal HA fitting.

Management of Bilateral Congenital Sensorineural Hearing Loss with Cochlear Implant Surgery in a child with Developmental Delay: A Case Report

Background : Most people have congenital hearing loss. This type of hearing loss happens during pregnancy. The causes are Prematurity, maternal hyperglycemia, anoxia before birth, genetics, and infectious disorders like rubella transmitted from mother to baby in utero. Early diagnosis of hearing loss in some neonates allows for timely treatment with cochlear implants or hearing aid treatment to support language development. Cochlear implants may enable better speech comprehension in both quiet and noisy situations with the aid of therapy. Case Presentation : We discuss Bilateral Congenital Profound Sensorineural Hearing Loss with Developmental Delay and Cochlear Implant Surgery. This 2.5-year-old female child registered in the ENT department, with the chief complaint of inability to hear by both ears and inability to speak. The child was aged 2.5 years and responded only to loud sounds. An examination and evaluation of a doctor confirmed the diagnosis of bilateral severe hearing loss with developmental delays. Therapeutic Management : Medication was given to a child as per the doctor’s order, Ivf DNS 400ml +Inj. KCL 4ml 8 Hourly, Inj. Cefuroxime 700 Mg Bd, Inj Pan 15mg Od, Inj. Emeset 1.5 Mg Tds, Inj. Neomol 20ml Tds, Inj. Dexa 2 Mg Bd, SypIbugesic Plus 7ml Tds ,Syp. Taxol Dx 3.5 ml Tds Syp. Sinarest 1 Tsp, N/D Otrivin (P) 2 Drops Tds. Child was treated with cochlear implant surgery. After treatment child’s condition improved. Conclusion : Timely intervention can be beneficial to improve the hearing sense and ultimately development of child with Sensorineural hearing loss.

Prevalence of Cognitive Impairment and Its Association With Hearing Loss Among Adults Over 50 Years of Age: Results From a Population-Based Survey in Santiago, Chile

The purpose of this study was to estimate the prevalence of cognitive impairment and explore its association with hearing loss and other sociodemographic and clinical risk factors, using an objective measurement of hearing levels, in adults over 50 years of age. A population-based survey was completed in Santiago, Chile between December 2019 and March 2020. Participants were screened for cognitive impairment using the Short Chilean Mini-Mental State Examination and hearing levels were assessed with tonal audiometry (hearTest). Data on demographic, socioeconomic, and clinical characteristics were collected. A total of 538 persons completed the assessment. The prevalence of cognitive impairment in the 50+ population was 9.3% (95% confidence interval [CI] [5.8, 14.7]). Cognitive impairment was significantly higher in individuals with any level of hearing loss (odds ratio [OR] = 2.19, 95% CI [1.00, 4.80], adjusted for age, sex, education, socioeconomic position [SEP], and head trauma). Subjects with hearing loss and who reported any use of hearing aids (16% of the sample) had a lower risk of cognitive impairment (OR of nonusers 3.64, 95% CI [1.00, 13.28], adjusted for age, sex, education, SEP, and head trauma). Strategies for addressing cognitive impairment should further explore the integration of early diagnosis of hearing loss and the regular use of hearing aids.

Risk Factors for Hearing Loss in High-Risk Neonates at a Tertiary Care Centre in Central India: A Prospective Observational Study

Introduction: Hearing impairment and deafness are global issues that affect 5% of the world’s population, with nearly 34 million children requiring rehabilitation to address their hearing loss (HL). An approximately 10-20 fold higher incidence of audiological risk factors has been noted in Neonatal Intensive Care Units (NICUs), which translates that 2-5% of all newborns in NICUs may be affected. Early diagnosis of HL and intervention can be advantageous from the timely fitting of hearing aids or cochlear implants. Aim: The aim of this study was to evaluate the risk factors for HL in NICUs and Special Neonatal Care Units (SNCUs) at a tertiary care hospital in Central India. Materials and Methods: This prospective, observational, single-centre study was conducted from October 2019 to September 2021 in the Department of Paediatrics at Indira Gandhi Government Medical College and Hospital, Nagpur, Maharashtra, India. A total of 319 neonates at high risk for HL, discharged from NICUs and SNCUs, were included in the study. The association between risk factors like prematurity, low birth weight (LBW), Toxoplasmosis, Rubella, Cytomegalovirus, Herpes Simplex (TORCH) infections, neonatal asphyxia, neonatal sepsis, meningitis, exchange transfusion, assisted ventilation, and HL were studied. The chi-square test was used to assess the association between SNHL and risk factors. Results: The incidence of Sensorineural Hearing Loss (SNHL) was 3.76%. Neonatal asphyxia (p-value &lt;0.01), exchange transfusion (p-value &lt;0.001), TORCH infections (p-value &lt; 0.001), meningitis (p-value &lt;0.001), and assisted ventilation (p-value &lt;0.001) were found to be significant risk factors associated with SNHL. Conclusion: Based on the assessment of risk factors, it was concluded that neonates should undergo hearing screening tests within the first month of life, and a diagnosis should be made by three months of age. This allows specialists to initiate treatment and intervention by six months of age, helping children with impaired hearing avoid the harmful consequences of semantic deprivation.

ASSOCIATION BETWEEN BODY MASS INDEX AND HEARING LOSS : A CROSSSECTIONAL STUDY IN A TERTIARY CARE HOSPITAL.

Hearing loss is a growing important global public health concern. Emerging evidence implicates high BMI as a risk factor for hearing loss. Identication of modiable factors for hearing loss that could be useful to prevent or at least delay the condition. We aimed to assess relation between body mass index and hearing loss and also to asses the parameters closely associated with obesity such as lipid parameters, blood sugars and their association on hearing threshold.In this cross sectional study conducted for a period of 6 months on 460 subjects presented with hearing loss, tinnitus and vertigo. BMI of each individual was calculated and categorized. They were subjected to audiometric evaluation.Overweight (88.1%) and obese participants (85.5%) showed association with hearing loss. Mild and moderate hearing loss was more common in both over weight and obese patients with signicant p value (&lt;0.001). BMI categories with deranged RBS, deranged lipid parameters there was association with hearing loss. Our study demonstrated association was found between obesity and hearing loss.We emphasize that obesity and various risk factors associated with obesity may play role in hearing loss, So early screening of obese subjects may provide early diagnosis of hearing loss.

Hearing Loss in Adults With Alström Syndrome-Experience From the UK National Alström Service.

To characterize the patterns of hearing loss and methods of hearing rehabilitation in the UK national cohort of adults with Alström syndrome. Retrospective review of electronic patient records. UK National multi-disciplinary team (MDT) Alström service held at the Queen Elizabeth Hospital, Birmingham. Forty one adult patients with a diagnosis of Alström syndrome, confirmed via ALMS1 gene sequencing, are under ongoing review within the UK National MDT Alström service. Magnitude and type of hearing loss were analyzed using patients' audiometric data. Deterioration of hearing was calculated using serial pure tone audiograms. Methods of hearing rehabilitation used by patients and potential candidacy for cochlear implantation were analyzed. Of 34 patients with available audiograms, all had sensorineural hearing loss (SNHL). Dual sensory (visual and hearing) loss was present in 32/34 (94%) patients. Hearing deteriorated with advancing age, at 1.23 dB/yr. Severe- profound SNHL was present in 9/34 (26%) cases. Air conduction hearing aids were used in 27/34 (79%) cases, and cochlear implants in 2/34 (5%). Alström syndrome is an ultra-rare genetic disorder with progressive, debilitating multi-system manifestations, including SNHL. The UK National MDT Alström service represents one of the largest reported adult cohorts in the world. SNHL in this group was ubiquitous, showing a rapid decline in hearing with age. Annual audiometric assessment to enable early diagnosis of hearing loss and optimum rehabilitation are paramount to minimize the impact of hearing loss in this condition.

CHIRP AUDITORY STEADY -STATE RESPONSE AS A HEARING SCREENING TOOL IN HIGH- RISK INFANTS

Background:Newborn hearing screening with automated procedures usually uses broad band stimuli, such as click. Studies using the Auditory Steady-State Response (ASSR) that used specific frequency chirps named narrow band CE-chirps have shown obvious diagnostic results. Objective:To assess the value of the binaural multi-frequency chirp ASSR as a hearing screening tool. To compare chirp ASSR and click ABR as a tool for early diagnosis of hearing loss in infants with risk factors for hearing loss.Methodology: Two groups of infants were studied, group I (28 infants with risk factors) with one or more risk factor for permanent hearing loss, group II (ten infants without risk factors). Their age ranges from one to six months. Assessment of middle ear pressure using immittancemetry, and hearing threshold estimation using Click ABR and Chirp ASSR were done. Results: When comparing hearing threshold of [group (I) infants with risk factors], and [group (II) infants without risk factors] it was found that there was no statistically significant difference when hearing was evaluated by using click ABR or Chirp ASSR.By comparing average ASSR (Average thresholds of1000, 2000 and 4000 HZ) threshold with ABR threshold, there was no statistically significant difference. There was high positive correlation between ABR threshold and average ASSR. Conclusion: Chirp ASSR can be used for assessment of hearing thresholds in infants with the same accuracy of Click ABR thresholds, with more advantage perceived by ASSR in the form of frequency specific threshold estimation. The binaural multi-frequency chirp ASSR can be used as a hearing screening tool.

Postnatal hearing loss: a study of children who passed neonatal TEOAE hearing screening bilaterally

Background Universal newborn hearing screening (UNHS) contributes to the early diagnosis of hearing loss. However, not all permanent pediatric hearing impairments can be identified by UNHS. Aims/objective To investigate children who have successfully passed the UNHS, but have later-onset hearing loss at an early stage. Methods UNHS of children, was reviewed retrospectively from databases at Karolinska University Hospital, Sweden. Gender, age, the reason for contact, the first contact and the most recent audiogram, the hearing diagnosis, the degree of hearing loss when they were enrolled in hearing rehabilitation, and the hearing aids they used were analysed. Results 63 children who had passed the UNHS at birth and were diagnosed with a hearing impairment at a later stage were included in the study. The average age was 3.3 and 3.9 years old when the children were diagnosed and were finally enrolled in the hearing habilitation, respectively. The reasons for diagnostic evaluation of a suspected hearing loss at present study are preschool hearing tests at the Child Health Care Centres, parents suspect, and/or delayed speech and language development. Conclusions and significance Our findings suggest that a passed UNHS does not exclude a future delayed onset of hearing loss, particularly in children with risk factors.

Newborn Hearing Screening: Analysing the Effectiveness of Early Detection of Neonatal Hearing Loss in a Hospital in Greece.

IntroductionThe introduction of newborn hearing screening programs (NHSP) has drastically contributed to the early diagnosis of hearing loss (HL) in children, with the prospect of children developing speech as early as possible. This retrospective study aims to present and discuss the preliminary results of the NHSP at the University Hospital of Patras, Greece, highlighting the strengths and weaknesses of the program. The evaluation of the implementation of NHSP is important to confirm the effectiveness of the process and elaborate system failures.MaterialsThe study describes the results of previous data collected from the NHSP in the Rio hospital of Patra and analyzed the conditions of the sample collected. The random sample involved newborns born between November 2018 - December 2020 at the University Hospital in Patra, Greece, which was assessed using transient evoked otoacoustic emissions (TEOAEs). Testing was performed twice per week on Thursday and Friday with a random sample, specifically examining the babies in the hospital these days. From the 2014 newborns assessed, 1491 were healthy neonates, while the other 523 required hospitalization in the neonatal unit.ResultsIn total, there were 2014 live births; 1491 healthy neonates were screened with TEOAEs. Of them, 44 did not pass the first test. After retesting one month later, 31 passed the test, while the other 13 were referred to a hearing centre for further audiological testing with auditory brainstem response (ABR) tests. Two infants never showed up for the follow-up appointment. Of the remaining 11, six infants had normal hearing, three had otitis media with effusion or other conductive HL. The last two infants had HL. Specifically, one had bilateral sensorineural HL greater than 40db, and one had unilateral sensorineural HL greater than 40db.Risk factors were identified in 523 newborns admitted to the unit. The most common risk factors identified were the use of ototoxic drugs, low Apgar scores, and prematurity. Of all the newborns, 491 passed the test the first time, and the rest 32 infants came back 1-2 months after leaving the neonatal unit. All the babies who had failed in the first screening test appeared for the follow-up appointment for the second screening test. Of these, 24 babies passed the test, but eight did not. Of these, four were diagnosed with media otitis with effusion or other conductive HL. Sensorineural HL was identified in the last four babies using ABR tests. In detail, two had unilateral sensorineural HL greater than 40db, while two had bilateral sensorineural HL greater than 40db.ConclusionIn conclusion, we found that for the NHS programs to be effective, they must be implemented long-term and have monetary support. Early diagnosis and cochlear implantation are the keys to excellent outcomes. Cooperation between different specialties and a patient-centred approach will help physicians holistically face neonatal HL. Building trust between the parents and doctor is essential for the program's success and reducing the lost-to-follow-up rate. To run a successful program, trained staff, equipment, and financial support are required. However, the gold standards for the success of the program are proper implementation of the program, close follow-up, strict adherence to the guidelines in the neonatal intensive care unit (NICU), and the early detection and diagnosis of HL.

Study on audiometer calibration at the extended high-frequency range 8–16 kHz

Audiometry is a branch of acoustical measurements that concentrated on testing the devices related to hearing and used in medical part such as audiometer. It is substantial to calibrate the audiometer to assure the measurement traceability and to safe human health. So, it is considered as a major claim. The importance of calibration comes from the request for advanced instrument to test accurately the hearing loss for human, especially for those people who might be at risk due to noise. The conventional audiometry covered from 125 to 8 kHz, while the extended high-frequency (EHF) audiometry above 8 up to 20 kHz, the last-mentioned type may be useful in early diagnosis of hearing loss. The audiometer calibration performed using ear simulator, standard or working microphone, sound level meter, and frequency analyzer according to IEC60645-1. The physical parameters such as sound pressure level, frequency and distortion may be affected by environmental conditions such as temperature change. The gained results observed that the measured value of all the tests performed was affected by temperature variation. There were deviations observed from each performed test’s nominal values in the calibration process as the temperature varies from 16 to 40°C. So, this is a detailed study for the significance, method, and uncertainty estimation for audiometer calibration covering the EHF ranges within the temperature from 16 to 40°C.

Potenciales Evocados Auditivos Continuos

El diagnóstico precoz de la hipoacusia debe llevarse acabo de forma temprana ante las implicaciones que la falta de estimulación auditiva tiene sobre el desarrollo del lenguaje y el habla. Hasta los seis meses las audiometrías conductuales no resultan fiables por lo que a estas edades los recién nacidos son evaluados mediante las Emisiones Otoacústicas (EOAE) y los Potenciales Evocados del Tronco Cerebral (PETC). Sin embargo, estas técnicas no son capaces de derivar información selectiva de la partición coclear. Los Potenciales Evocados Auditivos Continuos (PEAC) son una respuesta cerebral evocada periódica en el tiempo cuya frecuencia permanece constante en amplitud y fase. Con propósitos audiométricos los PEAC tiene varias ventajas sobre los PEATC. Primero a través de procedimientos estadísticos se determina la presencia de respuesta aumentando la fiabilidad y validez de la técnica. Segundo, los PEAC permiten el empleo de tonos específicos en frecuencia. Tercero, los PEAC permiten derivar una información específica en frecuencia de la partición coclear. En este artículo se revisan los principios y fundamentos de esta técnica electrofisiológica.

Molecular alteration in the Gap Junction Beta 2 (GJB2) gene associated with non-syndromic sensorineural hearing impairment.

Non-syndromic sensory neural hearing defect is one of the genetic diseases inherited from parents to offerings. The autosomal recessive form affects a large population worldwide and has become a major concern in the social and professional lives of many people. There are many factors and genes which are involved in hearing loss but the Gap Junction Beta 2 (GJB2) gene which encodes the connexin 26 protein, is a major cause of non-syndromic recessive deafness (NSRD). This study aims to record and analyze GJB2 gene mutations in the hearing-impaired population of North Karnataka, India. In this study, we included 368 congenitally hearing-impaired children from North Karnataka, India, under 18 years of age. After thorough clinical examinations, patient's history and proper audiological results, peripheral blood samples were collected and subjected to genetic analysis. We recorded that 54.8% of the NSRD cases have an autosomal recessive mutation in the coding region of the GJB2 gene. The frequency of W24X (25%) mutation was found to be high in the present study population. From this study we can suggest that, identifying this mutation in new-borns definitely helps in the early diagnosis of hearing loss.

Hearing Loss Effect on the Educational Process in Children and Adolescents

There are about 466 million people (6.1% of global population) with hearing impairment registered in the world according to the data from WHO experts. Their number exceeds 13 million in Russian Federation, and more than 1 million are children. The results of the universal audiological screening program for newborns has shown that 1 child per 1000 newborns is born deaf, another 2–3 children grow deaf during the first years of life. The number of people with hearing impairment will reach 900 million people by 2050 according to the WHO forecasts. Prevention and early diagnosis of hearing loss and rehabilitation of children with such disorders are necessary for avoiding problems with social integration. This review presents the analysis of cognitive abilities development in children and adolescents with different types and degrees of hearing loss with regard to different approaches in their management and rehabilitation.