Introduction Of Newborn Hearing Screening Research Articles

Hearing loss in neonates and infants.

Hearing in neonates and infants is crucial for their development of language and communication skills. Unless hearing loss is appropriately managed early, it can cause a significant socioeconomic burden considering its detrimental impact on the child's development and its common nature. It is also the most common congenital sensory deficit, with an approximate incidence of 1.5 per 1,000 newborns. Its etiologies are heterogeneous: genetic causes are reportedly involved in up to 80% of cases, while congenital cytomegalovirus infection is the leading environmental factor contributing to congenital hearing loss. The introduction of newborn hearing screening using automated auditory brainstem response and/or automated otoacoustic emission in many developed countries has helped detect and manage hearing loss early. Current auditory rehabilitation options such as cochlear implantation implementing cutting-edge technologies can treat almost all degrees of hearing loss, emphasizing the importance of early hearing detection and intervention. Rapidly developing genetic diagnostic technologies and future cutting-edge treatment options, including gene therapy, will shed light on the future management of hearing loss in neonates and infants.

The hope and burden of early intervention: Parents' educational planning for their deaf children in post-1960s Australia

PurposeThis article examines the educational decision-making of hearing parents for their deaf children born during a period (1970–1990s) before the introduction of new-born hearing screening in New South Wales, where the study was conducted, and prior to the now near-universal adoption of cochlear implants in Australia.Design/methodology/approachWe present findings from an oral history study in which parents were invited to recall how they planned for the education of their deaf children.FindingsWe propose that these oral histories shed light on how the concept, early intervention – a child development principle that became axiomatic from about the 1960s – significantly shaped the conduct of parents of deaf children, constituting both hope and burden, and intensifying a focus on early decision-making. They also illustrate ways in which parenting was shaped by two key structural shifts, one, being the increasing enrolment of deaf children in mainstream rather than separate classrooms and the other being the transformation of deafness itself by developments in hearing assistance technology.Originality/valueThe paper contributes to a sociological/historical literature of “parenting for education” that almost entirely lacks deaf perspectives and a specialist literature of parental decision-making for deaf children that is almost entirely focussed on the post cochlear implant generation. The paper is distinctive in its treatment of the concept of “early intervention” as a historical phenomenon rather than a “common sense” truth, and proposes that parents of deaf children were at the leading edge of late-20th and early-21st century parenting intensification.

Development and Implementation of a Low-Cost Tracking System after Newborn Hearing Screening in Upper Austria: Lessons Learned from the Perspective of an Early Intervention Provider.

More than one decade after the introduction of newborn hearing screening in Upper Austria, most children were still older than 6 months at enrolment in early intervention. In this study, under the guidance of health authorities, a revised screening and tracking protocol was developed by a network of early intervention providers and representatives of ENT, obstetrics, and pediatrics, including screening professionals and parents of children with hearing loss. Critical process indicators following internationally recommended benchmarks were defined and collected annually by the health authorities. Due to data protection issues, the data collection system was not personalized. Regular network meetings, case-oriented meetings, and screener training sessions were held. As a result, even without additional costs and within the legal constraints related to data protection in Austria, the proportion of children enrolled in early intervention before 6 months of age was significantly increased from 26% to 81% in two representative birth cohorts before and after the introduction of the new protocol, respectively. The coverage for bilateral screening increased from 91.4 to 97.6% of the total number of births.

Translation and validation of the revised version of the LittlEARS® early speech production questionnaire (LEESPQ®), in Dutch-speaking children with normal hearing

Introduction With the introduction of newborn hearing screening, children with different degrees of hearing loss can receive hearing aids and cochlear implants in the early months after birth, which is also the case in the Netherlands and in Flanders, the Dutch speaking part of Belgium. Materials and methods This has intensified the need for a validated questionnaire in the Dutch language to assess the speech development of children under the age of two. The LittlEARS Early Speech Production Questionnaire® (LEESPQ®), which was originally developed and validated in German language, was translated and validated into the Dutch language. Questionnaires of 355 normal hearing children were analysed. Results Total score was highly correlated with age (r = .775) and a normative curve was created. Internal consistency was reached with a high value of α = 0.870, which indicates that the questionnaire almost exclusively assesses speech production ability. The Dutch version of the LEESPQ®, is reliable, consistent and independent of gender or lingual status. As such, the Dutch LEESPQ®, may be a useful tool for language monitoring for children from birth to 18 months of age. Conclusion The Dutch LittlEARS Early Speech Production Questionnaire®, was found to be a reliable and valid tool to assess and monitor the early speech and language production skills in children up to 18 months of age.

Vestibular evaluation in patients with congenital profound hearing loss using ice cold water caloric test and rotational chair test

Introduction: With the introduction of newborn hearing screening, hearing problems are often detected at the neonatal stage and in early infancy, and holistic approach including not only auditory but also vestibular function is needed. This study hopes to establish some corelation between cochlear and vestibular system dysfunction in a patient born with congenital profound hearing loss (CPHL). Materials and Methods: In this observational and descriptive study conducted at a tertiary care hospital, we evaluated eighty patients with bilateral CPHL from 2016 to 2018. After taking a detailed history and thorough ear, nose, and throat examination, the patients underwent vestibular function evaluation by ice cold water caloric test (ICCT) and rotational chair test (RCT) in the outpatient department, and the duration of nystagmus was calculated based on which hypoactive or absent vestibular function was found out. Results: In the present study, out of eighty patients with CPHL, vestibular function of 62 (77.50%) was normal, 9 (11.25%) hypoactive, and 9 (11.25%) with absent vestibular function. Conclusion: In the outpatient department where sophisticated equipment and laboratory facilities are not available, ICCT can be used to determine the vestibular function and also RCT can be used. From the present study, it can be concluded that vestibular dysfunction is present in 22.50% of patients with CPHL with the use of ICCT and RCT. There is a strong possibility of genetic origin etiology for vestibular dysfunction. The present study also concludes that consanguinity may be major etiological factor for vestibular dysfunction.

Hearing loss in children with congenital cytomegalovirus infection: an 11-year retrospective study based on laboratory database of a tertiary paediatric hospital.

SUMMARYCongenital cytomegalovirus infection is considered the main cause of infantile non-genetic neurosensory hearing loss. Although this correlation was described more than 50 years ago, the natural history of internal ear involvement has not yet been fully defined. Hearing loss is the most frequent sequela and is seen in a variable percentage up to 30%; the hearing threshold is characterised by fluctuations or progressive deterioration. The purpose of this study was to evaluate the prevalence of hearing loss in cases of congenital CMV infection from Modena county, starting from the database of the microbiology and virology reference laboratory. All children undergoing urine testing for suspected CMV infection or viral DNA testing on Guthrie Card in the period between January 2004 and December 2014 were enrolled in the study. Family paediatricians were contacted and asked about clinical information on the possible presence at birth or subsequent occurrence of hearing loss, excluding cases where this was not possible. The results showed an annual prevalence of congenital cytomegalovirus infection among suspected cases that was stable over time despite the progressive increase in subjects tested. The prevalence of hearing loss was in line with the literature, whereas in long-term follow-up cases of moderate, medium-to-severe hearing loss with late onset were not detected. The introduction of newborn hearing screening in the county has allowed early diagnosis of hearing loss at birth as non-TEOAE-born births underwent a urine virus test. Moreover, despite all the limitations of the study, we can conclude that European epidemiological studies are needed to better define the relationship between congenital CMV infection and internal ear disease as the impact of environmental and genetic factors is still not entirely clarified.

Bilateral congenital deafness: What investigations should be performed?

The introduction of newborn hearing screening has led to earlier identification of children with congenital sensorineural hearing loss (SNHL). Aetiological clarification offers several benefits. There is currently a lack of agreement on which examinations should be recommended. Descriptive review of the literature reporting investigations performed to establish the aetiology of congenital SNHL and comparison of the management policy in Swiss referral centres. PubMed Search from 1985 to March 2016 with specific search terms; study selection according to inclusion/exclusion criteria; narrative analysis by use of defined criteria and question-naire. Ninety-two studies were finally included in this review. Forty studies investigated more than a single aetiology. Overall frequencies of aetiological parameters investigated were: genetic (47 studies), radiological (35), ophthalmic (35), serological (32), cardiac (25), renal (14), endocrine (12), neurological (8). Most of the studies were retrospective and various limitations such as poor population description, incomplete data or deficiencies in methodological quality were frequently detected. The variability detected in the investigative approach chosen by Swiss referral centres reflects the heterogeneous data seen in the literature. The evidence in the literature regarding an appro-priate evaluation is mostly of low quality and difficult to assess owing to high heterogeneity. Nevertheless, imaging, genetic testing, neuropaediatric and ophthalmological evaluations, electrocardiograms and cytomegalovirus analysis have been identified as examinations to be included in the assessment of children with congenital SNHL. There is a need for international consensus on the various issues of such an evaluation, such as choice of investigations and diagnostic criteria.

Risk Factors for Congenital Hearing Loss: Which Are the Most Relevant?

AbstractIntroduction:Congenital hearing loss is a multifactorial disease that affects mostly premature newborns exposed to certain risk factors. The recent widespread introduction of newborn hearing screening in Tîrgu Mureş, Romania prompted the need for a multidisciplinary centralized study on the subject.Case series presentation:We collected data from 340 neonates born in 2014 who had undergone neonatal hearing screening with otoacoustic emissions. Our focus group consisted of 137 neonates with a gestational age of less than 37 weeks. We collected data on the pregnancy, birth, and perinatal period, and then analyzed the impact of infections, treatments — with or without ototoxic potential, hypoxia, mechanical ventilation, intrauterine malnutrition, and the presence of malformations. Premature neonates showed a significantly higher number of REFER results than those with a normal gestational age. The rates of low birthweight, head circumference, and infant length; low Apgar scores; hypoxia in the perinatal period; the presence of placental pathology, mechanical ventilation, and perinatal infections; and the concomitant potentially ototoxic treatment were significantly higher in the REFER group. The age of the mother, bilirubin levels, and oxytocin use during birth did not prove to be relevant.Conclusions:Known risk factors can cause hearing loss in a considerable number of newborns and mandate a multidisciplinary approach to the problem at hand. The referral of these patients to an ENT specialist and their correct management according to an individualized hearing recovery plan is crucial

Detection Rates of Cortical Auditory Evoked Potentials at Different Sensation Levels in Infants with Sensory/Neural Hearing Loss and Auditory Neuropathy Spectrum Disorder

With the introduction of newborn hearing screening, infants are being diagnosed with hearing loss during the first few months of life. For infants with a sensory/neural hearing loss (SNHL), the audiogram can be estimated objectively using auditory brainstem response (ABR) testing and hearing aids prescribed accordingly. However, for infants with auditory neuropathy spectrum disorder (ANSD) due to the abnormal/absent ABR waveforms, alternative measures of auditory function are needed to assess the need for amplification and evaluate whether aided benefit has been achieved. Cortical auditory evoked potentials (CAEPs) are used to assess aided benefit in infants with hearing loss; however, there is insufficient information regarding the relationship between stimulus audibility and CAEP detection rates. It is also not clear whether CAEP detection rates differ between infants with SNHL and infants with ANSD. This study involved retrospective collection of CAEP, hearing threshold, and hearing aid gain data to investigate the relationship between stimulus audibility and CAEP detection rates. The results demonstrate that increases in stimulus audibility result in an increase in detection rate. For the same range of sensation levels, there was no difference in the detection rates between infants with SNHL and ANSD.

Neugeborenen-Hörscreening – Ergebnisse einer Elternbefragung in Sachsen-Anhalt

Background: In recent years quality assurance has become an essential part of today's health-care system in the wake of the modern patient-oriented quality management. With the statutory introduction of newborn hearing screening (NHS) in 2009, a quality assurance of these early detection methods has become necessary. The aim of the study was to determine patient satisfaction in relation to the NHS in Saxony-Anhalt. Patients/Methods: During the period from November 2013 to April 2014, 394 parents were retrospectively interviewed about their experiences and expectations in relation to the NHS, using a standardised questionnaire. In total, 21 child care centres and 6 paediatric primary care centres from all over Saxony-Anhalt were involved. Results: It turns out that the majority of parents are satisfied with the NHS and 97.7% are in favour of the offer of an NHS. Of the surveyed parents, 69.3% felt the information as sufficient. However, only 66.2% of parents took a closer look at the leaflet issued by the G-BA. In addition, 17.7% of respondents are dissatisfied with the professional competence of the examining staff. Conclusion: The study shows that the general attitude among parents towards newborn hearing screening was very positive. They felt reassured by it although there are some aspects still open to criticism.

Does the introduction of newborn hearing screening improve vocabulary development in hearing-impaired children? A population-based study in Japan

ObjectivePermanent hearing impairment has a life-long impact on children and its early identification is important for language development. A newborn hearing screening (NHS) program has started in Okayama Prefecture, Japan, in 1999 to detect hearing impairment immediately after birth. We aim to examine the effect of this screening program on vocabulary development in pre-school children in a before and after comparative study design. MethodsA total of 107 5-year-old children who graduated from Okayama Kanariya Gakuen (an auditory center for hearing-impaired children) between 1998 and 2011 were enrolled in this study. The pre-NHS group (n=40) was defined as those who graduated between 1998 and 2003, while the post-NHS group (n=67) was defined as those who graduated between 2004 and 2011. The primary outcome was receptive vocabulary, which was assessed by the Picture Vocabulary Test [score <18 (low) vs. score ≥18 (high)]. The secondary outcome was productive vocabulary, or the number of productive words, which was assessed by an original checklist [<1773 words (low) vs. ≥1773 (high)]. We calculated odds ratios and 95% confidence intervals for vocabulary development and compared both groups. ResultsThe adjusted Picture Vocabulary Test score and number of productive words were significantly higher (p<0.01) in the post-NHS group than the pre-NHS group. Odds ratios were 2.63 (95% confidence interval: 1.17–5.89) for receptive vocabulary and 4.17 (95% confidence interval: 1.69–10.29) for productive vocabulary. ConclusionsThe introduction of NHS in Okayama Prefecture significantly improved both receptive and productive vocabulary development in hearing-impaired children.

Analysis of Click-Evoked Auditory Brainstem Responses Using Time Domain Cross-Correlations Between Interleaved Responses

The rapidly evolving field of early diagnostics after the introduction of newborn hearing screening requires rapid, valid, and objective methods, which have to be thoroughly evaluated in adults before use in infants. The aim was to study cross-correlation analysis of interleaved auditory brainstem responses (ABRs) in a wide dynamic range in normal-hearing adults. Off-line analysis allowed for comparison with psychoacoustical click threshold (PCT), pure-tone threshold, and determination of ABR input/output function. Specifically, nonfiltered and band-pass filtered ABRs were studied in various time segments along with time elapsed for ensemble of sweeps reaching a specific detection criterion. Fourteen healthy normal-hearing subjects (18 to 35 years of age, 50% females) without any history of noise exposure participated. They all had pure-tone thresholds better than 20 dB HL (125 to 8000 Hz). ABRs were recorded in both ears using 100 μsec clicks, from 71.5 dB nHL down to -18.5 dB nHL, in 10 dB steps (repetition rate, 39 Hz; time window, 15 msec; filter, 30 to 8000 Hz). The number of sweeps increased from 2000 at 71.5 dB nHL, up to 30000 at -18.5 dB nHL. Each sweep was stored in a data base for off-line analysis. Cross-correlation analysis between two subaverages of interleaved responses was performed in the time domain for nonfiltered and digitally band-pass filtered (300 to 1500 Hz) entire and time-windowed (1 to 11 and 5 to 11 msec) responses. PCTs were measured using a Bekesy technique with the same insert phone and stimulus as used for the ABR (repetition rate, 20 Hz). Time elapsed (≈ number of accepted sweeps/repetition rate) for the ensemble of sweeps needed to reach a cross-correlation coefficient (ρ) of 0.70 (=3.7 dB signal-to-noise ratio [SNR]) was analyzed. Mean cross-correlation coefficients exceeded 0.90 in both ears at stimulus levels ≥11.5 dB nHL for the entire nonfiltered ABR. At 1.5 dB nHL, mean(SD) ρ was 0.53(0.32) and 0.44(0.40) for left and right ears, respectively (n = 14) (=0 dB SNR). In comparison, mean(SD) PCT was -1.9(2.9) and -2.5(3.2) dB nHL for left and right ears, respectively (n = 14), while mean pure-tone average (500 to 2000 Hz) was 2.5 dB HL (n = 28). Almost no effect of band-pass filtering or reduced analysis time window existed. Average time elapsed needed to reach ρ = 0.70 was approximately 20 seconds or less at stimulus levels ≥41.5 dB nHL, and ≈30 seconds at 31.5 dB nHL. The average (interpolated) stimulus level corresponding to ρ=0.70 for the entire nonfiltered ABR was 6.5 dB nHL (n = 28), which coincided with the estimated psychoacoustical threshold for single clicks. ABR could be identified in a short period of time using cross-correlation analysis between interleaved responses. The average stimulus level corresponding to 0 dB SNR in the entire nonfiltered ABR occurred at 1.5 dB nHL, 4 dB above the average PCT. The mean input/output function for the ensemble of sweeps required to reach ρ = 0.70 increased monotonically with increasing stimulus level, in parallel with the ABR based on all sweeps (≥1.5 dB nHL). Time domain cross-correlation analysis of ABR might form the basis for automatic response identification and future threshold-seeking procedures.

人工内耳手術を施行した進行性および遅発性難聴症例の検討

The introduction of newborn-hearing screening has enabled early childhood hearing loss to be diagnosed and increased the number of children undergoing early care. Bilateral hearing loss is found in 0.08% of newborns and children whose hearing loss progresses or onset is delayed account for 4 to 30% of all pediatric hearing impairment. Children with perinatal risk factors tend to have deteriorated hearing or delayed-onset hearing loss in early childhood, necessitating audiometric follow-up. We also are aware of some children without risk factors who develop hearing impairment during infancy or early childhood. Hearing deterioration may be difficult to diagnose objectively, especially in young children, the presence of risk factors must be determines as soon as possible, especially given the lack of hearing management and close examination of children without apparent risk factors. We retrospectively studied children born from April 1998 to March 2007 and undergoing cochlear implantation as of April 2008. Among cases, we focused on 10 whose hearing impairment advanced during infancy -4 with risk factors known before hearing deterioration progressed, and 6 cases thought not to have any risk factors. We detected enlarged vestibular acquaduct in 3 of these 6 cases, and 3 more of whom had no risk factors -2 passing newborn-hearing screening and 1 in whom such screening detected hemilateral hearing loss. Our results underscore the need for early temporal computed tomography for detecting enlarged vestibular aquaduct. Even children with mild or hemilateral hearing loss should undergo audiometric and developmental testing at least every 6 months up to going to elementary school. Children suspected of impaired hearing should undergo thorough hearing tests regardless of newborn hearing-screening results to catch any problems early. Appropriate regular hearing and language development check-up tests must also be developed.

Early intervention for children who are deafblind

Deafblindness (multi-sensory impairment) is a very rare disability causing extreme developmental disadvantage. All areas of development are affected from birth, jeopardising children’s personal development and their early family relationships. Professionals with expert knowledge of deafblindness are scarce because of its low incidence. Other complex, low-incidence disabilities share the problems of global developmental impact and limited community knowledge of conditions.Limited research has been undertaken regarding the benefits of early intervention for children with deafblindness. Effective early intervention is needed for this group, especially as the national introduction of newborn hearing screening will lower the average age of diagnosis. The complexity of the disability means that many professionals are usually involved in delivering intervention, necessitating effective multidisciplinary coordination.In this paper, the effects of deafblindness on development are summarised and the literature on early intervention for children with deafblindness is reviewed, together with an overview of findings from work with children with single sensory impairments and anecdotal evidence from programmes in other countries with well-developed services for children with deafblindness. The review indicates that the provision of early intervention for children with deafblindness should be specialist, coordinated and family-focused. The requirements for children with other complex low-incidence disabilities are likely to be similar.