Universal Newborn Hearing Screening Program Research Articles

Lessons from LOCHI.

Lessons from LOCHI.

A conceptual framework for rationalized and standardized Universal Newborn Hearing Screening (UNHS) programs.

Congenital hearing loss is the most frequent birth defect. The American Academy of Pediatrics and the Joint Committee on Infant Hearing established quality of care process indicators for Universal Newborn Hearing Screening starting from 1999. In a previous systematic review of Universal Newborn Hearing Screening studies we highlighted substantial variability in program design and in reported performance data. In order to overcome these heterogeneous findings we think it is necessary to optimize the implementation of Universal Newborn Hearing Screening programs with an appropriate application of the planning, executing, and monitoring, verifications and reporting phases. For this reason we propose a conceptual framework that logically integrates these three phases and, consequently, a tool (a check-list) for their rationalization and standardization.Our paper intends to stimulate debate on how to ameliorate the routine application of high quality Universal Newborn Hearing Screening programs. The conceptual framework is proposed to optimize, rationalise and standardise their implementation. The checklist is intended to allow an inter-program comparison by removing heterogeneity in processes description and assessment.Electronic supplementary materialThe online version of this article (doi:10.1186/s13052-016-0223-1) contains supplementary material, which is available to authorized users.

Improving regional universal newborn hearing screening programmes in Italy.

SUMMARYThe Universal Newborn Hearing Screening (UNHS) programme aims at achieving early detection of hearing impairment. Subsequent diagnosis and intervention should follow promptly. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for early Identification, Intervention and Care of Hearing Impaired Children", the limitations and strengths of current UNHS programs in Italy have been analysed by a group of professionals working in tertiary centres involved in regional UNHS programmes, using SWOT analysis and a subsequent TOWS matrix. Coverage and lost-to-follow up rates are issues related to UNHS programmes. Recommendations to improve the effectiveness of the UNHS programme have been identified. The need for homogeneous policies, high-quality information and dissemination of knowledge for operators and families of hearing-impaired children emerged from the discussion.

Aetiologic diagnosis of hearing loss in children identified through newborn hearing screening testing.

SUMMARYWith the implementation of universal newborn hearing screening (UNHS) programmes and early diagnosis and treatment of hearing problems, the need has clearly emerged to implement and carry out a systematic and coordinated protocol for the aetiological diagnosis of permanent hearing impairment (PHI). Within the framework of the Italian Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for early Identification, Intervention and Care of Hearing Impaired Children", it has been decided to consider the problems relative to aetiological diagnosis of child PHI within UNHS programmes. The specific objective was to apply a shared diagnostic protocol that can identify the cause in at least 70% of cases of PHI. For this part of the project, four main recommendations were identified that can be useful for an efficient aetiological diagnosis in children affected by PHI and that can offer valid suggestions to optimise resources and produce positive changes for third-level audiologic centres.

An international partnership analysis of a cohort of Vietnamese children with hearing impairment

Aim: A better understanding of the hearing disability situation would advance hearing health care in the developing world. Vietnam is a lower middle income country with hearing health care in its early stages of development. This study examined the situation (degree, types, and causes) of hearing impairment, the effects of age and gender on hearing impairment, and the age of identification and its relationship with degree of hearing impairment, in a sample of Vietnamese children with hearing loss.Method: Sixty-nine participants aged from 20 to 129 months with prelingual-onset hearing loss were assessed during Global Foundation For Children With Hearing Loss international partnership activities. Data on hearing assessment findings, including case history and pure-tone audiometric outcomes, were analyzed.Results: Results showed that the hearing loss of participants ranged from moderate to profound levels (mean pure-tone average in better ear = 95.9 dB HL, range = 51.7–125.0 dB, SD = 17.37). Sensorineural pathologies accounted for 80% of identified hearing loss. Maternal rubella was the most commonly identified cause of hearing loss, with this disease reported as confirmed in 32% of children and suspected in another 13% of the participants. Genetic factors were suspected in at least 13% of children. Age and gender showed no statistically significant associations with degree of hearing impairment. The average age of identification was 23.2 months (range = 2–68 months, SD = 12.82) and a greater proportion (65%) of children with profound hearing loss than those with milder degrees of hearing loss were noted.Conclusion: To reduce the prevalence of childhood hearing loss and its adverse long-term effects, improved immunization programs against infectious diseases, further development of universal newborn hearing screening and early intervention programs, and greater access to hearing technology in Vietnam are highly recommended.

Influência dos indicadores de risco nas diferentes etapas da Triagem Auditiva Neonatal

RESUMO Objetivo Determinar os indicadores de risco para deficiência auditiva, que afetam as diferentes etapas de avaliação de um programa de triagem auditiva neonatal universal. Métodos Estudo retrospectivo longitudinal de triagem auditiva neonatal, realizado em 832 neonatos nascidos em hospital público terciário, no período de janeiro a dezembro de 2012. O exame de Emissões Otoacústicas Evocadas por Estímulo Transiente foi realizado na primeira avaliação auditiva de todos os neonatos. Nos casos de “falha” na primeira avaliação, foi aplicado um reteste e, quando houve a permanência da “falha”, foi realizado o Potencial Evocado Auditivo de Tronco Encefálico (PEATE). Os neonatos com indicador de risco para deficiência auditiva realizaram o PEATE independente do resultado das emissões otoacústicas. Resultados A presença de, ao menos, um indicador de risco para deficiência auditiva, associado ou não a malformações craniofaciais, síndromes genéticas e peso menor que 1500 g ao nascimento, aumentaram significativamente as chances de “falha” na avaliação por emissões otoacústicas. Meningite e malformações craniofaciais aumentaram de maneira expressiva as chances de PEATE alterado. Dois neonatos com emissões otoacústicas normais apresentaram diagnóstico de neuropatia auditiva. Conclusão A malformação craniofacial é um indicador fortemente associado ao diagnóstico da surdez, independente de a triagem auditiva ter sido realizada por emissões otoacústicas ou por PEATE em suas diferentes etapas, o que justifica o monitoramento contínuo e sistemático do serviço de triagem, na busca da melhoria da qualidade do programa de saúde auditiva do neonato.

Science, Not Philosophy, Will Help Deaf and Hard-of-Hearing Children Reach Their Potential

Survival, developmental outcomes, and quality of life have been transformed over the past 30 years for infants suffering a wide range of previously devastating conditions. Children affected by prematurity, cystic fibrosis, and congenital heart disease now survive and indeed thrive into adulthood. Technical advances have proceeded alongside coordinated programs of multicenter registries and trials, driven by clinicians and researchers motivated to not only continually push the boundaries of postdiagnosis treatment but to show that children reap the benefits. The past 30 years have also heralded astonishing advances for children born deaf and hard of hearing. The first pediatric cochlear implantation in 1985 was followed by the discovery of the first of many genes for nonsyndromic hearing impairment1,2 in the 1990s. Widespread implementation of universal newborn hearing screening programs in the early 2000s means that hearing impairment is now routinely diagnosed in the first 3 postnatal months. For the first time in history, we have the opportunity to learn how to prevent language delay, so that … Address correspondence to Melissa Wake, MD, Centre for Community Child Health, Royal Children’s Hospital, Flemington Rd, Parkville 3052, Australia. E-mail: melissa.wake{at}rch.org.au

Speech Recognition and Parent Ratings From Auditory Development Questionnaires in Children Who Are Hard of Hearing.

Progress has been made in recent years in the provision of amplification and early intervention for children who are hard of hearing. However, children who use hearing aids (HAs) may have inconsistent access to their auditory environment due to limitations in speech audibility through their HAs or limited HA use. The effects of variability in children's auditory experience on parent-reported auditory skills questionnaires and on speech recognition in quiet and in noise were examined for a large group of children who were followed as part of the Outcomes of Children with Hearing Loss study. Parent ratings on auditory development questionnaires and children's speech recognition were assessed for 306 children who are hard of hearing. Children ranged in age from 12 months to 9 years. Three questionnaires involving parent ratings of auditory skill development and behavior were used, including the LittlEARS Auditory Questionnaire, Parents Evaluation of Oral/Aural Performance in Children rating scale, and an adaptation of the Speech, Spatial, and Qualities of Hearing scale. Speech recognition in quiet was assessed using the Open- and Closed-Set Test, Early Speech Perception test, Lexical Neighborhood Test, and Phonetically Balanced Kindergarten word lists. Speech recognition in noise was assessed using the Computer-Assisted Speech Perception Assessment. Children who are hard of hearing were compared with peers with normal hearing matched for age, maternal educational level, and nonverbal intelligence. The effects of aided audibility, HA use, and language ability on parent responses to auditory development questionnaires and on children's speech recognition were also examined. Children who are hard of hearing had poorer performance than peers with normal hearing on parent ratings of auditory skills and had poorer speech recognition. Significant individual variability among children who are hard of hearing was observed. Children with greater aided audibility through their HAs, more hours of HA use, and better language abilities generally had higher parent ratings of auditory skills and better speech-recognition abilities in quiet and in noise than peers with less audibility, more limited HA use, or poorer language abilities. In addition to the auditory and language factors that were predictive for speech recognition in quiet, phonological working memory was also a positive predictor for word recognition abilities in noise. Children who are hard of hearing continue to experience delays in auditory skill development and speech-recognition abilities compared with peers with normal hearing. However, significant improvements in these domains have occurred in comparison to similar data reported before the adoption of universal newborn hearing screening and early intervention programs for children who are hard of hearing. Increasing the audibility of speech has a direct positive effect on auditory skill development and speech-recognition abilities and also may enhance these skills by improving language abilities in children who are hard of hearing. Greater number of hours of HA use also had a significant positive impact on parent ratings of auditory skills and children's speech recognition.

Evaluating reporting and process quality of publications on UNHS: a systematic review of programmes.

BackgroundCongenital hearing loss is one of the most frequent birth defects, and Early Detection and Intervention has been found to improve language outcomes. The American Academy of Pediatrics (AAP) and the Joint Committee on Infant Hearing (JCIH) established quality of care process indicators and benchmarks for Universal Newborn Hearing Screening (UNHS). We have aggregated some of these indicators/benchmarks according to the three pillars of universality, timely detection and overreferral. When dealing with inter-comparison, relying on complete and standardised literature data becomes crucial.The purpose of this paper is to verify whether literature data on UNHS programmes have included sufficient information to allow inter-programme comparisons according to the indicators considered.MethodsWe performed a systematic search identifying UNHS studies and assessing the quality of programmes.ResultsThe identified 12 studies demonstrated heterogeneity in criteria for referring to further examinations during the screening phase and in identifying high-risk neonates, protocols, tests, staff, and testing environments. Our systematic review also highlighted substantial variability in reported performance data. In order to optimise the reporting of screening protocols and process performance, we propose a checklist. Another result is the difficulty in guaranteeing full respect for the criteria of universality, timely detection and overreferral.ConclusionsStandardisation in reporting UNHS experiences may also have a positive impact on inter-program comparisons, hence favouring the emergence of recognised best practices.Electronic supplementary materialThe online version of this article (doi:10.1186/s12887-015-0404-x) contains supplementary material, which is available to authorized users.

Newborn hearing screening: analysis and outcomes after 100,000 births in Upper-Normandy French region

ObjectivesNeonatal hearing impairment is a common disorder with a prevalence of 1 to 2‰ worldwide, with significant consequences on overall development when rehabilitated too late. New-born hearing screening has been implemented in the 1990s in most European countries and the USA. The Upper-Normandy region of France has been conducting a pilot program since 1999. The aim of this prospective study was to evaluate and critically analyse it. MethodsThe Upper-Normandy universal new-born hearing screening program is performed in two steps. Between 1999 and 2004, first, we administered a Transient Evoked Oto Acoustic Emission (TEOAE) test was administered a few days after birth for healthy newborns without risk factors. For newborns admitted to a neonatal intensive care unit (NICU) or presenting risk factors, was administered an automated auditory brainstem response (AABR) test prior to discharge. Second, newborns who failed the initial hearing screening were retested as outpatients using TEOAE. Since 2004, infants who failed the initial screen were tested with AABR 3 to 4 weeks later as outpatients, providing an opportunity to compare the two protocols. ResultsOverall screening coverage in the Upper-Normandy region is 99.8%. First step coverage is 99.58% in well-infant nurseries and 97.09% in the NICU. The test–retest procedure during the first step and the use of AABR for the second resulted in higher follow-up rates and lower false positive rates. ConclusionsThe Upper-Normandy region universal newborn hearing screening program facilitated diagnosis and rehabilitation of infants before age of 9 months, most notably when severe to profound hearing impairment was found.

Government-funded universal newborn hearing screening and genetic analyses of deafness predisposing genes in Taiwan

ObjectiveTo investigate the association of eight connexin genes (GJB2, GJB4, GJA1P1, GJB6, GJB3, GJA1, GJB1, and GJC3) and the SLC26A4 gene with congenital hearing impairment among infants in a universal newborn hearing screening program. MethodFrom September 2009 to October 2013, the consecutive neonates born in all six branches of Taipei City Hospital were enrolled. Infants who failed the newborn hearing screening and were diagnosed with hearing impairment underwent the genetic analyses. Result15,404 neonates were born at Taipei City Hospital, and 15,345 neonates underwent newborn hearing screening. Among them, 32 infants were diagnosed with unilateral or bilateral hearing impairment. 26 of them underwent analyses of the connexin genes and the SLC26A4 gene. Of the connexin genes, two infants carried a GJB3 mutation (heterozygous c.580G>A and heterozygous c.520G>A, respectively). Only one infant carried a GJB2 mutation (homozygous c.235delC). One infant carried a GJA1P1 mutation (heterozygous c.929delC) and another carried a GJB4 mutation (heterozygous c.302G>A). Additionally, one infant carried a GJA1P1 novel variant (heterozygous c.1081C>T). Another infant carried a GJA1 novel variant (heterozygous c.1-33C>G). Of the SLC26A4 gene, one infant carried heterozygous c.919-2A>G mutation and a novel variant (heterozygous c.164+1G>C), and high-resolution computed tomography (HRCT) of the temporal bone revealed bilateral enlarged vestibular aqueducts. One infant carried heterozygous c.919-2A>G mutation and no inner ear anomalies were demonstrated by HRCT of the temporal bone. Another infant carried a novel variant (heterozygous c.818C>T). ConclusionThese results provide a genetic profile of the connexin genes and SLC26A4 gene among infants with hearing impairment detected by a universal newborn hearing screening program in Taiwan. Further studies and long-term follow up of this cohort are warranted to determine the pathogenicity of each variants and the long-term hearing consequence.

Hearing Screening Using Auditory Steady State Responses Obtained by Simultaneous Air- and Bone-Conduction Stimuli

Introduction and objectivesMinimising false positives rates is an important goal of universal newborn hearing screening programmes. An adequate way for reaching that goal could be differentiating between transient conductive hearing losses (false positives) and permanent sensorineural hearing impairments (true positives) by means of a methodology that studies electrophysiological responses obtained using both air- and bone-conduction stimuli.Our objective was to evaluate the efficiency of an automated hearing screening test based on auditory steady state responses obtained using simultaneous air- and bone-conduction stimuli. MethodsA sample of 80 high risk babies less than 2 months of born were screened using the automatic screening test. A confirmatory clinical and electrophysiological evaluation was used as the gold standard. ResultsThe estimated diagnostic efficiency of this screening test was equivalent (100% sensitivity and 97.7% specificity) to the efficiency reported for otoacoustic emissions and automated auditory brainstem responses. The introduction of bone conduction in the screening reduced the false positive rate from 13.3% to 2.2%. The test duration was 5.3 (±1.9)min. In 34% of babies only one repetition of the test was needed to raising the result. ConclusionsThe screening test performed quite well in this initial clinical trial, differentiating transient conductive hearing losses from permanent neurosensory impairments and improving the diagnostic efficiency of auditory steady state responses.

Risk indicators for congenital and delayed-onset hearing loss.

To evaluate risk indicators for congenital and delayed onset hearing loss in a cohort of newborns who underwent newborn hearing screening, and to evaluate the impact of use of the Joint Committee on Infant Hearing (JCIH) recommendations on requirements for ongoing monitoring of infants identified as at risk for hearing loss. Cohort of 26,341 newborns entered in a prospectively collected database as part of the University of Michigan Universal Newborn Hearing Screening program, with 90 patients identified. Logistic regression analysis was used to evaluate putative risk indicators for congenital and delayed onset hearing loss. An estimate of the cost burden of ongoing monitoring imposed by the use of differing risk indicators was performed. After controlling for the impact of other risk indicators, intensive care unit length of stay greater than 5 days and exposure to loop diuretics are not associated with an increased risk of congenital or delayed onset hearing loss. Inclusion of these risk indicators as a requirement for ongoing audiologic monitoring results in a high monitoring cost per additional case identified. This study confirms that the majority of the risk indicators currently recommended by the JCIH are effective at identifying infants at increased risk of congenital and delayed onset hearing loss. However, use of neonatal intensive care unit length of stay greater than 5 days and exposure to ototoxic medications are associated with small gains in the number of infants correctly identified as at risk of hearing loss. Further evaluation of the utility of these risk indicators, preferably with a diversity of patient population and healthcare settings, is warranted.

Pediatric hearing loss.

On the basis of strong research, universal newborn screening should be performed before age 1 month with repeat or follow-up testing for those who do not pass performed before age 3 months and intervention started before age 6 months. On the basis of strong research and consensus statement, tympanostomy tubes should be considered for individuals with bilateral persistent middle ear effusion for 3 months or greater and a documented conductive hearing loss. On the basis of consensus statement, all children with suspected hearing loss should have an age appropriate hearing test. On the basis of strong research, the most common form of congenital hearing loss is genetic. Most of this is nonsyndromic hearing loss.

The Big Screen Difference

The Big Screen Difference

O-036 Hearing Loss By Week Of Gestation And Categories Of Birth Weight In Very Preterm Neonates: A Population-based Study

Background and aims Studies have shown an association between Neonatal hearing loss (NHL) and preterm birth and low birth weight, but the prevalence of NHL more specific within these categories is not known. Aim of this study is to present the risk of NHL by week of gestation and categories of birth weight in an nationwide cohort of neonates born Methods Results of the two-stage Automated Auditory Brainstem Response (AABR) Universal Newborn Hearing Screening Program in Dutch neonatal intensive care units and diagnostic examination were centrally registered between October 1998 and December 2012 and included in this study. NHL was defined as impaired when the neonates’ conventional Auditory Brainstem Response (ABR) level exceeded 35 dB in one (unilateral) or two (bilateral) ears at diagnostic examination. Birth weight was stratified into Results In total 18,564 neonates with a gestational age between 24.0 and 31.9 weeks were eligible for this study. Logistic regression analyses revealed significant associations between gestational age, birth weight and both unilateral and bilateral NHL (all p Conclusions Gestational age and birth weight are independently associated with NHL in a dose-response relationship. This information can be used to gain insight into health and related costs associated with very preterm births.

Defaulters of Universal Newborn Hearing Screening Program at Tertiary Hospital, Malaysia: The Associated Factors

Objectives: Describe the sociodemographic of defaulters in the Universal Newborn Hearing Screening Program at UKMMC and to identify the associated factors. Methods: A cross-sectional study was conducted at UKMMC from December 2011 until May 2013. Defaulter was defined as a patient who failed to attend the first stage hearing screening at UKMMC and also defaulted follow-up at the Audiology Unit between January 2010 and May 2011. The information was obtained through telephone inquiries to parents using the set of questionnaires. The factors studied were awareness of hearing loss, distance to the hospital, appointment date, and financial and transportation difficulties. Results: A total of 280 respondents were interviewed. The majority of the respondents were Malays (71.79%) in the middle income category. Nonawareness of the child’s hearing loss was the main associated factor (n = 205, 73.21%). The other associated factors were: appointment date was not given (n = 173, 61.8%), distance to the hospital (n = 126, 45%), appointment date given is too far away (n = 57, 20.4%), financial difficulties (n = 59, 21.1%), followed by respondents who do not have their own transport (n = 46, 16.4%). Conclusions: Nonawareness of the child’s hearing loss by his or her parents was the major factor to the high default rate. Therefore, education and empowerment programs should be introduced and implemented at UKMMC in order to improve awareness among parents.

Auditory Neuropathy Spectrum Disorder among DPOAEs Screened Infants

Objective : Auditory neuropathy spectrum disorder (ANSD) characterized by absent or severely abnormal auditory brainstem response (ABR) with intact outer hair cells functions, as evidenced by the presence of evoked otoacoustic emissions (OAEs) and/or cochlear microphonics. In Universal Newborn Hearing Screening (UNHS), all infants screened for hearing loss either by OAEs or ABR or both of them. The purpose of this work was to study ANSD among infants passed OAEs based UNHS program. Design : UNHS distortion product OAEs (DPOAEs) based program were done for 10281 infants within six years duration. Click evoked ABR were performed to high risk for hearing loss infants and on parents concern about hearing loss for their passed UNHS program children among attendee of audiology and ORL clinics. Tympanograms and behavioral thresholds were performed for the study group. Results : Thirteen infants met the inclusion criteria of ANSD were discovered among passed UNHS program infants (0.12%). Unilateral ANSD were diagnosed in 2 infants (the other ears were normal in one infant and ABR was preserved down to mild hearing loss in the other infant). All ANSD infants had absent click evoked ABR at the maximum presentation level of 100 dBnHL, the acoustic reflexes were absent while DPOAEs were presented in all affected ears. Discussion and Conclusion : In spite of passing UNHS OAEs based program still some infants are missed for the early diagnosis of ANSD which mandate special concern in the screening procedures to be more inclusive for the diagnosis of those infants.

Cribado auditivo mediante potenciales evocados auditivos de estado estable obtenidos por estimulación simultánea de la vía aérea y la ósea

Introduction and objectivesMinimising false positives rates is an important goal of universal newborn hearing screening programmes. An adequate way for reaching that goal could be differentiating between transient conductive hearing losses (false positives) and permanent sensorineural hearing impairments (true positives) by means of a methodology that studies electrophysiological responses obtained using both air- and bone-conduction stimuli.Our objective was to evaluate the efficiency of an automated hearing screening test based on auditory steady state responses obtained using simultaneous air- and bone-conduction stimuli. MethodsA sample of 80 high risk babies lees than 2 months of born were screened using the automatic screening test. A confirmatory clinical and electrophysiological evaluation was used as the gold standard. ResultsThe estimated diagnostic efficiency of this screening test was equivalent (100% sensitivity and 97.7% specificity) to the efficiency reported for otoacoustic emissions and automated auditory brainstem responses. The introduction of bone conduction in the screening reduced the false positive rate from 13.3% to 2.2%. The test duration was 5.3 (±1.9)min. In 34% of babies only one repetition of the test was needed to raising the result. ConclusionsThe screening test performed quite well in this initial clinical trial, differentiating transient conductive hearing losses from permanent neurosensory impairments and improving the diagnostic efficiency of auditory steady state responses.

Investigação etiológica da deficiência auditiva em neonatos identificados em um programa de triagem auditiva neonatal universal

Objetivo descrever os resultados da investigação etiológica da deficiência auditiva realizada em neonatos rastreados em um programa de triagem auditiva neonatal universal. Métodos estudo descritivo, transversal e prospectivo. Foram incluídos no estudo todos os neonatos diagnosticados com deficiência auditiva identificados em um programa de triagem auditiva neonatal universal no período de agosto de 2003 a dezembro de 2006. A provável etiologia da deficiência auditiva foi determinada após anamnese detalhada realizada pelo médico otorrinolaringologista; pesquisa das sorologias para toxoplasmose, rubéola, citomegalovírus, herpes, sífilis e HIV; tomografia dos ossos temporais e exames genéticos. Resultados foram diagnosticados 17 sujeitos com deficiência auditiva no período estudado. 64.7% dos casos estudados apresentaram como provável etiologia causas pré-natais, 29.4% causas peri-natais e um sujeito (5,9%) apresentou etiologia desconhecida. Das causas pré-natais, 36.4% tiveram origem genética confirmada e 36.4% etiologia presumida de hereditariedade. Foi confirmada a presença de infecções congênitas em 18.2% dos casos e um sujeito (9%) apresentou anomalia craniofacial como provável etiologia. O grau de perda auditiva mais frequente observado nos sujeitos estudados foi o profundo (47,1%). Conclusão a maior ocorrência de etiologias observada neste estudo foram as de origem pré-natal, seguida das de origem peri-natal.