Prevalence of moderate or greater permanent childhood hearing impairment and effectiveness of targeted surveillance for babies who pass newborn hearing screening.

OBJECTIVE: To assess the utility of targeted surveillance for the identification of moderate to profound PCHI in babies who pass newborn hearing screening in England and have risk factors. DESIGN: Retrospective analysis. STUDY SAMPLE: A total of 3,957,891 children born 01/04/2012-31/03/2018 in England. RESULTS: A total of 7148 PCHI cases were identified (1.81 per 1,000 babies). 6,707 followed an immediate referral from the screen (1 per 16 referrals), 51 followed targeted surveillance referral (1 per 540 referrals) and 390 without a referral. Audiology uptake was higher following an immediate referral (96.7% overall, 77.2% within NHSP-defined timescales) than following targeted surveillance (63.8% overall, 51.1% within 52 weeks of birth). The screening was 94.5% sensitive overall, with similar sensitivities for each of the risk factors. General linearised logistic regression models identified syndrome as the risk factor with the highest odds ratio (14.08 for all babies, 22.19 for babies without immediate referral). Close family history of hearing loss was the next highest (10.93 for all babies, 12.29 for babies without immediate referral). CONCLUSION: The evidence for a targeted surveillance programme, based on risk factors, for babies in England who pass the newborn screen is not strong.

Protocol and programme factors associated with referral and loss to follow-up from newborn hearing screening: a systematic review.

BACKGROUND: An effective newborn hearing screening programme has low referral rate and low loss to follow-up (LTFU) rate after referral from initial screening. This systematic review identified studies evaluating the effect of protocol and programme factors on these two outcomes, including the screening method used and the infant group. METHODS: Five databases were searched (latest: April 2021). Included studies reported original data from newborn hearing screening and described the target outcomes against a protocol or programme level factor. Studies were excluded if results were only available for one risk condition, for each ear, or for < 100 infants, or if methodological bias was observed. Included studies were evaluated for quality across three domains: sample, screening and outcome, using modified criteria from the Ottawa-Newcastle and QUADAS-2 scales. Findings from the included studies were synthesised in tables, figures and text. RESULTS: Fifty-eight studies reported on referral rate, 8 on LTFU rate, and 35 on both. Only 15 studies defined LTFU. Substantial diversity in referral and LTFU rate was observed across studies. Twelve of fourteen studies that evaluated screening method showed lower referral rates with aABR compared to TEOAE for well babies (WB). Rescreening before hospital discharge and screening after 3 days of age reduced referral rates. Studies investigating LTFU reported lower rates for programmes that had audiologist involvement, did not require fees for step 2, were embedded in a larger regional or national programme, and scheduled follow-up in a location accessible to the families. In programmes with low overall LTFU, higher LTFU was observed for infants from the NICU compared to WB. CONCLUSION: Although poor reporting and exclusion of non-English articles may limit the generalisability from this review, key influential factors for referral and LTFU rates were identified. Including aABR in WB screening can effectively reduce referral rates, but it is not the only solution. The reported referral and LTFU rates vary largely across studies, implying the contribution of several parameters identified in this review and the context in which the programme is performed. Extra attention should be paid to infants with higher risk for hearing impairment to ensure their return to follow-up.

A Study of Middle Ear Diseases in Children with Congenital Bilateral Severe to Profound Sensorineural Hearing Loss.

(1) To find the presence of middle ear diseases present in the patients with congenital bilateral severe to profound SNHL (2) If it poses as a threat to rehabilitative efforts for the existing severe to profound SNHL. Study setting: Department of ENT, C U Shah medical college and hospital. Study design: Prospective study Study population: Patients attending ENT department with complaints of congenital bilateral severe to profound sensorineural hearing loss. A total of 50 cases were studied during the study period. Methods of data collection: The Proforma was designed based on objective of the study. Detailed history was taken followed by thorough ENT and systemic examinations. Otoscopy and otoendoscopy were carried out and all patients were subjected to hearing tests consisting of Pure Tone Audiometry (PTA), Impedance Audiometry (IA), Brainstem Evoked Response Audiometry (BERA) and Oto Acoustic Emissions (OAE). HRCT and MRI scanning of temporal bones of all the patients was included as a part of the routine workup. Out of the 50 children with bilateral congenital hearing loss studied in this study, 13(26%) children were observed to have concurrent middle ear pathologies. 2 patients had bilateral retracted drum with tympanosclerosis; 3 had bilateral retraction pocketswith mastoiditis; 1 had bilateral SOM; 1 had right SOM and left retracted drum; 2 had bilateral PSQ cholesteatoma; 1 had left SOM and right sided tympanic membrane perforation, 3 had right sided tympanic membrane perforation with left sided normal ears. Children with congenital bilateral severe to profound hearing loss should be examined for middle ear pathologies, which can most often be overlooked otherwise, hence rendering the patient unfit for definitive management of the severe to profound SNHL in the form of Hearing Aid trial or Cochlear Implantation, further delaying the development of speech. Hence, all children with congenital bilateral severe to profound hearing loss should undergo regular screening for assessment of middle ear pathologies with prompt treatment when any middle ear pathology is encountered, therefore rendering the patient fit for fitting of cochlear implantation at the earliest possible to decrease permanent impairment of speech.

[Follow-up II of newborn hearing screening : Evaluation of a follow-up II facility after implementation of newborn hearing screening in Germany]. / Follow-up-II des Neugeborenen-Hörscreenings : Evaluation der Ergebnisqualität einer Follow-up-II-Einrichtung nach Einführung des Neugeborenen-Hörscreenings in Deutschland.

BACKGROUND: With the implementation of newborn hearing screening, evaluation in terms of quality and goal achievement was required. The present study evaluates a follow-up II facility from 2009 to 2016. METHODS: Data of 2705 newborns were retrospectively evaluated. The annual number of patients was analyzed, as well as the median age at first presentation, at diagnosis, and at treatment, each according to the reason for presentation and the diagnosis. RESULTS: From 2009 to 2016, the number of presented newborns increased by 91.4%. Newborns with abnormal initial screening or risk factors were presented significantly later than those for initial screening (median 5.3 and 8.0 vs. 4.6 weeks, respectively; p < 0.001). Permanently or transiently hearing-impaired patients were presented and diagnosed significantly later than those with normal hearing (age at initial presentation 6.1 and 7.6 vs. 5.4 weeks, p < 0.01 and p < 0.001, respectively; age at diagnosis 11.4 and 23.1 vs. 5.9 weeks, respectively; p < 0.001). Permanent hearing loss was treated at the age of 14.1 weeks. From 2009 to 2014, the age at first presentation and at diagnosis increased and subsequently mostly decreased until 2016. CONCLUSION: The age at first presentation and at diagnosis depends on the reason for presentation and on the diagnosis. Despite increasing patient numbers, the Joint Federal Committee (Gemeinsame Bundesausschuss, G­BA) targets were met due to effective and efficient organizational structuring of the follow-up II facility. However, early admission to a follow-up II facility is a prerequisite for the success of newborn hearing screening.

Assessment of hearing screening programmes across 47 countries or regions III: provision of childhood hearing screening after the newborn period.

OBJECTIVE: To inventory provision and features of childhood hearing screening after the newborn period (CHS), primarily in Europe. DESIGN: From each participating country or region, experts provided information through an extensive questionnaire: implementation year, age at screening, test method, pass criteria, screening location, screener profession, and quality indicators: coverage, referral, follow-up and detection rates, supplemented by literature sources. STUDY SAMPLE: Forty-two European countries or regions, plus Russia, Malawi, Rwanda, India, and China. RESULTS: CHS was performed universally with pure-tone audiometry screening (PTS) in 17 countries or regions, whereas non-universal CHS was performed in eight with PTS or whisper tests. All participating countries with universal PTS had newborn hearing screening. Coverage rate was provided from three countries, detection rate from one, and referral and follow-up rate from two. In four countries, universal PTS was performed at two ages. Earliest universal PTS was performed in a (pre)school setting by nurses (n = 9, median age: 5 years, range: 3-7), in a healthcare setting by doctors and nurses (n = 7, median age: 4.5 years, range: 4-7), or in both (n = 1). CONCLUSIONS: Within universal CHS, PTS was mostly performed at 4-6 years by nurses. Insufficient collection of data and monitoring with quality indicators impedes evaluation of screening.

Assessment of hearing screening programmes across 47 countries or regions II: coverage, referral, follow-up and detection rates from newborn hearing screening.

OBJECTIVES: To assess the performance of newborn hearing screening (NHS) programmes, through selected quality measures and their relationship to protocol design. DESIGN: NHS coverage, referral, follow-up and detection rates were aggregated. Referral rates were compared to age at screening step 1, number of steps, and test method: OAE or aABR. STUDY SAMPLE: A questionnaire on existing hearing screening was completed by experts from countries in Europe, plus Russia, Malawi, Rwanda, India and China. RESULTS: Out of 47 countries or regions, NHS coverage rates were reported from 26, referral rates from 23, follow up from 12 and detection rates from 13. Median coverage rate for step 1 was 96%. Referral rate from step 1 was 6-22% where screening may be performed <24 h from birth, 2-15% for >24 h, and 4% for >72 h. Referral rates to diagnostic assessment averaged 2.1% after one to two steps using OAE only, 1.7% after two steps including aABR, and 0.8% after three to four steps including aABR. Median detection rate for bilateral permanent hearing impairment ≥40dB was 1 per 1000 infants. CONCLUSION: Referral rates were related to age, test method and number of screening steps. Quality measures were not available for many NHS programmes.

Assessment of hearing screening programmes across 47 countries or regions I: provision of newborn hearing screening.

OBJECTIVES: Newborn hearing screening (NHS) varies regarding number and type of tests, location, age, professionals and funding. We compared the provision of existing screening programmes. DESIGN: A questionnaire containing nine domains: demography, administration, existing screening, coverage, tests, diagnosis, treatment, cost and adverse effects, was presented to hearing screening experts. Responses were verified. Clusters were identified based on number of screening steps and use of OAE or aABR, either for all infants or for well and high-risk infants (dual-protocol). STUDY SAMPLE: Fifty-two experts completed the questionnaire sufficiently: 40 European countries, Russia, Malawi, Rwanda, India and China. RESULTS: It took considerable effort to find experts for all countries with sufficient time and knowledge. Data essential for evaluation are often not collected. Infants are first screened in maternity wards in most countries. Human development index and health expenditure were high among countries with dual protocols, three screening steps, including aABR, and low among countries without NHS and countries using OAE for all infants. Nationwide implementation of NHS took 6 years, on average. CONCLUSION: The extent and complexity of NHS programmes are primarily related to health expenditure and HDI. Data collection should be improved to facilitate comparison of NHS programmes across borders.

Permanent childhood hearing impairment in infants admitted to the neonatal intensive care unit: nested case-control study.

The prevalence of permanent childhood hearing impairment (PCHI) in infants admitted to a neonatal intensive care unit (NICU) is higher than that in the general population. Our study objective was to identify risk factors associated with PCHI in infants who required admission to the NICU for more than 48 h. We performed a case-control study, including infants of all gestational ages who were admitted to NICU for more than 48 h and who underwent newborn hearing screening between 2005 and 2019. Infants admitted to NICU and diagnosed with PCHI by formal audiology were classified as "cases". The "controls" were infants who were admitted to NICU and did not have PCHI. Cases and controls (1:4) were matched based on their birth gestation, birthing place, and treating NICU. The prevalence of PCHI in infants admitted to NICU was 6.3% as compared with our general population prevalence of 0.25%. There were 77 cases and 269 controls during the study period. The median age at diagnosis of PCHI in these infants was 132 days (interquartile range 75.5-518.5). Using regression analysis, "any ventilation episodes", presence of seizures, and major congenital anomalies were significantly associated with PCHI in infants of all gestational ages. There were higher prevalence of PCHI in preterms (<32 weeks) who received furosemide and lower prevalence with antenatal use of magnesium sulphate.Conclusions: In our study, the prevalence of hearing loss was high in infants admitted to NICU. Gestation-specific risk factors identified in this case-control study would help in counselling of parents. What is Known: • In the UK, 1-2/1000 infants are born with hearing loss and infants admitted to the neonatal unit for 48 h or more have increased prevalence of hearing loss (1 in 100 live births). • Identification of risk factors in infants admitted to neonatal unit would help with risk stratification and further management. What is New: • In our study, infants admitted to the neonatal unit had higher prevalence of hearing loss (6.3 in 100 live births). • In infants across all gestational age "any ventilation episodes", presence of seizures, and severe congenital anomalies were associated with a statistically significant increase in prevalence of hearing loss. Higher prevalence of hearing loss was noted in preterm infants (<32 weeks) who received furosemide treatment and lower prevalence was noted with antenatal use of magnesium sulphate.

Evaluación de un programa piloto para trabajar habilidades narrativas en niños con hipoacusia usuarios de ayudas auditivas / Evaluation of a pilot program: developing narrative skills in children with hearing aids or cochlear implant

El Discurso Narrativo (DN) es una unidad lingüística compleja utilizada en ciertos contextos y que refleja la organización del pensamiento. La evidencia científica muestra que la población sorda, usuaria de ayudas auditivas, presenta dificultades en los diferentes niveles del lenguaje, tanto expresivos como comprensivos, incluida la habilidad para narrar. Además, existe evidencia de que la intervención terapéutica ayudaría a mejorar su rendimiento. Sin embargo, los datos disponibles sobre las características y abordaje del DN en esta población son escasos. El objetivo del estudio es evaluar un programa piloto para trabajar habilidades narrativas en niños chilenos usuarios de ayudas auditivas. Se estudiaron 22 niños con un promedio de edad de 6,5 años, adaptados con audífonos y/o implante coclear. Se aplicó a este grupo de niños una evaluación inicial del DN utilizando el instrumento Evaluación del Discurso Narrativo (EDNA), obteniéndose la Etapa y Desempeño narrativo de cada niño. Luego, se creó y aplicó individualmente un programa de estimulación del discurso narrativo de 12 sesiones una vez por semana. Finalmente, se repitió la evaluación al final del programa. Se encontraron diferencias significativas entre los resultados obtenidos previo y posterior a la implementación del programa de estimulación. En relación con la Etapa del DN, antes de la intervención el 45,5% de los niños no estructuraba, lo cual se redujo a un 9.1% en la evaluación final. En cuanto al Desempeño, previo a la intervención el 72,7% de los niños presentaba un "déficit narrativo", lo cual se redujo a un 18,2% posterior a la aplicación del programa.

Narrative discourse is considered a linguistic unit that is used in a specific communicative context, being an indicator of thinking organization. Previous evidence shows how hearing aid users, have difficulties with different language skills, both expressive and comprehensive, including the ability to narrate. Additionally, there is evidence showing how therapeutic intervention would help to improve their narrative performance. However, the information available about the discursive skilland the effect ofstimulation programs on it in hearing impaired children is scarce. Accordingly, the present study aims to explore narrative performance in hearing impaired children users of hearing aids/cochlear implants, before and after a narrative speech stimulation program. Twenty-two children diagnosed with bilateral hearing loss users of hearing aids/cochlear implants with a mean age of 6.5 years were included. An initial assessment of the narrative skills was performed using Narrative Discourse Assessment (EDNA), which provided a narrative Stage and a Total score. A twelve-session stimulation program was developed and individually administered to children once a week. Finally, an assessment was performed after the program ended. In the initial assessment, 45.5% of children did not have a structured narrative speech, a percentage that was reduced to a 9.1% in the final evaluation. Statistically significant differences were observed on the EDNA scores when comparing initial and final assessments. The results obtained in the present investigation show how children who use hearing aids/cochlear implants improved significantly their narrative abilities after participating in a pilot narrative speech stimulation program.

Clinicians' views of using cortical auditory evoked potentials (CAEP) in the permanent childhood hearing impairment patient pathway.

Objective: To obtain clinicians' views on the use of cortical auditory evoked potentials (CAEP) in the clinical pathway.Design: A questionnaire aimed at clinicians who use the HEARLab system with the Aided Cortical Assessment (ACA) Module. Results compared for Australians (where HEARLab produced) to other countries.Sample: The questionnaire was completed by 49 clinicians; 33 from Australia and 13 clinicians outside of Australia and 3 clinicians, destination unknown.Results: The findings of this research demonstrated that clinicians using CAEPs found them valuable for clinical practice. CAEPs were used to verify or modify hearing aid fittings and were used for counselling parents to reinforce the need for hearing aids. With the use of speech token as the stimulus clinicians had more relevant information to increase confidence in decision-making on paediatric hearing management.Conclusions: The main benefit from the use of CAEPs (using speech token stimuli) was for infant hearing aid fitting programmes, to facilitate earlier decisions relating to hearing aid fitting, for fine-tuning the aids and as an additional measure for cochlear implant referrals.

Early hearing detection and intervention in South Africa: Exploring factors compromising service delivery as expressed by caregivers.

AIM: The main aim of this study was to explore factors compromising early intervention (EI) service delivery to hearing impaired children in South Africa, as expressed by their caregivers. METHODS: Within a qualitative survey design, a sample of 19 hearing impaired children's caregivers completed structured self-administered questionnaires on factors that they perceive compromise EI for their children. These caregivers included mothers, fathers, grandparents, and legal guardians or adoptive parents of children with hearing impairment. Descriptive analysis of the data was undertaken. RESULTS: Findings indicated various factors compromising EI as reported by caregivers. These included limited availability of appropriate schools and health care facilities for their hearing impaired children; long distances between the few services that are available and the places of residence of the service users; significant costs linked to the services (such as medical expenses, boarding school facilities costs); limited skills and knowledge of professionals and teachers regarding hearing impairment; inconsistent and conflicting professional opinions about the child's diagnosis and treatment; as well as limited community awareness about hearing impairment along with services available for hearing impaired children. CONCLUSION: These findings have important clinical, training, policy, and advocacy implications within the South African context; if both access to and success within the EI services will be successful.

Outcomes of early intervention for deaf children with additional needs following an Auditory Verbal approach to communication.

OBJECTIVES: To determine and compare the rates of progress made by pre-school aged children with all degrees and types of hearing impairment and deafness, both with and without additional needs as catalogued using SNOMED CT, at the end of a non-statutory programme of individualised Auditory Verbal (AV) intervention. METHODS: An audit was conducted using a retrospective and comparative study design to examine spoken language outcomes in children who had spent more than two years on an AV programme and had completed their programmes between January 2007 and December 2017. The children were stratified according to i) whether they achieved age appropriate language (AAL) (n =102) or not (n =27); ii) whether they had deafness alone (n = 77) or deafness with additional needs (n =52); and iii) whether children with additional needs achieved AAL (n= 27) or not (n =25). Children undertook standardised spoken language assessments on joining the AV programme and then at intervals of at least 6 months for the duration of their programme. Derived measures of rates of language development (RLD) were used to compare the groups at i) the outset (initial RLD), and ii) the conclusion of the AV programme (programme RLD). RESULTS: Overall, 79% of children within this cohort achieved age appropriate spoken language scores. Children with additional needs (40%) embarked on a non-statutory AV programme at a significantly older age (corrected for prematurity), with significantly lower initial RLD and, as a group, attained significantly lower programme RLD compared with children with deafness alone. One in two of the children with additional needs reached AAL by the end of their individualised programme. The children with additional needs also demonstrated a highly significant increase in their mean programme RLD compared with the mean initial RLD indicating an acceleration in acquiring spoken language competencies while on the AV programme. CONCLUSIONS: For deaf children with additional needs who stay on an AV programme for more than two years, listening and spoken communication is significantly enhanced. Specific access to the AV approach in addition to generic, statutory early intervention could facilitate deaf children with additional needs to achieve or approach AAL. Ensuring families have access to effective early intervention increases the chances that i) a suitable communication approach is adopted at the earliest opportunity, and ii) a child with additional needs acquires listening and spoken language at a rate commensurate with their full potential. Applying the SNOMED CT framework as a means of categorising children's additional needs will enable more effective comparisons across studies from different centres around the world.

Achieving effective hearing aid fitting within one month after identification of childhood permanent hearing impairment.

Diagnosis of child permanent hearing impairment (PHI) can be made with extreme timeliness compared to the past thanks to improvements in PHI identification through newborn hearing screening programmes. It now becomes essential to provide an effective amplification as quickly as possible in order to restore auditory function and favour speech and language development. The early fitting of hearing aids and possible later cochlear implantation indeed prompts the development of central auditory pathways, connections with secondary sensory brain areas, as well as with motor and articulatory cortex. The aim of this paper is to report the results of a strategic analysis that involves identification of strengths, weaknesses, opportunities and threats regarding the process of achieving early amplification in all cases of significant childhood PHI. The analysis is focused on the Italian situation and is part 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".

Management challenges of congenital & early onset childhood hearing loss in a sub-Saharan African country.

BACKGROUND: Hearing impairment is a hidden human disability with potentially catastrophic and age long consequences. This study highlighted the challenges associated with the management of congenital and early onset childhood hearing loss in a sub-Saharan African country. METHODS: A retrospective descriptive study of children seen between January 2008 and December 2013 RESULT: A total of 223 children consisting of 124 (55.6%) males with (M:F) of 1.3:1. Age ranged 1-15 years (mean±SD; 6.39±4.37 years) and age group 1-5 years constituted the largest proportion (56.5%). Congenital causes, febrile illness and hypoxia were the leading causes of HI. Over 93% had moderately severe to profound hearing loss and 64.6% had delayed speech development. Majority (99.3%) with congenital/perinatal onset of HL had significantly delayed speech development and 99.3% of HL due to ototoxicity and infective causes had peri/post lingual speech impairment. Larger percentage of patients presented late; 16.6% of patients with congenital/perinatal onset of HL presented within the first year, >41% presented after the fifth year. Less than 5% had hearing aid fitted and patients with profound hearing impairment were referred for cochlear implant CONCLUSION: The burden of congenital and early onset hearing impairment is high and management outcomes are unsatisfactory in our locality, Challenges associated with managing such children were discussed, and suggestions/strategies for better management and outcome were made.

Screening, rubella vaccination, and childhood hearing impairment in Taiwan.

Childhood hearing impairment (CHI) is a major developmental disability, but data at the national level are limited, especially those on the changes in the prevalence over time. In Taiwan, the government began to certify disabled residents for providing various services in 1980 and maintains a registry of certified cases, which provides a rare opportunity for studying the trends of CHI prevalence. Using the registry data, we estimated the prevalence of CHI by age and severity and explored factors affecting its changes over time. From 2000 to 2011, the registered cases under 17 years old ranged from 3427 to 4075. The overall prevalence increased from 2000 to 2006, but then decreased till 2011. While the prevalence of mild CHI increased over the years, such a pattern was not observed in moderate or severe CHI. In general, the overall prevalence increased over the years in the age groups <3 years, 3-5 years, and 6-11 years (p<0.01), and the largest increase was observed in the age group <3 years, particularly after the promotion of screening by the government in 2003. The decrease after 2006 was mainly attributable to decreases in the age groups 12-14 (with a decreasing trend from 2001, p<0.01) and 15-17 years (with a decreasing trend from 2004, p<0.01). The timing was related to the implementation of a nationwide rubella vaccination program. Similar decreases had been observed in countries with rubella vaccination programs.