Hearing characteristics and otoradiological abnormalities in three patients with novel pathogenic variants of KMT2D-related Kabuki syndrome.

BACKGROUND: Kabuki syndrome 1 (KS1; OMIM:147920), which is characterized by distinctive dysmorphic facial features (such as arched eyebrows, long palpebral fissures with eversion of the lower lid, and large protuberant ears), intellectual disability, short stature, and dermatoglyphic and skeletal abnormalities, is brought on by pathogenic variants in KMT2D (OMIM:602113). In this work, three individuals with novel pathogenic KMT2D gene variants had their longitudinal audiological manifestations and ear structural characteristics outlined. METHODS: The longitudinal audiological data from neonatal hearing screening and a battery of several hearing tests were evaluated. The battery of hearing tests included tympanometry, distortion product otoacoustic emission (DPOAE), click-evoked air-conduction auditory brain-stem response (AC-ABR), click-evoked bone-conduction auditory brain-stem response (BC-ABR), narrow band CE-chirp auditory steady-state response (NB CE-chirp ASSR), and pure-tone audiometry (PTA). Phenotype identification and whole exome sequencing (WES) were performed on recruited individuals. RESULTS: All three patients (two females and on male; last evaluations at 14 months, 11 months, and 5.7 years, respectively) failed the newborn hearing screening, and the audiological follow-up data revealed mild to profound fluctuating hearing loss, which was directly influenced by the incidence and severity of otitis media with effusion (OME). When OME occurred, the AC-ABR thresholds increased from 30-75 dBnHL to 45-90 dBnHL. The threshold for the BC-ABR and BC-PTA was between 25 and 50 dBnHL, indicating mild to moderate sensorineural hearing loss (SNHL). The high-resolution computed tomography (HRCT) pictures indicated that all three patients had middle and inner ear abnormalities. Middle ear anomalies showed as diminished mastoid gasification and ossicle dysplasia. Cochlear dysplasia, a dilated vestibule, fusion of the vestibule with the horizontal semicircular canals, and a short and thick horizontal semicircular canal were visible on images of the inner ear. This study recruited three individuals with three novel pathogenic variants (c.5104C>T, c.10205delA, and c.12840delC) of KMT2D who were identified at ages 27 days, 2 months, and 5.5 years. CONCLUSIONS: Hearing characteristics of three individuals with three novel pathogenic variants of KMT2D range from mild to profound fluctuating hearing loss with mild to moderate SNHL. HRCT scans showed that all three individuals had anatomical middle and inner ear abnormalities. KS 1 patients must get clinical therapy for OME, frequent auditory monitoring, and prompt intervention.

Neonatal hearing screening - does failure in TEOAE screening matter when the AABR test is passed?

PURPOSE: Newborns who fail the transient evoked otoacoustic emissions (TEOAE) but pass the automatic auditory brainstem response (AABR) in universal newborn hearing screening (UNHS), frequently have no further diagnostic test or follow-up. The present study aimed to investigate whether hearing loss might be missed by ignoring neonatal TEOAE failure in the presence of normal AABR. METHODS: A retrospective analysis was conducted in newborns presenting between 2017 and 2021 to a tertiary referral centre due to failure in the initial UNHS. The main focus was on infants who failed TEOAE tests, but passed AABR screening. The clinical characteristics and audiometric outcomes were analysed and compared with those of other neonates. RESULTS: Among 1,095 referred newborns, 253 (23%) failed TEOAE despite passing AABR screening. Of the 253 affected infants, 154 returned for follow-up. At 1-year follow-up, 46 (28%) achieved normal audiometric results. 32 (21%) infants had permanent hearing loss (HL) confirmed by diagnostic ABR, 58 (38%) infants had HL solely due to middle ear effusion (MEE), and for 18 (12%) infants HL was suspected without further differentiation. The majority of permanent HL was mild (78% mild vs. 13% moderate vs. 9% profound). The rate of spontaneous MEE clearance was rather low (29%) leading to early surgical intervention in 36 children. The profile of the risk factors for hearing impairment was similar to that of newborns with failure in both, TEOAE and AABR; however, there was a stronger association between the presence of risk factors and the incidence of HL (relative risk 1.55 vs. 1.06; odds ratio 3.61 vs. 1.80). CONCLUSION: In newborns, the discordance between a "refer" in TEOAE and a "pass" in AABR screening is associated with a substantial prevalence of hearing impairment at follow-up, especially in the presence of risk factors.

Training spatial hearing in unilateral cochlear implant users through reaching to sounds in virtual reality.

BACKGROUND AND PURPOSE: Use of unilateral cochlear implant (UCI) is associated with limited spatial hearing skills. Evidence that training these abilities in UCI user is possible remains limited. In this study, we assessed whether a Spatial training based on hand-reaching to sounds performed in virtual reality improves spatial hearing abilities in UCI users METHODS: Using a crossover randomized clinical trial, we compared the effects of a Spatial training protocol with those of a Non-Spatial control training. We tested 17 UCI users in a head-pointing to sound task and in an audio-visual attention orienting task, before and after each training.
Study is recorded in clinicaltrials.gov (NCT04183348). RESULTS: During the Spatial VR training, sound localization errors in azimuth decreased. Moreover, when comparing head-pointing to sounds before vs. after training, localization errors decreased after the Spatial more than the control training. No training effects emerged in the audio-visual attention orienting task. CONCLUSIONS: Our results showed that sound localization in UCI users improves during a Spatial training, with benefits that extend also to a non-trained sound localization task (generalization). These findings have potentials for novel rehabilitation procedures in clinical contexts.

Precisión diagnóstica de una versión abreviada de las pruebas de patrones de frecuencia y patrones de duración para la evaluación del ordenamiento auditivo temporal / Diagnostic accuracy of an abbreviated version of the frequency pattern and duration pattern tests for the evaluation of auditory temporal ordering

Introducción: Las pruebas de patrones de frecuencia y patrones de duración son consideradas los gold standard de evaluación del ordenamiento auditivo temporal. A pesar de su amplia difusión, la extensión y duración de estas pruebas dificultan su utilización dentro de baterías de evaluación del procesamiento auditivo. Sin embargo, dadas sus características estructurales, pareciera ser posible reducirlas sin perder su precisión diagnóstica. Objetivo: Determinar las propiedades diagnósticas de versiones abreviadas de las pruebas de patrones de frecuencia y patrones de duración. Material y Método: Se realizó un estudio transversal de diseño observacional analítico. Se evaluaron 166 oídos de 88 sujetos con edades entre 18 y 33 años, los cuales fueron clasificados con normalidad o alteración del ordenamiento auditivo temporal. Se utilizaron las pruebas originales de Auditec de 30 ítems por oído como referencia y los primeros 10 ítems de cada oído como versión abreviada. Resultados: La versión abreviada de la prueba de patrones de frecuencia obtuvo una sensibilidad de un 94,33%, una especificidad del 94,29% y un área bajo la curva ROC de 0,980. La versión abreviada de la prueba de patrones de duración obtuvo una sensibilidad de un 89,58%, especificidad del 71,88% y un área bajo la curva ROC de 0,916. Ambas versiones tuvieron un índice de concordancia adecuado. Conclusiones: Las versiones abreviadas de la prueba de patrones de frecuencia y patrones de duración cuentan con excelentes propiedades diagnósticas para la evaluación del ordenamiento auditivo temporal y pueden ser utilizadas de manera intercambiable con las versiones originales.

Introduction: The frequency and duration pattern tests are considered the gold standard for assessing auditory temporal ordering. Despite their wide dissemination, the length and duration of these tests make it difficult to use them within auditory processing assessment batteries. However, given their structural characteristics, reducing them without losing their diagnostic accuracy seems possible. Aim: Determine the diagnostic properties of abbreviated versions of the Frequency Patterns and Duration Patterns tests. Material and Method: A cross-sectional study with an analytical observational design was carried out. 166 ears of 88 subjects aged between 18 and 33 years were evaluated, classified as normal or altered in temporal auditory order. The original Auditec tests of 30 items per ear were used as a reference, and the first 10 items of each ear as an abbreviated version. Results: The abbreviated version of the frequency patterns test obtained a sensitivity of 94.33%, a specificity of 94.29%, and an area under the ROC curve of 0.980. The abbreviated version of the duration pattern test obtained a sensitivity of 89.58%, a specificity of 71.88%, and an area under the ROC curve of 0.916. Both versions had an adequate concordance index. Conclusion: The abbreviated versions of the frequency patterns and duration patterns test have excellent diagnostic properties for assessing auditory temporal ordering and can be used interchangeably with the original versions.

[Analysis of clinical audiological characteristics in 868 children referred from maternal and child institutions].

Objective:To investigate the clinical audiological characteristics of children referred from maternal and child institutions and analyze the high risk factors of hearing loss, so as to provide scientific basis for further improvement of children's ear and hearing care. Methods:The subjects of this study were 868 children who were referred by maternal and child institutions in Beijing to the otology outpatient of Beijing Tongren Hospital, Capital Medical University for hearing diagnosis. All subjects underwent acoustic immittance, auditory brainstem response, distortion products otoacoustic emission and other audiological tests. Children were divided into groups according to the age of diagnosis: 0-<3 months group（242 cases）, 3-<6 months group（328 cases）, 6-<12 months group（180 cases）, ≥12 months group（118 cases）, the results of hearing diagnosis, hearing loss degree and types, the relationship between high risk factors and hearing loss in each group were compared and analyzed. Results:The age of diagnosis of 868 children was（7.13±8.29） months. 488 cases with hearing loss accounted for 56.22% and 380 cases with normal hearing accounted for 43.78%. Proportion of different degree of hearing loss of 792 ears from high to low was as follows: mild, 366 ears（46.21%）; moderate, 214 ears（27.02%）; severe, 151 ears（19.07%）; profound, 61 ears（7.70%）. There were statistically significant differences in the proportion of different hearing loss degree among 0-<3 months group, 3-<6 months group, 6-<12 months group and ≥12 months group（P<0.001）. Pairwise comparison between groups showed that the proportion of mild hearing loss of 0-<3 months group was higher than that in the other three groups（P<0.05）, there was no significant difference of moderate hearing loss among all groups（P>0.05）, the proportion of severe hearing loss of ≥12 months group was higher than that of 0-<3 months group（P<0.05）. The proportion of profound hearing loss with 0-<3 months group was lower than the other three groups（P<0.05）. In 792 ears with hearing loss, sensorineural hearing loss accounted for 67.42%, conductive hearing loss accounted for 20.71% and mixed hearing loss accounted for 11.87%. Among 98 cases with high risk factors for hearing loss, 58 cases（59.18%） were diagnosed with hearing loss. The incidence of hearing loss with high risk factors ranked from high to low was: craniofacial malformation（93.75%）, family history/congenital genetic syndrome（61.11%）, neonatal intensive care unit（NICU） hospitalization（46.43%） and others（20.00%）. Conclusion:Referrals from maternal and child institutions play an important role in the early detection of children with mild to moderate sensorineural hearing loss. Children with craniofacial malformation, family history/congenital genetic syndrome, hospitalization history of NICU and other high risk factors have a high incidence of hearing loss and should be attached with great importance.

Availability of data for cost-effectiveness comparison of child vision and hearing screening programmes.

OBJECTIVE: For cost-effectiveness comparison of child vision and hearing screening programmes, methods and data should be available. We assessed the current state of data collection and its availability in Europe. METHODS: The EUSCREEN Questionnaire, conducted in 2017-2018, assessed paediatric vision and hearing screening programmes in 45 countries in Europe. For the current study, its items on data collection, monitoring and evaluation, and six of eleven items essential for cost-effectiveness analysis: prevalence, sensitivity, specificity, coverage, attendance and loss to follow-up, were reappraised with an additional questionnaire. RESULTS: The practice of data collection in vision screening was reported in 36% (N = 42) of countries and in hearing screening in 81% (N = 43); collected data were published in 12% and 35%, respectively. Procedures for quality assurance in vision screening were reported in 19% and in hearing screening in 26%, research of screening effectiveness in 43% and 47%, whereas cost-effectiveness analysis was performed in 12% for both. Data on prevalence of amblyopia were reported in 40% and of hearing loss in 77%, on sensitivity of screening tests in 17% and 14%, on their specificity in 19% and 21%, on coverage of screening in 40% and 84%, on attendance in 21% and 37%, and on loss to follow-up in 12% and 40%, respectively. CONCLUSIONS: Data collection is insufficient in hearing screening and even more so in vision screening: data essential for cost-effectiveness comparison could not be reported from most countries. When collection takes place, this is mostly at a local level for quality assurance or accountability, and data are often not accessible. The resulting inability to compare cost-effectiveness among screening programmes perpetuates their diversity and inefficiency.

Evaluación de la competencia pragmática de cuatro adultos españoles con discapacidad auditiva congénita a través del Protocolo Rápido de Evaluación Pragmática - Revisado / Assessing the pragmatic competence of four spanish adults with congenital hearing loss through Protocolo Rápido de Evaluación Pragmática - Revisado

Numerosos estudios apuntan a las dificultades del lenguaje oral que pueden presentar las personas con discapacidad auditiva a lo largo de su desarrollo. No obstante, se conoce poco acerca del nivel de competencia pragmática que alcanzan y cómo esta área se desarrolla. En esta investigación se pretenden abordar las características pragmáticas de cuatro adultos españoles con discapacidad auditiva congénita a través del Protocolo Rápido de Evaluación Pragmática Revisado (PREP-R), que evalúa diferentes niveles de pragmática: textual, enunciativa e interactiva y, además,aporta un índice de habilidad pragmática general, específica y de base gramatical. Los participantes fueron evaluados mediante videograbaciones de muestras de lenguaje espontáneo en conversación con un familiar. Los resultados indican que, en general, los cuatro sujetos presentan un buen nivel de competencia pragmática, que se manifiesta a la hora de ajustar los actos de habla. Sin embargo, para regular su lenguaje, tienden a utilizar conductas compensatorias como: estrategias verbales que les permiten ganar tiempo extra para la construcción de sus emisiones, empleo de actos verbales y/o paraverbales compensatorios y el uso de gestos que completan su producción verbal. Estos datos indican que, aunque los participantes de este estudio presentan buenas habilidades pragmáticas, es necesario seguir desarrollando estrategias a nivel de intervención que les permitan comunicarse sin dificultades en diferentes contextos y con distintos interlocutores.

Numerous studies reveal the oral language difficulties that people with hearing loss may present throughout their development. However, little is known about the level of pragmatic competence they achieve and how this area evolves. This research aims to address the pragmatic characteristics of four Spanish adults with congenital hearing loss through Protocolo Rápido de Evaluación Pragmática -Revisado (PREP-R, which can be translated as Quick Protocol for Pragmatic Evaluation -Revised). This test assesses different levels of pragmatics: textual, enunciative, and interactional, and also provides an indicator for general, specific, and grammatically-based pragmatic ability. The participants were assessed by videotaping spontaneous speech samples in conversation with a family member. The results indicate that, in general, the four subjects present an adequate level of pragmatic competence, which is manifested in their adjustment of speech acts. Nevertheless, they show a tendency to use compensatory behaviors toregulate their speech, such as verbal strategies that allow them to gain extra time to construct their utterances, compensatory verbal and/or paraverbal acts, and gestures that complement their verbal productions. These data indicate that, although the participants of this study show good pragmatic skills, it is necessary to continue developing intervention strategies that allow them to communicate without difficulties in different contexts and with different communication partners.

Analysis of Newborn Hearing Screening Results in South Korea after National Health Insurance Coverage: A Nationwide Population-Based Study.

Newborn hearing screening (NHS) has been covered by national health insurance since October 2018 in Korea. However, the results of the NHS are not reported due to the absence of a follow-up tracking system. This study analyzed the status and the predicted referral rates of NHS after the Korean national health insurance coverage by analyzing the National Health Insurance Service database in 2019 and 2020. The NHS coverage was 91.7% of total birth in 2019 and 92.1% in 2020. The predicted referral rate of NHS calculated by the duplicated NHS cases was 1.05% in 2019 and 0.99% in 2020. However, another predicted referral rate calculated by the number of diagnostic auditory brainstem responses (ABRs) performed was 1.44% in 2019 and 1.43% in 2020. The first NHS was performed within one day of birth for 96.5% of the babies and within three days of birth for 97%. However, diagnostic ABR was adequately performed within three months of birth for only 4.3%, while 82.3% performed the test after six months which delays appropriate intervention for hearing loss. National support such as national coordinators, follow-up tracking, and data management systems are needed for early hearing detection and intervention of newborns and infants in Korea.

Valores normativos de pruebas conductuales de procesamiento auditivo para niños chilenos entre 7 y 12 años: una propuesta inicial / Normative values of auditory processing behavioral tests for chilean children between 7 and 12 years old: an initial proposal

Resumen Introducción: El trastorno del procesamiento auditivo en niños está relacionado con trastornos de lenguaje, bajo rendimiento académico, trastornos de aprendizaje y dificultades psicosociales. Existe consenso internacional sobre la utilidad de las pruebas conductuales para su evaluación y diagnóstico. Objetivo: Establecer valores normativos para pruebas conductuales de procesamiento auditivo en población pediátrica chilena con neurodesarrollo y audición normal. Material y Método: Se realizó un estudio observacional de corte transversal. Participaron 153 sujetos entre 7 y 12 años de la Región Metropolitana. Se estudiaron las pruebas de patrones de frecuencia, habla filtrada y dígitos dicóticos. Se construyeron modelos de regresión fraccional para estimar los valores normativos y además se calcularon los puntajes de corte en los percentiles 2.5, 5 y 10. Resultados: No se evidenciaron diferencias significativas entre oídos para las pruebas estudiadas a excepción de la prueba de dígitos dicóticos. Se construyeron modelos únicos para las pruebas patrones de frecuencia y habla filtrada, y modelos independientes para cada oído para la prueba dígitos dicóticos. Todas las estimaciones resultaron significativas y tuvieron niveles aceptables de precisión. Conclusión: Se obtuvieron los valores normativos y puntajes de corte para las tres pruebas estudiadas. Los valores obtenidos fueron similares a los reportados en otras poblaciones considerando los efectos de oído, de edad, sexo y escolaridad.

Abstract Introduction: Auditory processing disorder in children is related to language disorders, poor academic performance, learning disorders, and psychosocial difficulties. There is international consensus on the usefulness of behavioral tests for their evaluation and diagnosis. Aim: To establish normative values for auditory processing behavioral tests in Chilean pediatric population with normal neurodevelopment and hearing thresholds. Material and Method: An observational cross-sectional study was carried out. One hundred fifty-three subjects between 7 and 12 years old from the Metropolitan Region participated. Frequency pattern tests, filtered speech, and dichotic digits were studied. Fractional regression models were built to estimate the normative values, and cut-off scores were also calculated at the 2.5, 5, and 10 percentiles. Results: There were no significant differences between ears for the tests studied except for the dichotic digits test. Single models were built for the frequency and filtered speech patterns tests and independent models for each ear for the dichotic digit test. All estimates were significant and had acceptable levels of precision. Conclusion: The normative values and cut-off scores were obtained for the three tests studied. The values obtained were similar to those reported in other populations considering the effects of ear, age, sex, and education.

A systematic review of newborn and childhood hearing screening around the world: comparison and quality assessment of guidelines.

BACKGROUND: This study aimed to assess the quality of global guidelines or consensus statements for newborn and childhood hearing screening, as well as to compare various guidelines between other countries and China. METHODS: A PROSPERO registered systematic review (number CRD42021242198) was conducted. Multiple electronic databases and government websites including PubMed, EMBASE, Web of Science, CENTRAL, Cochrane Library, and BMJ Best Practice were searched from inception until May 2021. The latest national and international guidelines, consensus statements, technical specifications, and recommendations regarding newborn or childhood hearing screening that were published in Chinese or English medical journals or elsewhere with the full version available online. The following information was extracted independently by two reviewers for comparative analysis: titles, authors, publication year, country, the source organization, and main key recommendations using systems for assigning the level of evidence and strength of recommendations. The quality of the guidelines was assessed by three independent reviewers using the Appraisal of Guidelines for Research and Evaluation, 2nd edition. Intraclass correlation coefficients (ICCs) were calculated to assess among-reviewer agreement. RESULTS: We assessed 15 newborn and 6 childhood hearing screening guidelines, respectively. Most newborn guidelines recommend the 1-3-6 guidelines and pre-discharge screening; however, the specific screening times differ. 93.33% of newborn hearing guidelines recommend "primary screening-re-screening-diagnosis-intervention" for well-babies while 73.33% of the guidelines recommend "initial screening-diagnosis-intervention" for newborns in neonatal intensive care unit (NICU); 33.33% of the newborn hearing guidelines recommended initial screening coverage of > 95% while 46.66% did not mention it. Further, 26.66% of the newborn hearing guidelines recommended a referral rate to diagnosis within 4% while 60% did not mention it. Regarding childhood hearing screening guidelines, the screening populations differed across guidelines (age range: 0-9 years); most guidelines recommend pediatric hearing screening for all preschoolers. Only 50% of the guidelines specify screening and re-screening techniques, including pure-tone hearing screening, OAE, tympanometry, and others. The "Clarity of Presentation" domain achieved the highest mean score, and the lowest was "Editorial Independence" both in newborn and childhood guidelines. Overall score of newborn hearing screening guidelines ranged from 3 (2018 Europe) to 7 (2019 America), with an average score of 5.33. Average score of childhood hearing screening guidelines was 4.78, with the score ranging from 4 (2017 England, 2012 Europe, 2016 WHO) to 6.67 (2011 America). ICC analysis revealed excellent agreement across 21 guidelines (> 0.75). CONCLUSIONS: These findings indicated newborn hearing screening guidelines had superior quality over childhood ones. Comparative analysis suggested that recommendations of the Chinese newborn and pediatric hearing screening protocols are consistent with the mainstream international opinion. Moreover, this analysis demonstrated that "Editorial Independence" and "Stakeholder Involvement" have the greatest opportunities for improvement. These results may help to advance the quality of hearing screening guidelines in clinical practice and guide evidence-based updates.

Prevalence and characteristics of hearing and vision loss in preschool children from low income South African communities: results of a screening program of 10,390 children.

BACKGROUND: The majority of children with sensory impairments live in low- and middle-income countries. More studies of hearing and vision impairment prevalence are needed, in order to generate more accurate estimates of trends in sensory impairments. This study aimed to estimate the prevalence and describe the characteristics of hearing and vision loss among preschool children (4-7 years) in an underserved South African community following community-based mobile health (mHealth) supported hearing and vision services. METHODS: A screening program of sensory impairments was undertaken of children attending preschools in the communities of Khayelitsha and Mitchell's Plain, Cape Town, from September 2017 until June 2019. Hearing and vision screening were done by trained community health workers using mHealth technology. Children who failed hearing and vision screening were seen for follow-up assessments at their preschools. Follow-up assessments were conducted using smartphones that host point-of-care validated and calibrated hearing and vision testing applications (hearTest app, hearX Group, South Africa and PeekAcuity app, Peek Vision, United Kingdom). Descriptive statistical analysis and logistic regression analysis were conducted after extracting data from a secure cloud-based server (mHealth Studio, hearX Group) to Microsoft Excel (2016). RESULTS: A total of 10,390 children were screened at 298 preschools over 22 months. Of the children screened, 5.6 and 4.4% of children failed hearing and vision screening respectively. Community-based follow-up hearing tests were done at the preschools on 88.5% (514) of children of whom 240 children (54.2% female) presented with hearing loss. A preschool-based follow-up vision test was done on 400 children (88.1%). A total of 232 children (46.1% female) had a vision impairment, and a further 32 children passed the test but had obvious signs of ocular morbidity. Logistic regression analysis found that age was a significant predictor of vision loss (p < 0.05), but not for hearing loss (p = 0.06). Gender was not a significant predictor of hearing (p = 0.22) or vision loss (p = 0.20). CONCLUSIONS: Hearing loss is prevalent in at least 22 per 1000 and vision loss in at least 23 per 1000 preschool children in an underserved South African community. Timely identification of sensory losses can be facilitated through community-based hearing and vision services supported by mHealth technology.

Cost-Effectiveness of Hearing Screening in Older Adults: A Scoping Review.

OBJECTIVES: Age is the most common predictor of hearing loss; however, many older adults are not screened. Hearing screening could improve healthcare access, participation, and outcomes. Establishing whether hearing screening in older adults is cost-effective could improve the availability and utilization of screening. METHODS: We searched nine databases in January 2020. Studies with populations aged 50+ and provided data on the cost-effectiveness of hearing screening were included in the review. RESULTS: Five studies met the inclusion criteria and all found hearing screening programs to be cost-effective compared to no hearing screening, regardless of screening method (i.e., instrument or strategy). The maximum number of repeated screenings, coupled with younger ages, was most cost-effective. DISCUSSION: This review suggests that hearing screening in older adults is cost-effective, however, the evidence is limited. There is pressing need for research focused on economic impacts of hearing healthcare in older adults to inform research, policy and practice.

Performance and characteristics of the Newborn Hearing Screening Program in Campania region (Italy) between 2013 and 2019.

PURPOSE: Universal newborn hearing screening (UNHS) in the first month of life is crucial for facilitating both early hearing detection and intervention (EHDI) of significant permanent hearing impairment (PHI). In Campania region, UNHS has been introduced in 2003 by the Regional Council Resolution and started on January 2007. The aim of this paper is to update a previous article describing the performance of the program since its implementation in the period between 2013 and 2019. METHODS: A longitudinal retrospective study was carried at the Regional Reference Center III on 350,178 babies born in the analysis period. The paper reports the main results of overall coverage, referral rate, lost-to-follow-up rate,yield for PHI and shall determine various risk factor associations with hearing impairment RESULTS: In Campania region, 318,878 newborns were enrolled at I level, with a coverage rate of 91.06%, 301,818 (86.18%) Well Infant Nurseries (WIN) and 17,060 (5.35%) Neonatal Intensive Care Unit (NICU) babies. PHI was identified in 413 children, 288 (69.73%) bilaterally and 125 (30.26%) unilaterally. The overall cumulative incidence rate of PHI was 1.29 per 1000 live-born infants (95% CI 1.17-1.42) with a quite steady tendency during the whole study period. CONCLUSIONS: This study confirms the feasibility and effectiveness of UNHS in Campania region also in a setting with major socioeconomic and health organization restrictions.The program meets quality benchmarks to evaluate the progress of UNHS. Nowadays, it is possible to achieve an early diagnosis of all types of HL avoiding the consequences of hearing deprivation.

Desempenho em leitura, ordenação temporal, motivação e queixas escolares: estudo preliminar / Reading performance, temporal ordering, motivation and school complaints: preliminary study

RESUMO Objetivo verificar a associação entre o desempenho em leitura de crianças e adolescentes e a presença de queixas escolares, habilidade auditiva de ordenação temporal e motivação escolar. Métodos estudo observacional analítico transversal, com amostra de conveniência composta por 36 participantes. Os instrumentos de coleta foram questionário de caracterização, Critério de Classificação Econômica do Brasil, avaliação auditiva, testes de Memória para Sons Verbais, Não Verbais em Sequência, Padrão de Frequência e de Duração, Desempenho Escolar, as Provas de Avaliação dos Processos de Leitura e a Escala para Avaliação da Motivação Escolar Infantojuvenil. A variável resposta deste estudo foi "processos de leitura". Os testes Qui-quadrado de Pearson e Exato de Fisher foram utilizados para as análises de associação. Resultados as análises de associação revelaram que a maioria dos participantes com alteração nas tarefas de leitura apresentou, também, adequação da ordenação temporal simples e inadequação da ordenação temporal complexa. Não houve associação com significância estatística entre o desempenho em leitura e a presença de queixas escolares, habilidade auditiva de ordenação temporal e motivação escolar. Contudo, observou-se que a maioria dos participantes com alteração na leitura apresentou queixa de dificuldades escolares, baixo desempenho acadêmico e motivação escolar média. Conclusão embora não tenha indicado significância estatística na maioria das associações realizadas, o presente estudo evidenciou que há relação entre o desempenho em leitura de crianças e adolescentes e a presença de queixas escolares, habilidade auditiva de ordenação temporal e motivação escolar.

ABSTRACT Purpose To verify the association between reading performance of children and adolescents and the presence of school complaints, hearing ordering ability and school motivation. Methods Cross-sectional observational study with a convenience sample composed of 36 participants. The instruments used were the Sociodemographic and School Characterization Questionnaire, the Economic Classification Criterion of Brazil, the auditory evaluation, the Memory Tests for Verbal and Non-verbal Sounds in Sequence, the Frequency and Duration Pattern Tests, the School Performance Test, the Assessment of Reading Processes and the Scale for Assessing Children's School Motivation. The response variable in this study was the reading process. Pearson's Chi-square and Fisher's Exact tests were used for association analyzes. Results The association analysis revealed that most participants who presented changes in reading tasks also presented adequacy of simple temporal ordering and inadequacy in complex temporal ordering. There was no statistically significant association between reading performance and the presence of school complaints, temporal ordering auditory ability and school motivation. However, it was observed that most participants with reading disorders complained of school difficulties, poor academic performance, average school motivation, adequacy of simple temporal ordering and inadequacy of complex temporal ordering. Conclusion Although it did not show statistical significance in most of the associations performed, the present study showed that there is an relationship between the reading performance of children and adolescents and the presence of school complaints, temporal ordering auditory ability and school motivation.

Decentralising paediatric hearing services through district healthcare screening in Western Cape province, South Africa.

BACKGROUND: Childhood hearing loss is a global epidemic most prevalent in low- and middle-income countries where hearing healthcare services are often inaccessible. Referrals for primary care services to central hospitals add to growing lists and delays the time-sensitive treatment of childhood hearing loss. AIM: To compare a centralised tertiary model of hearing healthcare with a decentralised model through district hearing screening for children in the Western Cape province, South Africa. SETTING: A central paediatric tertiary hospital in Cape Town and a district hospital in the South Peninsula region. METHODS: A pragmatic quasi-experimental study design was used with a 7-month control period at a tertiary hospital (June 2019 to December 2019). Decentralising was measured by attendance rates, travelling distance, number of referrals to the tertiary hospital and hearing outcomes. There were 315 children in the tertiary group and 158 in the district group. Data were collected from patient records and an electronic database at the tertiary hospital. RESULTS: Attendance rate at the district hospital was significantly higher (p 0.001). Travel distance to the district hospital was significantly shorter (p 0.001). Number of referrals to the tertiary hospital decreased significantly during the intervention period (p 0.001). Most children in both the tertiary and district groups (78.7% and 80.4%, respectively) passed initial hearing screening bilaterally. CONCLUSION: Hearing screening should be conducted at the appropriate level of care to increase access, reduce patient travelling distances and associated costs and reduce the burden on tertiary-level hospitals.

Procedimiento para el pesquisaje auditivo universal en la población neonatal, Cuba, 2018 / A Procedure for Universal Hearing Screening in the Neonatal Population, Cuba, 2018

Introducción: El pesquisaje auditivo neonatal es una de las técnicas más utilizadas para realizar el diagnóstico precoz y oportuno de las pérdidas auditivas que llevan a una discapacidad. Objetivo: Diseñar el procedimiento para la pesquisa auditiva universal en la población neonatal cubana. Métodos: Estudio de desarrollo tecnológico, realizado de enero a diciembre de 2017. Se seleccionaron expertos mediante la autoevaluación y el coeficiente de competencia de Kappa, quienes participaron en el diseño a través de la técnica de grupos nominales, para el análisis de los problemas que limitan el pesquisaje auditivo universal y las potencialidades científicas, organizativas y funcionales de la red de servicios de atención a los desórdenes auditivos. Con posterioridad valoraron el procedimiento de forma individual y se aplicó la técnica de grupos nominales para lograr consenso. La revisión bibliográfica permitió valorar los aciertos y dificultades de otros procedimientos establecidos en otros contextos. Resultados: Se obtuvo como resultado el procedimiento para el pesquisaje auditivo universal, estructurado en tres componentes: pesquisaje, diagnóstico y rehabilitación. Conclusiones: El procedimiento diseñado para la detección universal precoz neonatal de las pérdidas auditivas en la población neonatal cubana estandariza los procesos de prestación de servicios de salud, mediante la gestión integral para prevenir y controlar, en forma oportuna, los eventos que afectan la salud auditiva(AU)

Introduction: Neonatal hearing screening is one of the most widely used techniques for early and timely diagnosis of hearing losses that lead to disability. Objective: To design the procedure for universal hearing screening in the Cuban neonatal population. Methods: Technological development study carried out from January to December 2017. Experts were selected through self-evaluation and the Kappa competence coefficient. The experts participated in the design through the nominal groups technique, in view of the analysis of the problems limiting universal hearing screening and the scientific, organizational and functional potentialities of the healthcare network for hearing disorders. Subsequently, they assessed the procedure individually and the nominal groups technique was applied to achieve consensus. The bibliographic review allowed to assess the accomplishments and difficulties of other procedures established in other settings. Results: The procedure for universal hearing screening was obtained as a result, structured in three components: screening, diagnosis and rehabilitation. Conclusions: The procedure designed for universal early neonatal screening of hearing loss in the Cuban neonatal population standardizes the processes of providing healthcare, through comprehensive management to prevent and control, timely, events that affect hearing-related health(AU)

Community-Based Hearing and Vision Screening in Schools in Low-Income Communities Using Mobile Health Technologies.

Introduction Globally, more than 50 million children have hearing or vision loss. Most of these sensory losses are identified late due to a lack of systematic screening, making treatment and rehabilitation less effective. Mobile health (mHealth), which is the use of smartphones or wireless devices in health care, can improve access to screening services. mHealth technologies allow lay health workers (LHWs) to provide hearing and vision screening in communities. Purpose The aim of the study was to evaluate a hearing and vision school screening program facilitated by LHWs using smartphone applications in a low-income community in South Africa. Method Three LHWs were trained to provide dual sensory screening using smartphone-based applications. The hearScreen app with calibrated headphones was used to conduct screening audiometry, and the Peek Acuity app was used for visual acuity screening. Schools were selected from low-income communities (Gauteng, South Africa), and children aged between 4 and 9 years received hearing and vision screening. Screening outcomes, associated variables, and program costs were evaluated. Results A total of 4,888 and 4,933 participants received hearing and vision screening, respectively. Overall, 1.6% of participants failed the hearing screening, and 3.6% failed visual acuity screening. Logistic regression showed that female participants were more likely to pass hearing screening (OR = 1.61, 95% CI [1.11, 2.54]), while older children were less likely to pass visual acuity screening (OR = 0.87, 95% CI [0.79, 0.96]). A third (32.5%) of referred cases followed up for air-conduction threshold audiometry, and one in four (25.1%) followed up for diagnostic vision testing. A high proportion of these cases were confirmed to have hearing (73.1%, 19/26) or vision loss (57.8%, 26/45). Conclusions mHealth technologies can enable LHWs to identify school-age children with hearing and/or vision loss in low-income communities. This approach allows for low-cost, scalable models for early detection of sensory losses that can affect academic performance.

Use of Diagnostic Testing and Intervention for Sensorineural Hearing Loss in US Children From 2008 to 2018.

Importance: Early detection and intervention of pediatric hearing loss is critical for language development and academic achievement. However, variations in the diagnostic workup and management of pediatric sensorineural hearing loss (SNHL) exist. Objective: To identify patient and clinician factors that are associated with variation in practice on a national level. Design, Setting, and Participants: This cross-sectional study used the Optum claims database to identify 53 711 unique children with SNHL between January 1, 2008, and December 31, 2018. Main Outcomes and Measures: National use rates and mean costs for diagnostic modalities (electrocardiogram, cytomegalovirus testing, magnetic resonance imaging, computed tomography, and genetic testing) and interventions (speech-language pathology, billed hearing aid services, and cochlear implant surgery) were reported. The associations of age, sex, SNHL laterality, clinician type, race/ethnicity, and household income with diagnostic workup and intervention use were measured in multivariable analyses. Results: Of 53 711 patients, 23 735 (44.2%) were girls, 2934 (5.5%) were Asian, 3797 (7.1%) were Black, 5626 (10.5%) were Hispanic, 33 441 (62.3%) were White, and the mean (SD) age was 7.3 (5.3) years. Of all patients, 32 200 (60.0%) were seen by general otolaryngologists, while 7573 (14.10%) were seen by pediatric otolaryngologists. Diagnostic workup was received by 14 647 patients (27.3%), while 13 482 (25.1%) received intervention. Use of genetic testing increased (odds ratio, 1.22 per year; 95% CI, 1.20-1.24), whereas use of computed tomography decreased (odds ratio, 0.93 per year; 95% CI, 0.92-0.94) during the study period. After adjusting for relevant covariables, children who were seen by pediatric otolaryngologists and geneticists had the highest odds of receiving workup and intervention. Additionally, racial/ethnic and economic disparities were observed in the use of most modalities of diagnostic workup and intervention for pediatric SNHL. Conclusions and Relevance: This cross-sectional study identified factors associated with disparities in the diagnostic workup and intervention of pediatric SNHL, thus highlighting the need for increased education and standardization in the management of this common sensory disorder.

Teacher Prescreening for Hearing Loss in the Developing World.

The goal of this prospective cohort study was to characterize the ability of teachers to identify schoolchildren at risk of hearing loss in order to maximize hearing screening efficiency in low-resource settings. At 4 semirural schools in Malindi, Kenya, preselected schoolchildren perceived as hearing impaired were compared to children thought to have normal hearing using portable audiometry. Eight of 127 children (54% male) failed hearing screening, all of who were identified by schoolteachers as having a high risk of hearing loss. Thus, for every 5 children prescreened by schoolteachers, an average of 1 child would be identified as having hearing loss. Overall, teacher prescreening had a 100% hearing loss identification rate and a 20% referral rate. In conclusion, in resource-limited settings, where universal hearing screening is challenging, teachers can effectively identify children with hearing loss for early intervention.

Comprehensive Community Screening of Otological Patients by Trained Technicians Using a Telemedicine Device: An Efficient and Cost-Effective Way to Triage Patients With Ear Diseases.

OBJECTIVE: To emphasize the benefits of tele-otology in community screening of patients with ear diseases. METHODS: A retrospective study of all patients screened and treated under the Shruti tele-otology program between 2013 and 2019 was conducted. It involved screening, diagnosis, medical management, surgical intervention, and rehabilitation using hearing aid. The study focused on underprivileged and underserved community of rural and urban slums across 12 states of India. The study was conducted using a telemedicine device called ENTraview, that is, a camera-enabled android phone integrated with an otoscope and audiometry screening. RESULT: A total of 810 746 people were screened, and incidence of various ear diseases was recorded. Ear problems were found in 265 615 (33%) patients, of which 151 067 (57%) had impacted wax, 46 792(18%) had chronic suppurative otitis media, 27 875 (10%) had diminished hearing, 12 729 (5%) had acute otitis media and acute suppurative otitis media (ASOM), and 27 152 (10%) had problems of foreign body, otomycosis, and so on. Of the total 265 615 referred patients, 20 986 (8%) reported for treatment and received treatment at a significantly reduced cost through Shruti program partners. The conversion rate of nonsurgical and surgical procedure was also compared, and it was found that, while 9% of the patients opted for nonsurgical treatment, only 3% opted for surgery in the intervention group giving a significant P value of .00001. CONCLUSION: The potential for telemedicine to reduce inequalities in health care is immense but remains underutilized. Shruti has largely been able to bridge this gap as it is an innovative, fast, and effective programs that address the ear ailment in the community.