Neonatal hearing screening

The aim of this study is to develop a mobile auditory training application based on gaming for children aged 3–5 years using cochlear implants and to evaluate its usability.

4 games were developed in the scope of the application World of Sounds; the crucible sound for auditory awareness, mole hunting for auditory discrimination, find the sound for auditory recognition, and choo-choo for auditory comprehension. The prototype was applied to 20 children with normal hearing and 20 children with cochlear implants, all of whom were aged 3–5. The participants were asked to fill out the Game Evaluation Form for Children. Moreover, 40 parents were included in the study, and the Evaluation Form for the Application was applied.

According to the form, at least 80% of children using cochlear implants, and all children in the healthy group, responded well to the usability factors. All factors were obtained as highly useable by parents of the children using cochlear implants. The results indicated that in the healthy group, the usefulness and motivation factors were above moderate, and the other factors were highly useable. In the mole-hunting game, there was no significant difference between the groups in the easy level of the first sub-section (p > 0.05). There was a significant difference between the groups in terms of the other sub-sections of the mole-hunting game and all sub-sections of the crucible sound, find the sound, and the choo-choo games (p < 0.05). While there was no correlation between duration of cochlear implant use and ADSI scores and the third sub-section of the crucible sound game (p > 0.05); a correlation was found in the other sub-sections of crucible sound and all sub-sections of the mole hunting, find the sound, and Choo-Choo games (p < 0.05).

It is thought that the application World of Sounds can serve as an accessible option to support traditional auditory rehabilitation for children with cochlear implants.

The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution.

We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher’s exact test and continuous variables by Mann–Whitney t-test (p ≤ 0.05).

A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p = 0.0130), prolonged course of hospitalization (p = 0.0011), duration of mechanical ventilation (p = 0.0046), requirement for tracheostomy (p = 0.0013), and duration of loop diuretic (p = 0.0005) and aminoglycoside therapy (p = 0.0003).

We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language.

The objective of this review was to provide a critical overview of our current understanding on the neurocognitive, neuromotor, and neurobehavioral development in congenital diaphragmatic hernia (CDH) patients, focusing on three interrelated clinical issues: (1) comprehensive outcome studies, (2) characterization of important predictors of adverse outcome, and (3) the pathophysiological mechanism contributing to neurodevelopmental disabilities in infants with CDH. Improved survival for CDH has led to an increasing focus on longer-term outcomes. Neurodevelopmental dysfunction has been recognized as the most common and potentially most disabling outcome of CDH and its treatment. While increased neuromotor dysfunction is a common problem during infancy, behavioral problems, hearing impairment and quality of life related issues are frequently found in older children and adolescence. Intelligence appears to be in the low normal range. Patient and disease specific predictors of adverse neurodevelopmental outcome have been defined. Imaging studies have revealed a high incidence of structural brain abnormalities. An improved understanding of the pathophysiological pathways and the neurodevelopmental consequences will allow earlier and possibly more targeted therapeutic interventions. Continuous assessment and follow-up as provided by an interdisciplinary team of medical, surgical and developmental specialists should become standard of care for all CDH children to identify and treat morbidities before additional disabilities evolve and to reduce adverse outcomes.

Presentamos los resultados de aplicar durante 42 meses un programa de detección precoz universal de la hipoacusia neonatal. Nuestro objetivo ha consistido en diagnosticar y tratar todas las hipoacusias permanentes unilaterales o bilaterales ≥ 40 dB nHL.

La estrategia de detección consta de 2 fases de cribado con otoemisiones acústicas transitorias (OEAT) y una fase de diagnóstico con potenciales evocados auditivos del tronco cerebral (PEATC). Los PEATC se realizaron tanto a los que “no pasaban” el cribado con OEAT como a los neonatos con factores de riesgo de hipoacusia.

De los 4.568 recién nacidos (RN) vivos, hemos evaluado al 97,6 %. Un 3,7 % de los nacidos presentaba factores de riesgo de hipoacusia. El 91,7 % de neonatos “pasó” la primera OEAT en ambos oídos, y tras la segunda sólo se derivó a la fase de diagnóstico al 1,3 %. Hemos detectado 2,7 hipoacusias severas-profundas bilaterales/1.000.

La finalidad de este programa reside en la aplicación precoz del tratamiento adecuado. Aunque resulta posible alcanzar los objetivos de cribado y diagnóstico de la hipoacusia neonatal, es mucho más complicada la fase de intervención, pues depende de diferentes disciplinas que deben actuar coordinadamente y múltiples factores ambientales y familiares difíciles de controlar. La efectividad terapéutica sigue siendo nuestro reto y el motivo de discusión para justificar plenamente los programas de detección precoz de la hipoacusia congénita.

We present the results from applying over a period of three and a half years a universal newborn hearing screening programme aimed at the early detection of hearing loss. Our goal has been to diagnose and treat all unilateral or bilateral permanent hearing losses ≥40 dB nHL.

The detection strategy consists of two screening phases with transient evoked otoacoustic emissions (TEOAE) and one diagnostic phase with auditory brainstem responses (ABR). ABR tests were performed on newborns “not passing” the TEOAE screening, as well as those with risk factors for hearing loss.

Of 4568 live newborns (NB), we evaluated 97.6 %. Of these, 3.7 % presented risk factors for hearing loss. The first TEOAE was “passed” in both ears by 91.7 % of the newborns; after the second TEOAE, only 1.3 % of cases were referred to the diagnostic phase. We detected 2.7/1000 cases of severe/profound bilateral hearing loss.

This programme was designed for early application of appropriate treatment. Although it is possible to reach the objectives of screening and diagnosis of newborn hearing loss, the intervention stage is much more complicated. This is because intervention depends on different disciplines that must act in co-ordination, as well as multiple environmental and family factors that are difficult to control. Therapeutic effectiveness still remains our challenge and a motive for discussion in fully justifying early congenital hearing loss screening programmes.

Universal hearing screening at the delivery centers enable early detection of hearing impairment. Since the 2002, hearing screening in neonates is performed in all Croatian delivery centers. The aim of this study was to propose the retest after 3 weeks period to those neonates with presumably hearing impairment demonstrated by evoked otoacoustic emission (E-OAE) method. Second aim was to asses the prevalence of hearing impairment in the Northern Adriatic region of Croatia.

Investigation has been conducted at the Department of Gynecology and Obstetrics, University Hospital in Rijeka, Croatia, in the period from 16 October 2002 to 31 December 2006. The hearing screening was performed in all newborns immediately before their discharge from the hospital, using the E-OAE method. Newborns with a possibility of hearing impairment were invited for retesting after 3 weeks, using the same method. Those newborns, whose repeated screening suggested a possible hearing impairment, were sent to an audiologist.

The hearing screening was performed by E-OAE method in 11.746 (98.9%) out of 11.869 newborns. The positive result indicating hearing impairment had 5.7% (676) children. These children where retested 3 weeks later using same method and only 128 (1.1%) of them still had positive results. Definite bilateral hearing impairment was confirmed by audiologists in seven children, which accounts for the total incidence of hearing impairment of 0.6‰.

Universal newborn hearing screening provides early detection of hearing loss. Retesting after 3 weeks with the same—E-OAE method we raised the specificity of E-OAE method, eliminating false-positive results (4.2%), decreased the attendance of healthy children to the audiologist center and most likely lowering the total costs and eventual parental and/or children stress.