Effect of Maternal Diabetes Mellitus on Neonatal Hearing.

Introduction: Gestational diabetes is a potential risk factor for neonatal hearing loss. Increased circulating sugars in mothers during pregnancy can impairs the micro circulation and can cause congenital anomalies of the inner ear resulting in congenital hearing loss. This study attempts to find the incidence of neonatal hearing loss among diabetic mothers. Methodology: This was a case control study with 86 neonates of diabetic mothers as cases and neonates of non diabetic mothers(n = 86) as controls. Antenatal diabetic history and sugar values of mothers were documented. Hearing status of the neonates were tested using DPOAE test and ABR test. DPOAE test was done on 3rd day and those who did not get a positive response underwent 2nd DPOAE and also ABR test if 2nd DPOAE was negative. Results: All neonates underwent DPOAE test and few were lost on follow up. First and second DPOAE showed a statistically significant difference between cases and controls. All babies who underwent ABR test had abnormal waveforms. 98% of cases showed moderate and severe bilateral hearing loss whereas all controls had only mild bilateral hearing loss. Discussion: This study showed a significantly higher percentage of abnormal hearing outcome among neonates of diabetic mothers than non diabetic mothers. This could be because of the toxic effects of maternal hyperglycemia on developing auditory system of the fetus. This study emphasis the need for better glycaemic control in diabetic pregnancy, the importance of early and mandatory hearing screening in newborns of diabetic mothers.

Factores de riesgo relacionados con hipoacusia en recién nacidos mediante tamizaje neonatal auditivo en el departamento del Atlántico (Colombia), 2019-2020

Objetivos: Identificar los factores de riesgo relacionados con hipoacusia en recién nacido mediante tamizaje neonatal auditivo en el departamento del Atlántico (Colombia) en el periodo 2019-2020. Materiales y métodos: Estudio observacional, analítico, de casos y controles anidado en una cohorte. Los recién nacidos fueron atendidos en 4 instituciones de III nivel de complejidad en Atlántico entre 2019 y 2020. Se incluyeron 32 casos correspondientes a registros según criterios de la Organización Mundial de la Salud (OMS), sugestivos de alteración auditiva mediante la prueba de otoemisiones acústicas y 28 controles seleccionados de la misma población fuente, donde se originaron los casos en el mismo período. Se aplicaron los criterios de exclusión. Las variables cualitativas se presentaron en tablas de frecuencia y el análisis estadístico se realizó en el programa STATGRAPHICS versión 16. Para establecer diferencia entre los 2 grupos se utilizó la prueba (X2), calculándose su respectivo odds ratio, con un intervalo del 95 % de confianza. Resultados: Los principales factores de riesgo encontrados en recién nacido con alteración auditiva sugestiva de hipoacusia con significancia estadística (p<0,05) fueron la estancia en la Unidad de Cuidados Neonatales: OR 4,2 IC95 % (1,4-12,4) y uso de aminoglucósidos: OR 3,2 IC95 % (1,03-10,1). Conclusiones: Se encontró asociación entre la estancia en la Unidad de Cuidados Neonatales y uso de aminoglucósidos con alteración auditiva sugestivo de hipoacusia. Se debe tener en cuenta estos factores de riesgo para ser identificados en forma oportuna y realizar el manejo adecuado, como por ejemplo, disminuyendo tiempo de exposición.

Objectives: Identify risk factors related to hearing loss in newborns through neonatal hearing screening in the Department of Atlantic in the period 2019-2020. Materials and methods: Observational, analytical, case-control study nested in a cohort. Newborns were cared for in 4 level III of complexity health institutions in Atlantic between 2019 and 2020. 32 cases were included corresponding to records according to World Health Organization (WHO) criteria, suggestive of hearing impairment through the otoacoustic emissions test and 28 controls selected from the same source population, where the cases originated in the same period. Exclusion criteria were applied. The qualitative variables were presented in frequency tables and the statistical analysis was carried out in the STAT-GRAPHICS version 16 program. To establish the difference between the 2 groups, the (X2) test was used, calculating their respective odds ratio with a CI 95 %. Results: The main risk factors found in newborns with hearing impairment suggestive of hearing loss with statistical significance (p<0,05) were the stay in the Neonatal Care Unit: OR 4,2 CI95% (1,4-12,4) and use of aminoglycosides: OR 3,2 CI95 % (1,03-10,1). Conclusions: An association was found between the stay in the neonatal care unit and the use of aminoglycosides with hearing impairment suggestive of hearing loss. These risk factors must be taken into account to be identified in a timely manner and to carry out adequate management, such as reducing exposure time.

Universal newborn hearing screening in the Italian Region of Sicily in 2018.

OBJECTIVES: We have clarified the role of Universal Neonatal Hearing Screening (UNHS) for both early diagnosis and rapid treatment in order to improve the prognosis of the deaf child and reduce patient management costs. Although in Sicily UNHS has been progressively implemented, there is scarce data in the literature on this matter. Therefore, the main objective was to collect in the year 2018 the following data: number of newborns screened for hearing loss, number of infants "referred" to transiently evoked otoacoustic emissions (TEOAE), number of infants with pathologic auditory brainstem response (ABR) and number of infants affected by permanent hearing loss. METHODS: UNHS monitoring was conducted through the collection of data through a questionnaire, which was analysed evaluating the effectiveness and adherence to the screening program prepared by the Department for Health Activities and the Epidemiological Observatory (DASOE). RESULTS: In 2018, there were 40,243 newborns in Sicily. A total of 37,562 newborns were screened (93.3%). There were 1,328 "referred" infants with TEOAE (3.5%). On the 2nd level, "referred" newborns examined were 1,080 of 1,328 expected (missing 248 "refer" newborns, equal to 18.6%). The number of "referred" infants confirmed with TEOAE was 113 of 1,080, while "referred" infants confirmed with ABR were 71. On the 3rd level, 67 of 71 were infants examined: 28 infants were suffering from monolateral hearing loss (13 slight/mild, 13 moderate, 1 severe and 1 profound) and 39 from bilateral hearing loss (1slight/mild, 19 moderate, 13 severe and 7 profound). Excluding 7 infants from the NICU, 60 of 37,562 infants had hearing loss (1.5%). CONCLUSIONS: The monitoring of the UNHS in Sicily has allowed obtaining the data of individual centres, absent in the literature to date, to verify the effectiveness of the screening, according to JCIH criteria, to highlight some criticalities and, finally, to propose possible solutions.

Does riboflavin depletion cause auditory neuropathy spectrum disorder in at risk neonates?

We present a new hypothesis for the pathogenesis of auditory neuropathy spectrum disorder (ANSD) in at risk neonates involving depletion of riboflavin. The association between neonatal hyperbilirubinemia and ANSD is well recognized, yet causation has not been proven. The risk of ANSD does not correlate clearly with severity of hyperbilirubinemia and ASND only occurs in a small proportion of hyperbilirubinemic neonates. Additional, perhaps co-dependent, factors are therefore likely to be involved in pathogenesis. The metabolism of bilirubin consumes riboflavin and levels of riboflavin are depleted further by phototherapy. The neonate may also be deficient in riboflavin secondary to maternal deficiency, and reduced intake or impaired absorption. We propose that riboflavin depletion may be a significant contributor to development of ANSD in at risk neonates. The basis of this hypothesis is the recent recognition that impairment of riboflavin metabolism caused by genetic mutations (SLC52A2 or AIMF1) also causes ANSD.

Vestibular Infant Screening - Flanders: The implementation of a standard vestibular screening protocol for hearing-impaired children in Flanders.

OBJECTIVES: The Vestibular Infant Screening - Flanders (VIS-Flanders) project aims to implement and refine a vestibular screening protocol for all children with neonatal hearing loss in Flanders (Belgium) to limit the impact of a vestibular dysfunction on the motor, cognitive and psychosocial development of hearing-impaired children. METHODS: Each child with a confirmed neonatal hearing loss in Flanders will undergo a vestibular screening at the age of 6 months in the reference centers involved in the neonatal hearing screening program. The cervical Vestibular Evoked Myogenic Potential (cVEMP) test will be used as a screening tool. The test is short, child-friendly, feasible at a young age and highly correlated with motor and balance performance. The results of an extensive follow-up protocol at the Ghent University Hospital will enable further refinement of the screening protocol. RESULTS: Data collection in all reference centers has started since June 2018. This paper outlines the rationale for the screening and the set-up of this four-year project. CONCLUSIONS: Current available literature strongly favors vestibular assessment in congenitally hearing-impaired children. A standard vestibular screening for these children should lead to early identification of vestibular deficits and subsequent prompt referral for further motor assessment and rehabilitation, in order to limit the impact of a vestibular dysfunction in developing children and improve their quality of life.

Detección temprana de la hipoacusia con emisiones acústicas / Early hearing loss detection through otoacoustic emissions

Introducción: La hipoacusia neonatal es considerada un problema de salud pública. Su diagnóstico precoz y rehabilitación adecuada previenen la consecuencia más importante de la hipoacusia infantil: crecer sin un lenguaje. Objetivo: Analizar los resultados obtenidos en el Programa Universal de Detección Precoz de la Hipoacusia Infantil implementado en el Complejo Hospitalario Universitario Insular Materno Infantil de Las Palmas de Gran Canaria desde enero de 2007 hasta diciembre de 2013. Material y método: Se estudiaron 44.597 recién nacidos, mediante otoemisiones acústicas en ambas fases del programa. La fase diagnóstica es llevada cabo en la Unidad de Hipoacusia del Servicio de Otorrinolaringología. Resultados: Desde enero de 2007 y diciembre de 2013 nacieron 46.587 niños, se le practicaron el screening a 44.597, de los cuales, 41.621 presentan OEA positivas, 1.233 tienen OEA ausentes. En la segunda fase 8.193 presentaron otoemisiones positivas en ambos oídos, 649 no pasaron la segunda fase. Presentaron factores de riesgo para hipoacusia un total de 9.581 niños. La media de derivación a la fase diagnóstica fue de 5.69%, alcanzó una cobertura del 95,73%. 32 niños fueron diagnosticados entre los 6 y 8 meses ingresaron al Programa de Implantes Cocleares siendo intervenidos quirúrgicamente entre los 9 y 16 meses. Conclusión: El programa es adecuado a nuestro funcionamiento, alcanzando el mismo un porcentaje de cobertura superiordel 95% valor ampliamente satisfactorio teniendo en cuenta las recomendaciones de la CODEPEH.

Introduction: Neonatal hearing loss is a public health problem early diagnosis and (re) habilitation adequate prevent the most important consequence of infant hearing loss: growing up without a language. Aim: To analyze the results obtained in the Universal Program for Early Detection of Infant Hearing Loss implemented in the Complejo Hospitalario Universitario Insular Materno Infantil, in Las Palmas de Gran Canaria, from January 2007 to December 2013. Material and method: We studied otoacoustic emissions in both phases ofthe program for 44 597 newborns. The diagnostic phase was carried out at the Hearing Loss Unit, Department of Otorhinolaryngology. Results: Between January 2007 and December 2013, 46,587 children were born, 44,597 underwent screening, of which 41,621 have positive otoacustic emissions and in 1233 these are absent. In 8,193 the secondphase gave positive otoacustic emissions in both ears and 649 failed the second phase. 9,581 newborns presented risk factors associated with hearing loss. The average referral to diagnostic phase was 5.69%, the program reacheda coverage of 95.73%. 32 children diagnosedbetween 6and8months entered the Cochlear Implant Program being operated on between 9 and 16 months. Conclusion: The Program in our hospital has proved adequate, achieving a coverage rate above 95%, highly satisfactory value considering the recommendations of the CODEPEH.

Evolution of Italian Universal Newborn Hearing Screening Programs.

BACKGROUND: The aim of this study was to evaluate the state of implementation of the Universal Newborn Hearing Screening Programs in Italy and to determine the effect that an ad hoc legislation may have on the percentage of infants screened for detection of hearing impairment in nurseries. MATERIAL AND METHODS: Italian Newborn Hearing Screening data were obtained during four national surveys (years 2003, 2006, 2008, and 2011). The screening rates obtained by the Regions which adopted or did not adopt a legislation to increase the newborns' coverage were compared. RESULTS: In 2011, the average coverage rate was 78.3%, but in 12 out of 20 Regions it exceeded 95%. Coverage rate was greater in Regions that implemented an ad hoc legislation compared to Regions that did not. As a matter of fact, Regions which passed the legislation screened more than 95% of infants, whereas Regions without legislation reported a mean screening rate of nearly 67% of newborns. CONCLUSION: Current results seem to confirm that a specific legislation might have a decisive effect on the increase of rate of coverage of newborn hearing screenings.

Bone conduction noise exposure via ventilators in the neonatal intensive care unit.

OBJECTIVES/HYPOTHESIS: To demonstrate that neonatal ventilators can expose patients to high noise levels through bone conduction (BC) as well as air conduction (AC). STUDY DESIGN: Observational study. METHODS: Three ventilators and various settings on a positive airway pressure machine (continuous, high bilevel, and low bilevel pressure) were tested. A sound level meter was used to measure the noise levels at a set distance from the ventilator to represent AC, on the ventilator circuit to represent BC at the alveolus, and within the ventilator circuit. RESULTS: The BC sound levels (74.1, 81.1, 86, 89.2 dBC) were significantly higher than the AC sound levels (72.8, 72.9, 70, 71.7 dBC) for the jet ventilator, continuous positive airway pressure setting, low bilevel setting, and high bilevel setting, respectively (P < .001). The sound level within the ventilator circuit ranged from 94.9 to 113.2 dBC depending on the machine/setting and was significantly louder than both AC or BC for all machines/settings (P < .001). CONCLUSIONS: There are concerning ventilator dependent noise levels present on and within ventilation circuitry that could be presented to the infant via BC. LEVEL OF EVIDENCE: NA

The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

INTRODUCTION AND OBJECTIVE: Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. METHOD: This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. RESULTS: Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. CONCLUSIONS: The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated.

Survey on Infant Hearing Loss at Caritas Baby Hospital in Bethlehem-Palestine.

This study describes the epidemiology of infants' hearing loss (IHL) among patients under 3 months of age at Caritas Baby Hospital, the only pediatric hospital in Palestine. It was aimed to demonstrate that IHL is a major health problem in Palestine and to assess the first available data of the newborn hearing screening program conducted between September 25, 2006 and December 31, 2011. Data was uploaded and analyzed using Microsoft Excel and the Statistical Package for the Social Sciences software (SPSS version 21). A total of 8144 infants were tested, 4812 (59%) were males and 3332 (41%) were females. As to their origin, 72% (5886) came from the Bethlehem district, 25% (2044) from the Hebron district, while 3% (214) from the other Palestinian districts (Jericho, Ramallah, Nablus, Jenin and Jerusalem). The transient evoked otoacoustic emissions (TEOAEs) and the automated auditory brainstem response were used according to the manufacturer guidelines. The results were interpreted according to the indications of the American Academy of Pediatrics, the National Institutes of Health, and the European Consensus Development Conference on Neonatal Hearing Screening. Out of the 8144 infants tested, 1507 (14.6%) did not pass the 1(st) test, 477 (32.8%) of these 1507 infants failed retesting, while 498 (33%) patients were lost to follow-up. Only 152 (31.9%) patients that failed retesting went to an audiologist. The audiologist evaluation revealed that 101 (66.4%) patients presented with a mild-moderate or profound hearing loss according to the Bureau International of Audiophonologie standards, 44 (28.9%) patients had otitis media, whereas 7 cases (4.7%) had no hearing disorders. The overall unadjusted percentage of hearing loss was 1.24%, and the adjusted overall percentage was 1.85%. The chart review showed that jaundice, sepsis, prematurity, lung disease were more common among the affected patients. The high prevalence of childhood deafness in Palestine is of utmost importance and deserves immediate attention on the part of the Palestinian government. Meanwhile, Caritas Baby Hospital undertook to set up a newborn hearing screening unit utilizing the TEOAE method.

Evaluation of very low birth weight (≤ 1,500 g) as a risk indicator for sensorineural hearing loss.

INTRODUCTION AND OBJECTIVE: Hearing plays an essential role in the acquisition, development and maintenance of the properties of the speech and language. Birth weight is an indicator of biological maturation of the newborn. Premature newborns with very low birth weight (VLBW<1,500 g) constitute a group with the highest risk of sensorineural hearing loss. Our objective was to ascertain the degree of hearing loss, sensorineural hearing loss and presence of the association to other risk factors for hearing loss in VLBW infants included in the Universal Hearing Loss Screening Programme at the University Mother-Child Hospital of Gran Canaria (Spain) in the 2007-2010 period. MATERIAL AND METHODS: This was a retrospective study of 364 infants with VLBW, measured by transient evoked otoacoustic emissions and auditory brainstem response. RESULTS: There were 112 newborn (30.8%) referred for auditory brainstem response. A diagnosis of hearing loss was given to 22 newborns (2.2%), 14 had conductive hearing loss and 8, sensorineural hearing loss (SNHL), of which 2 had bilateral profound hearing loss. The VLBW newborn presented the association to another risk factor in more than a quarter of the sample studied. All those diagnosed with SNHL were premature. CONCLUSIONS: The percentage of VLBW newborns diagnosed with hearing loss is higher than expected in the general population. All those diagnosed with SNHL were premature and presented one or 2 hearing risk factors associated with VLBW.

Neonatal screening for hearing loss: pilot study from a tertiary care centre.

Many developed countries have well established universal neonatal hearing screening programs. In India, the viability of such a program, in an already overburdened health system is indeed a challenge. This cross sectional study was undertaken to evaluate the possible burden of hearing loss among neonates born at a tertiary care hospital in Southern India. Five hundred neonates were screened with automated distortion product otoacoustic emission (aDPOAE) for hearing loss, 9.2% of whom had one or more high risk factors. Although 6.4% had hearing loss at initial assessment, only 1.6% had hearing loss on retesting with aDPOAE. Retesting with OAE before an automated Auditory brainstem response (aABR) helped to exclude patients without hearing loss. The frequency of moderate to moderately severe hearing loss in this study was 0.6%. This pilot study underscores the importance of the introduction of screening for congenital deafness in specialized centers in India, despite its challenges.

Cochlear Implant

The development and improvement of cochlear auditory prostheses have radically reshaped the management of children and adults with significant hearing loss. Rapid evolution in the candidacy criteria and the technology has resulted in a large number of individuals who have benefited from implantation. Cochlear implants replace the normal inner ear by transforming acoustic sound signals into electric stimuli and deliver them to the auditory nerve. The decision to implant in a patient is a medical and surgical one. Candidacy issues, device selection, and planning for postoperative rehabilitation are complex, which require a dedicated cochlear implant team. Medical and surgical evaluation includes all aspects of cochlear implant candidacy, including critical analysis of hearing tests, motivation of the patient and family, and status of language development. Postoperative speech recognition results are variable. The expected results depend heavily on the environment in which cochlear implants are used, as well as on case selection. In children, implantation before the age of 2 years provides a distinct advantage over later implantation in cases of early-onset deafness. Postimplantation rehabilitation can be important for some adult implant recipients, but appears to be critical for children to optimize the usefulness of an implant. For early diagnosis of neonatal deafness and early implantation, a national neonatal hearing screening program is mandatory.

Incidence of hearing loss and importance of risk factors in the neonatal intensive care unit / 소아과

PURPOSE: To assess the incidence of neonatal hearing loss in a neonatal intensive care unit and the relative importance of risk factors for hearing imparement in a neonatal intensive care unit which the Joint Committee on Infant Hearing(JCIH) had recommended. METHODS: One thousand, two hundred and one newborns admitted to the Good Moonhwa Intensive Care Unit from May 2003 to December 2005 were assesed using the automated auditory brainstem response(AABR). The screening was performed on those aged more than 36 weeks and weighing more than 2,200 g. We divided the infants into two groups, 'pass' and 'refer'. The 'refer' group were retested one month later, and if classified as 'refer' during the retest, were referred to a hearing impairment clinic. RESULTS: From the 1,201 neonates, 1,187(98.8 percent) passed the test and 14(1.2 percent) failed. 293(24.4 percent) of the 1,201 neonates had a risk factor for hearing impairment; 282(96.2 percent) passed the test and 11(3.8 percent) failed. The group with risk factors were shown to have a higher incidence of hearing loss(P<0.001). The neonates in the refer group were shown to have a higher incidence of ototoxic drugs(P<0.001), low birth weight(<1,500 g)(P<0.001) and craniofacial anomalies(P=0.007). On the other hand, there were no statistical differences between the pass and refer groups in congenital infection, hyperbilirubinemia, bacterial meningitis, low Apgar scores, prolonged mechanical ventilation and syndromes known to include hearing loss. CONCLUSION: In order to identify hearing-impaired infants within an appropriate period, neonatal hearing screening tests and identification of the risk factors for neonatal hearing loss are important.

Evaluación auditiva neurosensorial en un grupo de prematuros del programa madre canguro / Sensorineural auditory evaluation in a group of preterm neonates

Entre agosto de 1989 y diciembre de 1991 se buscó asociación entre algunos factores de riesgo comunes a los prematuros con peso menor de 1.500 g al nacer y el hecho de presentar disminución de la agudeza auditiva, medida con los potenciales evocados del tallo cerebral a los 4 meses y luego entre los 8 y los 14 meses. Los factores de riesgo tenidos en cuenta en 133 prematuros del "Programa madre canguro" del Instituto de los Seguros Sociales, en Medellín, fueron: Peso inferior a 1.500 g, hiperbilirrubinemia superior a 10

A study was carried out to investigate the association between risk factors commonly found in preterm neonates with very low birth-weight (Iess than 1.500 g) and decreased auditory acuteness; the latter was measured according to brain stem evoked potentiaJs (BERA test) In 133 preterm neonates belonging to the "Kangaroo mother program", in 'Medellín,Colombla. The following risk factors we~ evaluated: Hyperbilirrubinemia, hyaline membrane, birth weight lower than 1.500g, fetal distress, hypoglycemia, use of aminoglycosldes, thlrd trimester hemorrhage, and maternal high blood presssure. Abnormal results in BERA test were found in 54 cases (40.6%) at 4 months and in only 10 (7.5%) in the final evaluation performed between 8 and 14 months of age. No association was found between risk factors and decreased auditory acuteness; the shift from abnormal to normal auditory acuteness can be explained by brain maturation factors In the preterm infant. Causes for final deficits remain to be investigated; follow-up programs including auditory tests in this group of children at risk should be established