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Introduction: The infant-toddler meaningful auditory integration scale (IT-MAIS) can evaluate toddlers and infants' early auditory development in aspects of detection, discrimination, and identification of sounds. The present study aims to evaluate the reliability and criterion validity of the Persian version of the IT-MAIS (IT-MAIS-P) for children with hearing loss (HL). Materials and Methods: In this study, due to the limitations of COVID-19 and the lack of access to samples, 23 available children with HL were sampled. The cause of participants› HL was 43.5% congenital, 30.4% acquired, and 26.1% unknown. The Mean±SD of the age of these children was 21.6±6.3 months. To evaluate the predictive validity and concurrent validity, the speech intelligibility rating (SIR) and categories of auditory performance (CAP) were used, respectively. Also, to assess the inter-rater reliability, both parents of each child completed the IT-MAIS-P. Results: A significant correlation (concurrent validity) was observed between the IT-MAIS-P and the CAP scores (r=0.87, P<0.001). Predictive validity was not confirmed by comparing it with the SIR (P>0.05). Also, the inter-rater reliability (kappa=0.44, P<0.001) of the IT-MAIS-P was confirmed. Conclusion: The IT-MAIS-P is a valid tool to investigate the progress of auditory skills in infants, although the predictive validity was not confirmed. [ABSTRACT FROM AUTHOR]

Parents of children with hearing loss (HL) often navigate an unfamiliar and uncertain path. This qualitative study investigated the nature of parental uncertainty: (1) immediately after a child's HL was identified; (2) prior to primary school entry; and (3) during primary school. Open-ended questions in online surveys were completed by participants (N = 61) with: (a) personal experience as a parent of a child with HL (n = 35); (b) professional experience with children who have HL (n = 19); or (c) a combination of both parental and professional experience (n = 7). Six major themes were abstracted using thematic analysis, including uncertainty about: (1) the Pervasive Impact of HL, (2) the Nature and Causes of HL, (3) Developmental Impacts, (4) Family Life, (5) Family Decision-Making, and (6) Education and Support Services. The salience of these depended on the stage in time following identification. [ABSTRACT FROM AUTHOR]

Permanent childhood hearing impairment (PCHI) represents the most frequent sensory pathology at birth. PCHI has a relevant psychological impact on the life of both the affected children and their families. Thus, the aim of this work is to explore the degree of parental distress felt by mothers of a deaf or hard-of-hearing child, to determine if this stress is associated with variables related to the children's health (e.g., the severity of hearing loss, presence of other conditions, difficulty with treatment options, difficulty with rehabilitation) or family characteristics such as socio-economic and educational status. The study used the Parenting Stress Index–Short Form (PSI-SF) questionnaire administered to mothers. The results were analyzed in relation to variables such as parents' education level, number of children, severity of hearing loss, presence of other chronic conditions, presence of cognitive delay, familiarity with hearing loss, time of diagnosis, use of prosthetics, and start in a rehabilitation program. The data indicate a correlation between maternal stress levels and low-educational levels, as well as the presence of congenital infections and cognitive delay. These results highlight the need for a comprehensive physical and psychological approach for hearing-impaired children, as stress factors can affect the adherence to effective rehabilitation. [ABSTRACT FROM AUTHOR]

Objective: To assess the conditions based on Early Language Development Test (TEDIL-3) scores after rehabilitation in children with hearing loss who continue special education in a single centre in Mardin, a city in southeast Türkiye. Material and Methods: Demography, auditory findings, depression status of 53 (53% male, 47% female, aged 3-7 years) children, given a special education, in July and October 2021, were evaluated by TEDIL-3 test. Group P consisted of patients with scores above average and F group consist of children having scores below average on the TEDIL-3 test. Scores in different characteristics and between groups were compared with analysis of correlations and factor analysis. Results: As a result of the study, 30 (57%) children have been found to be in Group P, 23 (43%) of them in Group F. Statistical significance and higher scores were seen in children with families with monolanguage than bilanguage (p<0.05). Diagnosis age was higher in Group F (mean±SD; 18.5±18.1) than group P (mean±SD; 7.5±11.4). Cochlear implant age was correlated with diagnostic age (p=0.013, n=35, r=0.64). Although neonatal hearing screening (NHS) does not have prognostic significance, in the NHS, 41.5% of children were false positives and 7.5% of them had no application to NHS (p>0.05). Conclusion: Bilingualism and the higher age of diagnosis in hearing loss are major negative factors that we encounter in the auditory rehabilitation by TEDIL scores in Mardin. Correct and compulsory implementation in NHS and raising awaireness on both issues will contribute to auditory rehabilitaion in children in Mardin. [ABSTRACT FROM AUTHOR]

The academic achievement of deaf and hard-of-hearing (DHH) students depends on the interaction of many factors, including student and family characteristics and experiences inside and outside school. Several questionnaires have previously been designed for evaluating the listening skills of deaf English-speaking children in school environments. This study's objective was to validate an Arabic questionnaire to evaluate deaf Egyptian Arabic-speaking children's listening skills. The Arabic questionnaire for listening skills in the school environment was adapted by translating items from an English questionnaire into Arabic. The questionnaire was administered to 70 Arabic-speaking Egyptian children 7–12 years, including 30 children with typical hearing, 30 deaf children fitted with hearing aids, and ten deaf children with cochlear implants. Typical hearing children demonstrated the best performance with a non-significant difference between children who use cochlear implants and hearing aids. The degree of hearing loss and language therapy duration were the best predictors of deaf Egyptian children's listening skills. The adapted Arabic questionnaire is a valid and reliable functional assessment tool that can be used to evaluate and monitor Arabic-speaking deaf students' listening skills in the school environment. [ABSTRACT FROM AUTHOR]

The aim of this article is to increase awareness of language practices in the deaf community that affect communication needs and health outcomes, focusing particularly on the prevalence of bilingualism among deaf adults. Language deprivation and poor health outcomes in the deaf population are risks that cannot be addressed solely by hearing intervention. We propose that bilingualism acts as a protective measure to minimize the health risks faced by deaf individuals. Provision of culturally and linguistically appropriate services to deaf stakeholders, and particularly hearing families of deaf children, requires familiarity with the developmental and social ramifications of bilingualism. [ABSTRACT FROM AUTHOR]

Introduction: To assess the performance of a tablet-based tele-audiometry method for automated hearing screening of schoolchildren through a comparison of the results of various hearing screening approaches.Methods: A total of 244 children were evaluated. Tablet-based screening results were compared with gold-standard pure-tone audiometry. Acoustic immittance measurements were also conducted. To pass the tablet-based screening, the children were required to respond to at least two out of three sounds for all the frequencies in each ear. Several hearing screening methods were analysed: exclusively tablet-based (with and without 500 Hz checked) and combined tests (series and parallel). The sensitivity, specificity, positive and negative predictive values and accuracy were calculated.Results: A total of 9.43% of children presented with mild to moderate conductive hearing loss (unilateral or bilateral). Diagnostic values varied among the different hearing screening approaches that were evaluated: sensitivities ranged from 60 to 95%, specificities ranged from 44 to 91%, positive predictive values ranged from 15 to 44%, negative predictive values ranged from 95 to 99%, accuracy values ranged from 49 to 88%, and area under curve values ranged from 0.690 to 0.883. Regarding diagnostic values, the highest results were found for the tablet-based screening method and for the series approach.Discussion: Compared with the results obtained by conventional audiometry and considering the diagnostic values of the different hearing screening approaches, the highest diagnostic values were generally obtained using the automated hearing screening method (including 500 Hz). Thus, this application, which was developed for the tablet computer, was shown to be a valuable hearing screening tool for use with schoolchildren. Therefore, we suggest that this hearing screening protocol has the potential to improve asynchronous tele-audiology service delivery. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study is to investigate the effect of hearing loss during early childhood on the development of children according to the age of diagnosis, amplification and training. Methods: In the study, a total of 169 children with bilateral sensorineural hearing loss, who have the chronological average age of 26.4 months, have been examined. All children are unilateral cochlear implant users and have no known additional impairments and/or diseases other than their hearing loss. The development of children with hearing loss, divided into three groups according to age of diagnosis, amplification, and training has been identified using the Denver Development Screening Test-II (DDST-II), and then, general development and sub-development results of these age characteristics have been compared accordingly. Results: As a result of the study, 105 (62.1%) out of 169 children identified with DDST-II have been found to be normal, 48 (28.4%) of them suspicious, and 16 (9.5%) children are found to be abnormal. It appears that the children who have been diagnosed before 6 months, instrumented between 3 and 6 months, and started to auditory-verbal training are revealed to have normal skills in their personal–social, language, fine, and gross motor field capabilities. As the age of diagnosis and intervention is delayed, the rate of delay in the development domains seems to be increasing, which is statistically significant (p < 0.001). Conclusions: Children with hearing loss develop similar outcomes in comparison with their normal auditory peers once they are diagnosed before 6 months and benefit from early intervention services. [ABSTRACT FROM AUTHOR]

There is a strong association between access to early intervention (EI) services by 6 months of age and improved speech and language outcomes for children with permanent hearing loss (PHL). This study identified factors impacting on age of engagement and timing of engagement in EI services by families of 377 children with PHL. This retrospective cohort study included a group of children diagnosed with PHL born between 2011 and 2013 in Brisbane, Australia. The study investigated the association of demographic, social and diagnostic characteristics of children with age of engagement and timing of engagement in EI services. Factors significantly associated with later age of engagement, longer time between diagnosis and engagement, and no engagement with EI services included premature birth, unilateral hearing loss, mild hearing loss and not using a hearing aid. This study provided evidence for the efficacy of tailored support for families to achieve the best possible communication outcomes for their children with PHL. [ABSTRACT FROM AUTHOR]

Abstract Objective This study aimed to determine and describe hearing loss among preschool children in a South African community representative of typical low- and middle income countries (LMIC). Method Children between the ages of 3–6 years received a hearing screening at their early childhood development (ECD) center. If a child failed the hearing screening, he/she was seen for a follow-up rescreen and diagnostic assessment if necessary at their ECD center or closest referral clinic. Diagnostic testing consisted of otoscopy, tympanommetry and pure-tone diagnostic audiometry. Results A total of 6424 children were screened at ECD centers with a referral rate of 24.9%. Follow-up assessments were conducted on 45.3% (725) of these children. Diagnostic testing revealed that 9.3% of children presented with impacted cerumen and 18.7% presented with a hearing loss (56.5% binaural). Binary logistic regression revealed no gender or age effects (p > 0.05). Conductive hearing loss (65.2%) was the most common type of hearing loss found in children. Conclusions Most preschool children who failed the hearing screening and received a diagnostic assessment were in need of intervention services for conductive hearing losses, followed by sensorineural and mixed losses. [ABSTRACT FROM AUTHOR]

Abstract Background Seven hundred children were recalled for hearing screening at age 2–3 years due to a problem with their newborn hearing screen. They had all been well babies with no identified risk factors for hearing loss and hence were not scheduled for targeted follow-up to retest hearing. Methods There were 485 children (69%) that attended the recall. The average age was 36 months (SD 3.7). Family ethnicity was Pacific Island (36%), Asian (26%), NZ European (13%), and Māori (11%), and there was a high level of deprivation in the study population. Children were screened using distortion product otoacoustic emission (DPOAE) and a parent or caregiver completed a 14-item questionnaire about ear health. The children that did not pass screening were given appointments for audiology testing. Children with hearing loss and/or middle ear problems were referred for otolaryngology review and further hearing assessments. Results About one third (36%; n = 176) of children did not pass DPOAE screening; 82 (17%) had abnormal type B tympanograms and hearing loss; 29 underwent insertion of ventilation tubes, and one had a perforated tympanic membrane. There was a significant association between failed tympanometry and hearing loss (Chi-squared = 16.67, p <.001). Five children had permanent sensorineural hearing loss (SNHL), two of whom required cochlear implants for idiopathic hearing loss, with no specific risk factors. Overall 380 of 485 children screened were deemed to have normal hearing (i.e. 22% failed hearing). From the questionnaire, 15% of the caregivers with no suspicion of hearing problems did have children with significant hearing loss. Regression analysis showed that Pacific/Māori ethnicity was significantly associated with risk of hearing loss, together with questionnaire items identifying hearing problems and breathing problems. Conclusions There is a high proportion of children in South Auckland with unsuspected hearing loss; a different approach to hearing screening is warranted for this population with high rates of middle ear disease at age 3. [ABSTRACT FROM AUTHOR]

Abstract Objectives To evaluate the relation between ventilation tube insertion, otitis media with effusion duration and otologic outcomes in unilateral cleft lip and/or cleft palate children from infancy to teenage age. Design and population Retrospective longitudinal charts review of patients from the multidisciplinary cleft team of the University Hospital of Lausanne over a 30-year period. 146 charts from consecutive patients with non-syndromic unilateral cleft lip and/or cleft palate who were born between January 1986 and January 2003 were included. Results The earlier in life a cleft child experience his first otitis media with effusion (OME), the worse his long-term hearing will be. Along with the age of onset of OME, we disclosed an influence of the duration of OME without ventilation tube (VT) insertion on short and long-term hearing outcomes. Different patterns were observed between cleft palate (CP) and cleft lip palate children (CLP), with a higher incidence of otitis media with effusion for the CLP group than the CP group. Direct positive relationship between VT insertion and hearing were disclosed and evaluation of long-term complications did not reveal significant relation with VT insertion. Of note, OME in CLP children led to a higher rate (but not statistically significant) of chronic ear complications than in the CP group, that may indicate more persistent OME or different adverse effect on the middle ear mucosa between CP and CLP children. Conclusions Individualized counseling should take into account different factors such as the type of cleft, the age of onset of OME and duration of OME, keeping in mind the adverse effect of persistent middle ear fluid. In the present report, results prone an early ventilation tube insertion to prevent short and long-term injury to the middle ear homeostasis, hearing loss and related issues. [ABSTRACT FROM AUTHOR]

Abstract Objective Most studies on sudden sensorineural hearing loss (SSNHL) do not differentiate the outcomes within varied affected ears in children. The present study was designed to determine the clinical differences between unilateral and bilateral SSNHL in children. Methods The clinical data, from a total of 101 pediatric patients with SSNHL, was retrospectively analyzed from January 2003 to December 2016. The main outcome measures included basic characteristics, etiology, clinical symptoms and treatment courses. Results When the bilateral group (n = 28) was compared to the unilateral group (n = 73), neither gender nor onset of SSNHL was significantly different (p > 0.05 each); However, bilateral SSNHL tended to occur in younger ages (8.1 ± 4.0 yrs), with higher percentages of suspected etiologies (50%) and proportion of profound deafness (55.4%, p < 0.05 each). The short-term recovery rate was superior in the unilateral cases over the bilateral cases (37.0% vs. 12.5%, p < 0.05). Milder initial hearing threshold, early onset of treatment (5.6 ± 4.8 days) with unilateral involvement and an older age (11.3 ± 3.0 yrs) in bilaterally affected cases were associated with a better prognosis in this cohort. In addition, the unilateral group showed comparable outcomes, when sub-analyzed by comparison to that in either left- (n = 42) or right-sided (n = 31) SSNHL. Conclusion Although bilateral and unilateral pediatric SSNHL could cause partial to complete cochlear lesion, they may be relevant to distinct backgrounds. Our data also provides valuable information about demographics and outcomes of SSNHL in children. [ABSTRACT FROM AUTHOR]

Abstract Objectives To examine if the tablet-based Agilis Health Mobile Audiogram (Agilis Audiogram) is an effective and valid measure of hearing thresholds compared to a pure-tone audiogram in an adult and pediatric population. Methods Participants underwent an otologic exam, conventional audiometric evaluation and the self-administered Agilis Audiogram. We examined whether the difference of pure-tone average (PTA) between the two measurement techniques fell within the equivalence range of ±8 dB. The Agilis Audiogram was administered twice for each subject to assess test-retest reliability of the application. Results A total of 54 ears from 27 participants were evaluated. The average time to complete the self-administered Agilis Audiogram was 10 min. Among participants with normal hearing, the average PTA from conventional audiometric evaluation was 8.9 dB (±3.8) and the average PTA from the Agilis Audiogram was 8.5 dB (±4.5), with mean difference of 0.4 dB (±4.2; 95% CI -1.0 to 1.7 dB) falling within the equivalence range (−8 to 8 dB). Among participants with confirmed hearing loss, the average PTA was 22.5 dB (±17.1) from conventional audiometric evaluation and 24.3 dB (±16.6) from the Agilis Audiogram, with mean difference of −1.8 dB (±5.4; 95% CI -4.9 to 1.3 dB), falling within the equivalence range. Overall, there was a significant correlation between conventional audiometric evaluation and the Agilis Audiogram (Pearson correlation = 0.93; p < 0.001). Conclusion Thresholds obtained by the Agilis Audiogram were found to be a valid measure of hearing among adults with normal hearing and children with hearing loss in the mild-moderate range. [ABSTRACT FROM AUTHOR]

Objective: This is a retrospective review of the impact of an Auditory Brainstem Implant (ABI) on the audiological rehabilitation and tonal language development of pediatric patients with prelingual profound deafness in Hong Kong.Results: From January 2009 to February 2015, 11 pediatric patients with profound prelingual deafness received an ABI in Hong Kong (age range 1.67-3.75 years). Etiologies included Cochlear Nerve Deficiency in 7, Severe Cochlear Malformations in 2, and Retrocochlear Deafness in 2. All of them were rehabilitated in Cantonese, a dialect of Chinese. Standard pediatric cochlear implant outcome measurements were used in this study that comprised of the 7-Sound Detection, Syllable Identification, Vowel Identification, Consonant Identification, Tone Imitation, Tone Production and Speech Perception Category. Audiological rehabilitation and speech development outcomes were reviewed. Age-matched outcomes of pediatric cochlear implant users were used for comparisons.Conclusion: Encouraging results of speech development were found, especially with continued use of the ABI. There was considerable variation in outcomes. Children with coexisting developmental and nonauditory cognitive disabilities did not perform as well. Auditory brainstem implantation is a safe and beneficial treatment for profound prelingual deafness in Cantonese-speaking pediatric patients. [ABSTRACT FROM AUTHOR]

Objective To compare the English and non-English language performance of deaf or hard-of-hearing (DHH) children raised in homes where English was not the primary language to their typically hearing peers from similar language backgrounds. Methods Case control study of bilingual DHH children with unilateral or bilateral non-fluctuating hearing loss defined as the most recent PTA between 26 and 70 dB in one or both ears. Typically hearing controls included bilingual siblings and children recruited from the General Pediatrics practice. Subjects completed the OWLS-II, a validated English language proficiency tool. The subject's parents completed the Child Behavioral Checklist, an assessment of problem behavior; the Student Oral Language Observation Matrix (SOLOM), an assessment of the child's non-English home language; and a study questionnaire on the child's medical, social, and language history. Results 26 typically hearing controls, 15 children with bilateral hearing loss, and 18 children with unilateral hearing loss participated. The groups were similar in age, sex, insurance status, place of birth, age at arrival in the US, and maternal education status. Performance on the English language oral composite was significantly lower amongst the bilateral hearing loss group (BHL: 66.9, 95% CI [56.2–77.7]; UHL: 82.9, 95% CI [75.6–90.2]; NH: 84.4, 95% CI [79.5–89.3], p = 0.002). Performance on the SOLOM was significantly lower in bilateral hearing loss group. (BHL: 18.6, 95% CI [15.9–21.3]; UHL: 19.8, 95% CI [17.1–22.4]; NH: 22.3, 95% CI [20.6–24.0], p = 0.036). Conclusions Bilingual children with bilateral hearing loss are at increased risk for poor oral expressive and receptive language development. These children comprise a particularly vulnerable population who might benefit from additional focused interventions to support their language development. [ABSTRACT FROM AUTHOR]

Hearing loss results from a deviation in the perception of sounds in one's environment. A child may have hearing loss at a hearing threshold of ≥16 dB. Hearing loss is among the frequent disorders and affects lots of people globally. Hearing loss negatively affects many areas needed for classroom learning, language development, academic performance, and social development. These consequences can be reduced by early detection with appropriate audiological and speech therapy interventions. In developing countries like Ghana, there is no such laid down formal hearing screening programs for early detection of any signs of impairments. The present study was aimed at identifying the prevalence and causes of hearing loss among basic school children aged between 5 and 17 years at Freetown Basic School, Ho. The study sought to determine the causes and prevalence of hearing loss. Out of the 210 participants, 44 (21%) failed the audiometric screening test. Out of the 44 students who failed the audiometric screening test, 25 (11.9%) had hearing loss across all the test frequencies. Prevalence of hearing loss among basic school children was found to be 11.9%. This shows that hearing loss is not uncommon among school going children in the Ho Municipality. It was determined from the study that the prevailing causes of the hearing impairments may be attributable to abused use of ototoxic drugs and ear-aches and infections with majority of participants recording mild hearing loss. It can be concluded that hearing loss among school going children is preventable if appropriate measures are put in place. [ABSTRACT FROM AUTHOR]

Abstract: Cisplatin is an essential chemotherapeutic agent in the treatment of many pediatric cancers. Unfortunately, cisplatin‐induced hearing loss (CIHL) is a common, clinically significant side effect with life‐long ramifications, particularly for young children. ACCL05C1 and ACCL0431 are two recently completed Children’s Oncology Group studies focused on the measurement and prevention of CIHL. The purpose of this paper was to gain insights from ACCL05C1 and ACCL0431, the first published cooperative group studies dedicated solely to CIHL, to inform the design of future pediatric otoprotection trials. Use of otoprotective agents is an attractive strategy for preventing CIHL, but their successful development must overcome a unique constellation of methodological challenges related to translating preclinical research into clinical trials that are feasible, evaluate practical interventions, and limit risk. Issues particularly important for children include use of appropriate methods for hearing assessment and CIHL severity grading, and use of trial designs that are well‐informed by preclinical models and suitable for relatively small sample sizes. Increasing interest has made available new funding opportunities for expanding this urgently needed research. [ABSTRACT FROM AUTHOR]

Introduction Temporal bone fractures (TBF) are traditionally classified by their angle of fracture relative to the petrous ridge, and more recently by whether or not they violate the otic-capsule. This study compared rates of hearing loss (HL) and signs of otologic dysfunction among fracture types of both classification systems, within the pediatric population. Methods Pediatric patients were retrospectively characterized from a previously identified cohort of TBF patients, diagnosed from 2000 to 2014. CT scans were reviewed and TBFs were classified first as longitudinal (L), transverse (T) or mixed (M), and then as otic-capsule sparing (OCS) or otic-capsule violating (OCV). Medical records were reviewed, and rates of HL and presenting signs were compared among L, T and M fractures, and OCS and OCV fractures. Results Forty-three patients with 47 TBFs met the inclusion criteria. Eighteen, 4 and 25 TBFs were classified as L, T and M fractures, respectively. Thirty-three and 9 were classified as OCS, and OCV, respectively. Among 24 cases of HL: 20, 3, and 1 were conductive HL (CHL), sensorineural HL (SNHL) and mixed HL, respectively. Two cases of SNHL were found among OCV fractures, with none in OCS fractures (estimated difference 0.22; 95% confidence interval 0.01–0.60). Similar rates of CHL were found across L, T and M fractures (range 36–50%), and across OCV and OCS fractures (range 42–44%). Hemotympanum was the most common presenting sign, found in 68% of TBFs and 80% of CHL cases. There were no significant differences in the incidence of signs or symptoms between fracture types. Conclusions In our cohort, both the traditional and otic-capsule radiographic classification systems failed to predict the incidence of CHL and other otologic signs in the pediatric population. Though OCV fractures conferred an increased risk for developing SNHL, we found a lower incidence than anticipated given violation to the bony labyrinth. [ABSTRACT FROM AUTHOR]

Objective To examine the utility of the mismatch response (MMR) and acoustic change complex (ACC) for assessing speech discrimination in infants. Methods Continuous EEG was recorded during sleep from 48 (24 male, 20 female) normally hearing aged 1.77 to –4.57 months in response to two auditory discrimination tasks. ACC was recorded in response to a three-vowel sequence (/i/-/a/-/i/). MMR was recorded in response to a standard vowel, /a/, (probability 85%), and to a deviant vowel, /i/, (probability of 15%). A priori comparisons included: age, sex, and sleep state. These were conducted separately for each of the three bandpass filter settings were compared (1–18, 1–30, and 1–40 Hz). Results A priori tests revealed no differences in MMR or ACC for age, sex, or sleep state for any of the three filter settings. ACC and MMR responses were prominently observed in all 44 sleeping infants (data from four infants were excluded). Significant differences observed for ACC were to the onset and offset of stimuli. However, neither group nor individual differences were observed to changes in speech stimuli in the ACC. MMR revealed two prominent peaks occurring at the stimulus onset and at the stimulus offset. Permutation t-tests revealed significant differences between the standard and deviant stimuli for both the onset and offset MMR peaks (p < 0.01). The 1–18 Hz filter setting revealed significant differences for all participants in the MMR paradigm. Conclusion Both ACC and MMR responses were observed to auditory stimulation suggesting that infants perceive and process speech information even during sleep. Significant differences between the standard and deviant responses were observed in the MMR, but not ACC paradigm. These findings suggest that the MMR is sensitive to detecting auditory/speech discrimination processing. Significance This paper identified that MMR can be used to identify discrimination in normal hearing infants. This suggests that MMR has potential for use in infants with hearing loss to validate hearing aid fittings. [ABSTRACT FROM AUTHOR]

Abstract: Background: This study examined language development in young children with hearing loss and different types of additional disabilities (ADs). Method: A population‐based cohort of 67 children who were enrolled in the Longitudinal Outcomes of Children with Hearing Impairment study took part. Language ability was directly assessed at 3 and 5 years of age using the Preschool Language Scale, Fourth Edition and the Peabody Picture Vocabulary Test, Fourth Edition. Standard scores were used to enable comparison with age‐based expectations for typically developing children. Results: Analysis of variance showed that, across the total cohort, children's language scores remained stable over the 2‐year period. However, this overall stability masked a significant difference between children with different types of ADs; in particular, children with autism, cerebral palsy and/or developmental delay showed a decline in standard scores, whereas children with other disabilities showed a relative improvement. In addition, larger improvements in receptive vocabulary were associated with use of oral communication only. Conclusions: The results suggest that type of AD can be used to gauge expected language development in the population of children with hearing loss and ADs when formal assessment of cognitive ability is not feasible. [ABSTRACT FROM AUTHOR]

Environmental chemical exposure can cause neurotoxicity and has been recently linked to hearing loss in general population, but data are limited in early life exposure to lead (Pb) and cadmium (Cd) especially for children. We aimed to evaluate the association of their exposure with pediatric hearing ability. Blood Pb and urinary Cd were collected form 234 preschool children in 3–7 years of age from an electronic waste (e-waste) recycling area and a reference area matched in Shantou of southern China. Pure-tone air conduction (PTA) was used to test child hearing thresholds at frequencies of 0.25, 0.5, 1, 2, 4 and 8 kHz. A PTA ≥ 25 dB was defined as hearing loss. A higher median blood Pb level was found in the exposed group (4.94 ± 0.20 vs 3.85 ± 1.81 μg/dL, p < 0.001), while no significance was found for creatinine-adjusted Cd. Compared with the reference group, the exposed group had a higher prevalence of hearing loss (28.8% vs 13.6%, p < 0.001). The PTA in the left, right and both ears, and hearing thresholds at average low and high frequency, and single frequency of 0.5, 1 and 2 kHz were all increased in the exposed group. Positive correlations of child age and nail biting habit with Pb, and negative correlations of parent education level and child washing hands before dinner with Pb and Cd exposure were observed. Logistic regression analyses showed the adjusted OR of hearing loss for Pb exposure was 1.24 (95% CI: 1.029, 1.486). Our data suggest that early childhood exposure to Pb may be an important risk factor for hearing loss, and the developmental auditory system might be affected in e-waste polluted areas. [ABSTRACT FROM AUTHOR]

Deafness and hearing loss may have functional, economic, social and emotional impacts on humans, including the ability of an individual to communicate with others, feelings of isolation and frustration, and health sector costs. The World Health Organization reported that there are 32 million children worldwide with hearing loss. In order to investigate genetic mutations in children of 26 nationalities with hearing loss in Yunnan, Sanger sequencing was employed to screen for mutations in four of the most common pathological genes, including gap junction protein β2 and 3, solute carrier family 26 member 4 and mitochondrial DNA. Whole exome sequencing was used to detect the mutation in the proband of a family in which these four genes were normal. Subsequently, the mutation was identified by Sanger sequencing. The present study reports a novel mutation, c.718C>G; p. (Arg240Gly) in the melanogenesis associated transcription factor gene, in Han people with hearing loss. The results of the present study may provide parents and children an accurate diagnosis, which may allow physicians to how to rehabilitate children's hearing. [ABSTRACT FROM AUTHOR]

Objectives Platinum-based chemotherapy treatments are effective against a variety of pediatric malignancies. However, its use can lead to permanent hearing loss. The aim of this study was to evaluate the long-term effect of platinum chemotherapy on hearing and evaluate its progression. Methods Prospective cohort study. All records of pediatric patients receiving platinum-based chemotherapy between 2001 and 2006 were reviewed. Demographics and audiograms performed before, during, and following chemotherapy were analyzed. An updated audiogram and a video head impulse test were performed. A hearing ability questionnaire was also completed. Results Thirty-nine patients met the inclusion criteria. Of these, 12 patients were included in the study; 14 were deceased, 8 had incomplete data and 5 were excluded for other reasons. Median age at chemotherapy was 4.3 years (range 10 months–14.2 years). Seven patients had received cisplatin, two received carboplatin and three received both agents. Five had also received cranial irradiation. With a median follow-up time of 11.9 years, 58.3% had developed hearing loss and two patients wore bilateral hearing aids; 67% of the patients with hearing loss had worsening of their hearing in the long-term. All patients referred difficulties in various subscales measured by the questionnaire. Three patients had decreased vestibulo-ocular reflex gains. Conclusion Platinum-induced hearing loss in pediatric patients can be progressive and debilitating. A long term audiometric follow-up of at least 10 years is suggested for these patients. [ABSTRACT FROM AUTHOR]

Objective: To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes.Method: Study design: case–control study.Setting: A cochlear implantation tertiary referral center.Patients: All pediatric cochlear implantation recipients with Waardenburg syndrome, Usher syndrome, Dandy–Walker syndrome, or albinism. A control group was appropriately matched to the syndromic group with regard to age at implantation and duration of device use.Intervention: Cochlear implantation.Main outcome measures: Subjects’ auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group.Results: A total of 25 subjects (13 syndromic and 12 non-syndromic) participated in the study. Neither auditory ability nor speech intelligibility scores differed significantly by group. The final PTA of both the groups showed normal-to-mild hearing loss: 26 dB HL in the syndromic group and 23 dB HL for the control group.Conclusions: Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients’ sensory perception and lifestyle. [ABSTRACT FROM AUTHOR]

CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare neurological disorder, recently associated with the c.2452G > A hotspot mutation in the ATP1A3 gene, with sensorineural hearing loss as a prominent feature. We herein report on a girl who has experienced hearing loss for three years following an initial encephalitic episode when aged 15 months old. CAPOS was diagnosed only when she was six years old by targeted testing whilst she displayed optic atrophy, cerebellar signs and areflexia. CAPOS syndrome should be considered in the differential diagnosis of acquired childhood deafness, prompting clinicians to search for associated neurological features. [ABSTRACT FROM AUTHOR]

Purpose:Pilot study to assess the effect of augmentative and alternative communication technology to enhance language development in children who are deaf or hard-of-hearing. Materials and methods:Five children ages 5–10 years with permanent bilateral hearing loss who were identified with language underperformance participated in an individualized 24-week structured program using the application TouchChat WordPower on iPads®. Language samples were analyzed for changes in mean length of utterance, vocabulary words and mean turn length. Repeated measures models assessed change over time. Results:The baseline median mean length of utterance was 2.41 (range 1.09–6.63; mean 2.88) and significantly increased over time (p = 0.002) to a median of 3.68 at final visit (range 1.97–6.81; mean 3.62). At baseline, the median total number of words spoken per language sample was 251 (range 101–458), with 100 (range 36–100) different words spoken. Total words and different words significantly increased over time (β = 26.8 (7.1),p = 0.001 for total words;β = 8.0 (2.7),p = 0.008 for different words). Mean turn length values also slightly increased over time. Conclusions:Using augmentative and alternative communication technology on iPads®shows promise in supporting rapid language growth among elementary school-age children who are deaf or hard-of-hearing with language underperformance. [ABSTRACT FROM PUBLISHER]

Objective Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. Methods Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. Results 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. Conclusion Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children. [ABSTRACT FROM AUTHOR]

Objectives Deaf subjects with hearing aids or cochlear implants generally find it challenging to understand speech in noisy environments where a great deal of listening effort and cognitive load are invested. In prelingually deaf children, such difficulties may have detrimental consequences on the learning process and, later in life, on academic performance. Despite the importance of such a topic, currently, there is no validated test for the assessment of cognitive load during audiological tasks. Recently, alpha and theta EEG rhythm variations in the parietal and frontal areas, respectively, have been used as indicators of cognitive load in adult subjects. The aim of the present study was to investigate, by means of EEG, the cognitive load of pediatric subjects affected by asymmetric sensorineural hearing loss as they were engaged in a speech-in-noise identification task. Methods Seven children (4F and 3M, age range = 8–16 years) affected by asymmetric sensorineural hearing loss (i.e. profound degree on one side, mild-to-severe degree on the other side) and using a hearing aid only in their better ear, were included in the study. All of them underwent EEG recording during a speech-in-noise identification task: the experimental conditions were quiet, binaural noise, noise to the better hearing ear and noise to the poorer hearing ear. The subjects' Speech Recognition Thresholds (SRT) were also measured in each test condition. The primary outcome measures were: frontal EEG Power Spectral Density (PSD) in the theta band and parietal EEG PSD in the alpha band, as assessed before stimulus (word) onset. Results No statistically significant differences were noted among frontal theta power levels in the four test conditions. However, parietal alpha power levels were significantly higher in the “binaural noise” and in the “noise to worse hearing ear” conditions than in the “quiet” and “noise to better hearing ear” conditions (p < 0.001). SRT scores were consistent with task difficulty, but did not correlate with alpha and theta power level variations. Conclusion This is the first time that EEG has been applied to children with sensorineural hearing loss with the purpose of studying the cognitive load during effortful listening. Significantly higher parietal alpha power levels in two of three noisy conditions, compared to the quiet condition, are consistent with increased cognitive load. Specifically, considering the time window of the analysis (pre-stimulus), parietal alpha power levels may be a measure of cognitive functions such as sustained attention and selective inhibition. In this respect, the significantly lower parietal alpha power levels in the most challenging listening condition (i.e. noise to the better ear) may be attributed to loss of attention and to the subsequent fatigue and “withdrawal” from the task at hand. [ABSTRACT FROM AUTHOR]

Objectives Globally, newborn hearing screening (NHS) is variably incorporated into national healthcare systems. The authors reviewed the set-up and evolution process of a hospital-based NHS program in South Korea, where screening costs for low-income families are paid by the National Health Authority. Methods The NHS process for 13805 newborns delivered in a tertiary referral center of South Korea from 2005 through 2014 was reviewed. Hearing screening was conducted using automated auditory brainstem response (AABR); hearing loss was confirmed by auditory brainstem response for newborns who did not pass the screening test. Results The mean screening rate for hearing loss was 53.6% (7403 of 13805 newborns), which plateaued at 79.6% over time. Of the 14806 ears (7403 newborns), 1030 (7.0%) were assessed as “refer” on the first AABR, with 204 (1.4%) being assessed as “refer” on the second AABR. In hearing confirmation tests, 74 infants (1.0% of 7403 newborns) were diagnosed with hearing loss, including 13 infants (0.2%) with bilateral moderate to profound sensorineural hearing loss (SNHL). Hearing rehabilitation with long-term follow-up was confirmed in 11 infants. Conclusions In this hospital-based NHS program, the screening rate plateaued at ∼50% when the National Health Authority was not involved, but increased to ∼70% when the cost for low-income families was covered by the government. Among infants needing active hearing rehabilitation due to bilateral moderate to profound SNHL, 15% were lost to follow-up. These results demonstrate the need for a universal, mandatory NHS program to systematically register hearing-impaired infants within the government-sponsored public healthcare system. [ABSTRACT FROM AUTHOR]

This study sought to analyze and evaluate the accessibility, availability and quality of online information regarding the National Disability Insurance Scheme (NDIS) and hearing loss. The most common search engine keyword terms a caregiver may enter when conducting a web search was determined using a keyword search tool. The top websites linked with each keyword term were then evaluated using a subjective quality rating tool. It was found that only 8% of the websites evaluated referred users directly to the NDIS. The majority of websites (70%) scored in the fair range for overall quality with 15% of websites scoring poorly and 15% of websites scoring highly. However, the hierarchy in which websites were presented on Google when a search string was typed into the search bar was not necessarily associated with the highest quality website appearing first. Overall, whilst the quality of information regarding the NDIS was of a fair quality, the accessibility to this information by caregivers was found to be poor. [ABSTRACT FROM AUTHOR]

Objectives It is important for clinician such as speech-language pathologists and audiologists to develop more efficient procedures to assess the development of auditory, speech and language skills in children using hearing aid and/or cochlear implant compared to their peers with normal hearing. So, the aim of study was the comparison of the performance of 5-to-7-year-old Persian-language children with and without hearing loss in visual-only, auditory-only, and audiovisual presentation of sentence repetition task. Methods The research was administered as a cross-sectional study. The sample size was 92 Persian 5–7 year old children including: 60 with normal hearing and 32 with hearing loss. The children with hearing loss were recruited from Soroush rehabilitation center for Persian-language children with hearing loss in Shiraz, Iran, through consecutive sampling method. All the children had unilateral cochlear implant or bilateral hearing aid. The assessment tool was the Sentence Repetition Test. The study included three computer-based experiments including visual-only, auditory-only, and audiovisual. The scores were compared within and among the three groups through statistical tests in α = 0.05. Results The score of sentence repetition task between V-only, A-only, and AV presentation was significantly different in the three groups; in other words, the highest to lowest scores belonged respectively to audiovisual, auditory-only, and visual-only format in the children with normal hearing (P < 0.01), cochlear implant (P < 0.01), and hearing aid (P < 0.01). In addition, there was no significant correlationship between the visual-only and audiovisual sentence repetition scores in all the 5-to-7-year-old children (r = 0.179, n = 92, P = 0.088), but audiovisual sentence repetition scores were found to be strongly correlated with auditory-only scores in all the 5-to-7-year-old children (r = 0.943, n = 92, P = 0.000). Conclusions According to the study's findings, audiovisual integration occurs in the 5-to-7-year-old Persian children using hearing aid or cochlear implant during sentence repetition similar to their peers with normal hearing. Therefore, it is recommended that audiovisual sentence repetition should be used as a clinical criterion for auditory development in Persian-language children with hearing loss. [ABSTRACT FROM AUTHOR]

Objective Negative associations between hearing loss (HL) and behavioural and academic difficulties have been reported. However, most studies are based on small clinical samples. The aim of the present study was to investigate such associations using data from a large-scale non-clinical Danish birth cohort controlling for a large number of relevant confounding factors. Methods The study applied data from the Aarhus Birth Cohort's 10-12-year-old follow-up (N = 7599). Associations between parent-reported HL on the one hand, and parent- and teacher-reported behavioural difficulties measured by the Strengths and Difficulties Questionnaire (SDQ) and parent-reported academic difficulties on the other hand, were investigated. Results After controlling for relevant confounding factors, significant associations were observed between parent-reported HL and parent- and teacher-reported Externalising scores, and academic difficulties. Conclusions Childhood HL is associated with behavioural and academic difficulties. Parent reported HL in a non-clinical cohort is indicative for academic and behavioural difficulties. [ABSTRACT FROM AUTHOR]

Hearing loss among children, particularly those with severe and profound hearing impairment, has an effect on their communication and development, which in turn can have a significant impact on their parents. This study aimed to evaluate the quality of life of parents of children with hearing loss and identify the associated factors. A cross-sectional study was carried out involving parents of children that went through phonoaudiological therapy at a public university clinic in the city of São Paulo. The research instruments used were: the World Health Organization Quality of Life Instrument, Short Form (WHOQOL- bref), the Social Support Questionnaire 6 (SSQ-6) and the General Health Questionnaire (GHQ-12). Inferential statistical analysis was performed using the Student's t-test. The study included 29 parents, 26 mothers and 3 fathers, of 27 children with severe and profound hearing impairment. The mean of the domain scores of the WHOQOL-bref, on a scale of 0-100, ranged between 40-60 and the overall score was 53. The best performance was achieved in the physical domain (60.3) and the worst in the environment domain (40.5). The main factor associated with all domains of the WHOQOL-bref, as well as overall score was being satisfied with social support. [ABSTRACT FROM AUTHOR]

This study investigated the experience of families with young children with hearing loss participating in a self-directed funding scheme in Australia. Eleven hearing caregivers, one male, participated in qualitative semi-structured interviews. Analysis was undertaken using a phenomenological inspired thematic analysis approach. Major themes arising were: a perceived transfer of responsibility from the ‘system’ to the ‘family’ in order to get the best outcome for their child, an acknowledgement of having to be prepared for the planning session in order to make a strong case for resources, a sense of disappointment at a perceived lack of interest by staff in the individual circumstances of their child, as well as the challenges of dealing with information overload. The opportunity to engage with a trusted source prior to engaging with self-directed schemes as well as presenting information in a ‘just in time’ format may assist future families in navigating funding challenges for their child. [ABSTRACT FROM PUBLISHER]

This case report details a year-long phonological awareness (PA) intervention for pre-kindergarten children with hearing loss (CHL) who use listening and spoken language. All children wore cochlear implants and/or hearing aids. Intervention occurred for 15 min/day, 4 days per week across the pre-kindergarten school year and was delivered by classroom teachers of the deaf. At the beginning of the year, children's performance on measures of PA generally was low. After participating in the intervention, the majority of students performed within or above the developmental range. These findings indicate that PA training that is intense, modified for CHL, and transferable to mainstream setting is effective in increasing PA skills in CHL. [ABSTRACT FROM PUBLISHER]

The p.V37I (c.109G>A) mutation in the GJB2 gene is the common frequent cause of congenital deafness; however, its pathogenicity is debated. The present study investigated the prevalence of p.V37I in Chinese infants and young children and associated clinical characteristics. The subjects of the present study were screened for mutations in GJB2 (235delC, 299delAT, 176dell6, 35delG), SLC26A4 (IVS7-2A>G, 2168A>G), GJB3 (538C>T), and in the mitochondrial 12S rRNA gene (1555A>G, 1494C>T). Subjects with p.V37I underwent an audiological evaluation. GJB2 exon sequencing revealed that 20 subjects had p.V37I compound heterozygous mutations, one of whom had a family history; the mutations included c.235delC/p.V37I (n = 12), c.299AT/p.V37I (n = 7), and c.176del16/p.V37I (n = 1). Of the 20 subjects, 12 were referred for Universal Newborn Hearing Screening (UNHS). Nine of the 20 subjects had mild hearing loss in the better ear and 5 had moderate hearing loss in the better ear while 4 had normal hearing. Among subjects with the c.235delC/p.V37I mutation, 5 had mild hearing loss and 2 had moderate hearing loss while 3 had normal hearing. Among subjects with the c.299AT/p.V37I mutation, 3 had mld hearing loss and 3 had moderate hearing loss while 1 had normal hearing. One subject with the c.176del16/p.V37I mutation had mild hearing loss. Few studies have reported on the clinical characteristics of Chinese infants with p.V37I compound heterozygous mutations identified via screening for deafness genes and GJB2 sequencing. The c.235delC/p.V37I mutation was the most prevalent mutation found in subjects. The degree of hearing loss associated with p.V37I compound heterozygous mutations was mainly mild to moderate. [ABSTRACT FROM AUTHOR]

Background/Aim: There is currently no objective evaluation of hearing in patients with hydrocephalus (HCP), and we could not find any study in the literature comprising a sufficient number of patients with a high level of scientific evidence. In the current study, we used the auditory brainstem response (ABR) test to assess whether hearing function in patients with HCP is altered after ventriculoperitoneal shunt surgery. Methods: In total, 20 newborn patients with HCP (13 female, 7 male) were enrolled in this study. For each patient, ABR testing was performed at three time points: 1 day prior to the operation and on days 7 and 90 after the operation. ABRs using click stimuli of 90, 70, 50 and 30 dB nHL (normal hearing level) were achieved for V-wave latency, and I-III and I-IV interpeak latencies for both ears were recorded. Variance analysis for parametric data and Tukey's post hoc honest significant difference test were used to demonstrate the relationship between the results obtained from the different recording periods. Results were considered significant at p < 0.05, and 95% confidence intervals were calculated. Results: The mean values of the ABR tests were compared between the pre- and postoperative results, which showed an increase (faster transmission) of nerve conduction velocity of 0.2 ms. The results were not statistically significant for 50 and 90 dB (p > 0.05) but were significant for 30 and 70 dB (p < 0.05). Conclusion: Diagnosis in hydrocephalic patients is important not only for the treatment but also for the prevention of HCP- associated complications. Early treatment appears to be promising in terms of auditory benefit. Prompt diagnosis and treatment are therefore essential as soon as possible. [ABSTRACT FROM AUTHOR]

Objective This study aimed to describe the prevalence and characteristics of hearing loss in school-aged children in an urban South African population. Method Children from grade one to three from five schools in the Gauteng Province of South Africa formed a representative sample for this study. All children underwent otoscopic examinations, tympanometry and pure tone screening (25 dB HL at 1, 2 and 4 kHz). Children who failed the screening test and 5% of those who passed the screening test underwent diagnostic audiometry. Results A total of 1070 children were screened. Otoscopic examinations revealed that a total of 6.6% ears had cerumen and 7.5% of ears presented with a type-B tympanogram. 24 children (12 male, 12 female) were diagnosed with hearing loss. The overall prevalence of hearing loss was 2.2% with Caucasian children being 2.9 times more (95% confidence interval, 1.2–6.9) likely to have a hearing loss than African children. Conclusion Hearing loss prevalence in urban South African school-aged children suggest that many children (2.2%) are in need of some form of follow-up services, most for medical intervention (1.2%) with a smaller population requiring audiological intervention (0.4%). [ABSTRACT FROM AUTHOR]

Purpose To compare outcomes in pediatric patients suffering forceful head impact during recreational vehicle use to patients with forceful head impact from other mechanisms. Methods Retrospective cohort study of all patients 3–18 years old who suffered forceful head impact (any traumatic mechanism strong enough to result in a face or skull fracture) in our institutional trauma registry between January 2011 and September 2013. Results Out of 252 events involving forceful head impact, 64 events were a result of riding a recreational vehicle. Although there is no difference in rates of temporal bone fractures, recreational vehicle accidents have higher rates of otic capsule violation (21% vs. 5%) and higher rates of hearing loss (30% vs 16%) compared to patients with forceful head impact from other mechanisms. All incidents of otic capsule violation and sensorineural hearing loss in recreational vehicle accidents were associated with a temporal bone fracture. Conclusion Despite the increasing use of head protective gear while operating a recreational motor vehicle there is still heightened risk for temporal bone fractures and subsequent hearing loss. The comparative associations in this study suggest that helmets used with recreational vehicles do not protect the temporal bone thus leaving vital structures within the otic capsule at risk for damage and long term consequences. When treating these patients Otolaryngologists should be aware of the elevated risk of otic capsule violation and late hearing loss with temporal bone fractures. [ABSTRACT FROM AUTHOR]

Introduction The auditory brainstem response (ABR) test is used to identify hearing loss and measure hearing thresholds of infants and children who cannot be tested using standard behavioral hearing testing methods. In order for the ABR to yield useful data, a child must be asleep throughout the duration of the test. In many centers, this is achieved through the use of a general anesthetic, with its inherent risks and costs. Since 2004, ABRs have been routinely conducted at BC Children's Hospital in an ambulatory care setting under oral chloral hydrate sedation, with monitoring by a specialist nurse. The aim of this retrospective study was to assess the effectiveness and safety of nurse-led sedation with chloral hydrate for ABR testing at our tertiary pediatric center. Methods Medical and audiology records were reviewed for children aged 6 months to 17 years who underwent ABR testing from 2004 to 2012. We reviewed the dosage of drug used, condition of the child after chloral hydrate administration, adverse effects, audiological results, patients’ vital signs, and the effectiveness of the sedative in keeping the child asleep throughout the duration of the test. Frequency distributions were derived for adverse outcomes. Results 725 ABR records encompassing 635 children (multiple ABR tests in some children) were reviewed. The average dose of chloral hydrate used was 52 mg/kg. The majority of sedated ABR's (80.8%) were completed without any incident. Significant events [apnea and/or bradycardia], minor complications [vomiting, hypoxemia, prolonged sedation, and/or tachypnea] and restlessness were noted in 3.4%, 6.2%, and 5.0% of the cases, respectively. The majority of these issues resolved without medical intervention, such as the need to provide supplementary oxygen. In 95.9% of ABRs, chloral hydrate was successful in sedating the child adequately to answer the audiological question. Conclusions This forms the largest study to date on oral sedation for ABR testing. Based on our results, the use of chloral hydrate in the presence of a sedation nurse was a safe and reliable method of performing ABR in infants and children. This may be of significant value to centres worldwide exploring alternatives to general anesthesia for ABR testing. [ABSTRACT FROM AUTHOR]

Introduction: Hearing loss from birth up to the age of 3 years has a negative effect on speech/language development and results in sensory, cognitive, emotional, and academic defects in adulthood by causing delayed development of communicative-linguistic abilities. The present study was performed in order to assess the effect of early intervention on language development in Persian children aged 6-7 years with severe sensorineural hearing loss. Materials and Methods: Thirty boys and girls aged 6-7 years participated in this study, all of them had severe congenital sensorineural hearing loss in both ears. All children were using bilateral behind-the-ear hearing aid, and had similar economic/socio-cultural backgrounds. Subjects were categorized into two groups based on the age of identification/intervention of hearing loss (3-6 and 12-15 months of age). The Persian TOLD-P3 test was used to evaluate language development in all subjects. Data collection was accomplished by observation, completion of questionnaires, and speech recording. Results: There was a significant difference in language development in 11 sub-tests and five lingual gains on the Persian TOLD-P3 test between early (3-6 months of age) and late identified/intervened (12-15 months of age) hearing-impaired children (P 0.05). Early identified/intervened hearingimpaired children had a notable preference in all assessed sub-tests and lingual gains. Conclusion: Early identification/intervention of hearing loss before the age of 6 months has a significant positive effect on a child's language development in terms of picture/relational/oral vocabulary, grammatical comprehension, sentence combining, grammatical completion, phonologic analysis, word differentiation, word production, semantics, and syntax. Moreover, early identification/ intervention of hearing loss develops the hearing-impaired child's lingual gains in visual vocabulary, grammatical completion, word differentiation, phonologic analysis, and word production. [ABSTRACT FROM AUTHOR]

OBJECTIVE: In auditory neuropathy (AN) a dyssynchrony in the nerve conduction of the auditory nerve fibers is observed. Typically, patients with AN exhibit moderate to profound sensorineural hearing loss, and treatment using cochlear implants (CIs) or hearing aids should be performed as early as possible for a better hearing rehabilitation. The aim of this study is to evaluate the satisfaction level of patients with AN spectrum disorder treated using CIs. The Satisfaction with Amplification in Daily Life questionnaire was selected to evaluate 10 patients with AN treated using CIs. MATERIAL and METHODS: Clinical study of patients with AN spectrum disorder submitted to CI. A retrospective data analysis, genetic and clinical evaluation in a tertiary referral center was done. RESULTS: The means of the subscales for positive effects, services and costs, negative factors, and personal image were 6.15, 4.6, 3.26, and 3.33, respectively. CONCLUSIONS: Patients with AN treated using CIs consider themselves satisfied. [ABSTRACT FROM AUTHOR]

Objective Guidelines exist for evaluation and management of tinnitus in adults; however lack of evidence in children limits applicability of these guidelines to pediatric patients. Objective of this study is to determine the incidence of inner ear anomalies detected on imaging studies within the pediatric population with tinnitus and evaluate if presence of hearing loss increases the rate of detection of anomalies in comparison to normal hearing patients. Methods Retrospective review of all children with diagnosis of tinnitus from 2010 to 2015 ;at a tertiary care academic center. Results 102 pediatric patients with tinnitus were identified. Overall, 53 patients had imaging studies with 6 abnormal findings (11.3%). 51/102 patients had hearing loss of which 33 had imaging studies demonstrating 6 inner ear anomalies detected. This is an incidence of 18.2% for inner ear anomalies identified in patients with hearing loss (95% confidence interval (CI) of 7.0–35.5%). 4 of these 6 inner ear anomalies detected were vestibular aqueduct abnormalities. The other two anomalies were cochlear hypoplasia and bilateral semicircular canal dysmorphism. 51 patients had no hearing loss and of these patients, 20 had imaging studies with no inner ear abnormalities detected. There was no statistical difference in incidence of abnormal imaging findings in patients with and without hearing loss (Fisher's exact test, p ;= ;0.072.) Conclusion There is a high incidence of anomalies detected in imaging studies done in pediatric patients with tinnitus, especially in the presence of hearing loss. [ABSTRACT FROM AUTHOR]

Background: Understanding the risk factors for hearing loss is essential for designing the Belgian newborn hearing screening programme. Accordingly, they needed to be updated in accordance with current scientific knowledge. This study aimed to update the recommendations for the clinical management and follow-up of newborns with neonatal risk factors of hearing loss for the newborn screening programme in Belgium.Methods: A literature review was performed, and the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system assessment method was used to determine the level of evidence quality and strength of the recommendation for each risk factor. The state of scientific knowledge, levels of evidence quality, and graded recommendations were subsequently assessed using a three-round Delphi consensus process (two online questionnaires and one face-to-face meeting).Results: Congenital infections (i.e., cytomegalovirus, toxoplasmosis, and syphilis), a family history of hearing loss, consanguinity in (grand)parents, malformation syndromes, and foetal alcohol syndrome presented a 'high' level of evidence quality as neonatal risk factors for hearing loss. Because of the sensitivity of auditory function to bilirubin toxicity, hyperbilirubinaemia was assessed at a 'moderate' level of evidence quality. In contrast, a very low birth weight, low Apgar score, and hospitalisation in the neonatal intensive care unit ranged from 'very low' to 'low' levels, and ototoxic drugs were evidenced as 'very low'. Possible explanations for these 'very low' and 'low' levels include the improved management of these health conditions or treatments, and methodological weaknesses such as confounding effects, which make it difficult to conclude on individual risk factors. In the recommendation statements, the experts emphasised avoiding unidentified neonatal hearing loss and opted to include risk factors for hearing loss even in cases with weak evidence. The panel also highlighted the cumulative effect of risk factors for hearing loss.Conclusions: We revised the recommendations for the clinical management and follow-up of newborns exhibiting neonatal risk factors for hearing loss on the basis of the aforementioned evidence-based approach and clinical experience from experts. The next step is the implementation of these findings in the Belgian screening programme. [ABSTRACT FROM AUTHOR]

The objective of this study is to investigate the effects of IDDM on the function of the auditory pathway from the cochlea to the auditory cortex in child patients. Totally, 140 ears of 70 children with IDDM and 60 ears of 30 age and gender-matched healthy controls were included in the study. The ages of patients and controls ranged from 4 to 14 years. Audiological assessment including pure-tone audiometry, otoacoustic emission testing (OAE) and auditory brainstem response testing (ABR) has been performed to all participants. There was no significant difference between the patients and controls regarding pure-tone thresholds on audiometry ( p > 0.05). The latencies of waves I, III and V on ABR were not significantly different between the patients and controls ( p > 0.05). The amplitudes on DPOAE testing obtained from both groups were not significantly different at the frequencies of 2,000 and 4,000 Hz ( p > 0.05). However, the DPOAE amplitudes of the patients at 1,000 Hz were significantly lower than those of controls at the same frequency ( p = 0.03). There was no difference between the patients who had chronic disease (>5 years) and healthy controls regarding pure-tone audiometry, ABR and DPOAE testing results ( p > 0.05). In the light of the findings obtained with pure-tone audiometry, and OAE and ABR testing, it seems that auditory system is spared in children with IDDM at clinical level. Diabetes control and chronicity of the disease do not impact on the auditory system except for a subclinical involvement in the apical portion of the cochlea. [ABSTRACT FROM AUTHOR]

Objectives To investigate the overall prevalence of cochlear dead regions in children with moderate to severe sensorineural hearing impairment. Methods Threshold-equalizing noise (or TEN) test was administered on thirty sensorineural hearing impaired children (8 girls, 22 boys), aged 5–14 years (mean ± SD 8.5 ± 2.8). Results Classifying by subject, 76% tested positive for cochlear dead regions in one or both ears at least at one frequency. Classifying by ears, 58.3% had dead regions at one frequency or more. Classifying by the number of frequencies were tested, 20% met the criteria for a dead region. The difference between mean absolute thresholds in two groups was statistically significant at 1000 Hz and below ( p < 0.05). Conclusion The results indicated a relatively high prevalence of dead regions in children with sensory neural hearing impairment, especially for frequencies at which the hearing loss exceeds 70 dB HL. [ABSTRACT FROM AUTHOR]

Objectives To assess the extended interval regimen gentamicin associated ototoxicity in neonatal intensive care unit using hearing tests. Methods Two hundred and twenty neonates admitted to neonatal intensive care were assessed; 110 neonates who had received gentamicin and 110 neonates who had not received gentamicin served as control group. Gentamicin group were further subdivided according to the duration of treatment into 50 neonates who had received gentamicin for 5 days or less and 60 neonates who had received gentamicin for more than 5 days. TEOAEs were used for hearing screening. Auditory brain response was performed 3 months later for failed cases to confirm the hearing impairment. Results Three neonates failed TEOAEs screening in each group but hearing impairment was confirmed in one neonate only (0.9%) in each group (gentamicin and control groups). Neonates who received gentamicin for more than 5 days showed comparable results as regard TEOAEs or ABR results with those who received gentamicin for 5 days or less, and control group. Conclusions Extended interval dosing of gentamicin therapy in neonates does not increase the incidence of hearing loss. This suggests that hearing loss in neonatal intensive care unit may be attributed to factors other than gentamicin treatment. [ABSTRACT FROM AUTHOR]