ArticleLiterature Review

Benefits of early intervention for children with hearing loss

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Abstract

This article discusses the relationship between language development and degree of hearing loss and how these factors relate to the age of identification of hearing loss. An interesting effect caused by the interaction of these variables has been discussed in this article. The relationship of speech production, degree of hearing loss, language development, and age of identification are also presented. Social-emotional development of deaf and hard-of-hearing children and the variables that impact this development, in particular the strong relationship with language development, is included.

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... In total, 20 patients (13 female and 7 male, 65 and 35%, respectively) met the inclusion criteria for this study. The mean age of the patients on the day of surgery was 10.45 ± 5.82 days (range [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. ...
... The resulting neurological damage to the central auditory processes can affect speech and language development [15] . Hearing loss is often not noticeable in infants but may lead to speech and language delay which, if not corrected, can lead to social and emotional problems and difficulty at school [16] . Delaying the diagnosis of hearing loss in children can also exacerbate the negative effects. ...
... Delaying the diagnosis of hearing loss in children can also exacerbate the negative effects. Hearing loss in children should be detected as early as possible in order to help them to communicate [16] . The development of the maximum level of hearing and communication skills in infants is essential to neural development [17] . ...
Article
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.
... [7][8][9][10] Morbidity is greater when HL remains undetected and/or untreated, particularly for the developing child. [11][12][13] In addition, therapy-related ototoxicity can initially arise or progress years after completion of treatment. [1,5,[14][15][16][17][18] Thus, atrisk populations need long-term follow-up of audiological function. ...
... Speech and language development may be particularly affected when HL is acquired in early childhood. [11][12][13] The frequencies most important for understanding speech are 500-3,000 Hz. In young children, however, ability to hear frequencies between 4,000 and 8,000 Hz is also vital for proper language acquisition and speech development, since auditory and language processing is not yet mature and young children do not have the language base to "fill in the gaps" when portions of speech are not perceived. ...
... [8] Early detection and intervention of HL is critical in young children to help minimize the negative consequences of HL on speech, language, academic, and psychosocial outcomes. [11][12][13] In older children and adults, language acquisition may not be an issue, but they are still at risk for reduced educational achievement, social isolation, emotional difficulties, and poorer healthrelated quality of life when hearing is affected. [7,9] Furthermore, in the adolescent and adult populations, HL is associated with depression, underemployment, and reduced earnings. ...
Article
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Hearing loss (HL) is common in childhood cancer survivors exposed to platinum chemotherapy and/or cranial radiation and can severely impact quality of life. Early detection and appropriate management can mitigate academic, speech, language, social, and psychological morbidity resulting from hearing deficits. This review is targeted as a resource for providers involved in aftercare of childhood cancers. The goal is to promote early identification of survivors at-risk for HL, appropriate evaluation and interpretation of diagnostic tests, timely referral to an audiologist when indicated, and to increase knowledge of current therapeutic options.
... C hildhood hearing impairment is the result of the overlapping factors of genetic predisposition, the intrauterine environment, perinatal, and postnatal factors. Worldwide reporting of hearing loss finds that the prevalence of moderate and severe bilateral hearing deficit (>40 dB) is 1-3 per 1,000 live births in well baby nursery population (1,2) and 2-4 in 100 infants in an intensive care population (3)(4)(5)(6). The numbers given above signify that hearing impairment is one of the most common potentially disabling conditions present in infancy and one of the most frequent congenital anomalies (7)(8)(9). ...
... The major objective of UNHS is to identify children with all kinds and degrees of hearing impairment, both bilateral and unilateral and to lower the age at the time of diagnosis for early hearing amplification, to maximize their linguistic competence and literacy development (14). Yoshinga Itano reported that at 2 y of age infants with hearing loss, identified before the age of 6 mo, had better mean scores in expressive and receptive language than those identified later (4,15,16). ...
... At the beginning screening was performed in some countries by using the behavioral distraction test when the infant was 6-9 mo old. However, it has been shown in many studies that only children identified with hearing impairment early and provided with hearing amplification prior to 6 mo of age had a better chance of developing skills equivalent to their peers (4,16,34). The discovery of OAEs enabled development of an easy and effective method for hearing screening in infants in the first days of life. ...
Article
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The incidence of sensorineural hearing loss ranges from 1 to 3 per 1000 live births in term healthy neonates, and 2-4 per 100 in high-risk infants, a ten-fold increase. Early identification and intervention with hearing augmentation within 6 months yields optimal effect. If undetected and without treatment, significant hearing impairment may negatively impact speech development and lead to disorders in psychological and mental behaviors.Hearing screening programs in newborns enable detection of hearing impairment in the first days after birth. Programs to identify hearing deficit have significantly improved over the 2 decades, and their implementation continues to grow throughout the world. Initially based on risk factors, these programs identified only 50-75% of infants with hearing loss. Current recommendations are to conduct universal hearing screening in all infants. Techniques used primarily include automated auditory brainstem responses and otoacoustic emissions, that provide noninvasive recordings of physiologic auditory activity and are easily performed in neonates and infantsThe aim of this review is to present the objectives, benefits and results of newborn hearing screening programs including the pros and cons of universal versus selective screening. A brief history and the anticipated future development of these programs will also be discussed.
... Congenital hearing loss is a common and important health problem and one of the most common neurosensory handicaps in newborns and children (Yoshinaga-Itano, 1999;de Aledo Linos, 2001;Sivalal, 2005). Therapies for newborns with bilateral hearing loss are important during their first 24 weeks of life as otherwise a serious delay in speech and intellectual development has to be expected, see Yoshinaga-Itano (1999). ...
... Congenital hearing loss is a common and important health problem and one of the most common neurosensory handicaps in newborns and children (Yoshinaga-Itano, 1999;de Aledo Linos, 2001;Sivalal, 2005). Therapies for newborns with bilateral hearing loss are important during their first 24 weeks of life as otherwise a serious delay in speech and intellectual development has to be expected, see Yoshinaga-Itano (1999). ...
... The consequences of being deaf-mute are special schools and care, social isolation, and no exploitation of potential skills. Thus there are serious medical and economical consequences for the entire society due to this problem, see Yoshinaga-Itano (1999); de Aledo Linos (2001). ...
... Hearing deficit is one of the most common developmental disorders and deafness is the most common sensory disorder. The incidence of hearing deficit based on the universal neonatal screening programs is estimated to be 1-3 per 1000 live births in well baby nursery population and 2-4 in 100 infants in an intensive care population [1][2][3][4][5]. ...
... Unilateral hearing deficit does not usually cause so many problems as bilateral hearing impairment. Early and adequate intervention is required to minimize future problems with speech and language development [3,13,36,37]. Taking into account, that in most children with sensorineural hearing deficit the result of hearing screening was positive we can conclude that universal newborn hearing screening is of crucial importance as it allows for early diagnosis and enables intervention in the first months of life. ...
Article
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Objective: The aim of this study was to analyze infants diagnosed with sensorineural or conductive hearing deficit and to identify risk factors associated with these defects. Material and methods: A retrospective analysis of infants diagnosed with hearing deficit based on the database of the universal newborn hearing screening program and medical records of the patients. Results: 27 935 infants were covered by the universal neonatal hearing screening program. 109 (0.39%) were diagnosed with hearing deficit and referred for treatment and rehabilitation. 56 (51.4%) children were diagnosed with conductive, 38 (34.9%) with sensorineural and 15 (13.8%) with mixed type of hearing deficit. Children with sensorineural hearing deficit more frequently suffered from hyperbilirubinemia (p < 0.05), while infants with conductive hearing loss were more frequently diagnosed with isolated craniofacial anomalies (p < 0.05). The prevalence of other risk factors did not differ between the groups. Sensorineural hearing deficit occurred almost 3 times more often bilaterally than unilaterally (p < 0.05). In other types of hearing deficit, the difference was not significant. In children with conductive and mixed type of hearing loss the impairment was mainly mild while among those with sensorineural hearing deficit in almost 45% it was severe and profound (p < 0.05). When analyzing the consistency between hearing screening test by means of otoacoustic emissions and the final diagnosis of hearing deficit we found that the highest agreement rate was observed in children with sensorineural hearing loss (p < 0.01). Conclusions: The prevalence of most risk factors of hearing deficit was similar in children with sensorineural, conductive and mixed type of hearing loss, only hyperbilirubinemia seemed to predispose to sensorineural hearing deficit and isolated craniofacial malformations seemed to be associated with conductive hearing loss. Sensorineural hearing deficit usually occurred bilaterally and was severe or profound, while conductive and mixed type of hearing deficit were most often of mild degree. Most children with the final diagnosis of sensorineural hearing deficit had positive result of hearing screening by means of otoacoustic emissions.
... Más aún, está establecido que intervención (tratamiento) antes de los 6 meses de edad mejora significativamente el desarrollo del lenguaje en relación a quienes son tratados después, e inclusive puede lograrse comunicación y desarrollo dentro de límites normales para la edad (12,13). ...
... De allí la necesidad de implementar programas de chequeo auditivo universal de los recién nacidos y diagnosticarlos antes de los tres meses de edad y proveer intervención apropiada antes de los 6 meses de edad (12,13). ...
Article
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Es un honor para mí el participar como editor invitado de esta edición de la Revista Médica de Clínica Las Condes dedicada a Otología. He elegido escribir esta introducción en una forma general que describa los diversos temas que son tratados en detalle en los trabajos científicos presentados a continuación. Dada la orientación de esta revista -la revista científica de mayor circulación en Chile- los temas están realizados en una forma que puedan ser de interés tanto para médicos generales como especialistas.
... Symptoms of late-onset SNHL may emerge within 3 years after birth and at least half of SNHL children exhibit further hearing deterioration as they age (Demmler, 1991;Dahle et al., 2000;Morton and Nance, 2006;Goderis et al., 2016). While there is currently no effective therapy offered to SNHL children, early identification and non-pharmacological interventions can reduce the hearing impairment and improve the language skills and psychological development of the young patients (Robinshaw, 1995;Yoshinaga-Itano, 1999). ...
... These programs must begin early to prevent or reduce early-onset and late-onset complications. In the case of CMV-associated SNHL, the programs need to begin before crucial brain development for language and speech skills occurs (Robinshaw, 1995;Yoshinaga-Itano, 1999;Belec and Brogan, 2011). Furthermore, these programs need to focus on the molecular identification of HCMV infection itself rather than the complications associated with HCMV infection. ...
Article
Human cytomegalovirus (HCMV), a herpesvirus, is an important human pathogen that causes asymptomatic infections in healthy or immunocompetent individuals but can lead to severe and potentially life-threatening complications in immune-immature individuals such as neonates or immune-compromised patients such as organ-transplant recipients and HIV-positive individuals. Congenital HCMV infection represents a significant public health issue and poses substantial healthcare and economic burden to society. This virus causes the most common viral congenital infection worldwide, and is the leading non-genetic cause of sensorineural hearing loss in children in developed countries. Congenital HCMV infection is believed to fulfill the criteria of the American College of Medical Genetics to be considered as a condition targeted for a newborn screening program. This is because congenital HCMV infection can be identified during a time (within 2 days after birth) at which it would not ordinarily be detected clinically, and there are demonstrated benefits of early detection, timely intervention, and efficacious treatment of the condition. Recent progresses in developing polymerase chain reaction-based approaches to detect HCMV in samples obtained from newborns have generated much excitement in the field. In this review, we highlight the recent progress in diagnostic techniques that could potentially be used for the detection of HCMV infection in neonates and its direct implications in public health settings for diagnosing congenital HCMV infection.
... Early detection of hearing loss is extremely important in pediatric patients due to its documented impact on speech and language development, literacy development (Yoshinaga-Itano, 1999, 2003, and scholastic achievement (Moeller et al., 2007). Children and teens with CF will likely experience EHF hearing loss that accumulates over time into adulthood (Al-Malky et al., 2015Garinis et al., 2017). ...
... Forcucci and Stark (1972) conducted audiometric, ototologic, and speech-language examinations in a group of 31 children with CF, and 22% had deficient speechlanguage development. Due to the cumulative ototoxic nature of IV-AG, individuals with CF are at a higher risk for developing EHF hearing loss can progress to the SF region and ultimatley impact speech and language development, literacy development, scholastic achievement, and overall quality of life (Moeller et al., 2007;Yoshinaga-Itano, 1999, 2003. Studies have shown EHF information aides in vowel and consonant identification when access to SF energy is restricted or degraded (Lippmann, 1996;Vitela et al., 2015) or when extended bandwidth hearing aids improve audibility of EHFs (Seeto & Searchfield, 2018). ...
Article
Purpose The purpose of this study is to better understand the prevalence of ototoxicity-related hearing loss and its functional impact on communication in a pediatric and young adult cohort with cystic fibrosis (CF) and individuals without CF (controls). Method We did an observational, cross-sectional investigation of hearing function in children, teens, and young adults with CF (n = 57, M = 15.0 years) who received intravenous aminoglycoside antibiotics and age- and gender-matched controls (n = 61, M = 14.6 years). Participants completed standard and extended high-frequency audiometry, middle ear measures, speech perception tests, and a hearing and balance questionnaire. Results Individuals with CF were 3-4 times more likely to report issues with hearing, balance, and tinnitus and performed significantly poorer on speech perception tasks compared to controls. A higher prevalence of hearing loss was observed in individuals with CF (57%) compared to controls (37%). CF and control groups had similar proportions of slight and mild hearing losses; however, individuals with CF were 7.6 times more likely to have moderate and greater degrees of hearing loss. Older participants displayed higher average extended high-frequency thresholds, with no effect of age on average standard frequency thresholds. Although middle ear dysfunction has not previously been reported to be more prevalent in CF, this study showed that 16% had conductive or mixed hearing loss and higher rates of previous otitis media and pressure equalization tube surgeries compared to controls. Conclusions Individuals with CF have a higher prevalence of conductive, mixed, and sensorineural hearing loss; poorer speech-in-noise performance; and higher rates of multiple symptoms associated with otologic disorders (tinnitus, hearing difficulty, dizziness, imbalance, and otitis media) compared to controls. Accordingly, children with CF should be asked about these symptoms and receive baseline hearing assessment(s) prior to treatment with potentially ototoxic medications and at regular intervals thereafter in order to provide otologic and audiologic treatment for hearing- and ear-related problems to improve communication functioning.
... [15,16] In the present study, the commonest type of hearing loss was sensorineural 297 (97.4%). This was consistent with the findings from similar studies by Christine et al., [2] Ozturk et al., [10] Nasser et al. [11] with 94%, 92.2%, and 98% respectively. Sensorineural hearing loss is the commonest type of hearing loss. ...
Article
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Background: Hearing impairment in children is an important public health problem. Children with hearing impairment are educated in special schools (schools for the deaf) and in our locality all are assumed to be deaf without necessarily subjecting them to audiological evaluation in some cases. The objective of this study was to identify the cause of hearing impairment, type and degree of hearing loss, and use of hearing aids among students attending special schools for the deaf in Kaduna. Patients and Methods: This was a cross-sectional study conducted over a period of 4 months between February and May, 2014. Students aged 7-18 years attending Kaduna State Special Education School for the deaf, Katsina road, and demonstration school for the deaf, Kawo Kaduna, were recruited for the study. A questionnaire investigating the student’s biodata, causes of hearing impairment, and use of hearing aids was administered. This was followed by ear examination and pure tone audiometric tests. The data were analyzed using Statistical Package for the Social Science version 16. Results: A total of 307 participants were recruited as the study group. Among them, 58.6% were males and 41.4% were females. The mean age of the study subjects was 13.5 ± 3.6 standard deviation (SD) years. Only 27.7% of the study population have a known cause of their hearing impairment. Two hundred and ninety seven (97.4%) of the students had sensorineural hearing loss, while out of the remaining subjects 5 (1.6%) have conductive and 3 (0.9%) have mixed hearing losses. Two hundred and sixty eight (87.3%) of the participants had profound hearing loss while 37 (12%) had mild-to-severe hearing loss. Two students (0.7%) were found to have normal hearing. No student was found to be using hearing aid. Conclusion: In majority of the students, the cause of the hearing impairment is unknown. Some of the students have residual hearing and none of them is using hearing aids. Further evaluation and possible amplification may be required to rehabilitate some of these students into normal schools. Key words: Audiometry, schools for the deaf, deafness, hearing aid, hearing impairment
... Do ğuştan İK' lı olan bir ço cu ğun er ken ta nı, ci haz la ma ve re habi li tas yon ile eği ti mi ve sos yal ha ya ta ka tı lı mı ne ka dar ko lay sa, geç dö nem de bu sü re cin ger çek leş me si di ğer kurum lar için o de re ce ma li yet li, zor ve İE' ler ve ai le le ri için de o de re ce olum suz dur. 2,11,12 Bu bağ lam da çağ daş ve de mok ra tik bir hu kuk dev le ti olan Tür ki ye Cum huri ye ti'n de sağ lık hiz me ti nin bu alan da ki ye ter li li ği ya da ye ter siz li ği, önü müz de ki yıl lar da sık ola rak sor gu la nacak ve bel ki de (en azın dan ku rum sal bağ lam da) so rumlu luk da va la rı na he def ola cak tır. ...
Article
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Hearing loss (HL) is a symptom associated with various impairments such as speech and learning, so that their contribution to daily life activities could be limited. Hearing disabled people (HDP) could face to two options: joining to social life by using hearing aids (HA) or being sign language users as members of a different pyscho-sociocultural subsociety (the Deaf). These options are not only related with age-on set, level or etiology of HL, but more with health system of the country. Availability of early detection and ideal fitting, special and general education systems, in addition to social awareness level, are of major importance. Purpose of this paper is to review the studies presenting general view of the HL and HDP in Turkey. Thus, as national new born hearing screening program covers throughout the country, it could be possible to provide both quantitative and qualitative perspective to the future plans in this field.
... Early detection through universal newborn hearing screening (UNHS) programs offers opportunities for early access to hearing devices and specialized communication intervention. There is now clear evidence that supports the effectiveness of early fitting of hearing devices and early enrolment in specialized intervention for improving outcomes (Holzinger, Fellinger, & Beitel, 2011;Vohr et al., 2011;Yoshinaga-Itano, 1999). While decisions about device fitting are guided by evidence-based protocols on audiological management (American Academy of Audiology Task Force on Pediatric Amplification, 2013), there is a lack of high-quality evidence on how communication skills can be best learnt or taught in the presence of hearing loss (Fitzpatrick et al., 2016). ...
Article
To explore the factors influencing parents’ choice of communication mode during early education of their child with hearing loss. Qualitative descriptive analysis of semi-structured interviews with parents of children with hearing loss. Fourteen parents of children who participated in the Longitudinal Outcomes of Children with Hearing Impairment study. Four themes emerged from thematic analysis of the interview data: (1) parents draw on a variety of experiences and information to make decisions; (2) parents’ preferred outcomes for their children drive their choices; (3) child’s preference and proficiency drive parental choice; and (4) parents’ fears and worries influence decisions. The results reinforced the importance of parents receiving unbiased, descriptive information as well as evaluative information from professionals, so that they could consider all options in making a decision that met their needs. Parents also require continual support for implementation of their choices as they adjust to their children’s changing communication needs. Parent decisions around communication mode are rarely made in isolation, but occur within a larger decision-making matrix that include device choices, early intervention agency choices, and ‘future-proofing’ the child’s ongoing communication needs. Abbreviations: AH: Australian Hearing; EIA: early intervention agency; LOCHI: longitudinal outcomes of children with hearing impairment; NSW: New South Wales; PCHL: permanent childhood hearing loss; QLD: Queensland: UNHS: universal newborn hearing screening; VIC: Victoria
... 1,4,7,8,11,57 Çocuğun yaşamında işitme kaybı ne kadar erken ortaya çıkarsa, çocuğun gelişimi üzerindeki etkileri de o derecede ciddi ve sorun ne kadar erken tanımlanabilirse, bu etkilerin önlenmesi de o derecede başarılı olacaktır. 1,4,57,58 Bu noktada odyolojik değerlendirmeler, işitme kaybının çocuk üzerindeki etkileri ile ilgili çok önemli bilgiler sağlamakla birlikte, kapsamlı bir görüntü sunmazlar. 59,60 İşitme testleri, çocuğun işitme kaybı ile ilgili niceliksel bilgiler sağlar; işitme kaybının çocuğun günlük yaşamı üzerindeki etkileri veya işitme kaybının yarattığı yetersizlikler ile ilgili bilgiler sağlamaz. ...
Article
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Abstract: Among the society’s “special” members affected from various deficiencies, the ones affected from auditory deficiency (AAD) have a special place. By leading to different lingual and learning processes, congenital auditory deficiency and auditory deficiency that emerged in early childhood created different social life preferences and demands, and the tools for quality of life are suitable for evaluating the service offered to those children. Moreover, the auditory deficiency becomes a psychological and social deficiency through the decrease in auditory perception as the age advances, and what can show us the dimensions of the “being happy and healthy” perception, which those people think they lose due to their auditory deficiency, is the assessment tools for the quality of life.While the quality of life has been used to be a topic of psychology and sociology at the beginning, it has become a fundamental concept of the health sciences in course of time. Behind this increasing interest lies the fact that the quality of life is considered to be the result of medical interventions and is also contributes to the variables such as the functionality of senses or organs and the lifetime that are the reference points of traditional biomedical perspective. The scales for quality of life developed specifically for auditory deficiency are very important and necessary in assessing the positive effects of medical, rehabilitative, educational and social services offered to individuals, especially ones having auditory deficiencies, in otology and neurootology and audiology applications.Within this respect, translating those tools into Turkish and applying them together with general quality of life and quality of life in general health scales to large masses and adopting the values specific to our country will close the gap between clinic success and patient satisfaction especially in otology, neuro-otology and audiology. Özet: Toplumun değişik yetersizliklerden etkilenen “özel” bireyleri arasında işitsel yetersizlikten etkilenmiş (İYE) olanlar son derece özel bir yer tutmaktadır. İşitme kaybı doğuştan ve erken çocuklukta ortaya çıkan şekliyle, farklı dil ve öğrenme süreçlerine yol açarak farklı sosyal yaşantı tercihlerine ve dolayısıyla da taleplere yol açmaktadır ki bu bağlamda yaşam kalitesi araçları bu çocuklara sunulan hizmetin değerlendirilmesi için son derece uygundur. Yaşam kalitesi başlangıçta psikoloji ve sosyolojinin konusu iken zamanla sağlık bilimleri ile ilgili de temel bir kavram halini almıştır. Artan bu ilginin temelinde yaşam kalitesinin tıbbi müdahalelerin bir sonucu olarak ele alınması ve geleneksel biyomedikal bakış açısının dayanak noktaları olan organların veya duyuların işlevselliği ve yaşam süresi gibi değişkenlere de katkıda bulunuyor olması bulunmaktadır .İşitsel yetersizliğe özel geliştirilmiş yaşam kalitesi ölçekleri, hem otoloji ve nöorotoloji hem de odyoloji uygulamalarında İYE bireyler başta olmak üzere bireylere sunulan tıbbi, rehabilitatif, eğitim ve sosyal hizmetlerin olumlu etkilerinin değerlendirilmesinde son derece önemli ve gereklidir. İşitsel yetersizliğe özel geliştirilmiş yaşam kalitesi ölçekleri, hem otoloji ve nöorotoloji hem de odyoloji uygulamalarında İYE bireyler başta olmak üzere bireylere sunulan tıbbi, rehabilitatif, eğitim ve sosyal hizmetlerin olumlu etkilerinin değerlendirilmesinde son derece önemli ve gereklidir. Bu bağlamda, bu derleme de temel amaç, genel yaşam kalitesi ve genel sağlıkta yaşam kalitesi ölçekleriyle birlikte geniş kitlelere uygulanarak ülkemize özel değerlerin belirlenmesi başta otoloji, nörootoloji ve odyoloji olmak üzere klinik başarıyla hasta memnuniyeti arasındaki farkın ortadan kalkmasını sağlamaktır.
... Oyler & McKay, 2008;Tharpe, 1999Tharpe, , 2008. However, evidence suggests that children with MHL can benefit from early intervention, and the appropriateness of waiting for a measurable deficit before intervening has been called into question Moeller, 2000;Yoshinaga-Itano, 1999;Yoshinaga-Itano, Sedley, Coulter, & Mehl, 1998). Despite this growing evidence, as indicated by Kochkin et al. (2007) and others (e.g., Holstrum et al., 2008;McKay, Gravel, & Tharpe, 2008), many children with MHL still experience a failure-based approach to intervention. ...
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Purpose: A representative sample of the literature on minimal hearing loss (MHL) was reviewed to provide evidence of challenges faced by children with MHL and to establish the need for evidence-based options for early intervention. Method: Research articles published from 1950 to 2013 were searched in the Medline database using the keywords minimal hearing loss, unilateral hearing loss, and mild hearing loss. References cited in retrieved articles were also reviewed. Results: In total, 69 articles contained relevant information about pediatric outcomes and/or intervention for unilateral hearing loss, 50 for mild hearing loss, and 6 for high-frequency hearing loss. Six challenges associated with MHL emerged, and 6 interventions were indicated. Evidence indicates that although some individuals may appear to have no observable speech-language or academic difficulties, others experience considerable difficulties. It also indicates that even though children with MHL may appear to catch up in some areas, difficulties in select domains continue into adulthood. Conclusions: Evidence indicates significant risks associated with untreated MHL. Evidence also demonstrates the need for early intervention and identifies several appropriate intervention strategies; however, no single protocol is appropriate for all children. Therefore, families should be educated about the impact of MHL and about available interventions so that informed decisions can be made.
... The data presented in this review encourage close follow-up of CF patients on aminoglycoside treatment and demonstrates the importance of developing specific screening protocols. Screening is vital for further interventions to minimize linguistic and cognitive developmental delays secondary to SNHL, 39 adding to the overall quality of life of CF patients. To reach this goal, further prospective studies that follow patients long term with HFPTA and OAEs in addition to conventional PTA are warranted. ...
Article
To review the role of routine hearing screening for sensorineural hearing loss (SNHL) in children with cystic fibrosis (CF) who have been on aminoglycoside therapy. PubMed, Cochrane, Scopus, and Ovid databases. A systematic review of the literature was performed in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. A comprehensive search was performed from 1970 to 2014. Randomized controlled trials, case-control studies, cohort studies, and case series including pediatric subjects with baseline auditory evaluations were included. Twelve studies (1979-2014) were reviewed. The study population included 762 children (5 months-20 years). Hearing screening measures included pure-tone audiometry (PTA) at standard ± high frequency threshold (HFPTA) (12/12), distortion product otoacoustic emissions (DPOAE) (4/12), transient-evoked otoacoustic emissions (1/12), and automated auditory brainstem response (1/12). The overall prevalence of SNHL ranged from 0% to 29%. However, on subset analysis of children with greater than 10 courses of intravenous (IV) aminoglycosides, up to 44% had SNHL. Eight studies recommended hearing screening in CF children on aminoglycosides; of these, two studies recommended screening even without aminoglycoside exposure, and four studies made no recommendations. HFPTA was the most commonly recommended screening measure followed by DPOAEs. This systematic review supports a recommendation for clinicians to perform routine hearing screening in children with CF during and after aminoglycoside exposure based on the high prevalence of SNHL in this population. Future studies should define the optimal timing for hearing screening during and after aminoglycoside therapy in children with CF. NA Laryngoscope, 2015. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.
... The mean time of the final diagnosis and intervention in all groups of infants was 89 th day of life. This is appropriate time according to the standards of care and it enables an early intervention within 6 months of age [5,31]. ...
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Objectives The incidence of sensorineural hearing loss is between 1 and 3 per 1000 in healthy neonates and 2–4 per 100 in high-risk infants. The national universal neonatal hearing screening carried out in Poland since 2002 enables selection of infants with suspicion and/or risk factors of hearing loss. In this study, we assessed the incidence and risk factors of hearing impairment in infants ≤33 weeks’ gestational age (wga). Methods We analyzed the database of the Polish Universal Newborns Hearing Screening Program from 2010 to 2013. The study group involved 11438 infants born before 33 wga, the control group—1487730 infants. Screening was performed by means of transient evoked otoacoustic emissions. The risk factors of hearing loss were recorded. Infants who failed the screening test and/or had risk factors were referred for further audiological evaluation. Results Hearing deficit was diagnosed in 11% of infants ≤25 wga, 5% at 26–27 wga, 3.46% at 28 wga and 2–3% at 29–32 wga. In the control group the incidence of hearing deficit was 0.2% (2.87% with risk factors). The most important risk factors were craniofacial malformations, very low birth weight, low Apgar score and mechanical ventilation. Hearing screening was positive in 22.42% newborns ≤28 wga and 10% at 29–32 wga and in the control group. Conclusions Hearing impairment is a severe consequence of prematurity. Its prevalence is inversely related to the maturity of the baby. Premature infants have many concomitant risk factors which influence the occurrence of hearing deficit.
... Nevertheless, consensus appears to exist on the importance of providing as many listening opportunities to the child as possible. Different emphases are placed according to the disciplines involved, for example early diagnosis, early fitting of hearing aids and cochlear implants, and early initiation of speech therapy (Yoshinaga-Itano, 1999;Connor et al., 2000). The importance of characteristics of the child's social context is also recognised, more specifically when teachers, families and others in the community provide clear speech to the child, with a high frequency, and on a level adapted to the child's equivalent language age, this could improve spoken language development (Bronfenbrenner, 1986;Blamey, 2003). ...
Article
The context of congenital deafness has changed under the influence of recent technological innovations in the field of medicine. The implementation of universal neonatal hearing screening programmes in many countries has allowed for early care intervention, and cochlear implants offer new possibilities for hearing and spoken language development. Members of the Deaf community have challenged the medical approach of deafness and emphasised a perspective in which deafness is not considered as an impairment, but as a characteristic of a cultural-linguistic minority. Today, little is known about parents' perspective on their child's care trajectory. Given the different conceptual approaches of deafness and the fact that over 90 percent of deaf children are born to hearing parents who are unfamiliar with the issue, it is important to acquire sociological insight in parents' care-related experieneces and decisions. This dissertation adresses this issue, based on a sociological analysis of data collected among hearing and deaf parents in Flanders, Belgium. The results demonstrate the importance of parents' conceptualisation of deafness for their care-related experiences, preferences and decisions. Moreover, an explanatory model was developed for parents' care-related decisions, which supports policy and professional practice as well as scientific progress in the general field of disability research.
... Ce qui fait qu'un temps précieux est perdu pour la stimulation et le développement de l'audition. L'effet positif d'une stimulation et d'un traitement précoces par prothèses acoustiques est prouvé par rapport à une intervention plus tardive [9]. ...
... Damit geht wertvolle Zeit für eine entsprechende Förderung und die Gehörentwicklung verloren. Der positive Effekt einer Frühförderung und frühen Versorgung mit Hörhilfen im Vergleich zu einer erst später einsetzenden Intervention ist belegt [9]. ...
... Worldwide reporting of hearing loss finds that the prevalence of moderate and severe bilateral hearing deficit (> 40 dB) is 1-3 per 1,000 live births in well baby nursery population [1,2] and 2 -4 per 100 infants in an high risk/NICU babies [3][4][5][6][7]. These numbers signify that hearing impairment is one of the most common potentially disabling conditions in infancy and most frequent congenital anomalies which usually goes undiagnosed [8][9][10]. ...
... In order to achieve effective treatment, congenital or perinatal hearing loss should be recognised within three months of life, with confirmative audiological diagnosis and early intervention before the 6th month of age 4 . Early treatment is essential, as the first year of life is critical for normal development of speech and language, as well as intellectual and emotional growth [5][6][7] . Preterm infants, with an increased risk for SNHL and auditory neuropathy spectrum disorders, are screened with auditory brainstem response (ABR) which allows objective and accurate assessment of the hearing function, with normal variation according to age due to physiological maturation of the auditory pathway 7 . ...
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Plain-language-summary This study investigated hearing threshold changes during the first year of corrected age (CA) in infants admitted in a neonatal intensive care unit (NICU). In 5 years, 239 infants with birth weight (BW) ≤ 1,000 gm and/or gestational age (GA) ≤ 30 weeks were enrolled. Hearing was evaluated by oto-acoustic emission (OAEs) before discharge and auditory brainstem response (ABR) within 3 months of CA. Infants affected by unilateral or bilateral hearing loss were addressed to audiological follow-up until definitive diagnosis (within 6 months of CA). Changes in hearing threshold were also carefully analysed. 207 (86.6%) infants had normal hearing while 32 infants (13.4%) showed hearing loss (HL) at the confirmative ABR evaluation (9 mild, 16 moderate, 4 severe, 3 profound). The latter showed lower GA (27.7 ± 2 vs 28.4 ± 1.2; p = 0.0061) and BW (950 ± 390 vs 1,119 ± 326 gm; p = 0.0085). At final evaluation, 15 infants (47%) recovered a normal hearing. HL was confirmed in 17 patients. Among these, 3 infants were addressed to audiological follow-up (one case of mild unilateral hearing loss (UHL) and two with moderate UHL), while in 14 cases (44%) with bilateral sensory neural hearing loss (SNHL) (7 moderate, 4 severe, 3 profound) hearing aids were prescribed. They showed significantly lower GA and longer hospital stay in the NICU in comparison with infants without indication for audiological habilitation (18 infants) (GA 26.2 ± 2.2 weeks vs 28.4 ± 2.4; p = 0.01; NICU stay 132 ± 67 vs 59 ± 7; p = 0.0002). Definitive diagnosis was obtained at 5.9 ± 1.3 months of CA. Our study confirms the importance of audiological surveillance in preterm newborns. Hearing thresholds of preterm infants with hearing loss can change during the first year of CA and we observed normalisation in 47% of our patients. Most vulnerable to permanent SNHL were very preterm infants with a longer NICU stay, while a shorter stay represents a favourable prognostic factor for hearing improvement.
... One such case of CHL was seen due to extramedullary hematopoiesis of the middle ear in a patient with SCD. 11 Hearing loss presents as an especially important consequence in pediatric patients due to the importance of hearing and its role in social and language development. Unfortunately, apart from newborn screening exams, audiograms testing for hearing loss are not routinely performed in patients with SCD. ...
Article
Objectives To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. Study Design Meta‐analysis. Methods We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. Results We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta‐analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. Conclusion This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 2020
... 6,49,[106][107][108][109][110][111][112][113][114][115][116][117] Auditory development is complexly related to language development and auditory sensory deprivation during early sensitive periods of brain development has been shown to increase risk for LD. [46][47][48][118][119][120][121][122] Additionally, neonatal or early auditory dysfunction as evaluated by ABR has been associated with LD at later ages. 46,47,123 Therefore, UHB may be associated with LD as a corollary. ...
Article
Bilirubin-induced brain injury in the neonatal period has detrimental effects on neurodevelopment that persist into childhood and adulthood, contributing to childhood developmental disorders. Unconjugated bilirubin is a potent antioxidant that may be useful for protecting against oxidative injuries, but it becomes a potent neurotoxin once it crosses the blood brain barrier. Because bilirubin toxicity involves a myriad of pathological mechanisms, can damage most types of brain cells, and affects brain circuits or loops that influence cognition, learning, behavior, sensory, and language, the clinical effects of bilirubin-induced neurotoxicity are likely to be manifold. One possible effect that several experts have identified is bilirubin-induced neurological dysfunction (subtle kernicterus). However, the underlying biological mechanisms or pathways by which subtle kernicterus could lead to developmental disorders has not been elucidated previously. Our aim in this review is to describe a spectrum of developmental disorders that may reflect subtle kernicterus and outline plausible biological mechanisms for this possible association. We review existing evidence that support or refute the association between unconjugated hyperbilirubinemia and developmental disorders, and limitations associated with these studies.
... Hearing loss affects children's cognitive, emotional, and social development, and educational, societal, and financial outcomes [1,2]. Early identification and intervention through universal newborn hearing screening (NHS) has been shown to ameliorate these risks [3,4]. However, as many as 36% of U.S. children referred on NHS are lost to follow-up [5]. ...
Article
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Introduction: Hearing loss substantially impacts pediatric development, and early identification improves outcomes. While intervening before school-entry is critical to optimize learning, early-childhood hearing screening practices are highly variable. Conditioned play audiometry (CPA) is the gold standard for preschool hearing screening, but otoacoustic emission (OAE) testing provides objective data that may improve screening outcomes. Objectives: To compare outcomes of a community-based low-income preschool hearing program before and after implementation of OAE in a single-visit, two-tiered paradigm. We hypothesized that this intervention would reduce referral rates and improve follow-up while maintaining stable rates of diagnosed sensorineural hearing loss. Methods: We performed a cohort study of 3257 children screened from July 2014-June 2016. Department of Public Health data were analyzed pre- and post-implementation of second-line OAE testing for children referred on CPA screening with targeted follow-up by DPH staff. Primary outcomes included referral rates, follow-up rates, and diagnosis of sensorineural hearing loss. Results: Demographics, pure-tone pass rates, and incidence of newly-diagnosed permanent hearing loss were similar across years. After intervention, overall pass rates increased from 92% to 95% (P = 0.0014), while only 0.7% remained unable to be tested (P<0.0001). 5% of children were unable to be tested by CPA screening but passed OAE testing, obviating further evaluation. Referral rate decreased from 8% to 5% (P = 0.0014), and follow-up improved from 36% to 91% (P<0.0001). Identification of pathology in children with follow-up increased from 19% to over 50%. Further, disparities in pass rates and ability to test seen in Year 1 were eliminated in Year 2. Conclusion and relevance: In a community setting, implementation of second-line OAE screening for CPA referrals reduced referral rates, increased identification of hearing loss, reduced outcome disparities, and improved follow-up rates. This study provides lessons in how to improve outcomes and reduce disparities in early-childhood hearing screening.
... The earlier a child's hearing loss is identified, and intervention provided, the better the overall outcome. [8][9][10] However, delays are common since without systematic newborn screening, hearing loss in children is commonly unaddressed during the first 2 years of life, 11 which represents a crucial period from a developmental perspective. In the case of mild or unilateral hearing loss, this period can be even longer and very often intervention may be delayed until children reach school age. ...
Article
Aim: To review existing guidelines for universal newborn hearing screening (UNHS), identify those that provide comprehensive and clear recommendations on the subject, and provide a brief overview. Method: A scoping literature review was performed in PubMed, the Guidelines International Network library, and national guideline databases to identify guidelines on newborn hearing screening developed or updated between 2004 and 2019. The quality of the guidelines was checked with the Checklist for the Quality Assessment of Guidelines (AGREE II). Results: Six guidelines met all the inclusion criteria. All six were based on the 1-3-6 benchmark (screening completed by 1mo, audiological diagnosis by 3mo, enrolment in early intervention by 6mo). However, the guidelines varied in terms of their recommendations for the application of screening methods, role of health professionals in the screening process, and quality. Based on the AGREE II score, flexibility, adaptability, and foundation role for all other guidelines, the 2019 guidelines of the Joint Committee on Infant Hearing position statement were identified as the most appropriate to be recommended for adaptation by countries or programmes. Interpretation: The diversity in the existing guidance can be confusing for countries and institutions that are planning to develop universal hearing screening programmes. As more countries and organizations develop their newborn hearing screening programmes, they will need examples to emulate. This review provides an evaluation of the quality, comprehensiveness, and applicability of existing clinical guidelines that can serve as a facilitator for countries, institutions, or organizations in their planning and implementation of a UNHS programme.
... HL affects one in 1000 newborns [11]. However, the heterogeneity of etiologies leading to HL provides a challenge for effective diagnosis and treatment. ...
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Hearing loss (HL) is a common sensory disorder. More than half of HL cases can be attributed to genetic causes. There is no effective therapy for genetic HL at present, early diagnosis to reduce the incidence of genetic HL is important for clinical intervention in genetic HL. Previous studies have identified 111 nonsyndromic hearing loss genes. The most frequently mutated genes identified in NSHL patients in China include GJB2, SLC26A4, and the mitochondrial gene MT-RNR1. It is important to develop HL gene panels in Chinese population, which allow for etiologic diagnosis of both SHL and NSHL. In this study, a total of 220 unrelated Han Chinese patients with bilateral progressive SNHL and 50 unrelated healthy controls were performed Single nucleotide polymorphism (SNP) genotyping using an improved multiplex ligation detection reaction (iMLDR) technique, is to simultaneously detect a total of 32 mutations in ten HL genes, covering all currently characterized mutations involved in the etiology of nonsyndromic or syndromic hearing loss in the Chinese population. The 49 positive samples with known mutations were successfully detected using the iMLDR Technique. For 171 SNHL patients, gene variants were found in 57 cases (33.33%), among which, 30 patients carried mutations in GJB2, 14 patients carried mutations in SLC26A4, seven patients carried mutations in GJB3, and six patients carried mutations in MT-RNR1. The molecular etiology of deafness was confirmed in 12.9% (22/171) of patients carried homozygous variants. These results were verified by Sanger sequencing, indicating that the sensitivity and specificity of the iMLDR technique was 100%. We believe that the implementation of this population-specific technology at an efficient clinical level would have great value in HL diagnosis and treatment.
... 4-7,14-16 Doğduktan sonra en geç 6 ay içinde işitme engeli tanısı konan ve işitme cihazı uygulanıp işitme ve konuşma eğitimi alan bebeklerin konuşma becerisi normal işiten yaşıtlarına benzer düzeyde gelişebilir. [1][2][3][4][5][6][7]14 Erken tanılama, işitme zorluğu çeken çocuklarda yaşamları boyunca onları etkileyebilecek olan konuşma, dil gelişimlerinde, öğrenme becerilerinde, sosyal ve mesleki alanlardaki olumsuz etkileri ortadan kaldırabilmektedir. 13 Erken teşhis ve erken müdahalenin sonuçları Tablo 2'de gösterilmektedir. ...
Article
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Ülkemizde son derece az sayıda bulunan eğitim odyolojisi uzmanlarının, önemli görevlerinden biri işitme kayıplı bireylerin eğitim gereksinimlerinin değerlendirmek, belirlenen ihtiyaçlar doğrultusunda rehabilitasyon hizmetlerini sağlamak, ayrıca hem işitme kayıplı bireyin hem de ailesinin rehberlik-danışmanlık hizmetlerini yürütmektir. Artık biliyoruz ki ülkemizde de yürütülmekte olan Yeni Doğan İşitme Tarama programları ile işitme kaybının tanı yaşı 3-4 aya indirilmeye başlanmıştır. Erken tanılama ve erken cihazlandırma ile işitme yetersizliği olan çocukların sesle tanışmaları sağlanmaktadır. Ancak çocukların sesi duymaya başlaması konuşmayı öğrenmeleri için yeterli değildir. Hemen arkasından başlayacak özel eğitim ve rehabilitasyon hizmetleri sonucunda çocuk konuşmayı öğrenmekle birlikte tüm gelişim alanlarında da yaşıtlarını yakalayabilmektedir. Eğitim odyolojisi uzmanları işitme kayıplı çocukların gerektiğinde özel eğitim hizmetlerine yönlendirilmesi ve izlenmesi hizmetlerini de yürütmektedirler.
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Purpose: Patients treated with cranial radiation therapy (RT) are at risk for sensorineural hearing loss (SNHL). Although SNHL is often characterized as a delayed consequence of anticancer therapy, longitudinal reports of SNHL in childhood cancer survivors treated with contemporary RT are limited. We report the incidence, onset, severity, and long-term trajectory of SNHL among children receiving RT. Potential risk factors for SNHL were also identified. Patients and methods: Serial audiologic testing was conducted on 235 pediatric patients who were treated with conformal or intensity-modulated RT as part of an institutional phase II trial for localized primary brain tumors, including craniopharyngioma, ependymoma, and juvenile pilocytic astrocytoma. All but one patient had measurable cochlear radiation dose (CRD) greater than 0 Gy. The median follow-up from RT initiation to latest audiogram was 9 years with a median of 11 post-RT audiograms per patient. Audiograms were classified by the Chang Ototoxicity Grading Scale. Progression was defined by an increase in Chang grade from SNHL onset to the most recent evaluation. Results: At last evaluation, SNHL was prevalent in 14% of patients: 2.1% had mild and 11.9% had significant SNHL requiring hearing aids. Median time from RT to SNHL onset was 3.6 years (range, 0.4 to 13.2 years). Among 29 patients with follow-up evaluations after SNHL onset, 65.5% experienced continued decline in hearing sensitivity in either ear and 34.5% had no change. Younger age at RT initiation (hazard ratio [HR], 2.32; 95% CI, 1.21 to 4.46), higher CRD (HR, 1.07; 95% CI, 1.03 to 1.11), and cerebrospinal fluid shunting (HR, 2.02; 95% CI, 1.07 to 3.78) were associated with SNHL. Conclusion: SNHL is a late effect of RT that likely worsens over time. Long-term audiologic follow-up for a minimum of 10 years post-RT is recommended.
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Bone conduction (BC) auditory brainstem response (ABR) is an important factor in determining the type of hearing loss especially in infants and other difficult-to-test populations. However, multiple constraints including technical difficulties make evaluating BC ABR less feasible in the clinic, and there is also lack of information that guides clinicians how to evaluate it. The purpose of this study is to compare the latency of wave V in BC and air conduction (AC) ABR in normal hearing infants and gather reference values for wave V latency of BC ABR.
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In most classrooms, where information is presented orally via spoken language, accurate knowledge of a student's hearing status is crucial so that the interdisciplinary team can ensure appropriate service provision. Audiologists play a key role on the interdisciplinary team to provide other professionals with information about children's hearing status, communication needs, device use, and intervention strategies. Conversely, audiologists gain valuable information and strategies from other team members.
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The aim of this study was to evaluate the status of early hearing detection and intervention after newborn hearing screening (NHS) in South Korea. A retrospective review of Korean national health insurance service data of all infants receiving the 4-month old national infant health checkup between 2010 and 2016 from a nationwide population-based database was conducted. Based on the results of the NHS-administered hearing questionnaires as part of the national infant health checkup, individuals were classified into “pass” (1,730,615 infants) or “refer” (10,941 infants) groups. Next, an analysis was conducted of age and the frequencies of tracking audiologic tests and surgeries of the middle ear (ME) and cochlear implants (CI). Diagnostic auditory brainstem response and audiometry, and surgeries of ME and CI were significantly performed more and earlier in the refer group compared with the pass group. For infants in the pass group who were presumed to have delayed or acquired hearing loss, the time of the first audiology tests and CI surgery was significantly delayed compared to those in the refer group; the average ages for first CI were 37 and 52 months in the refer group and pass group, respectively. Therefore, for early detection of delayed-onset hearing loss, regular hearing screening programs should be considered throughout the preschool ages.
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The use of simulation in audiology (AUD) and speech-language pathology (SLP) fields is less than its use in other health professions. This study aimed to examine the effect of the use of simulation training sessions versus video simulation case scenarios on AUD and SLP undergraduate students’ confidence in knowledge and professional competencies of the 1–3–6 early hearing detection and intervention (EHDI) timeline. A randomized pretest-posttest control group study design was used to assess self-ratings of confidence for specific knowledge and professional competencies. Thirty-four undergraduate students (AUD = 24; SLP = 10) participated in this study. The mean age of the participants was 20.09 years (SD = ±0.79, range: 19–21 years). All AUD and SLP participants attended a lecture and then were divided into two groups: the experimental group had simulation activities, while the control group watched video-recorded scenarios followed by a discussion session. Statistically significant differences (improvements) of students’ confidence in knowledge and professional competencies were detected in both groups. The mean difference between pre- and post-test scores for the experimental group was 0.59 (p < 0.001), and it was 0.91 (p < 0.001) for the control group. The use of simulation training sessions or video simulation case sceneries can facilitate student learning. Video simulation case scenarios are feasible for teaching the 1–3–6 EHDI timeline and offer similar situations to simulation training sessions. AUD and SLP programs are encouraged to use both teaching methods.
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Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive multiple congenital malformation and intellectual disability syndrome resulting from variants in DHCR7. Auditory characteristics of persons with SLOS have been described in limited case reports but have not been systematically evaluated. The objective of this study is to describe the auditory phenotype in SLOS. Age- and ability-appropriate hearing evaluations were conducted on 32 patients with SLOS. A subset of 21 had auditory brainstem response testing, from which an auditory neural phenotype is described. Peripheral or retrocochlear auditory dysfunction was observed in at least one ear of 65.6% (21) of the patients in our SLOS cohort. The audiometric phenotype was heterogeneous and included conductive, mixed, and sensorineural hearing loss. The most common presentation was a slight to mild conductive hearing loss, although profound sensorineural hearing loss was also observed. Abnormal auditory brainstem responses indicative of retrocochlear dysfunction were identified in 21.9% of the patients. Many were difficult to test behaviorally and required objective assessment methods to estimate hearing sensitivity. Individuals with SLOS are likely to have hearing loss that may impact communication, including speech and language development. Routine audiologic surveillance should be conducted to ensure prompt management of hearing loss.
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Purpose: The purpose of this study is to better understand the prevalence of ototoxicity-related hearing loss and its functional impact on communication in a pediatric and young adult cohort with cystic fibrosis (CF) and individuals without CF (controls). Method: Observational, cross-sectional investigation of hearing function in children, teens, and young adults with CF (n = 57, mean = 15.0 yr.) who received intravenous aminoglycoside antibiotics and age- and gender-matched controls (n = 61, mean = 14.6 yr.). Participants completed standard and extended high frequency audiometry, middle ear measures, speech perception tests, and a hearing and balance questionnaire. Results: Individuals with CF were 3 to 4 times more likely to report issues with hearing, balance, and tinnitus and performed significantly poorer on speech perception tasks compared to controls. A higher prevalence of hearing loss was observed in individuals with CF (57%) compared to controls (37%). CF and control groups had similar proportions of slight and mild hearing losses, however individuals with CF were 7.6 times more likely to have moderate and greater degrees of hearing loss. Older participants displayed higher average EHF thresholds, with no effect of age on average SF thresholds. Although middle ear dysfunction has not previously been reported to be more prevalent in CF, this study showed that 16% had conductive or mixed hearing loss and higher rates of previous otitis media and pressure equalization (PE) tube surgeries compared to controls. Conclusions: Individuals with CF have a higher prevalence of conductive, mixed and sensorineural hearing loss, poorer speech-in-noise performance and higher rates of multiple symptoms associated with otologic disorders (tinnitus, hearing difficulty, dizziness, imbalance and otitis media) compared to controls. Accordingly, children with CF should be asked about these symptoms, receive baseline hearing assessment(s) prior to treatment with potentially ototoxic medications, and at regular intervals thereafter in order to provide otologic and audiologic treatment for hearing and ear-related problems to improve communication functioning.
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Aim: The aim of this retrospective cohort study was to present the demographic and clinical features of newborns diagnosed with hearing loss and to evaluate the results of hearing rehabilitation in terms of auditory perception and speech and language skills. Materials and methods: Demographic data, auditory perception performances and receptive and expressive language skills of newborns were evaluated with Ling’s six sounds test, Infant-Toddler/Meaningful Auditory Integration Scale and Preschool Language Scale Fourth Edition on first admission and subsequently at 1st, 2nd, 3rd, 4th and 5th years. Results: A total of 30 newborns (10 girls, 20 boys), with an average age of 48.70±17.19 (range, 5 to 75) months at the initial evaluation-were included in this study. There was significant increases on the detection and recognition performance of Ling’s six sounds and IT-MAIS/MAIS scores. These improvements were observed more remarkably especially in the first and second years. Receptive and expressive language performances of newborns were increasing gradually and reached to the level of chronologic age at the end of the second year. Conclusion: Neonatal hearing screening programs must be popularized for early diagnosis and referral of newborns with hearing loss. Increased awareness and improvement of communication on these topics are mandatory for achievement of better results.
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Objective: The aim of this study was to evaluate the GoldenGate microarray as a diagnostic tool and to elucidate the contribution of the genes on this array to the development of both nonsyndromic and syndromic sensorineural hearing loss in China. Methods: We developed a microarray to detect 240 mutations underlying syndromic and nonsyndromic sensorineural hearing loss. The microarray was then used for analysis of 382 patients with nonsyndromic sensorineural hearing loss (including 15 patients with enlarged vestibular aqueduct syndrome), 21 patients with Waardenburg syndrome, and 60 unrelated controls. Subsequently, we analyzed the sensitivity, specificity, and reproducibility of this new approach after Sanger sequencing-based verification, and also determined the contribution of the genes on this array to the development of distinct hearing disorders. Results: The sensitivity and specificity of the microarray chip were 98.73% and 98.34%, respectively. Genetic defects were identified in 61.26% of the patients with nonsyndromic sensorineural hearing loss, and 9 causative genes were identified. The molecular etiology was confirmed in 19.05% and 46.67% of the patients with Waardenburg syndrome and enlarged vestibular aqueduct syndrome, respectively. Conclusion: Our new mutation-based microarray comprises an accurate and comprehensive genetic tool for the detection of sensorineural hearing loss. This microarray-based detection method could serve as a first-pass screening (before next-generation-sequencing screening) for deafness-causing mutations in China.
Article
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.
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Objective: Assisted reproductive technologies (ART), especially intracytoplasmic sperm injection (ICSI), is associated with birth defects. However, there are few reports on hearing screening tests of these babies. We aimed to determine the association between ART and hearing loss in newborns. Materials and methods: This retrospective study examined 246 ICSI newborns between 2013 and 2015. All the patients conceived by ICSI. We examined the hearing screening results of the babies. Results: A total of 25 newborns did not pass the first screening test, including 17 by transient evoked otoacoustic emission (TEOAE) and 8 by auditory brainstem response (ABR). Ten babies did not pass the advanced examination. Total hearing loss affected 4% of ART babies. Conclusion: Hearing screening tests exposed deficient results in 4% of ICSI babies, which is ten times more frequent than spontaneously conceived newborns.
Chapter
Despite positive trends in the literature associated with early implantation, we cannot assume that children with cochlear implants (CIs) learn just like their hearing peers, or even just like each other. Schools that include children with CIs must consider a range of factors that influence academic and social outcomes, and balance each child’s need for challenge and support. Considerations include parent education and emotional support, language and literacy development, social emotional learning, motor and sensory development, working memory and other cognitive skills, classroom acoustics and modifications, individualized support services, classroom supports, and other ecological inputs.
Article
This investigation was conducted by a questionnaire that was administered to the parents of children suspected of hearing impairment and received hearing tests. A total of 31 completed and valid questionnaires were returned.The questions were about the background of the detection of their children's hearing loss, the otorhinolaryngologists' support at the result of their children's audiometry test and the information they received. Sixteen children were found having hearing loss as their parents suspected. Eleven cases had satisfaction of the otorhinolaryngologist's support, but Thirteen cases were dissatisfied. Twenty six cases obtained some information about the deafness or training method of hearing-impaired children from webpages or acquaintances.The results suggested that the support and the precise information from otorhinolaryngologists are needed to the patients' parents.
Article
In the present study, we compared the processing of both conventional and novel metaphors by deaf versus hearing young adults. Eighteen deaf participants with severe-to-profound hearing loss and 18 controls matched for age, sex, and years of education were presented with word pairs of 4 types (literal, conventional metaphors, novel metaphors, and unrelated word pairs) and were asked to perform a semantic judgment task. The word pairs were presented randomly, 1 word at a time, in the center of a computer screen. The reaction times and accuracy rates were collected. The groups of deaf and hearing participantsboth showed lower correct responses and longer reaction times for the novel metaphors than for the conventional metaphors. The percentages of correct scores on the semantic judgment task did not differ between the groups. However, the reaction times were significantly longer for the novel metaphors in the group of deaf individuals than in the group of hearing individuals. Thus, deaf young adults show the same pattern of metaphor processing as hearing peers, that is, novel metaphors are more difficult to process compared with conventional metaphors. However, processing of novel metaphors by deaf participants is achieved with more effort.
Article
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Congenital hearing impairment has a negative effect on the social and communication skills in the majority of children. We compared the development of children with various levels of hearing loss who were supplied with hearing aids and children with cochlear implants and those with no hearing problems. We verified the hypothesis about the bidirectional dependency relationship between the tested variables: the influence of mothers’ anxiety on their hearing impaired children’s development and the influence of the children’s developmental level on the anxiety level of their mothers. The participants were 94 children aged 12 to 24 months with various levels of hearing loss corrected with hearing aids or cochlear implants as well as their mothers. The level of anxiety in mothers was assessed using the State-Trait Anxiety Inventory (STAI). A cause and effect relationship was shown between the child’s hearing loss and their development in the communicative area as well as in the social and emotional area. It was found that children with profound hearing impairment with a cochlear implant functioned better than the group of children with similar or lower hearing impairment using hearing aids. The mothers’ anxiety conditioned the child’s poorer development, and the child’s delayed development had an influence on increasing the anxiety level in mothers. The level of profoundness of hearing loss weakens communicative as well as social and emotional development of children. There exists a bidirectional relationship between mothers’ anxiety and their children’s development.
Article
Objective: To compare the prevalence of disruptive behavior problems between preschool-aged children with hearing loss and normal hearing. Study design: Cross-sectional study. Setting: Tertiary academic center. Patients: Caregivers of children (2-5 yr old) with normal hearing (NH) (n = 39), hearing loss using hearing aid(s) (HA) (n = 29), or cochlear implant(s) (CI) (n = 21). Intervention(s): Demographic information and a mental health history were obtained. Child behavior and language development were assessed. Main outcome measure(s): The Young Child-Diagnostic Interview Schedule for Children-IV and the MacArthur-Bates Communication Development Inventory III. Results: Distributions of race, socioeconomic status, insurance status, and parental home situation (single versus two parent family) were similar across all groups. Parents of children with hearing loss were significantly more likely to report behavior problems (HA = 41%, CI = 38%) than parents of NH children (10%; p = 0.002). Children with hearing loss were significantly more likely to meet diagnostic criteria for oppositional defiant disorder (HA = 48%, CI = 48%) than NH children (23%; p = 0.02). More NH children (8%) than hearing impaired children (0%) had accessed mental health services (p = 0.08). NH children were found to have more advanced language development than hearing-impaired children (p < 0.01), but controlling for Communication Development Inventory III percentiles, the observed behavioral differences remained. Conclusion: Controlling for language development, children with hearing loss have higher prevalence of and impairment from disruptive behaviors than their NH peers. These children are less likely to receive appropriate behavioral interventions. Further research is warranted to investigate the impact of disruptive behaviors on speech and hearing rehabilitation. Methods to improve access to effective behavioral interventions in this population are needed.
Article
We describe a dominant Japanese patient with progressive conductive hearing loss who was diagnosed with NOG -related symphalangism spectrum disorder ( NOG -SSD), a spectrum of congenital stapes fixation syndromes caused by NOG mutations. Based on the clinical features, including proximal symphalangism, conductive hearing loss, hyper­opia, and short, broad middle, and distal phalanges of the thumbs, his family was diagnosed with stapes ankylosis with broad thumbs and toes syndrome (SABTT). Genetic analysis revealed a heterozygous substitution in the NOG gene, c.645C>A, p.C215* in affected family individuals. He had normal hearing on auditory brainstem response (ABR) testing at ages 9 months and 1 and 2 years. He was followed up to evaluate the hearing level because of his family history of hearing loss caused by SABTT. Follow-up pure tone average testing revealed the development of progressive conductive hearing loss. Stapes surgery was performed, and his post-operative hearing threshold improved to normal in both ears. According to hearing test results, the stapes ankylosis in our SABTT patient seemed to be incomplete at birth and progressive in early childhood. The ABR results in our patient indicated the possibility that newborn hearing screening may not detect conductive hearing loss in patients with NOG -SSD. Hence, children with a family history and/or known congenital joint abnormality should undergo periodic hearing tests due to possible progressive hearing loss. Because of high success rates of stapes surgeries in cases of SABTT, early surgical interventions would help minimise the negative effect of hearing loss during school age. Identification of the nature of conductive hearing loss due to progressive stapes ankylosis allows for better genetic counselling and proper intervention in NOG -SSD patients.
Article
Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board–approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a standardized protocol for ECG screening/follow-up.
Article
Leber congenital amaurosis (LCA) is a neurodegenerative disease of photoreceptor cells that causes blindness within the first year of life. It occasionally occurs in syndromic metabolic diseases and plurisystemic ciliopathies. Using exome sequencing in a multiplex family and three simplex case subjects with an atypical association of LCA with early-onset hearing loss, we identified two heterozygous mutations affecting Arg391 in β-tubulin 4B isotype-encoding (TUBB4B). Inspection of the atomic structure of the microtubule (MT) protofilament reveals that the β-tubulin Arg391 residue contributes to a binding pocket that interacts with α-tubulin contained in the longitudinally adjacent αβ-heterodimer, consistent with a role in maintaining MT stability. Functional analysis in cultured cells overexpressing FLAG-tagged wild-type or mutant TUBB4B as well as in primary skin-derived fibroblasts showed that the mutant TUBB4B is able to fold, form αβ-heterodimers, and co-assemble into the endogenous MT lattice. However, the dynamics of growing MTs were consistently altered, showing that the mutations have a significant dampening impact on normal MT growth. Our findings provide a link between sensorineural disease and anomalies in MT behavior and describe a syndromic LCA unrelated to ciliary dysfunction.
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To compare the language abilities of earlier- and later-identified deaf and hard-of-hearing children. We compared the receptive and expressive language abilities of 72 deaf or hard-of-hearing children whose hearing losses were identified by 6 months of age with 78 children whose hearing losses were identified after the age of 6 months. All of the children received early intervention services within an average of 2 months after identification. The participants' receptive and expressive language abilities were measured using the Minnesota Child Development Inventory. Children whose hearing losses were identified by 6 months of age demonstrated significantly better language scores than children identified after 6 months of age. For children with normal cognitive abilities, this language advantage was found across all test ages, communication modes, degrees of hearing loss, and socioeconomic strata. It also was independent of gender, minority status, and the presence or absence of additional disabilities. Significantly better language development was associated with early identification of hearing loss and early intervention. There was no significant difference between the earlier- and later-identified groups on several variables frequently associated with language ability in deaf and hard-of-hearing children. Thus, the variable on which the two groups differed (age of identification and intervention) must be considered a potential explanation for the language advantage documented in the earlier-identified group.
Article
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The high-risk registry was used as a screening device for identifying hearing loss for many decades in Colorado. It reportedly missed approximately 50% of all infants with congenital sensorineural hearing loss (Mehl & Thomson, 1998; Parving, 1993; Watkins, Baldwin, & McEnery, 1991). Little is known about the developmental characteristics of this population. This article describes children identified through the high-risk registry. These children have been divided into two groups according to their age of identification: (a) deaf and hard of hearing children identified before age 6 months, and (b) deaf and hard of hearing children identified between ages 7 and 18 months. The children identified before age 6 months and receiving intervention at an average of 2 to 3 months after identification of hearing loss had significantly higher levels of receptive and expressive language, personal-social development, expressive and receptive vocabulary, general development, situation comprehension, and vowel production. The high-risk registry used for newborn hearing screening has been replaced by universal newborn physiological hearing screening in the state of Colorado.
Article
Research in the area of language, speech, social-emotional development, and deafness has focused on a search for predictors of successful outcomes. Until recently, robust predictors of successful language outcomes were difficult to determine. The literature contains examples of children who are deaf or hard of hearing who are able to maintain developmental profiles similar to their hearing peers. Unfortunately, these children represent the minority of the population of children with significant hearing loss-not the majority. A series of developmental studies have been conducted at the University of Colorado on the language, speech, and social-emotional development of deaf and hard-of-hearing children in the State of Colorado. The vast majority of these children (99%) receive intervention services through the Colorado Home Intervention Program (CHIP). Several outcomes have been observed in following over 400 children in Colorado with significant hearing loss. There is a significantly higher number of children who have developed and maintained age-appropriate language skills, both orally and in sign language. There is a significantly more competent sign language level in children entering kindergarten. Most children with all degrees of hearing loss, except profound loss (when using traditional amplification), have developed intelligible speech by the time they enter kindergarten, regardless of their mode of communication and even when they have additional disabilities. In general, the research indicates that there are more children who are linguistically competent in two modalities, visual and auditory.
Article
In 1974, the Office of Demographic Studies, Gallaudet College, conducted a survey in which the teachers of 978 hearing-impaired students in special education programs across the United States rated the degree to which the speech of each student would be understood by the average person. These ratings were then analyzed for their relationships to other significant characteristics of the children in question. Among the findings were 1) that speech intelligibility does not improve with age, 2) that it declines as the severity of the hearing loss increases, 3) that higher speech intelligibility is generally related to greater degrees of hearing aid usage (which, however, is itself strongly related to degree of hearing loss), 4) that placement in a particlular type of educational program doesn't produce better intelligibility than placement in some other program type, 5) that black students are substantially less intelligible than students from other ethnic backgrounds, and that family income level is related to speech intelligibility. (Tables are provided for all study data). (DLS)
Article
The purpose of this series of studies was to demonstrate the comparability of this Colorado population of deaf or hard-of-hearing students with today's deaf or hard-of-hearing students and with those reported on in the literature. The students are described through their performance on the Test of Auditory Comprehension of Language (TACL) (Carrow-Woolfolk, 1973); The Test of Syntactic Abilities (Quigley, Steinkamp, Power, & Jones, 1978); words per T-unit (Hunt, 1965); Woodcock & Johnson's Psychoeducational Battery (WJPEB) paragraph comprehension, calculation, and proofing subtests (1979); A Speech Intelligibility Test for Deaf Children (the Clarke test) (Manger, 1972); and the Vineland Social Maturity Scale (the Vineland scale) (Doll, 1965). Additionally, the relationship between these assessments and the elaboration, sequencing, story-grammar, and total process score from the Colorado Process Analysis of Written Language (COPA) (Yoshinaga-Itano & Downey, 1992) is reported. The weakest relationship among these written-language strategies is with the Clarke test and the TACL, indicating that these metalinguistic/metacognitive written-language strategies are not strongly related to speech skills or syntactic structure comprehension through conversational dialogue. The WJPEB calculation subtest and the Vineland test have a surprisingly strong relationship with the COPA, indicating that the underlying cognitive strategies for these skills may be similar to those needed for written-language expression.
Article
Survey questionnaires eliciting incidence information about degree and type of hearing loss, educational placement, use of amplification, and other demographic data were completed by audiologists in 13 of the 15 Area Education Agencies in Iowa in an attempt to describe the characteristics of hearing-impaired children in public-school settings. The information revealed patterns of hearing loss related to age and sex, use of hearing aids, and classroom placement that may be useful in planning support services for this population.
Article
Psychoeducational data were collected from the files of 1,250 hearing-impaired children in Iowa public schools in an effort to identify educational and linguistic profiles related to different degrees of hearing loss. The files of most mildly to moderately hearing-impaired children do not reflect complete assessment of language, academic, or intellectual skills even when support services are being provided. The data reveal deficits that often are inconsistent with the reports and patterns of achievement on which the allocation of support services for hearing-impaired children have been based. The appropriateness of many of the assessment tools in use is questionable.
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Can speech development at 36 months in children with hearing loss be predicted from information available in the second year of life? The Volta Review
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From phone to phoneme: Can we find meaning in babble? The Volta Review
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Is babble the gateway to speech for all children? A longtitudinal study of deaf and hard-of-hearing infants. The Volta Review
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From phone to phoneme: Can we find meaning in babble?
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