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      Hearing impairment and language delay in infants: Diagnostics and genetics

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          Abstract

          This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches.

          The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA).

          The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment.

          The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development.

          Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity.

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          Most cited references112

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          Long-term outcomes of congenital cytomegalovirus infection in Sweden and the United Kingdom.

          Congenital cytomegalovirus (CMV) is an important cause of neurological problems, particularly sensorineural hearing loss, but data on long-term sequelae and the impact of nonprimary maternal infection are limited. We report updated findings on childhood outcomes from 2 large prospective studies. Pregnant women in Malmö, Sweden, and London, United Kingdom, were included between 1977 and 1986, and newborns were screened for CMV (virus culture of urine or saliva). Cases and matched controls underwent regular, detailed developmental assessments up to at least age 5 years. One hundred seventy-six congenitally infected infants were identified among >50 000 screened (Malmö: 76 [4.6/1000 births]; London: 100 [3.2/1000 births]); 214 controls were selected. Symptoms were recorded in 11% of CMV-infected neonates (19/176) and were mostly mild; only 1 neonate had neurological symptoms. At follow-up, 7% of infants (11/154) were classified as having mild, 5% (7/154) moderate, and 6% (9/154) severe neurological sequelae. Four of 161 controls (2%) had mild impairment. Among children symptomatic at birth, 42% (8/19) had sequelae, versus 14% (19/135) of the asymptomatic infants (P = .006). All moderate/severe outcomes were identified by age 1; mild sequelae were first identified at age 2-5 years in 6 children, and age 6-7 years in 3. Among the 16 children with moderate/severe outcomes, 2 had mothers with confirmed and 7 with presumed nonprimary infection. Moderate or severe outcomes were reported in 11% of children with congenital CMV identified through population screening, all by 1 year; all impairment detected after this age was mild. Nonprimary infections contributed substantially to the burden of childhood congenital CMV disease.
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            Young adult academic outcomes in a longitudinal sample of early identified language impaired and control children.

            The long-term academic consequences of childhood language impairment are both theoretically and clinically important. An unbiased appraisal of these outcomes, however, requires carefully designed, longitudinal research. A group of children first identified as having speech and/or language impairment in a community-based, longitudinal study at 5 years of age and matched controls were re-examined during young adulthood (age 19). A comprehensive battery of speech and language, cognitive and achievement tests, psychiatric interviews, and questionnaires were completed by subjects, their parents and teachers. While children with early speech problems showed only a few academic differences from controls in young adulthood, early language impaired (LI) young adults lagged significantly behind controls in all areas of academic achievement, even after controlling for intelligence. Further, rates of learning disabilities (LD) were significantly higher in the LI group than both the controls and community base rates. Concurrent individual difference variables, including phonological awareness, naming speed for digits, non-verbal IQ, verbal working memory, and executive function, all contributed unique variance to achievement in specific areas. Early LI rather than speech impairment is clearly associated with continued academic difficulties into adulthood. These results speak to the need for intensive, early intervention for LI youngsters.
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              The Language Development Survey: a screening tool for delayed language in toddlers.

              L Rescorla (1989)
              This paper reports data from four studies using the Language Development Survey (LDS), a vocabulary checklist designed for use as a screening tool for the identification of language delay in 2-year-old children. A survey completed by the parent in about 10 min, the LDS displayed excellent reliability as assessed by Cronbach's alpha and test-retest techniques. Total vocabulary score as reported on the LDS was highly correlated with performance on Bayley, Reynell, and Preschool Language Scale expressive vocabulary items. The LDS was found to have excellent sensitivity and specificity for the identification of language delay, with a criterion of fewer than 50 words or no word combinations at 2 years yielding very low false positive and false negative rates. Data from three of these studies demonstrate the utility of the LDS as a screening tool for children attending public and private pediatric practices. Prevalence data using the LDS are reported comparing three different severity cutoffs for more than 500 children in seven survey samples.
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                Author and article information

                Journal
                GMS Curr Top Otorhinolaryngol Head Neck Surg
                GMS Curr Top Otorhinolaryngol Head Neck Surg
                GMS Curr Top Otorhinolaryngol Head Neck Surg
                GMS Current Topics in Otorhinolaryngology, Head and Neck Surgery
                German Medical Science GMS Publishing House
                1865-1011
                01 December 2014
                2014
                : 13
                : Doc05
                Affiliations
                [1 ]Department of Otorhinolaryngology, Cologne University Hospital. Köln, Germany
                Author notes
                *To whom correspondence should be addressed: Ruth Lang-Roth, Department of Otorhinolaryngology, Cologne University Hospital, Phoniatry & Pediatric Audiology, Cochlear Implant Center, Newborn Hearing Screening Center North Rhine, Kerpener Straße 62, 50937 Köln, Germany, E-mail: ruth.lang-roth@ 123456uni-koeln.de
                Article
                cto000108 Doc05 urn:nbn:de:0183-cto0001087
                10.3205/cto000108
                4273166
                25587365
                400c198e-86ec-4efd-8329-1030f7118210
                Copyright © 2014 Lang-Roth

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). You are free to copy, distribute and transmit the work, provided the original author and source are credited.

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                Categories
                Article

                Surgery
                newborn hearing screening,organization of newborn hearing screening in germany,pediatric audiology diagnosis,speech and language delay,genetic hearing disorders

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