Congenital CMV infection affects the general development of the brain and gives rise to a complex pattern of difficulties. Identifying comorbid conditions is very important, as children with associated difficulties and disabilities need more support than children with just hearing impairment. Congenital CMV infection needs to be considered in children with hearing impairment and/or balance disturbance and/or neurodevelopmental disabilities.

The importance that early auditory stimulation has on the infant's plastic brain with regard to optimal listening and language development is well-known. 1,2 During the first year of life, infants with normal hearing start to pay attention to the acoustic features of the speech they are surrounded by. They also develop phonology and acquire the statistical patterns that are important for word segmentation. 3 Children with congenital hearing loss are now identified earlier because of universal hearing screening programmes for newborn infants. 4 The recommended practice for newborn infants with hearing

Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.

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Word fluency was examined in 73 Swedish children aged 6-9 years divided into two age groups, 6-7 and 8-9 years; 34 deaf children with cochlear implants (CI) (15 girls/19 boys) and 39 age-matched children with normal hearing (NH) (20 girls/19 boys). One purpose was to compare the ability to retrieve words in two different word fluency tasks; one phonemically based (FAS letter fluency) and one semantically based (animal fluency). A second purpose was to examine retrieval strategies in the two tasks by conducting an analysis of clustering and switching of word sequences. In general we found that age was an important factor for word fluency ability, in both the CI and the NH groups. It was also demonstrated that children with CI aged 8-9 years retrieved significantly fewer words and used less efficient strategies in the retrieval process, especially on the phonemically based task compared to children with NH of the same ages, whereas children 6-7 years performed similarly in both groups regarding number of retrieved words and use of strategies. The results are discussed with respect to factors such as age differences in performance for children with CI, especially for the phonemically based task.

Background: Decision-making on treatment and (re)habilitation needs to be based on clinical expertise and scientific evidence. Research evidence for the impact of permanent unilateral hearing impairment (UHI) on children's development has been mixed and, in some of the reports, based on fairly small, heterogeneous samples. Additionally, treatment provided has been highly variable, ranging from no action taken or watchful waiting up to single-sided cochlear implantation. Published information about the effects of treatment has also been heterogeneous. Moreover, earlier reviews and meta-analyses published on the impact of UHI on children's development have generally focused on select areas of development. Objectives: This systematic review aimed to summarize the impact of children's congenital or early onset unilateral hearing impairment on listening and auditory skills, communication, speech and language development, cognitive development, educational achievements, psycho-social development, and quality of life. Methods: Literature searches were performed to identify reports published from inception to February 16th, 2018 with the main electronic bibliographic databases in medicine, psychology, education, and speech and hearing sciences as the data sources. PubMed, CINALH, ERIC, LLBA, PsychINFO, and ISI Web of Science were searched for unilateral hearing impairment with its synonyms and consequences of congenital or early onset unilateral hearing impairment. Eligible were articles written in English, German, or Swedish on permanent unilateral hearing impairments that are congenital or with onset before three years of age. Hearing impairment had to be of at least a moderate degree with PTA ≥ 40 dB averaged over frequencies 0.5 to 2 or 0.5 to 4 kHz, hearing in the contralateral ear had to have PTA 0.5-2 kHz or PTA 0.5-4 kHz ≤ 20 dB, and consequences of unilateral hearing impairment needed to be reported in an unanimously defined population in at least one of the areas the review focused on. Four researchers independently screened 1,618 abstracts and 566 full-text articles for evaluation of study eligibility. Eligible full-text articles were then reviewed to summarize the results and assess the quality of evidence. Additionally, data from 13 eligible case and multi-case studies, each having less than 10 participants, were extracted to summarize their results. Quality assessment of evidence was made adapting the Grades of Recommendations, Assessment, Development, and Evaluation (GRADE) process, and reporting of the results adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. Results: Three articles with the quality of evidence graded as very-low to low, fulfilled the eligibility criteria set. Due to the heterogeneity of the articles, only a descriptive summary could be generated from the results. Unilateral hearing impairment was reported to have a negative impact on preverbal vocalization of infants and on sound localization and speech perception both in quiet and in n...

This study examined verbal retrieval in participants with acquired moderate-to-severe sensorineural hearing impairment (M age = 63, M education level = 13 years) compared to participants with normal hearing thresholds (M age = 62, M education level = 14 years) using the letter and category fluency tasks. Analyses of number of words produced, clustering, and switching, were conducted. There was no significant difference between the groups in category fluency performance. In letter fluency, however, the participants with hearing impairment produced significantly fewer words than the normal hearing participants and their production was characterized by fewer switches. Regression analyses were conducted to examine the relationship between demographic, auditory, and cognitive variables and letter fluency performance in the two groups. Phonological skills and auditory acuity predicted letter fluency output only in participants with hearing impairment and a hearing-related link between phonological skills, working memory capacity, and letter fluency switching was found.