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Children
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Congenital Hearing Loss in Children
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Congenital Hearing Loss in Children

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Otolaryngology".

Deadline for manuscript submissions: closed (5 July 2024) | Viewed by 6885

Special Issue Editors

Dr. Silvia Palma

E-Mail Website
Guest Editor
Audiology, Primary Care Department, Azienda USL di Modena, Modena, Italy
Interests: cytomegalovirus infection; congenital hearing loss; communication skills in toddlers; middle ear diseases
Special Issues, Collections and Topics in MDPI journals
Prof. Dr. Alessandro Martini

E-Mail Website
Guest Editor
Department of Neurosciences (DNS), Otolaryngology Section, Padova University, Padova, Italy
Interests: hearing disorders; otology; cochlear implants; audiology; genetics
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Hearing loss is one of the most frequent congenital diseases in children. It has a reported prevalence of 1–3:1000 in “healthy babies” and a major prevalence, with variable results, in the neonatal intensive care unit population. As underlined by a recent WHO report, hearing loss, if unaddressed, can negatively impact many aspects of life, particularly language and speech development in children.

Implementing an ever-greater understanding of the prevalence and underlying mechanisms of sensorineural hearing loss improves the clinical decision-making prognosis and treatment of these conditions. Early diagnosis and medical intervention have been shown to lead to improved developmental outcomes. In addition, hearing technology, such as hearing aids and cochlear implants, is effective and can benefit children.

[*] Cutting-edge research: In developed countries, it has been reported that up to 80% of congenital hearing loss is due to genetic causes, with the remaining 20% being secondary to environmental or acquired causes.

 We are soliciting papers focused on implementing knowledge in the field, particularly the prevalence of acquired hearing loss cases: for example, from “TORCH” infection (toxoplasmosis, syphilis, rubella, cytomegalovirus, and herpes) or postnatal bacterial meningitis.

Dr. Silvia Palma
Prof. Dr. Alessandro Martini
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • congenital hearing loss
  • syndromes, genetics
  • epidemiology, cytomegalovirus
  • newborn hearing screening
  • infection

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Published Papers (4 papers)

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Research

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11 pages, 1273 KiB  
Article
Audiological Phenotypes of Connexin Gene Mutation Patterns: A Glance at Different GJB2/GJB6 Gene Mutation Profiles
by Leonardo Franz, Alessandro Incognito, Chiara Gallo, Licia Turolla, Elisa Scquizzato, Roberta Cenedese, Alessandro Matarazzo, Daniel Savegnago, Paolo Zanatta, Elisabetta Genovese, Cosimo de Filippis and Gino Marioni
Children 2024, 11(2), 194; https://doi.org/10.3390/children11020194 - 3 Feb 2024
Cited by 1 | Viewed by 1453
Abstract
GJB2 mutations are the most common cause of autosomal-recessive non-syndromic sensorineural hearing loss (SNHL). The available evidence shows large phenotypic variability across different genotypes and allelic variants. The aim of this study was to investigate the clinical and audiological features of a cohort [...] Read more.
GJB2 mutations are the most common cause of autosomal-recessive non-syndromic sensorineural hearing loss (SNHL). The available evidence shows large phenotypic variability across different genotypes and allelic variants. The aim of this study was to investigate the clinical and audiological features of a cohort of subjects with different GJB2/GJB6 gene mutation profiles from a tertiary referral center in Northeastern Italy. We considered 57 patients with GJB2/GJB6 mutations presenting with congenital, non-syndromic SNHL, mainly coming from the Veneto region (Italy). The samples were screened for mutations in exons 1 and 2 of the GJB2 gene and for the GJB6 gene deletion del (GJB6-D13S1830). Free-field and air-conduction frequency-specific thresholds and the pure-tone average (PTA) were considered in the statistical analysis. Five patients (8.87%) had connexin gene mutations in simple heterozygosis, 15 (26.31%) in compound heterozygosis, 34 (59.64%) in homozygosis, and 3 (5.26%) with digenic patterns. The frequency-specific air-conduction thresholds showed significantly different mean values across the different genotypes (Roy’s largest-root test, p = 0.0473). Despite the evidence already available on genetic SNHL, many new insights are to be expected. Further large-scale prospective studies including different populations are necessary to confirm these preliminary findings about the clinical and audiological features of patients with different GJB2/GJB6 gene mutation patterns. Full article
(This article belongs to the Special Issue Congenital Hearing Loss in Children)
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19 pages, 5652 KiB  
Article
EEG Alpha Band Responses Reveal Amplification Benefits in Infants with Hearing Loss
by Kristin Uhler, Daniel J. Tollin and Phillip M. Gilley
Children 2023, 10(3), 600; https://doi.org/10.3390/children10030600 - 21 Mar 2023
Cited by 1 | Viewed by 1691
Abstract
Our objective was to examine the effects of hearing aid amplification on auditory detection and discrimination in infants who were hard of hearing (IHH) using a physiological measure of auditory perception. We recorded EEG from 41 sleeping IHH aged 1.04 [...] Read more.
Our objective was to examine the effects of hearing aid amplification on auditory detection and discrimination in infants who were hard of hearing (IHH) using a physiological measure of auditory perception. We recorded EEG from 41 sleeping IHH aged 1.04 to 5.62 months while presenting auditory stimuli in a mismatch response paradigm. Responses were recorded during two listening conditions for each participant: aided and unaided. Temporal envelopes of the mismatch response in the EEG alpha band (6–12 Hz) were extracted from the latent, time-frequency transformed data. Aided alpha band responses were greater than unaided responses for the deviant trials but were not different for the standard trials. Responses to the deviant trials were greater than responses to the standard trials for the aided conditions but were not different for the unaided conditions. These results suggest that the alpha band mismatch can be used to examine both detection and discrimination of speech and non-speech sounds in IHH. With further study, the alpha band mismatch could expand and refine our abilities to validate hearing aid fittings at younger ages than current clinical protocols allow. Full article
(This article belongs to the Special Issue Congenital Hearing Loss in Children)
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Review

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15 pages, 1050 KiB  
Review
The Otoacoustic Emissions in the Universal Neonatal Hearing Screening: An Update on the European Data (2004 to 2024)
by Stavros Hatzopoulos, Ludovica Cardinali, Piotr Henryk Skarżyński and Giovanna Zimatore
Children 2024, 11(11), 1276; https://doi.org/10.3390/children11111276 - 23 Oct 2024
Viewed by 918
Abstract
Background: The reported data on European universal neonatal hearing screening (UNHS) practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected unofficial data from 38 collaborating European institutions. The objectives of this systematic review were as follows: (a) [...] Read more.
Background: The reported data on European universal neonatal hearing screening (UNHS) practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected unofficial data from 38 collaborating European institutions. The objectives of this systematic review were as follows: (a) to identify the most recent (in a 20-year span) literature information about UNHS programs in Europe and (b) to provide data on the procedures used to assess the population, the intervention policies, and on the estimated prevalence of congenital hearing loss with emphasis on the bilateral hearing loss cases. Methods: Queries were conducted via the Pubmed, Scopus and Google Scholar databases for the time period of 2004–2024. The Mesh terms used were “OAE”, “Universal Neonatal Hearing Screening”, “congenital hearing loss” and “well babies”. Only research articles and review papers of European origin were considered good candidates. The standard English language filter was not used, in order to identify information from non-English-speaking scientific communities and groups. Results: Very few data and reports were identified in the literature search. Eleven manuscripts were identified corresponding to eight UNHS programs. Except in Poland, most of the data refer to regional and not national programs. The screening coverage estimates of all programs exceed 90%; infants were mostly assessed by a three-stage protocol (TEOAE + TEOAE + AABR), followed by a clinical ABR test. The average prevalence (i.e., from well babies AND NICU infants) of bilateral hearing loss ranged from 0.5 to 20.94 per 1000 (Zurich sample). Infants presenting unilateral or bilateral hearing losses were first rehabilitated by hearing aids and consequently (>15 mo) by cochlear implants. Conclusions: Even though UNHS programs are well-established clinical practices in the European States, the amount of information in the literature about these programs is surprising low. The existing data in the timespan 2004–2024 corroborate the international UNHS data in terms of coverage and bilateral hearing loss prevalence, but there is a strong need to supplement the existing information with the latest developments, especially in the area of hearing loss rehabilitation. Full article
(This article belongs to the Special Issue Congenital Hearing Loss in Children)
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Other

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8 pages, 1427 KiB  
Case Report
A Peculiar Case of Ossicular Chain Fixation and Enlarged Vestibular Aqueduct
by Davide Brotto and Marzia Ariano
Children 2023, 10(2), 360; https://doi.org/10.3390/children10020360 - 11 Feb 2023
Viewed by 1701
Abstract
We present the case of a patient treated as affected by conductive hearing loss due to recurrent otitis, then as a juvenile otosclerosis, who was finally diagnosed as affected by bilateral ossicular chain fixation and enlarged vestibular aqueduct by means of cone-beam CT. [...] Read more.
We present the case of a patient treated as affected by conductive hearing loss due to recurrent otitis, then as a juvenile otosclerosis, who was finally diagnosed as affected by bilateral ossicular chain fixation and enlarged vestibular aqueduct by means of cone-beam CT. Full article
(This article belongs to the Special Issue Congenital Hearing Loss in Children)
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