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Congenital Hearing Loss in Children

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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

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Keywords

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

Published Papers (3 papers)

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11 pages, 1273 KiB

Open AccessArticle

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 - 03 Feb 2024

Viewed by 794

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 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

Open AccessArticle

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 1190

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 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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8 pages, 1427 KiB

Open AccessCase 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

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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.

(This article belongs to the Special Issue Congenital Hearing Loss in Children)

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