Diagnosis and Rehabilitation of Hearing Loss

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Medical Research".

Deadline for manuscript submissions: closed (30 November 2023) | Viewed by 2829

Special Issue Editor


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Guest Editor
Russian Medical Academy of Continuing Professional Education, Research Institute of Experimental and Clinical Audiology, Moscow, Russia
Interests: audiology; hearing disorders; auditory evoked potentials; auditory perception; speech intelligibility; deafness; hearing loss; electrophysiology; audiometry

Special Issue Information

Dear Colleagues,

According to WHO statistics, unaddressed hearing loss is the third largest cause of years lived with disability globally. Hearing loss, if not identified and addressed, can have far-reaching consequences, adversely affecting language development, psychosocial well-being, quality of life, educational attainment and economic independence at various stages of life. While the financial burden is enormous, what cannot be quantified is the distress caused by the loss of communication, education and social interaction that accompanies unaddressed hearing loss. Across the course of their lives, people with ear conditions or hearing loss can benefit greatly from effective and available interventions. The past few decades have seen game-changing advances in the field of hearing technology, diagnostics and telemedicine, with innovations that enable ear diseases and hearing loss to be identified at any age and in any setting. Medical and surgical management, hearing aids, cochlear implants and rehabilitative therapy are solutions which can ensure that people with ear diseases or hearing loss access education and communication, and thereby have the opportunity to fulfil their potential.

The Special Issue focuses on modern methods of the early diagnosis and rehabilitation of hearing loss and deafness, including the registration of auditory evoked responses to acoustic and electric stimulation, intracochlear electrocochleography and neuroimaging. A portion of the submitted manuscripts will be devoted to the rehabilitation of hearing loss and deafness with implantable technologies.

Dr. George A. Tavartkiladze
Guest Editor

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Keywords

  • cochlea
  • hearing loss
  • deafness
  • intracochlear electrocochleography
  • cochlear implantation
  • neuroimaging
  • auditory evoked potentials
  • genetics of hearing loss

Published Papers (3 papers)

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Research

15 pages, 2124 KiB  
Article
Early Detection of Hearing Loss among the Elderly
by Sol Ferrán, Raquel Manrique-Huarte, Janaina P. Lima, Carla Rodríguez-Zanetti, Diego Calavia, Constanza Jimena Andrade, David Terrasa, Alicia Huarte and Manuel Manrique
Life 2024, 14(4), 471; https://doi.org/10.3390/life14040471 - 4 Apr 2024
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Abstract
Background: Age-related hearing loss (ARHL) is a complex communication disorder that affects the cochlea and central auditory pathway. The goal of this study is to characterize this type of hearing loss and to identify non-invasive, inexpensive, and quick tests to detect ARHL among [...] Read more.
Background: Age-related hearing loss (ARHL) is a complex communication disorder that affects the cochlea and central auditory pathway. The goal of this study is to characterize this type of hearing loss and to identify non-invasive, inexpensive, and quick tests to detect ARHL among elderly adults, seeking to preserve quality of life and reduce the burden on healthcare systems. Methods: An observational, prospective study is conducted with >55-year-old subjects divided into the following groups: normal range (Group A), detected but not treated (Group B), and detected and treated (Group C). During follow-up, Speech Spatial Qualities (SSQ12), and Hearing Handicap Inventory in the Elderly Screening test (HHIE-S) questionnaires were assessed, along with hearing levels (hearing thresholds at 4 kHz were studied in more depth), and a series of tests and questionnaires to assess balance, cognitive level, level of dependence, and depression. Results: A total of 710 patients were included in this study. The duration of hearing loss (11.8 yr. in Group B and 21.0 yr. in Group C) and average time-to-treatment for Group C (14.1 yr.) are both protracted. Both of the used questionnaires show statistically significant differences among the groups, revealing greater handicaps for Group C. Audiometry performed at 4 kHz shows how hearing loss progresses with age, finding differences between men and women. There is a correlation between time-to-treatment in Group C and the cognitive test DSST (−0.26; p = 0.003). Conclusions: HHIE-S, SSQ12, and 4 kHz audiometry are sensitive and feasible tests to implement in screening programs. Full article
(This article belongs to the Special Issue Diagnosis and Rehabilitation of Hearing Loss)
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16 pages, 4736 KiB  
Article
A Novel COCH p.D544Vfs*3 Variant Associated with DFNA9 Sensorineural Hearing Loss Causes Pathological Multimeric Cochlin Formation
by Yingqiu Peng, Mengya Xiang, Ting Fan, Xiaofang Zhong, Aqiang Dai, Jialing Feng, Pengfei Guan, Jiamin Gong, Jian Li and Yunfeng Wang
Life 2024, 14(1), 33; https://doi.org/10.3390/life14010033 - 25 Dec 2023
Viewed by 908
Abstract
COCH (coagulation factor C homology) is one of the most frequently mutated genes of autosomal dominant non-syndromic hearing loss. Variants in COCH could cause DFNA9, which is characterized by late-onset hearing loss with variable degrees of vestibular dysfunction. In this study, we report [...] Read more.
COCH (coagulation factor C homology) is one of the most frequently mutated genes of autosomal dominant non-syndromic hearing loss. Variants in COCH could cause DFNA9, which is characterized by late-onset hearing loss with variable degrees of vestibular dysfunction. In this study, we report a Chinese family with a novel COCH variant (c.1687delA) causing p.D544Vfs*3 in the cochlin. Comprehensive audiometric tests and vestibular function assessments were taken to acquire the phenotypic profile of the subjects. Next-generation sequencing was conducted and segregation analysis was carried out using Sanger sequencing. The proband presented mild vestibular symptoms and normal functional assessment results in almost every test, while the variant co-segregated with hearing impairment in the pedigree. The variant was located beyond the vWFA2 domain, which was predicted to affect the post-translational cleavage of the cochlin via molecular modeling analysis. Notably, in the overexpressing study, by transient transfecting the HEK 293T cells, we found that the p.D544Vfs*3 variant increased the formation of multimeric cochlin. Our result enriched the spectrum of DFNA9-linked pathological COCH variants and suggested that variants, causative of cochlin multimerization, could be related to DFNA9 with sensorineural hearing loss rather than serious vestibular symptoms. Full article
(This article belongs to the Special Issue Diagnosis and Rehabilitation of Hearing Loss)
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0 pages, 420 KiB  
Article
Comparative Analysis of Intellectual Quotient in Developmental Population with Severe Hearing Loss: Hearing Aids vs. Cochlear Implant Users
by Arianna Di Stadio, Pietro De Luca, Valentina Ippolito, Paola Vedova, Sabina Garofalo, Rosaria Turchetta, Salvatore Ferlito and Antonio della Volpe
Life 2024, 14(1), 12; https://doi.org/10.3390/life14010012 - 20 Dec 2023
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Abstract
The development of language, memory and intellectual functions is linked to normal hearing and correct sounds interpretation. Hearing loss (HL), especially in its severe form, negatively affects the development of these functions. This prospective study aimed at comparing the Intelligent Quotients (IQ) of [...] Read more.
The development of language, memory and intellectual functions is linked to normal hearing and correct sounds interpretation. Hearing loss (HL), especially in its severe form, negatively affects the development of these functions. This prospective study aimed at comparing the Intelligent Quotients (IQ) of children with cochlear implants (CI) with the ones of people wearing hearing aids (HA) after one year of hearing rehabilitation. 21 subjects with severe/profound bilateral hearing loss (deafness) were included in this study. Eleven children with congenital profound HL underwent CI and ten children with moderate to severe HL (congenital and acquired) were rehabilitated by HA. Children’s IQs were assessed at enrolment (T0) and 12 months after hearing aids/CI use plus speech therapy. Statistical analyses were performed to analyze the data within and between groups. Comparison of IQs showed no statistically significant differences between CI and HA none at T0 and T1. The subtests showed lower scores in verbal comprehension and process speed index in patients treated with HA when compared to CI. This study showed that auditory rehabilitation can support the normal development of cognitive function in children between six and eight years of age. The use of the correct hearing aids based on the patient’s hearing thresholds is important to maximize the rehabilitation outcomes. Due to the small sample size, although stratified for age, our results must be considered preliminary and further analyses on larger samples are needed to confirm our data. Full article
(This article belongs to the Special Issue Diagnosis and Rehabilitation of Hearing Loss)
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