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Audiology Research
Volume 11
Issue 4
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Audiol. Res., Volume 11, Issue 4 (December 2021) – 19 articles

Cover Story (view full-size image): Misophonia is a disorder characterized by a disproportionate emotional response occurring in association with a decreased tolerance for certain sounds. In this study, we examined misophonic trigger sounds using state-of-the-art psychometric methods, including item factor analysis and item response theory models. This study adds valuable insight at the very core of misophonia: its trigger sounds. View this paper
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12 pages, 1055 KiB  
Article
Executive Functions and Deafness: Results in a Group of Cochlear Implanted Children
by Andrea De Giacomo, Alessandra Murri, Emilia Matera, Francesco Pompamea, Francesco Craig, Francesca Giagnotti, Roberto Bartoli and Nicola Quaranta
Audiol. Res. 2021, 11(4), 706-717; https://doi.org/10.3390/audiolres11040063 - 15 Dec 2021
Cited by 5 | Viewed by 2681
Abstract
Objects: This study aimed to evaluate the Executive Function (EF) domains in a group of profoundly deaf children treated with cochlear implant (CI) in comparison to normal hearing (NH) children. The secondary aim was to evaluate the influence exerted by the age at [...] Read more.
Objects: This study aimed to evaluate the Executive Function (EF) domains in a group of profoundly deaf children treated with cochlear implant (CI) in comparison to normal hearing (NH) children. The secondary aim was to evaluate the influence exerted by the age at cochlear implant activation on EFs. Materials and Methods: 32 children were enrolled into two groups: group A of 17 CI users with a mean age of 8.78 years and group B of 15 NH subjects with a mean age of 7.99 years (SD + 2.3). All subjects were tested using the following tests: the subtests for working memory of the neuropsychological evaluation battery for the developmental age (Batteria di valutazione neuropsicologica per l’età evolutive), inhibition and control of the impulsive response—CAF, and the tower of London test. Results: No children with CIs scored within the normal range in the tests administered for the evaluation of EF domains. The same scores were significantly lower when compared with scores obtained by NH children. Children with younger age at CI activation showed better executive performances in planning, working memory (backward digit span), and cognitive flexibility (categorical fluency). Conclusion: The results of this study highlight that cochlear implantation plays a role in improving hearing and consequently influences the development of EFs in deaf children. Full article
(This article belongs to the Special Issue Advances in Cochlear Implantation)
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15 pages, 2739 KiB  
Article
Intracorporeal Cortical Telemetry as a Step to Automatic Closed-Loop EEG-Based CI Fitting: A Proof of Concept
by Andy J. Beynon, Bart M. Luijten and Emmanuel A. M. Mylanus
Audiol. Res. 2021, 11(4), 691-705; https://doi.org/10.3390/audiolres11040062 - 13 Dec 2021
Viewed by 2540
Abstract
Electrically evoked auditory potentials have been used to predict auditory thresholds in patients with a cochlear implant (CI). However, with exception of electrically evoked compound action potentials (eCAP), conventional extracorporeal EEG recording devices are still needed. Until now, built-in (intracorporeal) back-telemetry options are [...] Read more.
Electrically evoked auditory potentials have been used to predict auditory thresholds in patients with a cochlear implant (CI). However, with exception of electrically evoked compound action potentials (eCAP), conventional extracorporeal EEG recording devices are still needed. Until now, built-in (intracorporeal) back-telemetry options are limited to eCAPs. Intracorporeal recording of auditory responses beyond the cochlea is still lacking. This study describes the feasibility of obtaining longer latency cortical responses by concatenating interleaved short recording time windows used for eCAP recordings. Extracochlear reference electrodes were dedicated to record cortical responses, while intracochlear electrodes were used for stimulation, enabling intracorporeal telemetry (i.e., without an EEG device) to assess higher cortical processing in CI recipients. Simultaneous extra- and intra-corporeal recordings showed that it is feasible to obtain intracorporeal slow vertex potentials with a CI similar to those obtained by conventional extracorporeal EEG recordings. Our data demonstrate a proof of concept of closed-loop intracorporeal auditory cortical response telemetry (ICT) with a cochlear implant device. This research breaks new ground for next generation CI devices to assess higher cortical neural processing based on acute or continuous EEG telemetry to enable individualized automatic and/or adaptive CI fitting with only a CI. Full article
(This article belongs to the Special Issue Advances in Cochlear Implantation)
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18 pages, 2897 KiB  
Article
Verification of a Mobile Psychoacoustic Test System
by Jordana C. Soares, Sangamanatha A. Veeranna, Vijay Parsa, Chris Allan, Winnie Ly, Minh Duong, Paula Folkeard, Sheila Moodie and Prudence Allen
Audiol. Res. 2021, 11(4), 673-690; https://doi.org/10.3390/audiolres11040061 - 13 Dec 2021
Cited by 2 | Viewed by 2741
Abstract
Many hearing difficulties can be explained as a loss of audibility, a problem easily detected and treated using standard audiological procedures. Yet, hearing can be much poorer (or more impaired) than audibility predicts because of deficits in the suprathreshold mechanisms that encode the [...] Read more.
Many hearing difficulties can be explained as a loss of audibility, a problem easily detected and treated using standard audiological procedures. Yet, hearing can be much poorer (or more impaired) than audibility predicts because of deficits in the suprathreshold mechanisms that encode the rapidly changing, spectral, temporal, and binaural aspects of the sound. The ability to evaluate these mechanisms requires well-defined stimuli and strict adherence to rigorous psychometric principles. This project reports on the comparison between a laboratory-based and a mobile system’s results for psychoacoustic assessment in adult listeners with normal hearing. A description of both systems employed is provided. Psychoacoustic tests include frequency discrimination, amplitude modulation detection, binaural encoding, and temporal gap detection. Results reported by the mobile system were not significantly different from those collected with the laboratory-based system for most of the tests and were consistent with those reported in the literature. The mobile system has the potential to be a feasible option for the assessment of suprathreshold auditory encoding abilities. Full article
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20 pages, 497 KiB  
Review
Benefits of Parent Training in the Rehabilitation of Deaf or Hard of Hearing Children of Hearing Parents: A Systematic Review
by Ilaria Giallini, Maria Nicastri, Laura Mariani, Rosaria Turchetta, Giovanni Ruoppolo, Marco de Vincentiis, Corrado De Vito, Antonio Sciurti, Valentina Baccolini and Patrizia Mancini
Audiol. Res. 2021, 11(4), 653-672; https://doi.org/10.3390/audiolres11040060 - 13 Dec 2021
Cited by 9 | Viewed by 3643
Abstract
The present study is a systematic review on the effectiveness of Parent Training (PT) and coaching in deaf and hard of hearing (DHH) rehabilitation programs which reviews and synthesizes the existing body of evidence to assess the benefits of these programs in enhancing [...] Read more.
The present study is a systematic review on the effectiveness of Parent Training (PT) and coaching in deaf and hard of hearing (DHH) rehabilitation programs which reviews and synthesizes the existing body of evidence to assess the benefits of these programs in enhancing parents’ sensitivity, responsivity and promoting language development in DHH children during the first years after HA fitting or CI activation. Five published studies met the Population, Intervention, Comparison and Outcomes (PICO) inclusion criteria and were eligible to be included, but heterogeneity in terms of the study design, interventions and outcomes did not allow for performing a meta-analysis. All included studies shared the view that a parent’s learning is a circular (rather than frontal) process, and the results appear promising in terms of enhancing parents’ responsiveness and promoting DHH child language development. Nevertheless, the available evidence was judged to not be robust enough due to limitations in the studies’ designs. Further high-quality evidence is needed to evaluate the true degree of clinical value and the cost effectiveness of PT programs aimed at increasing parents’ responsiveness to their DHH children. Full article
(This article belongs to the Special Issue Advances in Cochlear Implantation)
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14 pages, 2491 KiB  
Review
Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene
by Rosamaria Santarelli, Pietro Scimemi, Chiara La Morgia, Elona Cama, Ignacio del Castillo and Valerio Carelli
Audiol. Res. 2021, 11(4), 639-652; https://doi.org/10.3390/audiolres11040059 - 26 Nov 2021
Cited by 3 | Viewed by 4377
Abstract
Auditory Neuropathy (AN) is characterized by disruption of temporal coding of acoustic signals in auditory nerve fibers resulting in alterations of auditory perceptions. Mutations in several genes have been associated to the most forms of AN. Underlying mechanisms include both pre-synaptic and post-synaptic [...] Read more.
Auditory Neuropathy (AN) is characterized by disruption of temporal coding of acoustic signals in auditory nerve fibers resulting in alterations of auditory perceptions. Mutations in several genes have been associated to the most forms of AN. Underlying mechanisms include both pre-synaptic and post-synaptic damage involving inner hair cell (IHC) depolarization, neurotransmitter release, spike initiation in auditory nerve terminals, loss of auditory fibers and impaired conduction. In contrast, outer hair cell (OHC) activities (otoacoustic emissions [OAEs] and cochlear microphonic [CM]) are normal. Disordered synchrony of auditory nerve activity has been suggested as the basis of both the alterations of auditory brainstem responses (ABRs) and reduction of speech perception. We will review how electrocochleography (ECochG) recordings provide detailed information to help objectively define the sites of auditory neural dysfunction and their effect on receptor summating potential (SP) and neural compound action potential (CAP), the latter reflecting disorders of ribbon synapses and auditory nerve fibers. Full article
(This article belongs to the Special Issue Genetics of Hearing Loss)
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3 pages, 1154 KiB  
Editorial
Victor A. McKusick, the “Father of Medical Genetics”
by Alessandro Martini and Matteo Cassina
Audiol. Res. 2021, 11(4), 636-638; https://doi.org/10.3390/audiolres11040058 - 25 Nov 2021
Cited by 1 | Viewed by 2404 | Correction
Abstract
The Special Issue “Genetics of hearing loss” is dedicated to Victor A [...] Full article
(This article belongs to the Special Issue Genetics of Hearing Loss)
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7 pages, 234 KiB  
Commentary
Genetics & Epigenetics of Hereditary Deafness: An Historical Overview
by Alessandro Martini, Flavia Sorrentino, Ugo Sorrentino and Matteo Cassina
Audiol. Res. 2021, 11(4), 629-635; https://doi.org/10.3390/audiolres11040057 - 17 Nov 2021
Cited by 3 | Viewed by 3390
Abstract
Hearing loss (HL) is one of the most common sensory impairments worldwide and represents a critical medical and public health issue. Since the mid-1900s, great efforts have been aimed at understanding the etiology of both syndromic and non-syndromic HL and identifying correlations with [...] Read more.
Hearing loss (HL) is one of the most common sensory impairments worldwide and represents a critical medical and public health issue. Since the mid-1900s, great efforts have been aimed at understanding the etiology of both syndromic and non-syndromic HL and identifying correlations with specific audiological phenotypes. The extraordinary discoveries in the field of molecular genetics during the last three decades have contributed substantially to the current knowledge. Next-generation sequencing technologies have dramatically increased the diagnostic rate for genetic HL, enabling the detection of novel variants in known deafness-related genes and the discovery of new genes implicated in hearing disease. Overall, genetic factors account for at least 40% of the cases with HL, but a portion of affected patients still lack a definite molecular diagnosis. Important steps forward have been made, but many aspects still have to be clarified. In particular, the role of epigenetics in the development, function and pathology of hearing is a research field that still needs to be explored. This research is extremely challenging due to the time- and tissue-dependent variability of the epigenetic changes. Multisystem diseases are expected to be investigated at first: specific epi-signatures have been identified for several syndromic disorders and represent potential markers for molecular diagnostics. Full article
(This article belongs to the Special Issue Genetics of Hearing Loss)
11 pages, 1472 KiB  
Article
Skull Vibration Induced Nystagmus Test: Correlations with Semicircular Canal and Otolith Asymmetries
by Christol Fabre, Haoyue Tan, Georges Dumas, Ludovic Giraud, Philippe Perrin and Sébastien Schmerber
Audiol. Res. 2021, 11(4), 618-628; https://doi.org/10.3390/audiolres11040056 - 15 Nov 2021
Cited by 5 | Viewed by 3006
Abstract
Background: To establish in patients with peripheral vestibular disorders relations between skull vibration-induced nystagmus (SVIN) different components (horizontal, vertical, torsional) and the results of different structurally related vestibular tests. Methods: SVIN test, canal vestibular test (CVT: caloric test + video head impulse test: [...] Read more.
Background: To establish in patients with peripheral vestibular disorders relations between skull vibration-induced nystagmus (SVIN) different components (horizontal, vertical, torsional) and the results of different structurally related vestibular tests. Methods: SVIN test, canal vestibular test (CVT: caloric test + video head impulse test: VHIT), otolithic vestibular test (OVT: ocular vestibular evoked myogenic potential oVEMP + cervical vestibular evoked myogenic potential cVEMP) performed on the same day in 52 patients with peripheral vestibular diseases (age < 65 years), and 11 control patients were analyzed. Mixed effects logistic regression analysis was performed to assert whether the presence of nystagmus in SVIN (3D analysis) have an association with the presence of peripheral vestibular dysfunction measured by vestibular explorations (CVT or OVT). Results: We obtained different groups: Group-Co (control group), Group-VNT (dizzy patients with no vestibular tests alterations), Group-O (OVT alterations only), Group-C (CVT alterations only), Group-M (mixed alterations). SVIN-SPV horizontal component was significantly higher in Group-M than in the other groups (p = 0.005) and correlated with alterations of lateral-VHIT (p < 0.001), caloric test (p = 0.002) and oVEMP (p = 0.006). SVIN-SPV vertical component was correlated with the anterior-VHIT and oVEMP alterations (p = 0.007; p = 0.017, respectively). SVIN-SPV torsional component was correlated with the anterior-VHIT positivity (p = 0.017). SVIN was the only positive test for 10% of patients (83% of Group-VNT). Conclusion: SVIN-SPV analysis in dizzy patients shows significant correlation to both CVT and OVT. SVIN horizontal component is mainly relevant to both vestibular tests exploring lateral canal and utricle responses. SVIN-SPV is significantly higher in patients with combined canal and otolith lesions. In some patients with dizziness, SVIN may be the only positive test. Full article
(This article belongs to the Special Issue Skull Vibration-Induced Nystagmus Test)
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9 pages, 974 KiB  
Case Report
A Rare Case of Perrault Syndrome with Auditory Neuropathy Spectrum Disorder: Cochlear Implantation Treatment and Literature Review
by Francesca Forli, Luca Bruschini, Beatrice Franciosi, Roberta Battini, Gemma Marinella, Stefano Berrettini and Francesco Lazzerini
Audiol. Res. 2021, 11(4), 609-617; https://doi.org/10.3390/audiolres11040055 - 13 Nov 2021
Cited by 5 | Viewed by 3086
Abstract
Perrault syndrome (PRLTS) is a rare autosomal recessive disorder characterised by ovarian failure in females and sensorineural hearing loss (SNHL) in both genders. In the present paper we describe a child affected by PRLTS3, due to CLPP homozygous mutations, presenting auditory neuropathy spectrum [...] Read more.
Perrault syndrome (PRLTS) is a rare autosomal recessive disorder characterised by ovarian failure in females and sensorineural hearing loss (SNHL) in both genders. In the present paper we describe a child affected by PRLTS3, due to CLPP homozygous mutations, presenting auditory neuropathy spectrum disorder (ANSD) with bilateral progressive SNHL. This is the first case reported in the literature of an ANSD in PRLTS3. CLPP is a nuclear encoded mitochondrial protease directed at the mitochondrial matrix. It is encoded on chromosome 19. This protease participates in mitochondrial protein quality control by degrading misfolded or damaged proteins, thus maintaining the normal metabolic function of the cell. In PRLTS3, the peptidase activity of CLPP is suppressed. Neurological impairments involved in PRLTS3 suggest that the pathogenic mutations in CLPP might trigger a mitochondrial dysfunction. A comprehensive description of the clinical and audiological presentation, as well as the issues related to cochlear implant (CI) procedure and the results, are addressed and discussed. A brief review of the literature on this topic is also provided. Full article
(This article belongs to the Special Issue Genetics of Hearing Loss)
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6 pages, 245 KiB  
Article
Skull Vibration-Induced Nystagmus Test (SVINT) in Vestibular Migraine and Menière’s Disease
by Roberto Teggi, Omar Gatti, Marco Familiari, Iacopo Cangiano and Mario Bussi
Audiol. Res. 2021, 11(4), 603-608; https://doi.org/10.3390/audiolres11040054 - 8 Nov 2021
Cited by 4 | Viewed by 3179
Abstract
Background: Vestibular migraine (VM) and Menière’s disease (MD) are the two most frequent episodic vertigo apart from Benign Paroxysmal Positional Vertigo (BPPV) differential diagnosis for them may be troublesome in the early stages. SVINT is a newly proposed vestibular test, which demonstrated to [...] Read more.
Background: Vestibular migraine (VM) and Menière’s disease (MD) are the two most frequent episodic vertigo apart from Benign Paroxysmal Positional Vertigo (BPPV) differential diagnosis for them may be troublesome in the early stages. SVINT is a newly proposed vestibular test, which demonstrated to be fast and reliable in diagnoses above all of peripheral vestibular deficits. Methods: We retrieved clinical data from two groups of subjects (200 VM and 605 MD), enrolled between 2010 and 2020. Among others, these subjects were included when performing a SVINT. The purpose of the study is to assess if SVINT can be useful to differentiate the two episodic disorders. Results: 59.2% of MD subjects presented as positive with SVINT while only 6% did so with VM; among other tests, only video HIT demonstrated a different frequency in the two groups (13.1% and 0.5%, respectively), but the low sensitivity in these subjects makes the test unaffordable for diagnostic purposes. Conclusions: Since SVINT demonstrated to be positive in a peripheral vestibular deficit in previous works, we think that our data are consistent with the hypothesis that, in the pathophysiology of VM attacks, the central vestibular pathways are mainly involved. Full article
(This article belongs to the Special Issue Skull Vibration-Induced Nystagmus Test)
9 pages, 7856 KiB  
Article
Audiometric Characteristics and Tinnitus Features in a Cohort of 170 Spanish Patients
by María Cuesta and Pedro Cobo
Audiol. Res. 2021, 11(4), 594-602; https://doi.org/10.3390/audiolres11040053 - 3 Nov 2021
Cited by 4 | Viewed by 2823
Abstract
Background: Tinnitus is a rather prevalent, quite heterogeneous, and difficult to treat auditory disorder. The aim of this article is to provide the design and results of a cross-sectional study related to audiological and tinnitus features in a group of 170 Spanish patients. [...] Read more.
Background: Tinnitus is a rather prevalent, quite heterogeneous, and difficult to treat auditory disorder. The aim of this article is to provide the design and results of a cross-sectional study related to audiological and tinnitus features in a group of 170 Spanish patients. Methods: Audiometric characteristics were assessed on the basis of the pure-tone audiometry of both ears in 170 tinnitus patients and 85 control subjects. The audiometric status of each tinnitus participant was assessed on the basis of the average auditory threshold (AAT) in the whole frequency range (from 125 Hz to 8 kHz), and low (from 125 Hz to 2 kHz) and high (from 3 kHz to 8 kHz)-frequency intervals. Tinnitus features were evaluated through personal interview with patients and included tinnitus duration, laterality, pitch, sound, and distress (Tinnitus Handicap Inventory, THI). Correlational analysis was carried out between audiological (AAT) and tinnitus (THI) variables. Results: A very weak Spearman rank correlation factor is found between both variables. Conclusions: The subjective outcome of tinnitus distress (THI) was not correlated with the objective measure of hearing loss (AAT) in our cohort. Full article
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12 pages, 590 KiB  
Case Report
DFNA20/26 and Other ACTG1-Associated Phenotypes: A Case Report and Review of the Literature
by Ugo Sorrentino, Chiara Piccolo, Chiara Rigon, Valeria Brasson, Eva Trevisson, Francesca Boaretto, Alessandro Martini and Matteo Cassina
Audiol. Res. 2021, 11(4), 582-593; https://doi.org/10.3390/audiolres11040052 - 18 Oct 2021
Cited by 6 | Viewed by 3213
Abstract
Since the early 2000s, an ever-increasing subset of missense pathogenic variants in the ACTG1 gene has been associated with an autosomal-dominant, progressive, typically post-lingual non-syndromic hearing loss (NSHL) condition designed as DFNA20/26. ACTG1 gene encodes gamma actin, the predominant actin protein in the [...] Read more.
Since the early 2000s, an ever-increasing subset of missense pathogenic variants in the ACTG1 gene has been associated with an autosomal-dominant, progressive, typically post-lingual non-syndromic hearing loss (NSHL) condition designed as DFNA20/26. ACTG1 gene encodes gamma actin, the predominant actin protein in the cytoskeleton of auditory hair cells; its normal expression and function are essential for the stereocilia maintenance. Different gain-of-function pathogenic variants of ACTG1 have been associated with two major phenotypes: DFNA20/26 and Baraitser–Winter syndrome, a multiple congenital anomaly disorder. Here, we report a novel ACTG1 variant [c.625G>A (p. Val209Met)] in an adult patient with moderate-severe NSHL characterized by a downsloping audiogram. The patient, who had a clinical history of slowly progressive NSHL and tinnitus, was referred to our laboratory for the analysis of a large panel of NSHL-associated genes by next generation sequencing. An extensive review of previously reported ACTG1 variants and their associated phenotypes was also performed. Full article
(This article belongs to the Special Issue Genetics of Hearing Loss)
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15 pages, 1825 KiB  
Article
Item Response Theory Investigation of Misophonia Auditory Triggers
by Silia Vitoratou, Nora Uglik-Marucha, Chloe Hayes, Mercede Erfanian, Oliver Pearson and Jane Gregory
Audiol. Res. 2021, 11(4), 567-581; https://doi.org/10.3390/audiolres11040051 - 14 Oct 2021
Cited by 20 | Viewed by 5629
Abstract
Misophonia is characterised by a low tolerance for day-to-day sounds, causing intense negative affect. This study conducts an in-depth investigation of 35 misophonia triggers. A sample of 613 individuals who identify as experiencing misophonia and 202 individuals from the general population completed self-report [...] Read more.
Misophonia is characterised by a low tolerance for day-to-day sounds, causing intense negative affect. This study conducts an in-depth investigation of 35 misophonia triggers. A sample of 613 individuals who identify as experiencing misophonia and 202 individuals from the general population completed self-report measures. Using contemporary psychometric methods, we studied the triggers in terms of internal consistency, stability in time, precision, severity, discrimination ability, and information. Three dimensions of sensitivity were identified, namely, to eating sounds, to nose/throat sounds, and to general environmental sounds. The most informative and discriminative triggers belonged to the eating sounds. Participants identifying with having misophonia had also significantly increased odds to endorse eating sounds as auditory triggers than others. This study highlights the central role of eating sounds in this phenomenon and finds that different triggers are endorsed by those with more severe sound sensitivities than those with low sensitivity. Full article
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10 pages, 2590 KiB  
Review
The Neural Basis of Skull Vibration Induced Nystagmus (SVIN)
by Ian S. Curthoys
Audiol. Res. 2021, 11(4), 557-566; https://doi.org/10.3390/audiolres11040050 - 14 Oct 2021
Cited by 11 | Viewed by 3322
Abstract
I list a summary of the major clinical observations of SVIN in patients with total unilateral vestibular loss (TUVL) and show how basic results from neurophysiology can explain these clinical observations. The account integrates results from single neuron recordings of identified semicircular canal [...] Read more.
I list a summary of the major clinical observations of SVIN in patients with total unilateral vestibular loss (TUVL) and show how basic results from neurophysiology can explain these clinical observations. The account integrates results from single neuron recordings of identified semicircular canal and otolith afferent neurons in guinea pigs in response to low frequency skull vibration with evidence of the eye movement response in cats to selective semicircular canal stimulation (both individual and combined) and a simple model of nystagmus generation to show how these results explain most of the major characteristics of SVIN. Full article
(This article belongs to the Special Issue Skull Vibration-Induced Nystagmus Test)
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10 pages, 267 KiB  
Review
Hyperacusis in Autism Spectrum Disorders
by Ali A. Danesh, Stephanie Howery, Hashir Aazh, Wafaa Kaf and Adrien A. Eshraghi
Audiol. Res. 2021, 11(4), 547-556; https://doi.org/10.3390/audiolres11040049 - 14 Oct 2021
Cited by 21 | Viewed by 9956
Abstract
Hyperacusis is highly prevalent in the autism spectrum disorder (ASD) population. This auditory hypersensitivity can trigger pragmatically atypical reactions that may impact social and academic domains. Objective: The aim of this report is to describe the relationship between decreased sound tolerance disorders and [...] Read more.
Hyperacusis is highly prevalent in the autism spectrum disorder (ASD) population. This auditory hypersensitivity can trigger pragmatically atypical reactions that may impact social and academic domains. Objective: The aim of this report is to describe the relationship between decreased sound tolerance disorders and the ASD population. Topics covered: The main topics discussed include (1) assessment and prevalence of hyperacusis in ASD; (2) etiology of hyperacusis in ASD; (3) treatment of hyperacusis in ASD. Conclusions: Knowledge of the assessment and treatment of decreased sound tolerance disorders within the ASD population is growing and changing. Full article
10 pages, 1069 KiB  
Article
Audiological Performance of ADHEAR Systems in Simulated Conductive Hearing Loss: A Case Series with a Review of the Existing Literature
by Enrico Muzzi, Valeria Gambacorta, Ruggero Lapenna, Giulia Pizzamiglio, Sara Ghiselli, Igor Caregnato, Raffaella Marchi, Giampietro Ricci and Eva Orzan
Audiol. Res. 2021, 11(4), 537-546; https://doi.org/10.3390/audiolres11040048 - 13 Oct 2021
Cited by 3 | Viewed by 4198
Abstract
A new non-invasive adhesive bone conduction hearing device (ABCD) has been proposed as an alternative solution for reversible bilateral conductive hearing loss in recurrent or long-lasting forms of otitis media with effusion (OME) in children that cannot undergo surgical treatment. Our aim was [...] Read more.
A new non-invasive adhesive bone conduction hearing device (ABCD) has been proposed as an alternative solution for reversible bilateral conductive hearing loss in recurrent or long-lasting forms of otitis media with effusion (OME) in children that cannot undergo surgical treatment. Our aim was to assess the effectiveness of ABCD in children with OME. Twelve normal-hearing Italian-speaking volunteers, in whom a conductive hearing loss was simulated, participated in the study. The free-field average hearing threshold was determined and, to evaluate binaural hearing skills, loudness summation and the squelch effect were assessed. Five conditions were tested: (1) unaided without earplugs, (2) unaided with bilateral earplugs, (3) aided right ear with bilateral earplugs, (4) aided left ear with bilateral earplugs, and (5) bilateral aid with bilateral earplugs. Post-hoc analysis showed a significant statistical difference between plugged, unplugged, and each aided condition. The main results were a better loudness summation and a substantial improvement of the squelch effect in the bilaterally aided. Our results suggest that ABCD is a valid treatment for patients with conductive hearing loss that cannot undergo bone conduction implant surgery. It is also important to consider bilateral aids in order to deal with situations in which binaural hearing is fundamental. Full article
(This article belongs to the Special Issue Bone and Cartilage Conduction)
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13 pages, 2557 KiB  
Review
Genetics of Inner Ear Malformations: A Review
by Davide Brotto, Flavia Sorrentino, Roberta Cenedese, Irene Avato, Roberto Bovo, Patrizia Trevisi and Renzo Manara
Audiol. Res. 2021, 11(4), 524-536; https://doi.org/10.3390/audiolres11040047 - 12 Oct 2021
Cited by 8 | Viewed by 5066
Abstract
Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. [...] Read more.
Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations. Full article
(This article belongs to the Special Issue Genetics of Hearing Loss)
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16 pages, 533 KiB  
Review
Sound Localization and Lateralization by Bilateral Bone Conduction Devices, Middle Ear Implants, and Cartilage Conduction Hearing Aids
by Kimio Shiraishi
Audiol. Res. 2021, 11(4), 508-523; https://doi.org/10.3390/audiolres11040046 - 30 Sep 2021
Cited by 4 | Viewed by 4695
Abstract
Sound localization in daily life is one of the important functions of binaural hearing. Bilateral bone conduction devices (BCDs), middle ear implants, and cartilage conduction hearing aids have been often applied for patients with conductive hearing loss (CHL) or mixed hearing loss, for [...] Read more.
Sound localization in daily life is one of the important functions of binaural hearing. Bilateral bone conduction devices (BCDs), middle ear implants, and cartilage conduction hearing aids have been often applied for patients with conductive hearing loss (CHL) or mixed hearing loss, for example, resulting from bilateral microtia and aural atresia. In this review, factors affecting the accuracy of sound localization with bilateral BCDs, middle ear implants, and cartilage conduction hearing aids were classified into four categories: (1) types of device, (2) experimental conditions, (3) participants, and (4) pathways from the stimulus sound to both cochleae. Recent studies within the past 10 years on sound localization and lateralization by BCDs, middle ear implants, and cartilage conduction hearing aids were discussed. Most studies showed benefits for sound localization or lateralization with bilateral devices. However, the judgment accuracy was generally lower than that for normal hearing, and the localization errors tended to be larger than for normal hearing. Moreover, it should be noted that the degree of accuracy in sound localization by bilateral BCDs varied considerably among patients. Further research on sound localization is necessary to analyze the complicated mechanism of bone conduction, including suprathreshold air conduction with bilateral devices. Full article
(This article belongs to the Special Issue Bone and Cartilage Conduction)
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Review
The Enduring Controversy of Cervicogenic Vertigo, and Its Place among Positional Vertigo Syndromes
by Marcello Cherchi, Frank E. DiLiberto, Darío A. Yacovino and Sunit Das
Audiol. Res. 2021, 11(4), 491-507; https://doi.org/10.3390/audiolres11040045 - 26 Sep 2021
Cited by 3 | Viewed by 7123
Abstract
The idea of cervicogenic vertigo (CV) was proposed nearly a century ago, yet despite considerable scrutiny and research, little progress has been made in clarifying the underlying mechanism of the disease, developing a confirmatory diagnostic test, or devising an appropriately targeted treatment. Given [...] Read more.
The idea of cervicogenic vertigo (CV) was proposed nearly a century ago, yet despite considerable scrutiny and research, little progress has been made in clarifying the underlying mechanism of the disease, developing a confirmatory diagnostic test, or devising an appropriately targeted treatment. Given the history of this idea, we offer a review geared towards understanding why so many attempts at clarifying it have failed, with specific comments regarding how CV fits into the broader landscape of positional vertigo syndromes, what a successful diagnostic test might require, and some practical advice on how to approach this in the absence of a diagnostic test. Full article
(This article belongs to the Special Issue Advances in Positional Vertigo)
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