Audiology Research

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

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

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

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

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

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

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

There is an increasing need for state-of-the-art Central Auditory Processing assessment for Portuguese native speakers, applicable as early as possible. As a contribution to answering this need, this paper presents a new battery for Central Auditory Processing assessment for European Portuguese applicable to children aged 5 and above, named BAPA-PE, providing information regarding test selection and development. The battery consists of six behavioral tests: Staggered Spondaic Words (SSW) for European Portuguese, Filtered Speech, Speech in Noise, Detection Interval in Noise, Duration, and Frequency Pattern. The normative data for children aged 5 to 12 are also reported. A sample was obtained of 217 subjects without ear pathology and with typical development. Each age group was composed of at least 30 children. All children were evaluated using pure tone audiometry, speech audiometry, impedance, and otoacoustic emissions. Normative scores are reported for each of the six auditory processing tests. The assessment is applicable to young children (aged 5 and 6). The statistical analyses showed significant effects in scores of Age for all tests and of Ear for several tests. The main result from the work presented, the Auditory Processing Assessment Battery—European Portuguese (BAPA-PE), is available for clinical use with normative data. This battery is a new tool for behaviorism assessment of European Portuguese speakers with suspected central auditory pathology and for monitoring the results of auditory training. Full article

Hearing loss is one of the most common congenital sensory disorders. It can be associated with several comorbidities, in particular developmental disabilities (DD). In Emilia-Romagna (ER), a region in Northern Italy, Child and Adolescent Mental Health Services (CAMHS) provide the diagnostic framework and treatment for these conditions. The aim of the present study is to evaluate the prevalence of hearing loss, both isolated or in association with comorbidities, in the juvenile population. The study draws its data from the ER Childhood and Adolescent Neuropsychiatry Information System (SINPIAER), an Administrative Healthcare Database collecting the clinical data of all those who have attended CAMHS since 2010. The most frequent type of hearing loss was bilateral sensorineural hearing loss, which was present in 69–72% of the cases, while bilateral conductive hearing loss was the second most common type, ranging from 8 to 10%. Among DD, congenital malformations, mental retardation, visual impairment, and cerebral palsy were the most common. In particular, autism spectrum disorders show increasing incidence and prevalence among CAMHS users in ER region. In-depth knowledge of hearing loss epidemiology and related conditions, such as developmental disabilities, in the juvenile population is crucial for disease prevention, health planning, and resource allocation. Full article

Compare the sensitivity and specificity of cVEMP (500 Hz), oVEMP (500 Hz and 4 kHz) in the identification of SSCD. A secondary objective was to identify the influence of dehiscence size and location on cVEMP and oVEMP responses. Methods: Individuals with unilateral (n = 16) and bilateral (n = 10) scan confirmed SSCD were assessed using air-conducted cVEMP and oVEMP Results: For cVEMP, an amplitude cutoff of 286.9 μV or a threshold cutoff of 67.5 dBnHL revealed, respectively, a sensitivity of 75% and 70.6% and a specificity of 69.4% and 100%. For oVEMP (500 Hz), an amplitude cutoff of 10.8 μV or a threshold cutoff of 77.5 dBnHL revealed a sensitivity of 83.33% and a specificity of 87.5% and 80%, respectively. oVEMP (4 kHz), an amplitude cutoff of 3.1 μV, revealed a high specificity of 100% but a low sensitivity of 47.2%. A positive correlation was noted between the length of the SSCD and the cVEMP and oVEMP (500 Hz) thresholds and cVEMP amplitude. Conclusions: Our results support the use of oVEMP in the identification of SSCD. The presence of oVEMP (500 Hz) with an amplitude higher or equal to 10.8 μV, a threshold lower or equal to 77.5 dBnHL or oVEMP (4 kHz) amplitude of 3.1 μV represents the most useful to identify SSCD. Full article

Hearing loss (HL) affects 1–3 newborns per 1000 and, in industrialized countries, recognizes a genetic etiology in more than 80% of the congenital cases. Excluding GJB2 and GJB6, OTOA is one of the leading genes associated with autosomal recessive non-syndromic HL. Allelic heterogeneity linked to OTOA also includes genomic rearrangements facilitated by non-allelic homologous recombination with the neighboring OTOAP1 pseudogene. We present a couple of Italian siblings affected by moderate to severe sensorineural hearing loss (SNHL) due to compound heterozygosity at the OTOA locus. Multigene panel next-generation sequencing identified the c.2223G>A, p.(Trp741*) variant transmitted from the unaffected mother. Assuming the existence of a second paternal deleterious variant which evaded detection at sequencing, genomic array analysis found a ~150 Kb microdeletion of paternal origin and spanning part of OTOA. Both deleterious alleles were identified for the first time. This study demonstrates the utility of an integrated approach to solve complex cases and allow appropriate management to affected individuals and at-risk relatives. Full article

Hearing loss is the most common sensorial deficit in humans and one of the most common birth defects. In developed countries, at least 60% of cases of hearing loss are of genetic origin and may arise from pathogenic sequence alterations in one of more than 300 genes known to be involved in the hearing function. Hearing loss of genetic origin is frequently associated with inner ear malformations; of these, the most commonly detected is the enlarged vestibular aqueduct (EVA). EVA may be associated to other cochleovestibular malformations, such as cochlear incomplete partitions, and can be found in syndromic as well as non-syndromic forms of hearing loss. Genes that have been linked to non-syndromic EVA are SLC26A4, GJB2, FOXI1, KCNJ10, and POU3F4. SLC26A4 and FOXI1 are also involved in determining syndromic forms of hearing loss with EVA, which are Pendred syndrome and distal renal tubular acidosis with deafness, respectively. In Caucasian cohorts, approximately 50% of cases of non-syndromic EVA are linked to SLC26A4 and a large fraction of patients remain undiagnosed, thus providing a strong imperative to further explore the etiology of this condition. Full article

Objective: The purpose of this article is to describe BPPV in COVID-19 patients by discussing the possible mechanisms underlying the onset of this vertigo. Methods: We studied eight patients (4 F, 4 M, aged between 44 and 69 years) with COVID-19 infections complaining of vertigo. Patients were evaluated at the end of infection with an accurate clinical history, and the investigation of spontaneous, positional and positioning nystagmus. Results: The vestibular findings showed benign paroxysmal positional vertigo (BPPV) in all the patients. Three patients had a mild phenotype of the COVID infection, whereas five subjects were hospitalized for the COVID infection and in three cases intensive care was required. Vestibular evaluation showed an involvement of posterior semicircular canals in five patients and horizontal in three. Three patients were treated with the Epley maneuver, two with Semont, one with Lempert and two with Gufoni maneuvers. Conclusions: We hypothesize that BPPV in COVID-19 infections can be relate to drugs, prolonged bed rest and to direct damage by viral infection on the peripheral vestibular system and in particular on the otolitic membrane due to the cytopathic effect of the virus and to the inflammatory response. Studies on large series of patients are needed to confirm our preliminary observation and to better evaluate the pathophysiological mechanisms underlying BPPV in these patients. Full article

Hearing improvement represents one of the may valuable outcomes in microtia and aural atresia reconstruction surgery. Most patients with poor development in their hearing function have had a severe microtia. Conventional methods to improve hearing function are bone conduction and bone anchored hearing aids. Cartilage conduction hearing aids (CCHA) represents a new amplification method. This study assessed the outcomes and evaluated the impact and its safety in the patients with microtia and aural atresia whose hearing dysfunction did not improve after surgery for ear reconstruction in our hospital. Hearing functions were evaluated with pure tone audiometry or sound field testing by behavioral audiometry and speech audiometry before and after CCHA fitting. As a result, there was a significant difference between unaided and aided thresholds (p < 0.001). Speech recognition threshold and speech discrimination level also significantly improved with CCHA. The average functional gains of 14 ears were 26.9 ± 2.3 dB. Almost all parents of the patients reported satisfaction with the performance of CCHA, and daily communication in children with hearing loss also became better than usual. Full article

Acoustic coupling between microphone and loudspeaker is a significant problem in open-fit digital hearing aids. An open-fit compared to a close-fit hearing aid significantly lowers the signal quality and limits the achievable maximum stable gain. Adaptive feedback cancellation (AFC) enables an efficient approach to reduce the impact of acoustic coupling. However, without careful consideration, it can also introduce bias in estimating the feedback path due to the high correlation between the loudspeaker signal and the incoming signal, especially when the incoming signal is spectrally coloured, e.g., speech and music. The prediction error method (PEM) is well known for reducing this bias. The presented study aims to propose a switched PEM with soft-clipping (swPEMSC) that allows for further improvement in convergence/tracking rates, resulting in a better ability to recover from unstable/howling status. This swPEMSC employs a new update rule inspired by a soft-clipping based stability detector (SCSD). It allows to pick up either the PEMSC-NLMS or PEMSC-APA depending on the magnitude of the effective feedback signal; howling corresponds to a large feedback signal. The PEMSC-NLMS with a small step-size ensures a low steady-state error, but slow convergence/tracking rates, while PEMSC-APA with a large step-size allows for fast convergence/tracking rates, but a high steady-state error. By combining those approaches, the proposed approach can take advantage of good characteristics from both. Experimental results using different types of incoming signals and an abrupt change of feedback paths show that the swPEMSC can shorten unstable periods (howling) by improving the convergence and tracking rates while retaining a low steady-state error and good signal quality. Full article

Modulation of microphonics has recently been used to investigate the sensitivity of the utricle in the vestibular organ of the guinea pig. The same technique was used more than 30 years ago to obtain information on the processing of rotational stimuli in the horizontal semicircular canals of the pigeon. Data from that time were reanalysed to give a relation that describes the mechano-electrical transduction (MET) process in vestibular hair cells. Full article

Background: The purpose of this study was to investigate the effectiveness of aural-oral habilitation (AO) over the traditional speech-language therapy, based on the number of vocalization-volubility of a deaf child with late-mapping bilateral cochlear implants using sequential measurements. Methods: The spontaneous productions during child interactions were analyzed. The child (CY, 7;0 years old) with a mean unaided pure-tone average (PTA) hearing loss >80 dB HL was assessed by using an assessment battery. Study design consisted of two phases: (a) baseline (end of speech therapy) and (b) end of AO treatment. Protophones were analyzed via acoustical analysis using PRAAT software. Results: One-way repeated-measure ANOVAs were conducted within and between phases. The analyses revealed significant differences between the ‘phase’ and the vocalization outcome (F = 9.4, df = 1, p = 0.035). Post hoc analyses revealed the significant difference between the mean number of disyllable vocalizations of AO approach (p = 0.05). The mean number of vocalizations was calculated for each protophone type, but no other significant difference was measured. Conclusions: AO approach proved effective as measured through volubility. The outcome of this study is indicative and is a starting point for broader research. Full article

The effect of triazino-indole derivative (Trisan) on hypoxia-inducible factor (HIF) expression level in the organ of Corti, when administering it for therapeutic and preventive purposes, was investigated using an acoustic trauma model in experimental animals (female F1 hybrids of CBA and C57BL/6 lines). Cytoflavin was used as a comparator product. Study product Trisan (1% solution) was injected intravenously, intramuscularly and intraperitoneally, in the dose of 5, 7 and 10 mg/kg 2 h after the acoustic trauma for therapeutic purposes and in the dose of 5, 7 and 10 mg/kg for 3 days before the acoustic trauma for preventive purposes. IHC methods were used to investigate the organ of Corti. Trisan was observed to increase HIF expression in hair cells and neurons of the spiral ganglion in case of acoustic trauma. Depending on the dose, the increased HIF-1 expression in hair cells and spiral ganglion occurred both after therapeutic and preventive use of Trisan. Maximum HIF expression in hair cells and ganglion was noted at the therapeutic and preventive drug dose of 10 mg/kg. Following experimental results, we conclude that the otoprotective effect of triazino-indole derivative is realized via its effect on HIF metabolism, which makes it a target molecule for the drug. Full article

Ultrasound can deliver speech information when it is amplitude-modulated with speech and presented via bone conduction. This speech-modulated bone-conducted ultrasound (SM-BCU) can also transmit prosodic information. However, there is insufficient research on the recognition of vowel duration in SM-BCU. The aim of this study was to investigate the categorization of vowel durational changes in SM-BCU using a behavioral test. Eight Japanese-speaking participants with normal hearing participated in a forced-choice behavioral task to discriminate between “hato” (pigeon) and “haato” (heart). Speech signal stimuli were presented in seven duration grades from 220 ms to 340 ms. The threshold at which 50% of responses were “haato” was calculated and compared for air-conducted audible sound (ACAS) and SM-BCU. The boundary width was also evaluated. Although the SM-BCU threshold (mean: 274.6 ms) was significantly longer than the ACAS threshold (mean: 269.6 ms), there were no differences in boundary width. These results suggest that SM-BCU can deliver prosodic information about vowel duration with a similar difference limen to that of ACAS in normal hearing. Full article