Audiol. Res., Volume 11, Issue 3 (September 2021) – 17 articles

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

Background: The development of the global digit-in-noise test requires optimization of each language version on a group of normal-hearing native-speakers. An alternative solution may be an adaptive optimization during ongoing tests in a group of subjects with unknown hearing impairments. The objective of the research was to compare the optimization results between these groups. Methods: Digit triplets consisting of three pseudo-randomly selected digits were presented in speech-shaped noise at various signal-to-noise ratios (SNRs), according to the protocol of the final speech test. Digit-specific and position-specific speech reception thresholds (SRTs) were determined and compared between groups. Results: The study sample consisted of 82 subjects, 26 normal-hearing subjects and 56 patients with diverse hearing disorders. Statistically significant differences in digit-specific SRTs between the control and the investigated group were obtained for three digits in continuous noise (digits 0, 4, 6; p-value of 0.04, 0.03, 0.05) and two in modulated noise (digits 1 and 6; p-value of 0.05 and 0.01). An analysis including only ears with SRTs within the range of the normal hearing control group showed no statistically significant differences between digits. Conclusion: Optimization of speech material can be carried out in a group of subjects with unknown hearing impairments, provided the ears with scores outside normal range are rejected. Full article

Soft tissue conduction is an additional mode of auditory stimulation which can be initiated either by applying an external vibrator to skin sites not overlying skull bone such as the neck (so it is not bone conduction) or by intrinsic body vibrations resulting, for example, from the heartbeat and vocalization. The soft tissue vibrations thereby induced are conducted by the soft tissues to all parts of the body, including the walls of the external auditory canal. In order for soft tissue conduction to elicit hearing, the soft tissue vibrations which are induced must penetrate into the cochlea in order to excite the inner ear hair cells and auditory nerve fibers. This final stage can be achieved either by an osseous bone conduction mechanism, or, more likely, by the occlusion effect: the vibrations of the walls of the occluded canal induce air pressures in the canal which drive the tympanic membrane and middle ear ossicles and activate the inner ear, acting by means of a more air conduction-like mechanism. In fact, when the clinician applies his stethoscope to the body surface of his patient in order to detect heart sounds or pulmonary air flow, he is detecting soft tissue vibrations. Full article

Because cartilage conduction—the transmission of sound via the aural cartilage—has different auditory pathways from well-known air and bone conduction, how the output volume in the external auditory canal is stimulated remains unknown. To develop a simulator approximating the conduction of sound in ear cartilage, the vibrations of the pinna and sound in the external auditory canal were measured using pinna simulators made of silicon rubbers of different hardness (A40, A20, A10, A5, A0) as measured by a durometer. The same procedure, as well as a current calibration method for air conduction devices, was applied to an existing pinna simulator, the Head and Torso Simulator (hardness A5). The levels for vibration acceleration and sound pressure from these pinna simulators show spectral peaks at dominant frequencies (below 1.5 kHz) for the conduction of sound in cartilage. These peaks were likely to move to lower frequencies as hardness decreases. On approaching the hardness of actual aural cartilage (A10 to A20), the simulated levels for vibration acceleration and sound pressure approximated the measurements of human ears. The adjustment of the hardness used in pinna simulators is an important factor in simulating accurately the conduction of sound in cartilage. Full article

Background: There is growing evidence of otoneurological involvement of SARS-CoV-2, such as tinnitus and balance disorders and smell and taste disorders, but HL in COVID-19 patients has still been marginally studied. Investigating the role of SARS-CoV-2 as an aetiological factor of Sudden Sensorineural Hearing Loss (SSNHL) may offer the opportunity to address treatment strategies to maximize clinical recovery and avoid side effects. Methods and results: For this purpose, we will present case studies of five patients who experienced SSNHL during COVID-19. Patients were selected from COVID-19 positive adult subjects with mild clinical presentation, admitted to the outpatient Ear Nose and Throat Department of Cardarelli Hospital due to the onset of SSNHL during the infection. All underwent a complete audio-vestibular investigation before and after SSNHL treatment protocol. Each patient is described with a detailed analysis. Conclusions: SSNHL could be an occasional symptom of COVID-19, even in mild manifestations of the disease. Our experience leads us to underline the value of promptly recognizing and addressing this and other uncommon symptoms, giving patients the opportunity to receive early treatment. Full article

Background/Aim: the aim of this study was to assess the skull vibration-induced nystagmus test (SVINT) results and vestibular residual function after horizontal semicircular canal (HSCC) plugging. Methods: In this retrospective chart review performed in a tertiary referral center, 11 patients who underwent unilateral horizontal semicircular canal plugging (uHSCCP) for disabling Menière’s disease (MD) were included. The skull vibration-induced nystagmus (SVIN) slow-phase velocity (SPV) was compared with the results of the caloric test (CaT), video head impulse test (VHIT), and cervical vestibular-evoked myogenic potentials (cVEMP) performed on the same day. Results: Overall, 10 patients had a strong SVIN beating toward the intact side (Horizontal SVIN-SPV: 8.8°/s ± 5.6°/s), 10 had a significant or severe ipsilateral CaT hypofunction, 10 had an ipsilateral horizontal VHIT gain impairment, and 3 had altered cVEMP on the operated side. Five had sensorineural hearing worsening. SVIN-positive results were correlated with CaT and horizontal VHIT (HVHIT) results (p < 0.05) but not with cVEMP. SVIN-SPV was correlated with CaT hypofunction in % (p < 0.05). Comparison of pre- and postoperative CaT % hypofunction showed a significant worsening (p = 0.028). Conclusion: SVINT results in a human model of horizontal canal plugging are well correlated with vestibular tests exploring horizontal canal function, but not with cVEMP. SVINT always showed a strong lesional nystagmus beating away from the lesion side. SVIN acts as a good marker of HSCC function. This surgical technique showed invasiveness regarding horizontal canal vestibular function. Full article