Audiology Research

15 pages, 1524 KiB

Open AccessFeature PaperEditor’s ChoiceReview

Gene Therapy for Inherited Hearing Loss: Updates and Remaining Challenges

by Roni Hahn and Karen B. Avraham

Audiol. Res. 2023, 13(6), 952-966; https://doi.org/10.3390/audiolres13060083 - 4 Dec 2023

Cited by 7 | Viewed by 3732

Hearing loss stands as the most prevalent sensory deficit among humans, posing a significant global health challenge. Projections indicate that by 2050, approximately 10% of the world’s population will grapple with disabling hearing impairment. While approximately half of congenital hearing loss cases have [...] Read more.

Hearing loss stands as the most prevalent sensory deficit among humans, posing a significant global health challenge. Projections indicate that by 2050, approximately 10% of the world’s population will grapple with disabling hearing impairment. While approximately half of congenital hearing loss cases have a genetic etiology, traditional interventions such as hearing aids and cochlear implants do not completely restore normal hearing. The absence of biological treatment has prompted significant efforts in recent years, with a strong focus on gene therapy to address hereditary hearing loss. Although several studies have exhibited promising recovery from common forms of genetic deafness in mouse models, existing challenges must be overcome to make gene therapy applicable in the near future. Herein, we summarize the primary gene therapy strategies employed over past years, provide an overview of the recent achievements in preclinical studies for genetic hearing loss, and outline the current key obstacles to cochlear gene therapy. Full article

(This article belongs to the Special Issue Genetics of Hearing Loss—Volume II)

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16 pages, 501 KiB

Open AccessEditor’s ChoiceReview

Bedside Testing in Acute Vestibular Syndrome—Evaluating HINTS Plus and Beyond—A Critical Review

by Alexander A. Tarnutzer and Jonathan A. Edlow

Audiol. Res. 2023, 13(5), 670-685; https://doi.org/10.3390/audiolres13050059 - 1 Sep 2023

Cited by 8 | Viewed by 2348

Abstract

Acute vertigo and dizziness are frequent presenting symptoms in patients in the emergency department. These symptoms, which can be subtle and transient, present diagnostic challenges because they can be caused by a broad range of conditions that cut across many specialties and organ [...] Read more.

Acute vertigo and dizziness are frequent presenting symptoms in patients in the emergency department. These symptoms, which can be subtle and transient, present diagnostic challenges because they can be caused by a broad range of conditions that cut across many specialties and organ systems. Previous work has emphasized the value of combining structured history taking and a targeted examination focusing on subtle oculomotor signs. In this review, we discuss various diagnostic bedside algorithms proposed for the acutely dizzy patient. We analyzed these different approaches by calculating their area-under-the-curve (ROC) characteristics and sensitivity/specificity. We found that the algorithms that incorporated structured history taking and the use of subtle oculomotor signs had the highest diagnostic accuracy. In fact, both the HINTS+ bedside exam and the STANDING algorithm can more accurately diagnose acute strokes than early (<24 to 48 h after symptom onset) MRI with diffusion-weighted imaging (DWI). An important caveat is that HINTS and STANDING require moderate training to achieve this accuracy. Therefore, for physicians who have not undergone adequate training, other approaches are needed. These other approaches (e.g., ABCD2 score, PCI score, and TriAGe+ score) rely on vascular risk factors, clinical symptoms, and focal neurologic findings. While these other scores are easier for frontline providers to use, their diagnostic accuracy is far lower than HINTS+ or STANDING. Therefore, a focus on providing dedicated training in HINTS+ or STANDING techniques to frontline clinicians will be key to improving diagnostic accuracy and avoiding unnecessary brain imaging. Full article

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11 pages, 2267 KiB

Open AccessEditor’s ChoiceArticle

In Vivo Measurement of Ear Ossicle and Bony Wall Vibration by Sound Stimulation of Cartilage Conduction

by Hiroaki Yazama, Shiro Arii, Hideyuki Kataoka, Tasuku Watanabe, Ryo Kamitani and Kazunori Fujiwara

Audiol. Res. 2023, 13(4), 495-505; https://doi.org/10.3390/audiolres13040044 - 12 Jul 2023

Cited by 2 | Viewed by 1330

Abstract

The cartilage-conduction pathway was recently proposed as a third auditory pathway; however, middle-ear vibrations have not yet been investigated in vivo. We aimed to measure the ossicles and bone vibration upon cartilage-conduction stimulation with a non-contact laser Doppler vibrometer. We recruited adult patients [...] Read more.

The cartilage-conduction pathway was recently proposed as a third auditory pathway; however, middle-ear vibrations have not yet been investigated in vivo. We aimed to measure the ossicles and bone vibration upon cartilage-conduction stimulation with a non-contact laser Doppler vibrometer. We recruited adult patients with normal ear structures who underwent cochlear implant surgery at our hospital between April 2020 and December 2022. For sound input, a cartilage-conduction transducer, custom-made by RION Corporation (Tokyo, Japan), was fixed to the surface of the tragus and connected to an audiometer to regulate the output. A posterior tympanotomy was performed and a laser beam was directed through the cavity to measure the vibration of the ossicles, cochlear promontory, and posterior wall of the external auditory canal. Five participants (three men, mean age: 56.4 years) were included. The mean hearing loss on the operative side was 96.3 dB HL in one patient, and that of the other patients was off-scale. The vibrations were measured at a sound input of 1 kHz and 60 dB. We observed vibrations of all three structures, demonstrating the existence of cartilage-conduction pathways in vivo. These results may help uncover the mechanisms of the cartilage-conduction pathway in the future. Full article

(This article belongs to the Special Issue Bone and Cartilage Conduction—Volume II)

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13 pages, 1501 KiB

Open AccessFeature PaperEditor’s ChoiceReview

A Review of Neural Data and Modelling to Explain How a Semicircular Canal Dehiscence (SCD) Causes Enhanced VEMPs, Skull Vibration Induced Nystagmus (SVIN), and the Tullio Phenomenon

by Ian S. Curthoys, Christopher M. Smith, Ann M. Burgess and Julia Dlugaiczyk

Audiol. Res. 2023, 13(3), 418-430; https://doi.org/10.3390/audiolres13030037 - 2 Jun 2023

Cited by 4 | Viewed by 2405

Abstract

Angular acceleration stimulation of a semicircular canal causes an increased firing rate in primary canal afferent neurons that result in nystagmus in healthy adult animals. However, increased firing rate in canal afferent neurons can also be caused by sound or vibration in patients [...] Read more.

Angular acceleration stimulation of a semicircular canal causes an increased firing rate in primary canal afferent neurons that result in nystagmus in healthy adult animals. However, increased firing rate in canal afferent neurons can also be caused by sound or vibration in patients after a semicircular canal dehiscence, and so these unusual stimuli will also cause nystagmus. The recent data and model by Iversen and Rabbitt show that sound or vibration may increase firing rate either by neural activation locked to the individual cycles of the stimulus or by slow changes in firing rate due to fluid pumping (“acoustic streaming”), which causes cupula deflection. Both mechanisms will act to increase the primary afferent firing rate and so trigger nystagmus. The primary afferent data in guinea pigs indicate that in some situations, these two mechanisms may oppose each other. This review has shown how these three clinical phenomena—skull vibration-induced nystagmus, enhanced vestibular evoked myogenic potentials, and the Tullio phenomenon—have a common tie: they are caused by the new response of semicircular canal afferent neurons to sound and vibration after a semicircular canal dehiscence. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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18 pages, 1847 KiB

Open AccessEditor’s ChoiceArticle

Neuropsychological Functions and Audiological Findings in Elderly Cochlear Implant Users: The Role of Attention in Postoperative Performance

by Ilaria Giallini, Bianca Maria Serena Inguscio, Maria Nicastri, Ginevra Portanova, Andrea Ciofalo, Annalisa Pace, Antonio Greco, Hilal Dincer D’Alessandro and Patrizia Mancini

Audiol. Res. 2023, 13(2), 236-253; https://doi.org/10.3390/audiolres13020022 - 27 Mar 2023

Cited by 4 | Viewed by 2311

Abstract

Objectives: The present study aimed to investigate in a group of elderly CI users working memory and attention, conventionally considered as predictors of better CI performance and to try to disentangle the effects of these cognitive domains on speech perception, finding potential markers [...] Read more.

Objectives: The present study aimed to investigate in a group of elderly CI users working memory and attention, conventionally considered as predictors of better CI performance and to try to disentangle the effects of these cognitive domains on speech perception, finding potential markers of cognitive decline related to audiometric findings. Methods Thirty postlingually deafened CI users aged >60 underwent an audiological evaluation followed by a cognitive assessment of attention and verbal working memory. A correlation analysis was performed to evaluate the associations between cognitive variables while a simple regression investigated the relationships between cognitive and audiological variables. Comparative analysis was performed to compare variables on the basis of subjects’ attention performance. Results: Attention was found to play a significant role in sound field and speech perception. Univariate analysis found a significant difference between poor and high attention performers, while regression analysis showed that attention significantly predicted recognition of words presented at Signal/Noise +10. Further, the high attention performers showed significantly higher scores than low attentional performers for all working memory tasks. Conclusion: Overall findings confirmed that a better cognitive performance may positively contribute to better speech perception outcomes, especially in complex listening situations. WM may play a crucial role in storage and processing of auditory-verbal stimuli and a robust attention may lead to better performance for speech perception in noise. Implementation of cognitive training in auditory rehabilitation of CI users should be investigated in order to improve cognitive and audiological performance in elderly CI users. Full article

(This article belongs to the Special Issue Cognitive Decline within the Audiology Scope of Practice)

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16 pages, 1973 KiB

Open AccessEditor’s ChoiceReview

Cochlear Implantation in Intralabyrinthine Schwannoma: Case Series and Systematic Review of the Literature

by Sebastiano Franchella, Marzia Ariano, Francesca Bevilacqua, Stefano Concheri and Elisabetta Zanoletti

Audiol. Res. 2023, 13(2), 169-184; https://doi.org/10.3390/audiolres13020017 - 28 Feb 2023

Cited by 7 | Viewed by 2073

Abstract

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with [...] Read more.

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature. Full article

(This article belongs to the Special Issue Hearing and Balance in Acoustic Neuroma)

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11 pages, 2251 KiB

Open AccessEditor’s ChoiceArticle

Do Patients Aged 85 Years and above Benefit from Their Cochlear Implants?

by Karin Hallin, Ulrika Larsson and Nadine Schart-Morén

Audiol. Res. 2023, 13(1), 96-106; https://doi.org/10.3390/audiolres13010010 - 19 Jan 2023

Viewed by 4318

Abstract

The present study aims to investigate the usage and benefits of cochlear implants (CIs) in elderly patients aged ≥85 years, including their device-handling issues, follow-ups, and the influence on their well-being. The patients answered one questionnaire regarding quality of life, EQ5D-3L, and one [...] Read more.

The present study aims to investigate the usage and benefits of cochlear implants (CIs) in elderly patients aged ≥85 years, including their device-handling issues, follow-ups, and the influence on their well-being. The patients answered one questionnaire regarding quality of life, EQ5D-3L, and one questionnaire, obtained from the Swedish CI quality register, regarding usage, handling, satisfaction, remaining difficulties, etc. The medical records were searched for the implantation date, implant model, speech processor model, monosyllabic (MS) word scores, infections over the implant, and compliance regarding scheduled visits to the clinic. The results show that most elderly patients are satisfied full-time users of their implants. Even though most patients had no problems handling their CI, handling issues must be considered. Recurring guidance and training on device operation are needed. We suggest that follow-up visits are essentially needed for this group of patients on a regular basis. CI surgery is considered a safe treatment, even for the elderly. Upgrads to new external equipment (e.g., sound processors) should not be excluded because of their age. The results suggested that the CI positively affected their well-being. This study was approved by the Swedish Ethical Review Authority (5/10-2021, Dnr: 2021-04970). Full article

(This article belongs to the Special Issue Rehabilitation of Hearing Impairment)

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13 pages, 619 KiB

Open AccessEditor’s ChoiceReview

Intra-Operative Cochlear Nerve Function Monitoring in Hearing Preservation Surgery: A Systematic Review of the Literature

by Marzia Ariano, Sebastiano Franchella, Giulia Tealdo and Elisabetta Zanoletti

Audiol. Res. 2022, 12(6), 696-708; https://doi.org/10.3390/audiolres12060066 - 15 Dec 2022

Cited by 4 | Viewed by 1880

Abstract

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory [...] Read more.

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory brainstem responses), DENM (direct eighth cranial nerve monitoring), EcochG (electrocochleography), CNAP (cochlear compound nerve action potentials), DPOAE (distortion product otoacoustic emissions), PAMRs (postauricular muscle responses). The Cochrane library, Scopus, DynaMed, and PubMed databases were screened to obtain any relevant papers from October 2009 to the present day. Due to the heterogeneity of the existing studies in the literature, there is no way to tell whether a technique is better than another. All authors reported satisfactory outcomes with the cochlear nerve monitoring techniques tested, either alone or in combination. Full article

(This article belongs to the Special Issue Hearing and Balance in Acoustic Neuroma)

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9 pages, 248 KiB

Open AccessEditor’s ChoiceArticle

Postoperative Impact of Pontocerebellar Angle Surgery on the Quality of Life in Patients with Vestibular Schwannoma

by Valentina Foscolo, Luigi de Gennaro, Alessandra Murri, Luca Speranzon, Francesco Signorelli, Nicola Quaranta and Raffaella Messina

Audiol. Res. 2022, 12(6), 635-643; https://doi.org/10.3390/audiolres12060061 - 17 Nov 2022

Cited by 6 | Viewed by 2134

Abstract

Background: Vestibular Schwannomas are benign tumors arising from the VIII CN. Surgical treatment is indicated in case of tumors larger than 2.5 cm in the cerebellopontine angle or in the case of cranial nerve dysfunction. The aim of the present study was to [...] Read more.

Background: Vestibular Schwannomas are benign tumors arising from the VIII CN. Surgical treatment is indicated in case of tumors larger than 2.5 cm in the cerebellopontine angle or in the case of cranial nerve dysfunction. The aim of the present study was to evaluate the QoL by means of the PANQOL questionnaire in a group of surgically treated patients mainly affected by large and giant VS Methods: All patients underwent preoperative and postoperative otoneurological evaluation and gadolinium enhanced MRI and they completed, independently, the PANQOL questionnaire at last follow up. Results: 70% of patients presented with large Koos III or IV VS Each domain of PANQOL showed a strong correlation with the total PANQOL score. In relation to the postoperative facial nerve function, patients with poorer function showed significantly lower score in the facial dysfunction and pain, patients with postoperative balance problems showed a significantly lower PANQOL score for domains of balance and pain. Conclusions: This study showed that postoperative QoL of patients was acceptable even if there were some domains that were more affected, such as hearing and balance domains; therefore, the lowest scores suggest the need for vestibular rehabilitation programs and strategies that improve postoperative hearing. Full article

(This article belongs to the Special Issue Hearing and Balance in Acoustic Neuroma)

12 pages, 483 KiB

Open AccessEditor’s ChoiceArticle

Prevention of Recurrent Benign Paroxysmal Positional Vertigo: The Role of Combined Supplementation with Vitamin D and Antioxidants

by Giacinto Asprella Libonati, Antonello Leone, Salvatore Martellucci, Andrea Gallo, Roberto Albera, Sergio Lucisano, Maurizio Bavazzano, Giuseppe Chiarella, Pasquale Viola, Francesco Galletti, Francesco Freni, Francesco Ciodaro, Vincenzo Marcelli, Giuseppe Tortoriello, Leonardo Scotto di Santillo, Pasqualina Maria Picciotti, Jacopo Galli, Silvano Vitale, Nicola Quaranta, Giada Cavallaro, Paolo Gamba, Roberto Teggi, Iacopo Cangiano, Mario Faralli, Annalisa Barboni, Aldo Messina and Giusi Graziano Show full author list Hide full author list

Audiol. Res. 2022, 12(4), 445-456; https://doi.org/10.3390/audiolres12040045 - 22 Aug 2022

Cited by 9 | Viewed by 4559

Abstract

Benign paroxysmal positional vertigo (BPPV) usually has a favorable course, although it is possible to observe BPPV with a high recurrence rate. Previous studies suggested that vitamin D deficiency might affect BPPV recurrences, and oxidative stress might play a complementary role in BPPV [...] Read more.

Benign paroxysmal positional vertigo (BPPV) usually has a favorable course, although it is possible to observe BPPV with a high recurrence rate. Previous studies suggested that vitamin D deficiency might affect BPPV recurrences, and oxidative stress might play a complementary role in BPPV pathogenesis. This multicentric trial aimed to evaluate the effectiveness of oral nutritional supplementation with a compound of alpha-lipoic acid, Carnosine, and Zinc (LICA^® (Difass International, Coriano (RN), Italy)), vitamins of group B and vitamin D in preventing BPPV recurrences. A total of 128 patients with high recurrence-BPPV were randomized in three arms: Arm 1 consisted of subjects with “insufficient” or “deficient” vitamin D blood levels, treated with daily oral supplementation of LICA^®, vitamins of group B and vitamin D3 (800 UI), Arm 2 included BPPV subjects with “sufficient” vitamin D who did not receive any nutritional support, and Arm 3 included subjects with a “sufficient” serum concentration of vitamin D who received supplementation with a compound of LICA^® and Curcumin. After six months of follow-up, a significant reduction of BPPV relapses compared to the baseline was found only in Arm 1 (−2.32, 95% CI: 3.41–1.62, p-value < 0.0001). Study results suggested that oral nutritional supplementation with vitamin D3 plus antioxidants can prevent relapses in patients suffering from high recurrence-BPPV. Full article

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10 pages, 314 KiB

Open AccessEditor’s ChoiceReview

Dissociation between Caloric and Video Head Impulse Tests in Dizziness Clinics

by Sofia Waissbluth and Valeria Sepúlveda

Audiol. Res. 2022, 12(4), 423-432; https://doi.org/10.3390/audiolres12040043 - 8 Aug 2022

Cited by 6 | Viewed by 2799

Abstract

Vestibular assessment tests such as the video head impulse test (vHIT) for the horizontal semicircular canal, and caloric test (Cal), both evaluate horizontal canal function. One would assume that the outcomes for these tests should lead to concordant results, yet several studies have [...] Read more.

Vestibular assessment tests such as the video head impulse test (vHIT) for the horizontal semicircular canal, and caloric test (Cal), both evaluate horizontal canal function. One would assume that the outcomes for these tests should lead to concordant results, yet several studies have suggested that dissociation can occur in certain pathological conditions. As this topic remains inconclusive, this review aims to analyze the scientific evidence regarding the patterns of hypofunction observed in vHIT and Cal in different otoneurological diseases. A comprehensive review of the literature regarding dissociation between these tests in common neurotological diseases was carried out. Articles were analyzed when data for vHIT and Cal were described in a way that it was possible to calculate discordance rates; both retrospective and prospective studies were analyzed. In this review, the discordance rates were as follows: 56% in Ménière’s disease, 51.5% in vestibular migraine, 37.2% in vestibular schwannoma, and 20.8% in vestibular neuritis. These results highlight the benefit of using both Cal and vHIT, and that they are complementary tests. Full article

(This article belongs to the Special Issue Current Challenges and Advances in Inner Ear Disorders)

11 pages, 274 KiB

Open AccessEditor’s ChoiceArticle

Control of Disabling Vertigo in Ménière’s Disease Following Cochlear Implantation without Labyrinthectomy

by Andrea Canale, Giulia Dalmasso, Roberto Albera, Sergio Lucisano, George Dumas, Flavio Perottino and Andrea Albera

Audiol. Res. 2022, 12(4), 393-403; https://doi.org/10.3390/audiolres12040040 - 22 Jul 2022

Cited by 2 | Viewed by 2988

Abstract

Background: The placement of a cochlear implant (CI) can restore auditory function in the case of profound cochlear deafness, which may be due to Ménière’s disease (MD) or be associated with symptoms related to endolymphatic hydrops. The usual treatment of disabling vertigo in [...] Read more.

Background: The placement of a cochlear implant (CI) can restore auditory function in the case of profound cochlear deafness, which may be due to Ménière’s disease (MD) or be associated with symptoms related to endolymphatic hydrops. The usual treatment of disabling vertigo in MD is based on vestibular deafferentation by labyrinth ablation. The aim of the present study was to retrospectively evaluate the efficacy of the CI in the control of disabling vestibular manifestations in the case of MD unresponsive to medical treatments. Methods: A case series of five MD patients with disabling vestibular manifestations associated with profound hearing loss was included. A complete audio-vestibular evaluation was performed after CI positioning. Results: All patients reported clinical benefits after implant positioning: no vestibular crisis was reported after the surgery. The vHIT and the caloric test showed a normal function or a mild vestibular hypofunction. The auditory performances were comparable to those in the general implanted population. All patients reported subjective tinnitus reduction. Conclusions: To date, very few studies have reported vestibular outcomes in hydropic pathology on the implanted side; our results are encouraging. We can therefore confirm the efficacy and safety of the CI as a unique treatment for hearing loss, dizziness, and tinnitus in case of disabling cochlear hydrops, especially in those patients where the history of the disease requires preservation of the vestibular function. Full article

(This article belongs to the Special Issue Advances in Cochlear Implantation)

9 pages, 490 KiB

Open AccessEditor’s ChoiceReview

Definition of Tinnitus

by Aldo Messina, Alessandro Corvaia and Chiara Marino

Audiol. Res. 2022, 12(3), 281-289; https://doi.org/10.3390/audiolres12030029 - 23 May 2022

Cited by 8 | Viewed by 3000

Abstract

Tinnitus is generally defined as the perception of sound in the absence of vibration of an external elastic body. If this definition appears useful to differentiate tinnitus from somatosounds, it is not suitable for distinguishing it from psychiatric hallucinations. Nor does this solution [...] Read more.

Tinnitus is generally defined as the perception of sound in the absence of vibration of an external elastic body. If this definition appears useful to differentiate tinnitus from somatosounds, it is not suitable for distinguishing it from psychiatric hallucinations. Nor does this solution define a temporal limit of duration of the perception, which is important for distinguishing pathological tinnitus from those occasional noises that we all perceive from time to time. A complete definition appears necessary not only to achieve homogeneity in epidemiological studies but also to set up correct and personalized therapeutic schemes. An analogy with neuropsychiatric studies and, in particular, the concept of auditory hallucinosis are proposed by the authors to define tinnitus. According to the authors, tinnitus is auditory hallucinosis, and similarly, vertigo is spatial hallucinosis. Full article

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12 pages, 299 KiB

Open AccessEditor’s ChoiceReview

Audiovestibular Disorders after COVID-19 Vaccine: Is There an Association?

by Davide Pisani, Federico Maria Gioacchini, Pasquale Viola, Alfonso Scarpa, Alessia Astorina, Massimo Re, Gianmarco Marcianò, Francesco Manti, Roberta Anzivino and Giuseppe Chiarella

Audiol. Res. 2022, 12(3), 212-223; https://doi.org/10.3390/audiolres12030024 - 21 Apr 2022

Cited by 17 | Viewed by 5355

Abstract

The SARS-CoV-2 vaccination campaign is probably one of the most historic public hygiene measures in modern medicine. The drama of the pandemic has forced the scientific community to accelerate the development and commercialization of vaccines, thereby enhancing the phases of active surveillance. Among [...] Read more.

The SARS-CoV-2 vaccination campaign is probably one of the most historic public hygiene measures in modern medicine. The drama of the pandemic has forced the scientific community to accelerate the development and commercialization of vaccines, thereby enhancing the phases of active surveillance. Among the adverse events following immunization (AEFI) reported, those of an audiovestibular interest, such as sudden sensorineural hearing loss (SSNHL), tinnitus, dizziness, and vertigo, constitute a very small percentage. There are many plausible etiological hypotheses, and scientific research needs to pay more attention to the correct collection of data, which up until now have often been inadequate and fragmented, on which to base future studies. SSNHL, new onset tinnitus, vertigo, and dizziness require a prompt evaluation, while the proposed treatment is the same as it is for events unrelated to vaccination. These are uncommon adverse events, and the risk rates for these diseases have not increased in conjunction with the COVID-19 vaccinations, therefore there is no justification of any hesitation towards the vaccination campaign. Full article

(This article belongs to the Special Issue Audio-Vestibular Disorders in the COVID-19 Pandemics)

10 pages, 798 KiB

Open AccessEditor’s ChoiceReview

Atypical Positional Vertigo: Definition, Causes, and Mechanisms

by Sergio Carmona, Guillermo Javier Zalazar, Martin Fernández, Gabriela Grinstein and João Lemos

Audiol. Res. 2022, 12(2), 152-161; https://doi.org/10.3390/audiolres12020018 - 14 Mar 2022

Cited by 6 | Viewed by 5982

Abstract

Paroxysmal positional vertigo is a frequent cause for consultation. When approaching these patients, we try to differentiate central from peripheral causes, but sometimes we find manifestations that generate diagnostic doubts. In this review, we address atypical paroxysmal positional vertigo, reviewing the literature on [...] Read more.

Paroxysmal positional vertigo is a frequent cause for consultation. When approaching these patients, we try to differentiate central from peripheral causes, but sometimes we find manifestations that generate diagnostic doubts. In this review, we address atypical paroxysmal positional vertigo, reviewing the literature on the subject and giving a provisional definition of atypical positional vertigo as well as outlining its causes and pathophysiological mechanisms. Full article

(This article belongs to the Special Issue Advances in Positional Vertigo)

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24 pages, 1844 KiB

Open AccessFeature PaperEditor’s ChoiceReview

Usher Syndrome

by Alessandro Castiglione and Claes Möller

Audiol. Res. 2022, 12(1), 42-65; https://doi.org/10.3390/audiolres12010005 - 11 Jan 2022

Cited by 36 | Viewed by 12212

Abstract

Usher syndrome (USH) is the most common genetic condition responsible for combined loss of hearing and vision. Balance disorders and bilateral vestibular areflexia are also observed in some cases. The syndrome was first described by Albrecht von Graefe in 1858, but later named [...] Read more.

Usher syndrome (USH) is the most common genetic condition responsible for combined loss of hearing and vision. Balance disorders and bilateral vestibular areflexia are also observed in some cases. The syndrome was first described by Albrecht von Graefe in 1858, but later named by Charles Usher, who presented a large number of cases with hearing loss and retinopathy in 1914. USH has been grouped into three main clinical types: 1, 2, and 3, which are caused by mutations in different genes and are further divided into different subtypes. To date, nine causative genes have been identified and confirmed as responsible for the syndrome when mutated: MYO7A, USH1C, CDH23, PCDH15, and USH1G (SANS) for Usher type 1; USH2A, ADGRV1, and WHRN for Usher type 2; CLRN1 for Usher type 3. USH is inherited in an autosomal recessive pattern. Digenic, bi-allelic, and polygenic forms have also been reported, in addition to dominant or nonsyndromic forms of genetic mutations. This narrative review reports the causative forms, diagnosis, prognosis, epidemiology, rehabilitation, research, and new treatments of USH. Full article

(This article belongs to the Special Issue Genetics of Hearing Loss)

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9 pages, 1373 KiB

Open AccessFeature PaperEditor’s ChoiceArticle

Cochlear Implant in Patients with Intralabyrinthine Schwannoma without Tumor Removal

by Andrea Laborai, Sara Ghiselli and Domenico Cuda

Audiol. Res. 2022, 12(1), 33-41; https://doi.org/10.3390/audiolres12010004 - 10 Jan 2022

Cited by 14 | Viewed by 3923

Abstract

(1) Background: Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells. When they originate from neural elements within the vestibule or cochlea, they are defined as intralabyrinthine schwannomas (ILSs). Cochlear implant (CI) has been reported as a feasible [...] Read more.

(1) Background: Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells. When they originate from neural elements within the vestibule or cochlea, they are defined as intralabyrinthine schwannomas (ILSs). Cochlear implant (CI) has been reported as a feasible solution for hearing restoration in these patients. (2) Methods: Two patients with single-sided deafness (SSD) due to sudden sensorineural hearing loss and ipsilateral tinnitus were the cases. MRI detected an ILS. CI was positioned using a standard round window approach without tumor removal. (3) Results: The hearing threshold was 35 dB in one case and 30 dB in the other 6 mo after activation. Speech audiometry with bisillables in quiet was 21% and 27% at 65 dB, and the tinnitus was completely resolved or reduced. In the localization test, a 25.9° error azimuth was obtained with CI on, compared to 43.2° without CI. The data log reported a daily use of 11 h and 14 h. In order to not decrease the CI’s performance, we decided not to perform tumor exeresis, but only CI surgery to restore functional binaural hearing. (4) Conclusions: These are the sixth and seventh cases in the literature of CI in patients with ILS without any tumor treatment and the first with SSD. Cochlear implant without tumor removal can be a feasible option for restoring binaural hearing without worsening the CI’s performance. Full article

(This article belongs to the Special Issue Advances in Cochlear Implantation)

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12 pages, 650 KiB

Open AccessEditor’s ChoiceReview

Fifty Years of Development of the Skull Vibration-Induced Nystagmus Test

by Solara Sinno, Sébastien Schmerber, Philippe Perrin and Georges Dumas

Audiol. Res. 2022, 12(1), 10-21; https://doi.org/10.3390/audiolres12010002 - 30 Dec 2021

Cited by 2 | Viewed by 3046

Abstract

This review enumerates most of the studies on the Skull Vibration-Induced Nystagmus Test (SVINT) in the past 50 years from different research groups around the world. It is an attempt to demonstrate the evolution of this test and its increased interest around the [...] Read more.

This review enumerates most of the studies on the Skull Vibration-Induced Nystagmus Test (SVINT) in the past 50 years from different research groups around the world. It is an attempt to demonstrate the evolution of this test and its increased interest around the globe. It explores clinical studies and animal studies, both permitting a better understanding of the importance of SVINT and its pathophysiology. Full article

(This article belongs to the Special Issue Skull Vibration-Induced Nystagmus Test)

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9 pages, 268 KiB

Open AccessEditor’s ChoiceConcept Paper

Interdisciplinary Approaches to the Study of Listening Effort in Young Children with Cochlear Implants

by Amanda Saksida, Sara Ghiselli, Stefano Bembich, Alessandro Scorpecci, Sara Giannantonio, Alessandra Resca, Pasquale Marsella and Eva Orzan

Audiol. Res. 2022, 12(1), 1-9; https://doi.org/10.3390/audiolres12010001 - 21 Dec 2021

Cited by 2 | Viewed by 3714

Abstract

Very early bilateral implantation is thought to significantly reduce the attentional effort required to acquire spoken language, and consequently offer a profound improvement in quality of life. Despite the early intervention, however, auditory and communicative outcomes in children with cochlear implants remain poorer [...] Read more.

Very early bilateral implantation is thought to significantly reduce the attentional effort required to acquire spoken language, and consequently offer a profound improvement in quality of life. Despite the early intervention, however, auditory and communicative outcomes in children with cochlear implants remain poorer than in hearing children. The distorted auditory input via the cochlear implants requires more auditory attention resulting in increased listening effort and fatigue. Listening effort and fatigue may critically affect attention to speech, and in turn language processing, which may help to explain the variation in language and communication abilities. However, measuring attention to speech and listening effort is demanding in infants and very young children. Three objective techniques for measuring listening effort are presented in this paper that may address the challenges of testing very young and/or uncooperative children with cochlear implants: pupillometry, electroencephalography, and functional near-infrared spectroscopy. We review the studies of listening effort that used these techniques in paediatric populations with hearing loss, and discuss potential benefits of the systematic evaluation of listening effort in these populations. Full article

(This article belongs to the Special Issue Advances in Cochlear Implantation)

7 pages, 234 KiB

Open AccessEditor’s ChoiceCommentary

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 3572

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)

13 pages, 2557 KiB

Open AccessFeature PaperEditor’s ChoiceReview

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 5532

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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17 pages, 976 KiB

Open AccessEditor’s ChoiceArticle

Auditory Processing Disorder Test Battery in European Portuguese—Development and Normative Data for Pediatric Population

by Jorge Humberto Martins, Marisa Alves, Susana Andrade, Isabel Falé and António Teixeira

Audiol. Res. 2021, 11(3), 474-490; https://doi.org/10.3390/audiolres11030044 - 17 Sep 2021

Cited by 5 | Viewed by 4295

Abstract

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 [...] Read more.

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

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20 pages, 5938 KiB

Open AccessEditor’s ChoiceReview

Genetic Determinants of Non-Syndromic Enlarged Vestibular Aqueduct: A Review

by Sebastian Roesch, Gerd Rasp, Antonio Sarikas and Silvia Dossena

Audiol. Res. 2021, 11(3), 423-442; https://doi.org/10.3390/audiolres11030040 - 28 Aug 2021

Cited by 22 | Viewed by 7942

Abstract

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 [...] Read more.

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

(This article belongs to the Special Issue Genetics of Hearing Loss)

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5 pages, 212 KiB

Open AccessEditor’s ChoiceBrief Report

Benign Paroxysmal Positional Vertigo (BPPV) in COVID-19

by Pasqualina Maria Picciotti, Giulio Cesare Passali, Bruno Sergi and Eugenio De Corso

Audiol. Res. 2021, 11(3), 418-422; https://doi.org/10.3390/audiolres11030039 - 13 Aug 2021

Cited by 14 | Viewed by 6885

Abstract

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 [...] Read more.

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

(This article belongs to the Special Issue Advances in Positional Vertigo)

5 pages, 984 KiB

Open AccessEditor’s ChoiceTechnical Note

Modulation of Vestibular Microphonics: A Historical Note

by Hero P. Wit

Audiol. Res. 2021, 11(3), 384-388; https://doi.org/10.3390/audiolres11030036 - 6 Aug 2021

Viewed by 2151

Abstract

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 [...] Read more.

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

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15 pages, 2147 KiB

Open AccessEditor’s ChoiceArticle

Optimization of the Speech Test Material in a Group of Hearing Impaired Subjects: A Feasibility Study for Multilingual Digit Triplet Test Development

by Marcin Masalski, Martyna Adamczyk and Krzysztof Morawski

Audiol. Res. 2021, 11(3), 342-356; https://doi.org/10.3390/audiolres11030032 - 12 Jul 2021

Cited by 5 | Viewed by 3361

Abstract

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 [...] Read more.

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

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7 pages, 367 KiB

Open AccessEditor’s ChoiceReview

How Is the Cochlea Activated in Response to Soft Tissue Auditory Stimulation in the Occluded Ear?

by Miriam Geal-Dor and Haim Sohmer

Audiol. Res. 2021, 11(3), 335-341; https://doi.org/10.3390/audiolres11030031 - 9 Jul 2021

Cited by 2 | Viewed by 2405

Abstract

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, [...] Read more.

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

(This article belongs to the Special Issue Bone and Cartilage Conduction)

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14 pages, 3702 KiB

Open AccessEditor’s ChoiceArticle

Sudden Sensorineural Hearing Loss in Mild COVID-19: Case Series and Analysis of the Literature

by Filippo Ricciardiello, Davide Pisani, Pasquale Viola, Elisabetta Cristiano, Alfonso Scarpa, Antonio Giannone, Giuseppe Longo, Giuseppe Russo, Marco Bocchetti, Ciro Coppola, Marco Perrella, Flavia Oliva and Giuseppe Chiarella

Audiol. Res. 2021, 11(3), 313-326; https://doi.org/10.3390/audiolres11030029 - 1 Jul 2021

Cited by 34 | Viewed by 7727

Abstract

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 [...] Read more.

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

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10 pages, 566 KiB

Open AccessEditor’s ChoiceArticle

Sport as a Factor in Improving Visual Spatial Cognitive Deficits in Patients with Hearing Loss and Chronic Vestibular Deficit

by Giorgio Guidetti, Riccardo Guidetti and Silvia Quaglieri

Audiol. Res. 2021, 11(2), 291-300; https://doi.org/10.3390/audiolres11020027 - 19 Jun 2021

Cited by 4 | Viewed by 3005

Abstract

Hearing loss and chronic vestibular pathologies require brain adaptive mechanisms supported by a cross-modal cortical plasticity. They are often accompanied by cognitive deficits. Spatial memory is a cognitive process responsible for recording information about the spatial environment and spatial orientation. Visual-spatial working memory [...] Read more.

Hearing loss and chronic vestibular pathologies require brain adaptive mechanisms supported by a cross-modal cortical plasticity. They are often accompanied by cognitive deficits. Spatial memory is a cognitive process responsible for recording information about the spatial environment and spatial orientation. Visual-spatial working memory (VSWM) is a kind of short-term working memory that allows spatial information to be temporarily stored and manipulated. It can be conditioned by hearing loss and also well-compensated chronic vestibular deficit. Vestibular rehabilitation and hearing aid devices or training are able to improve the VSWM. We studied 119 subjects suffering from perinatal or congenital hearing loss, compared with 532 healthy subjects and 404 patients with well-compensated chronic vestibular deficit (CVF). VSWM was evaluated by the eCorsi test. The subjects suffering from chronic hearing loss and/or unilateral or bilateral vestibular deficit showed a VSWM less efficient than healthy people, but much better than those with CVF, suggesting a better multimodal adaptive strategy, probably favored by a cross-modal plasticity which also provides habitual use of lip reading. The sport activity cancels the difference with healthy subjects. It is therefore evident that patients with this type of deficit since childhood should be supported and advised on a sport activity or repeated vestibular stimulation. Full article

(This article belongs to the Special Issue Advances in Vestibular Rehabilitation—in Memory of Doctor Giorgio Guidetti)

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17 pages, 840 KiB

Open AccessEditor’s ChoiceArticle

Effect of Auditory Distraction on Working Memory, Attention Switching, and Listening Comprehension

by Naveen K. Nagaraj

Audiol. Res. 2021, 11(2), 227-243; https://doi.org/10.3390/audiolres11020021 - 28 May 2021

Cited by 5 | Viewed by 6133

Abstract

The effect of non-informational speech spectrum noise as a distractor on cognitive and listening comprehension ability was examined in fifty-three young, normal hearing adults. Time-controlled tasks were used to measure auditory working memory (WM) capacity and attention switching (AS) ability. Listening comprehension was [...] Read more.

The effect of non-informational speech spectrum noise as a distractor on cognitive and listening comprehension ability was examined in fifty-three young, normal hearing adults. Time-controlled tasks were used to measure auditory working memory (WM) capacity and attention switching (AS) ability. Listening comprehension was measured using a lecture, interview, and spoken narratives test. Noise level was individually set to achieve at least 90% or higher speech intelligibility. Participants’ listening comprehension in the presence of distracting noise was better on inference questions compared to listening in quiet. Their speed of information processing was also significantly faster in WM and AS tasks in noise. These results were consistent with the view that noise may enhance arousal levels leading to faster information processing during cognitive tasks. Whereas the speed of AS was faster in noise, this rapid switching of attention resulted in more errors in updating items. Participants who processed information faster in noise and did so accurately, more effectively switched their attention to refresh/rehearse recall items within WM. More efficient processing deployed in the presence of noise appeared to have led to improvements in WM performance and making inferences in a listening comprehension task. Additional research is required to examine these findings using background noise that can cause informational masking. Full article

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7 pages, 756 KiB

Open AccessEditor’s ChoiceSystematic Review

Meta-Analysis—Correlation between Spiral Ganglion Cell Counts and Speech Perception with a Cochlear Implant

by Yew-Song Cheng and Mario A. Svirsky

Audiol. Res. 2021, 11(2), 220-226; https://doi.org/10.3390/audiolres11020020 - 26 May 2021

Cited by 10 | Viewed by 3678

Abstract

The presence of spiral ganglion cells (SGCs) is widely accepted to be a prerequisite for successful speech perception with a cochlear implant (CI), because SGCs provide the only known conduit between the implant electrode and the central auditory system. By extension, it has [...] Read more.

The presence of spiral ganglion cells (SGCs) is widely accepted to be a prerequisite for successful speech perception with a cochlear implant (CI), because SGCs provide the only known conduit between the implant electrode and the central auditory system. By extension, it has been hypothesized that the number of SGCs might be an important factor in CI outcomes. An impressive body of work has been published on findings from the laborious process of collecting temporal bones from CI users and counting the number of SGCs to correlate those numbers with speech perception scores, but the findings thus far have been conflicting. We performed a meta-analysis of all published studies with the hope that combining existing data may help us reach a more definitive conclusion about the relationship between SGC count and speech perception scores in adults. Full article

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13 pages, 882 KiB

Open AccessEditor’s ChoiceReview

Review of Bone Conduction Hearing Devices

by Susan E. Ellsperman, Emily M. Nairn and Emily Z. Stucken

Audiol. Res. 2021, 11(2), 207-219; https://doi.org/10.3390/audiolres11020019 - 18 May 2021

Cited by 51 | Viewed by 9450

Abstract

Bone conduction is an efficient pathway of sound transmission which can be harnessed to provide hearing amplification. Bone conduction hearing devices may be indicated when ear canal pathology precludes the use of a conventional hearing aid, as well as in cases of single-sided [...] Read more.

Bone conduction is an efficient pathway of sound transmission which can be harnessed to provide hearing amplification. Bone conduction hearing devices may be indicated when ear canal pathology precludes the use of a conventional hearing aid, as well as in cases of single-sided deafness. Several different technologies exist which transmit sound via bone conduction. Here, we will review the physiology of bone conduction, the indications for bone conduction amplification, and the specifics of currently available devices. Full article

(This article belongs to the Special Issue Bone and Cartilage Conduction)

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8 pages, 247 KiB

Open AccessEditor’s ChoiceArticle

Benign Positional Paroxysmal Vertigo in Children

by Cristiano Balzanelli, Daniele Spataro and Luca Oscar Redaelli de Zinis

Audiol. Res. 2021, 11(1), 47-54; https://doi.org/10.3390/audiolres11010006 - 1 Feb 2021

Cited by 7 | Viewed by 3945

Abstract

The aim of this study was to assess the prevalence and analyze clinical parameters of benign positional paroxysmal vertigo (BPPV) in a pediatric age. A cohort of 423 children under the age of 15 (median age 11. interquartile range 9–13) was submitted to [...] Read more.

The aim of this study was to assess the prevalence and analyze clinical parameters of benign positional paroxysmal vertigo (BPPV) in a pediatric age. A cohort of 423 children under the age of 15 (median age 11. interquartile range 9–13) was submitted to vestibular assessment for balance disorders. Dix-Hallpike and Roll-Supine tests were performed to look for positioning nystagmus using video-infrared goggles. BPPV was found in 43 of 423 children evaluated for balance disorders (10.2%). There were 28 females (65.1%) and 15 (34.9%) males. The posterior canal was involved in 79% of cases and the horizontal canal in 21% of cases. No apogeotropic bilateral or anterior canal form were seen. Thus, BPPV is not an infrequent type of vertigo in children and must be evaluated as soon as possible in order to plan the most appropriate maneuver and restore daily activities as soon as possible, avoiding anxiety and fear. Full article

(This article belongs to the Special Issue Advances in Positional Vertigo)

7 pages, 247 KiB

Open AccessEditor’s ChoiceArticle

Human Leukocyte Antigen (HLA) Influence on Prognosis of Autoimmune Hearing Loss

by George Psillas, Paris Binos, Grigorios G Dimas, Michalis Daniilidis and Jiannis Constantinidis

Audiol. Res. 2021, 11(1), 31-37; https://doi.org/10.3390/audiolres11010004 - 25 Jan 2021

Cited by 8 | Viewed by 2827

Abstract

Background: To evaluate the effect of human leukocyte antigen (HLA) on hearing outcome in patients suffering from autoimmune hearing loss (AIHL). Materials and Methods: The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden, fluctuating, or quickly progressing (<12 [...] Read more.

Background: To evaluate the effect of human leukocyte antigen (HLA) on hearing outcome in patients suffering from autoimmune hearing loss (AIHL). Materials and Methods: The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden, fluctuating, or quickly progressing (<12 months) sensorineural hearing loss (uni-/bilateral). The molecular typing of HLA alleles was achieved by using polymerase chain reaction procedures. Patients underwent a tapering schema of steroid treatment and audiometric features were recorded. A logistic regression model was used to identify which HLA typing alleles were statistically significant in patients’ response to treatment. Results: Forty patients with AIHL were found to be carriers of HLA B27, B35, B51, C4, C7, and DRB1*04 alleles. No statistically significant influence of HLA B27, B35, B51, C4, C7, DRB1*04 HLA alleles typing was detected for the prognosis of AIHL. In these patients, the onset of AIHL was mainly progressive (53.8%), 29.2% of them had moderate hearing loss, and most of the cases had both bilateral hearing loss (62.5%) and downsloping audiogram (40%). Conclusion: The presence of HLA B27, B35, B51, C4, C7, and DRB1*04 alleles had no significant effect on a favorable outcome of AIHL. However, larger samples of patients are necessary in order to improve the knowledge about the HLA influence on the clinical course of AIHL. Full article

12 pages, 4805 KiB

Open AccessEditor’s ChoiceArticle

Towards Auditory Profile-Based Hearing-Aid Fitting: Fitting Rationale and Pilot Evaluation

by Raul Sanchez-Lopez, Michal Fereczkowski, Sébastien Santurette, Torsten Dau and Tobias Neher

Audiol. Res. 2021, 11(1), 10-21; https://doi.org/10.3390/audiolres11010002 - 16 Jan 2021

Cited by 7 | Viewed by 5213

Abstract

Background—The clinical characterization of hearing deficits for hearing-aid fitting purposes is typically based on the pure-tone audiogram only. In a previous study, a group of hearing-impaired listeners completed a comprehensive test battery that was designed to tap into different dimensions of hearing [...] Read more.

Background—The clinical characterization of hearing deficits for hearing-aid fitting purposes is typically based on the pure-tone audiogram only. In a previous study, a group of hearing-impaired listeners completed a comprehensive test battery that was designed to tap into different dimensions of hearing abilities. A data-driven analysis of the data yielded four clinically relevant patient sub-populations or “auditory profiles”. The purpose of the current study was to propose and pilot-test profile-based hearing-aid settings in order to explore their potential for providing more targeted hearing-aid treatment. Methods—Four candidate hearing-aid settings were developed and evaluated by a subset of the participants tested previously. The evaluation consisted of multi-comparison preference ratings that were carried out in realistic sound scenarios. Results—Listeners belonging to the different auditory profiles showed different patterns of preference for the tested hearing-aid settings that were largely consistent with the expectations. Conclusions—The results of this pilot evaluation support further investigations into stratified, profile-based hearing-aid fitting with wearable hearing aids. Full article

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