Hearing Loss: From Pathophysiology to Therapies and Habilitation

A special issue of Applied Sciences (ISSN 2076-3417). This special issue belongs to the section "Applied Biosciences and Bioengineering".

Deadline for manuscript submissions: 30 April 2024 | Viewed by 18288

Special Issue Editors


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Guest Editor
1. Shaare Zedek Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 91031, Israel
2. Director, Otology Unit and Cochlear Implant Center, Shaare Zedek Medical Center, Jerusalem 91031, Israel
3. President of the Israeli Society of Otoneurology
Interests: cochlear implants; otosclerosis; bone conduction; physiology of hearing; ototoxicity; hearing loss; tinnitus

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Guest Editor
1. Department of Communication Disorders, Steyer School of Health Professions, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv-Yafo 6997801, Israel
2. Dean of Innovation in Teaching and Learning, Tel Aviv University, Tel Aviv-Yafo 6997801, Israel
3. President of the European Federation of Auditory Societies (EFAS)
Interests: cochlear implants; auditory development; auditory learning; speech perception; auditory habilitation

Special Issue Information

Dear Colleagues,

While hearing loss is considered one of the most common disabilities, science and technology have provided us with means to significantly reduce its debilitating effects. Understanding the pathophysiology of hearing, along the auditory pathways from the ear to the brain, has enabled us to successfully manage hearing loss. Management includes audiological, medical and surgical therapy, a variety of auditory implantable devices and different habilitation protocols. Cochlear implants, bone-anchored hearing aids, middle-ear implants and auditory brainstem implants are examples of effective interventions for restoring hearing (to different degrees). Of these, cochlear implants are the most viable option for individuals with severe–profound sensorineural hearing loss who do not benefit from hearing aids, providing excellent functional results. However, even with cochlear implants, there is much to unravel and a need for improvement in many aspects.

This Special Issue aims to bring together knowledge from different disciplines that will allow the integration and a broad view of this multifaceted pathology. We invite submissions of manuscripts relevant to the aforementioned issues.

Prof. Dr. Ronen Perez
Prof. Dr. Liat Kishon-Rabin
Guest Editors

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Published Papers (9 papers)

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Research

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12 pages, 1571 KiB  
Article
The Effect of Noise on the Utilization of Fundamental Frequency and Formants for Voice Discrimination in Children and Adults
by Liat Kishon-Rabin and Yael Zaltz
Appl. Sci. 2023, 13(19), 10752; https://0-doi-org.brum.beds.ac.uk/10.3390/app131910752 - 27 Sep 2023
Viewed by 713
Abstract
The ability to discriminate between talkers based on their fundamental (F0) and formant frequencies can facilitate speech comprehension in multi-talker environments. To date, voice discrimination (VD) of children and adults has only been tested in quiet conditions. This study examines the effect of [...] Read more.
The ability to discriminate between talkers based on their fundamental (F0) and formant frequencies can facilitate speech comprehension in multi-talker environments. To date, voice discrimination (VD) of children and adults has only been tested in quiet conditions. This study examines the effect of speech-shaped noise on the use of F0 only, formants only, and the combined F0 + formant cues for VD. A total of 24 adults (18–35 years) and 16 children (7–10 years) underwent VD threshold assessments in quiet and noisy environments with the tested cues. Thresholds were obtained using a three-interval, three-alternative, two-down, one-up adaptive procedure. The results demonstrated that noise negatively impacted the utilization of formants for VD. Consequently, F0 became the lead cue for VD for the adults in noisy environments, whereas the formants were the more accessible cue for VD in quiet environments. For children, however, both cues were poorly utilized in noisy environments. The finding that robust cues such as formants are not readily available for VD in noisy conditions has significant clinical implications. Specifically, the reliance on F0 in noisy environments highlights the difficulties that children encounter in multi-talker environments due to their poor F0 discrimination and emphasizes the importance of maintaining F0 cues in speech-processing strategies tailored for hearing devices. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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10 pages, 886 KiB  
Article
Is Corticosteroid Treatment Beneficial in Sudden Sensorineural Hearing Loss? A Large Retrospective Study
by Itay Chen, Ronen Perez, Shalom Eligal, Ori Menahem, Riki Salem, Jean-Yves Sichel and Chanan Shaul
Appl. Sci. 2023, 13(14), 8546; https://0-doi-org.brum.beds.ac.uk/10.3390/app13148546 - 24 Jul 2023
Viewed by 2325
Abstract
The main treatment approaches for sudden sensorineural hearing loss (SSNHL) involve oral and intratympanic corticosteroids, but their efficacy remains controversial. The study objective was to evaluate the benefit of oral corticosteroids followed by intratympanic salvage treatment. This was conducted by comparing the hearing [...] Read more.
The main treatment approaches for sudden sensorineural hearing loss (SSNHL) involve oral and intratympanic corticosteroids, but their efficacy remains controversial. The study objective was to evaluate the benefit of oral corticosteroids followed by intratympanic salvage treatment. This was conducted by comparing the hearing results of post-treatment patients arriving early and pretreatment patients arriving late over the same time points after the onset of HL. A cohort of 776 patients with SSNHL was classified into four groups by time from onset of symptoms to the initiation of treatment (weeks). The post-treatment audiometry of those patients presenting during the first and second week post-HL was compared to the pretreatment audiometry of those presenting in weeks three and four. The post-treatment audiometry of week one and pretreatment audiometry of week three was conducted 17.2 ± 4 and 19.4 ± 3 (p = 0.13) days post-HL onset, respectively. The post-treatment audiometry of week two and pretreatment audiometry of week four was conducted on days 24.6 ± 4 and 25.2 ± 3 (p = 0.32). The pure-tune average for week one and three groups was 36.7 ± 28 and 37.5 ± 19 dB (p = 0.55), and for weeks 2 and 4, it was 31.7 ± 22 and 36.6 ± 23 dB (p = 0.1). Similarly, no significant differences in speech recognition threshold and speech discrimination were found. These results question the benefit of corticosteroid treatment for SSNHL and suggest that improvements may be due to the natural healing process. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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11 pages, 995 KiB  
Article
Medical Safety and Device Reliability of Active Transcutaneous Middle Ear and Bone Conducting Implants: A Long-Term Multi-Centre Observational Study
by Deborah Vickers, John Briggs, Wiebke Lamping, Rachel Andrew, Mina Bingham, Joseph Toner, Stacey Cooper, Patrick Spielman, Hashmat Ghulam, Terry Nunn, Abi Asher, Susan Eitutis, Mark Chung, Anna Skibinska, Robert Gardner, Francesca Routh, Tom Wilson, Lisa Kennedy, Shakeel Saeed and VERS Clinical Support Team
Appl. Sci. 2023, 13(14), 8279; https://0-doi-org.brum.beds.ac.uk/10.3390/app13148279 - 18 Jul 2023
Viewed by 804
Abstract
Active bone-conducting hearing devices (aBCHD; e.g., MEDEL Bonebridge® (BB)) and active middle ear implants (aMEI; e.g., MEDEL Vibrant Soundbridge® (VSB)) use radio frequency transmission to send information from an external microphone and sound processor to an internally implanted transducer. These devices [...] Read more.
Active bone-conducting hearing devices (aBCHD; e.g., MEDEL Bonebridge® (BB)) and active middle ear implants (aMEI; e.g., MEDEL Vibrant Soundbridge® (VSB)) use radio frequency transmission to send information from an external microphone and sound processor to an internally implanted transducer. These devices potentially have an advantage over devices with percutaneous links because the skin is closed over the implantable components, which should reduce the risk of skin problems and infection. On the other hand, surgical procedures are more complex, with a greater risk of damage due to surgery. The objectives of this research were to quantify the reliability and long-term survival of MEDEL VSB and BB devices, determine the adverse and serious adverse device-related complications, and consider associated causes. A multi-center observational retrospective and prospective study was conducted at eleven auditory implant centers in the United Kingdom. Data was collected using a surgical questionnaire and audiological reports. Data were obtained from patient notes or from prospective cases that had a minimum follow-up of one year post-implant. Consecutive patient records were reviewed. Datasets from 109 BB and 163 VSB were reviewed. Of these, 205 were retrospective case note reviews, and 67 were prospective cases. The mean follow-up was 4 and 6 years, respectively, for BB and VSB. Kaplan–Meier Survival analyses indicated that the BB survival was 97% and 93.3% at 1 and 5 years, respectively, and the VSB was 92.1% and 87% at the same time points. This is a large cohort study for the field and has indicated that BB and VSB are safe interventions. Care should be taken to monitor magnet strength in the first few months. For the majority of device-related effects, there was no apparent association with etiology. However, an interesting pattern emerged for individuals who exhibited an inflammatory response, e.g., adhesions or device extrusion, and those with a history of chronic suppurative otitis media. This should be considered in future work and is not surprising given that many VSB recipients have a complicated hearing history, often associated with otitis media. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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23 pages, 2953 KiB  
Article
Cortical Activation in Response to Speech Differs between Prelingually Deafened Cochlear Implant Users with Good or Poor Speech-in-Noise Understanding: An fNIRS Study
by Michal Levin, Michal Balberg and Yael Zaltz
Appl. Sci. 2022, 12(23), 12063; https://0-doi-org.brum.beds.ac.uk/10.3390/app122312063 - 25 Nov 2022
Cited by 3 | Viewed by 1667
Abstract
Cochlear implant (CI) users with prelingual deafness (hearing impairment started before language development was completed) show variable speech-in-noise (SIN) understanding. The present study aimed to assess cortical activation patterns to speech-in-quiet (SIQ) and SIN in prelingual CI users and compared to individuals with [...] Read more.
Cochlear implant (CI) users with prelingual deafness (hearing impairment started before language development was completed) show variable speech-in-noise (SIN) understanding. The present study aimed to assess cortical activation patterns to speech-in-quiet (SIQ) and SIN in prelingual CI users and compared to individuals with normal hearing (NH), using functional Near-Infrared Spectroscopy (fNIRS). Participants included 15 NH who listened to natural speech, 15 NH who listened via 8-channel noise-excited vocoder, and 14 prelingual CI users. fNIRS data were collected in a block design that included three conditions: SIQ, SIN in a signal-to-noise ratio of 0 dB, and noise. Speech reception thresholds in noise (SRTn) were also assessed. Results revealed different patterns of activation between the NH and CI participants in channels covering mainly the right and left middle temporal gyrus (MTG), depending on the SRTn of the CI users. Specifically, while the NH group showed large response to SIQ and SIN in the MTG areas, prelingual CI users with poor SRTn showed significantly smaller response to SIQ, and inversed response (a reduction in activation) to SIN in the same brain areas. These novel findings support the notion that the MTG can serve as a neural marker for speech understanding in CI patients. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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15 pages, 2028 KiB  
Article
Wideband Tympanometry in Adults with Severe to Profound Hearing Loss with and without Cochlear Implants
by Joseph Attias, Navid Shahnaz, Chamutal Efrat, Brian Westerberg, Jane Lea, Eytan David, Ofir Zavdy and Ohad Hilly
Appl. Sci. 2022, 12(17), 8879; https://0-doi-org.brum.beds.ac.uk/10.3390/app12178879 - 04 Sep 2022
Cited by 1 | Viewed by 2053
Abstract
Objectives—Cochlear implantation has been suggested to be associated with an air-bone gap, possibly secondary to increased middle- and inner-ear stiffness. To explore the effect of possible changes in mechanics due to cochlear implantation, we measured wideband tympanometry (WBT) in individuals with normal hearing [...] Read more.
Objectives—Cochlear implantation has been suggested to be associated with an air-bone gap, possibly secondary to increased middle- and inner-ear stiffness. To explore the effect of possible changes in mechanics due to cochlear implantation, we measured wideband tympanometry (WBT) in individuals with normal hearing and individuals with severe to profound sensorineural hearing loss who underwent unilateral or bilateral cochlear implantation. Our goal was to characterize differences in WBT patterns associated with severe to profound sensorineural hearing loss and with cochlear implantation. Design—The study participants were 24 individuals with normal hearing (48 ears) and 17 with cochlear implants, of which 15 were unilaterally implanted and two were implanted bilaterally. All the participants had normal otoscopy. In the implanted group, inner-ear anatomy was normal according to preoperative imaging. All participants underwent pure-tone audiometric assessment, standard tympanometry (226 Hz probe tone), and WBT to a click stimulus (analyzed from 226 to 8000 Hz). WBT was recorded at ambient and peak pressures. To elucidate the effect of sensorineural hearing loss on WBT, we compared normal-hearing ears with impaired non-implanted ears. The impact of cochlear implantation was assessed by comparing the WBT of the implanted and non-implanted ears of the same participants. Moreover, the effect of the ear (right vs. left) and test–retest reproducibility were evaluated. Results—WBT results for right and left ears showed no statistically significant differences in the normal hearing participants. There were no statistical differences shown between repeated measurements of WBT with removal and re-insertion of the probe in any of the groups—the normal ears, the implanted ears, and the non-implanted ears with hearing impairment. Peak compensated admittance (Ytm) in standard tympanometry was normal in 32 out of 34 (94%) ears with sensorineural hearing loss, with one CI user having bilateral negative middle-ear pressure. Compared to normal-hearing ears, ears with severe to profound SNHL, with or without cochlear implantation, showed a significant decrease in absorbance at high frequencies (4000–5000 Hz). Implanted ears, compared both to normal-hearing and contralateral-non-implanted ears, showed a significant decrease in absorbance at low frequencies (400–800 Hz) and an increase in absorbance around 1600 Hz. Conclusions—WBT analysis revealed that the implanted ears showed a significant decrease in absorbance at low frequencies (400–800 Hz) and an increase at 1600 Hz. This finding is likely a result of an increased stiffness due to the implantation surgery and the presence of the implant in the inner ear. In contrast, the specific decrease in absorbance around 4000–5000 Hz in the implanted and non-implanted ears compared to the normal ears is likely either due to aging or the impact of the severe-to-profound SNHL on the input impedance of the cochlea. The structural and functional changes of both the conductive system and the cochlea associated with aging and SNHL may change the wideband acoustic immittance of the ear and these alterations might be correlated with the hearing loss severity. Additional research is needed to determine how much hearing loss and age influence wideband acoustic immittance of the ear. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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15 pages, 1028 KiB  
Article
Consequences of Stapes Surgery on Tongue Morphological Characteristics in Narrow Band Imaging, Gustatory Function and General Sensation: A Prospective Tertiary Center Study
by Nina Božanić Urbančič, Domen Vozel, Nejc Steiner, Manja Hribar, Iztok Fošnarič, Robert Šifrer, Jure Urbančič and Saba Battelino
Appl. Sci. 2022, 12(7), 3248; https://0-doi-org.brum.beds.ac.uk/10.3390/app12073248 - 23 Mar 2022
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Abstract
(1) Background: Chorda tympani (CT) manipulation during stapes surgery affects its functions. We hypothesized that this alters tongue morphology and sensory functions. (2) Methods: Patients undergoing stapes surgery were tested 1 day preoperatively, 1 and 6 months postoperatively. Narrow band imaging contact endoscopy [...] Read more.
(1) Background: Chorda tympani (CT) manipulation during stapes surgery affects its functions. We hypothesized that this alters tongue morphology and sensory functions. (2) Methods: Patients undergoing stapes surgery were tested 1 day preoperatively, 1 and 6 months postoperatively. Narrow band imaging contact endoscopy (NBI) was used to determine the number of fungiform papillae (Npapillae) and the total score of blood vessel morphology (NBItotal). The taste was tested with taste strips. General sensation was tested with a static two-point discrimination. Tests were performed on ipsilateral and contralateral side of the tongue. (3) Results: 52 otosclerosis patients were included in the study. There was a statistically significant decrease of NBItotal (p = 0.005), Npapillae (p = 0.009), sensation of sweet (p = 0.003), salty (p = 0.035), sour (p = 0.036), and bitter taste (p = 0.013) within the test side during the follow-up. A statistically significant impact on presence of dysgeusia for sweet was found 1 month postoperatively (p < 0.005). Postoperative decrease in two-point discrimination score did not reach a statistical significance (p = 0.056). (4) Conclusions: CT manipulation affects fungiform papillae density, vascular patterns and taste sensation. The general sensation of the tongue is not influenced by CT manipulation. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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8 pages, 363 KiB  
Article
Prevalence of Hearing Loss in Dutch Newborns; Results of the Nationwide Well-Baby Newborn Hearing Screening Program
by Rosanne B. van der Zee, Noëlle N. Uilenburg, Catharina (Kitty) P. B. van der Ploeg and Evelien Dirks
Appl. Sci. 2022, 12(4), 2035; https://0-doi-org.brum.beds.ac.uk/10.3390/app12042035 - 16 Feb 2022
Cited by 2 | Viewed by 2131
Abstract
Background: Few studies report prevalence rates of hearing loss in newborns for nationwide populations. The Dutch well-baby newborn hearing screening covers almost all eligible children and has high participation rates for follow-up screening rounds and diagnosis. This allows calculating reliable prevalence rates of [...] Read more.
Background: Few studies report prevalence rates of hearing loss in newborns for nationwide populations. The Dutch well-baby newborn hearing screening covers almost all eligible children and has high participation rates for follow-up screening rounds and diagnosis. This allows calculating reliable prevalence rates of permanent neonatal hearing loss specified by severity. Methods: Results from the well-baby newborn hearing screening program and diagnostic follow-up of referred children from 2015 to 2019 were included in calculating prevalence rates. Hearing loss was classified according to the degree of severity. Results: A total of 99.7% of 833,318 children eligible for screening were included. A total of 0.3% were referred for audiological diagnostics. Permanent bilateral hearing loss of ≥40 dB was diagnosed in 23.7% of them and unilateral hearing loss in 14.4%. A prevalence rate of 1.23 per 1000 children was found, 0.46 for unilateral hearing loss and 0.76 for bilateral hearing loss. Moderate hearing loss is most common in children with bilateral hearing loss (0.47), followed by profound (0.21) and severe (0.06) hearing loss. In children with unilateral hearing loss, prevalence rates are the highest for profound hearing loss (0.21), followed by moderate (0.16) and severe (0.09) hearing loss. A total of 87.5% of the children were diagnosed within the age of 3 months. Conclusions: Because of the high quality of the Dutch well-baby hearing screening program, reported results approximate true prevalence rates of permanent hearing loss by severity. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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Review

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14 pages, 803 KiB  
Review
Preservation of Inner Ear Functions: Extending Glucocorticoid Therapy by Tissue-Protective α1-Antitrypsin
by Amit Amar, Eli C. Lewis, Daniel M. Kaplan and Sabri El-Saied
Appl. Sci. 2022, 12(18), 9359; https://0-doi-org.brum.beds.ac.uk/10.3390/app12189359 - 18 Sep 2022
Cited by 1 | Viewed by 2931
Abstract
Protecting tissues from excessive inflammation by glucocorticoids results in an effective blockade of inflammation; however, it does not instigate processes of inflammatory resolution or tissue repair. Moreover, glucocorticoids have side effects such as a susceptibility to infections. In otolaryngology—specifically, within the inner ear—surgical [...] Read more.
Protecting tissues from excessive inflammation by glucocorticoids results in an effective blockade of inflammation; however, it does not instigate processes of inflammatory resolution or tissue repair. Moreover, glucocorticoids have side effects such as a susceptibility to infections. In otolaryngology—specifically, within the inner ear—surgical and non-surgical pathologies include cochlear implantation, stapes surgery, perilymph fistulas and Meniere’s disease. For these, steroids are indicated in order to prevent excessive inflammation that might lead to hearing and vestibular failure. Unless tissue homeostasis is restored, the compromised tissue is at risk of a functional loss. α1-Antitrypsin (AAT) is a circulating inflammation-modulating molecule that rises during the molecular signs of a tissue injury; it manipulates inflammation towards an inflammatory resolution and advances tissue repair. Lifelong infusions of AAT are currently indicated for genetic AAT deficiencies and are safe. In the present review, we discuss the advantages and downfalls of glucocorticoid treatments across several surgical inner ear injuries alongside evidence of the beneficial attributes of treatments with AAT. Collectively, the present knowledge places AAT treatments, wither independent or in combination with glucocorticoids, as adding focus on tissue repair in the context of unmet medical needs in otolaryngology. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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11 pages, 2912 KiB  
Review
Otologic Manifestations of IgG4-Related Disease: Literature Review and Report of Two Cases
by Aviad Sapir, Daniel M. Kaplan, Benzion Samueli, Rosa Novoa, Ohad Hilly and Sabri El-Saied
Appl. Sci. 2022, 12(16), 8353; https://0-doi-org.brum.beds.ac.uk/10.3390/app12168353 - 21 Aug 2022
Cited by 1 | Viewed by 1818
Abstract
Background: IgG4-related disease (IgG4-RD) is an inflammatory process. The literature on IgG4-RD is rather limited, with mastoid involvement being uncommon. In such cases, presentation may mimic other middle ear and mastoid cavity pathologies. Objective: To summarize findings of patients with IgG4-RD [...] Read more.
Background: IgG4-related disease (IgG4-RD) is an inflammatory process. The literature on IgG4-RD is rather limited, with mastoid involvement being uncommon. In such cases, presentation may mimic other middle ear and mastoid cavity pathologies. Objective: To summarize findings of patients with IgG4-RD involving the mastoid. Methods: Description of two new cases and summary of findings with previous reports. Results: Nineteen cases of IgG4-RD with mastoid bone involvement were reported in the literature, the earliest appearing at the beginning of the previous decade. Most frequent symptoms included hearing deterioration, tinnitus and otalgia. In 58% of the cases, the process was restricted to otologic manifestations. In 7 out of 19 cases, tissue IgG4 levels were elevated. In all histopathologic samples taken from the operative sites, a dense lymphoplasmacytic cell infiltration was observed. Following definitive pathological diagnosis, the most common treatment was corticosteroids. Generally, the time from onset to final diagnosis was usually more than six months. The treatment is corticosteroids, followed by immunosuppressive agents such as rituximab, cyclophosphamide, and methotrexate. Conclusions: IgG4-RD involving the mastoid is a challenging condition, both in diagnosis and treatment. Hence, IgG4-RD should be included in the differential diagnosis of middle ear pathologies and include a multi-disciplinary team for treatment. Full article
(This article belongs to the Special Issue Hearing Loss: From Pathophysiology to Therapies and Habilitation)
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