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Medicina
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Metabolic Disorders and Sensorineural Hearing Loss
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Metabolic Disorders and Sensorineural Hearing Loss

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Endocrinology".

Deadline for manuscript submissions: closed (31 July 2023) | Viewed by 15883

Special Issue Editors

Prof. Dr. Elisabetta Genovese

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Guest Editor
Full Professor in Audiology, Department of Medical and Surgical Science for Children and Adults, University of Modena and Reggio Emilia, Modena, Italy
Interests: audiology; deafness; hearing disorders; audiometry; screening; speech intelligibility; psychoacoustics; speech science; language disorders; hearing loss
Prof. Dr. Daniele Monzani

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Guest Editor
Professor in Audiology, Head and Neck Department, University of Verona, Verona, Italy
Interests: otorhinolaryngology; audiology; orthodontics and digital dentistry
Special Issues, Collections and Topics in MDPI journals
Dr. Silvia Palma

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Guest Editor
Audiology, Primary Care Department, Local Health Unit of Modena, Modena, Italy
Interests: hearing loss; hearing disorders; ENT; otology; audiology; ear deafness; pediatric otolaryngology; auditory perception; audiometry
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Sensorineural hearing loss is an important public health problem whose prevalence has increased as life expectancy has become longer, resulting in a lower quality of life. Established causes of hearing loss include genetic predisposition, vascular causes, infections, ototoxic drugs and exposure to excessive noise, but the determinants of most cases of ageing-related hearing loss are not entirely certain.

Metabolic syndrome (MetS) is a cluster of co-occurring conditions that increase the risk of heart disease, stroke and type 2 diabetes, along with other conditions that affect the blood vessels, including hearing loss. Moreover, the number of components of the metabolic syndrome is positively correlated with the rate of sensorineural hearing loss.

Due to ageing of the world population, metabolic disorders are increasing, and with them the potential number of people affected by hearing loss.

To date, few studies have examined the link between metabolic disorders and the causal relationship between diabetes mellitus, and hearing loss has still not been established in large longitudinal cohort studies.

Prof. Dr. Elisabetta Genovese
Prof. Dr. Daniele Monzani
Dr. Silvia Palma
Guest Editors

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Keywords

  • hearing loss
  • tinnitus
  • cochlear damage
  • metabolic syndrome (diabetes mellitus, hypertension, dyslipidemia)
  • mitochondrial disease
  • kidney disease
  • cardiovascular disease
  • age-related hearing loss

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Published Papers (7 papers)

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Research

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16 pages, 376 KiB  
Article
Vascular Factors in Patients with Midlife Sensorineural Hearing Loss and the Progression to Mild Cognitive Impairment
by Valeria Del Vecchio, Laura Tricarico, Anna Pisani, Nicola Serra, Domenico D’Errico, Eugenio De Corso, Teresa Rea, Pasqualina M. Picciotti, Carla Laria, Giuseppe Manna, Annamaria Franzè, Rita Malesci and Anna Rita Fetoni
Medicina 2023, 59(3), 481; https://doi.org/10.3390/medicina59030481 - 28 Feb 2023
Cited by 2 | Viewed by 2029
Abstract
Background and Objectives: Midlife hearing loss (HL) has been considered as a major modifiable risk factor for a later-life progression to dementia. Our aim was to detect a link between precocious sensorineural hearing loss (SNHL) and mild cognitive impairment (MCI) and their [...] Read more.
Background and Objectives: Midlife hearing loss (HL) has been considered as a major modifiable risk factor for a later-life progression to dementia. Our aim was to detect a link between precocious sensorineural hearing loss (SNHL) and mild cognitive impairment (MCI) and their association to putative risk factors for a common pathology. Materials and methods: In this study, a retrospective case-control study was carried out. A total of 112 patients were enrolled as following: 81 patients with bilateral SNHL and 31 subjects with normal hearing, whose ages ranged from 50 to 65 years. Both groups performed pure tone audiometry, a tinnitus handicap inventory (THI), Mini-Mental State examination (MMSE), and the Montreal Cognitive Assessment (MoCA), Hospital Anxiety and Depression Scale (HADS-A and HADS-D). Results: The mean age was 58 ± 5.2 in SNHL patients and 53.2 ± 4.8 in the control group. The mean pure tone average in the SNHL group was 40.2 ± 18.7 dB HL on the right side and 41.2 ± 17.2 dB HL on the left side, while in the control group it was 12.5 ± 2.8 dB HL on right side and 12.4 ± 3.1 dB HL on left side. About 64% of patients with SNHL exhibited comorbidities, and the most common condition was hypertension. Altered MoCA test scores were significantly related to the pure tone averages in patients with SNHL compared to the control group (p = 0.0004), while the differences in the HADS-A and HADS-D were not significant. Furthermore, a significant correlation was observed in SNHL patients between an altered MoCA test and hypercholesterolemia (p = 0.043). Conclusions: Hearing impairment and screening tests to detect MCI should be considered in the midlife in order to carry out strategies to prevent the progression to dementia. Hypertension and hypercholesterolemia are two risk factors in the development of endothelial dysfunction, oxidative stress, and vascular inflammation, and may represent the common pathology linking the inner ear and brain damage. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)

Review

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25 pages, 1657 KiB  
Review
Polyphenols in Inner Ear Neurobiology, Health and Disease: From Bench to Clinics
by Naomi Osakabe, Sergio Modafferi, Maria Laura Ontario, Francesco Rampulla, Vincenzo Zimbone, Maria Rita Migliore, Tilman Fritsch, Ali S. Abdelhameed, Luigi Maiolino, Gabriella Lupo, Carmelina Daniela Anfuso, Elisabetta Genovese, Daniele Monzani, Uwe Wenzel, Edward J. Calabrese, R. Martin Vabulas and Vittorio Calabrese
Medicina 2023, 59(11), 2045; https://doi.org/10.3390/medicina59112045 - 20 Nov 2023
Cited by 2 | Viewed by 2246
Abstract
There is substantial experimental and clinical interest in providing effective ways to both prevent and slow the onset of hearing loss. Auditory hair cells, which occur along the basilar membrane of the cochlea, often lose functionality due to age-related biological alterations, as well [...] Read more.
There is substantial experimental and clinical interest in providing effective ways to both prevent and slow the onset of hearing loss. Auditory hair cells, which occur along the basilar membrane of the cochlea, often lose functionality due to age-related biological alterations, as well as from exposure to high decibel sounds affecting a diminished/damaged auditory sensitivity. Hearing loss is also seen to take place due to neuronal degeneration before or following hair cell destruction/loss. A strategy is necessary to protect hair cells and XIII cranial/auditory nerve cells prior to injury and throughout aging. Within this context, it was proposed that cochlea neural stem cells may be protected from such aging and environmental/noise insults via the ingestion of protective dietary supplements. Of particular importance is that these studies typically display a hormetic-like biphasic dose–response pattern that prevents the occurrence of auditory cell damage induced by various model chemical toxins, such as cisplatin. Likewise, the hormetic dose–response also enhances the occurrence of cochlear neural cell viability, proliferation, and differentiation. These findings are particularly important since they confirmed a strong dose dependency of the significant beneficial effects (which is biphasic), whilst having a low-dose beneficial response, whereas extensive exposures may become ineffective and/or potentially harmful. According to hormesis, phytochemicals including polyphenols exhibit biphasic dose–response effects activating low-dose antioxidant signaling pathways, resulting in the upregulation of vitagenes, a group of genes involved in preserving cellular homeostasis during stressful conditions. Modulation of the vitagene network through polyphenols increases cellular resilience mechanisms, thus impacting neurological disorder pathophysiology. Here, we aimed to explore polyphenols targeting the NF-E2-related factor 2 (Nrf2) pathway to neuroprotective and therapeutic strategies that can potentially reduce oxidative stress and inflammation, thus preventing auditory hair cell and XIII cranial/auditory nerve cell degeneration. Furthermore, we explored techniques to enhance their bioavailability and efficacy. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)
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11 pages, 304 KiB  
Review
Endothelial Dysfunction and Metabolic Disorders in Patients with Sudden Sensorineural Hearing Loss
by Giada Cavallaro, Alessandra Pantaleo, Vito Pontillo, Francesco Barbara, Alessandra Murri and Nicola Quaranta
Medicina 2023, 59(10), 1718; https://doi.org/10.3390/medicina59101718 - 26 Sep 2023
Cited by 1 | Viewed by 1502
Abstract
Sudden sensorineural hearing loss (SSNHL) is defined as a sensorineural hearing loss of 30 dB or greater on at least three contiguous audiometric frequencies occurring within a 72 h period. Although SSNHL is commonly encountered in clinical audiology and otolaryngology practice, its etiopathogenesis [...] Read more.
Sudden sensorineural hearing loss (SSNHL) is defined as a sensorineural hearing loss of 30 dB or greater on at least three contiguous audiometric frequencies occurring within a 72 h period. Although SSNHL is commonly encountered in clinical audiology and otolaryngology practice, its etiopathogenesis continues to be poorly understood. Scientific investigations have highlighted the vulnerability of cochlear microcirculation to blood flow alterations. Even mild hypoperfusion can lead to immediate dysfunction in the organ of Corti, given the heightened susceptibility of cochlear hair cells to hypoxia and ischemic damage. The purpose of this review paper is to present evidence of endothelial and vascular involvement in SSNHL and the risk factors, such as metabolic syndrome, that may negatively impact the inner ear’s vascular supply, influencing the onset pattern, incidence, and prognosis of SSNHL. By addressing these variables, we can deepen our comprehension of the mechanisms underlying SSNHL and potentially uncover strategies for prevention. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)
10 pages, 745 KiB  
Review
Cooling the Cochlea: Slowing Down Metabolism May Be a Way of Protecting Hearing from Surgical Trauma
by Andrew Bell and W. Wiktor Jedrzejczak
Medicina 2023, 59(7), 1187; https://doi.org/10.3390/medicina59071187 - 22 Jun 2023
Viewed by 1405
Abstract
Background and Objectives: This narrative review of the literature explores the effect of body temperature on hearing. In particular, its focus is on extended high frequency (EHF) hearing—the range beyond the standard audiometric limit of 8 kHz. Such high frequencies are the [...] Read more.
Background and Objectives: This narrative review of the literature explores the effect of body temperature on hearing. In particular, its focus is on extended high frequency (EHF) hearing—the range beyond the standard audiometric limit of 8 kHz. Such high frequencies are the first to be affected by noise-induced hearing loss, and so monitoring them can provide an early warning sign of incipient damage. Materials and Methods: This review builds on a personal literature database of 216 references covering the general topic of EHF hearing; the procedure was to then identify papers related to whole-body or cochlear cooling. A starting point was the paper by Munjal et al. who in 2013 reported changes of up to 15–30 dB in the EHF thresholds of subjects who had undergone cardiopulmonary bypass (CBP) surgery, which typically involves mild to moderate hypothermia—cooling of the blood—to reduce cellular oxygen demand and minimise tissue damage. Results: Reviewing the surrounding literature, we find that although CBP surgery by itself can impair hearing thresholds, lower body and cochlear temperatures in general provide neuroprotective effects. A connection between hearing loss and CBP surgery has been periodically documented, but the mechanism behind it has yet to be conclusively identified. Conclusions: The observations reviewed here tend to confirm the otoprotective effects of cooling. We consider that the high sensitivity of EHF thresholds to temperature is a major factor that has not been sufficiently recognised, although it has important implications for otological research and practice. Two important inferences are that, first, monitoring EHF thresholds might have considerable value in audiology, and, second, that lowering temperature of the cochlea during cochlear implantation might provide substantially better hearing preservation, as some researchers have already suggested. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)
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11 pages, 894 KiB  
Review
The Role of Primary Mitochondrial Disorders in Hearing Impairment: An Overview
by Virginia Fancello, Giuseppe Fancello, Silvia Palma, Daniele Monzani, Elisabetta Genovese, Chiara Bianchini and Andrea Ciorba
Medicina 2023, 59(3), 608; https://doi.org/10.3390/medicina59030608 - 19 Mar 2023
Cited by 3 | Viewed by 2583
Abstract
Background. Defects of mitochondrial DNA (mtDNA) involved in the function of the mitochondrial electron transport chain can result in primary mitochondrial diseases (PMDs). Various features can influence the phenotypes of different PMDs, with relevant consequences on clinical presentation, including the presence of [...] Read more.
Background. Defects of mitochondrial DNA (mtDNA) involved in the function of the mitochondrial electron transport chain can result in primary mitochondrial diseases (PMDs). Various features can influence the phenotypes of different PMDs, with relevant consequences on clinical presentation, including the presence of hearing impairment. This paper aims to describe the hearing loss related to different PMDs, and when possible, their phenotype. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline until December 2022. A total of 485 papers were identified, and based on specified criteria, 7 were included in this study. Results. A total of 759 patients affected by PMDs and hearing loss were included. The age of patients ranged from 2 days to 78 years old, and the male-to-female ratio was 1.3:1. The percentage of subjects affected by hearing loss was 40.8%, (310/759), and in most cases, hearing impairment was described as sensorineural, bilateral, symmetrical, and progressive, with different presentations depending on age and syndrome severity. Conclusions. PMDs are challenging conditions with different clinical phenotypes. Hearing loss, especially when bilateral and progressive, may represent a red flag; its association with other systemic disorders (particularly neuromuscular, ocular, and endocrine) should alert clinicians, and confirmation via genetic testing is mandatory nowadays. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)
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9 pages, 314 KiB  
Review
Diabetes Mellitus and Hearing Loss: A Complex Relationship
by Federico Maria Gioacchini, Davide Pisani, Pasquale Viola, Alessia Astorina, Alfonso Scarpa, Fernanda Asprella Libonati, Michele Tulli, Massimo Re and Giuseppe Chiarella
Medicina 2023, 59(2), 269; https://doi.org/10.3390/medicina59020269 - 30 Jan 2023
Cited by 6 | Viewed by 2699
Abstract
Background and Objectives: Discussion is open about the relationship between diabetes (DM) and hearing loss (HL). There is a lot of evidence in the literature suggesting a causal link between these conditions, beyond being considered simple comorbidities. The difficulty in identifying populations [...] Read more.
Background and Objectives: Discussion is open about the relationship between diabetes (DM) and hearing loss (HL). There is a lot of evidence in the literature suggesting a causal link between these conditions, beyond being considered simple comorbidities. The difficulty in identifying populations free from confounding factors makes it difficult to reach definitive conclusions on the pathophysiological mechanisms at play. Nonetheless, there is numerous evidence that demonstrates how the population affected by DM is more affected by sensorineural HL (SNHL) and exhibit a higher prevalence of idiopathic sudden sensorineural HL (ISSNHL). Materials and Methods: Articles reporting potentially relevant information were reviewed, and the most significant results are discussed in this article. Starting from the possible mechanisms relating to auditory impairment in the diabetic condition, this article summarizes the studies on auditory evaluation in subjects with DM1 and DM2 and addresses the relationship between DM and ISSNHL. Results: DM is considered a risk factor for SNHL, although some studies have reported no relationship when the associations were adjusted for age, gender, and hypertension. Macro and microvascular insults that cause decreased blood flow, oxygen exchange, and ion transport are major complications of hypertension and DM and can have a direct effect on the sensory and support cells of the cochlea. Conclusions: Given the difficulty of carrying out studies on populations without confounding factors, new laboratory studies are strongly required to clarify which specific physiopathological mechanisms underlie the diabetic damage caused to the hearing organs and how pharmacological management may contribute to counteracting the pathophysiological effects of the diabetic condition on the auditory system. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)

Other

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11 pages, 1547 KiB  
Case Report
Temperature-Sensitive Auditory Neuropathy: Report of a Novel Variant of OTOF Gene and Review of Current Literature
by Francesca Forli, Silvia Capobianco, Stefano Berrettini, Luca Bruschini, Silvia Romano, Antonella Fogli, Veronica Bertini and Francesco Lazzerini
Medicina 2023, 59(2), 352; https://doi.org/10.3390/medicina59020352 - 13 Feb 2023
Viewed by 2475
Abstract
Background and objectives: Otoferlin is a multi-C2 domain protein implicated in neurotransmitter-containing vesicle release and replenishment of the cochlear inner hair cell (IHC) synapses. Mutations in the OTOF gene have been associated with two different clinical phenotypes: a prelingual severe-to-profound sensorineural hearing [...] Read more.
Background and objectives: Otoferlin is a multi-C2 domain protein implicated in neurotransmitter-containing vesicle release and replenishment of the cochlear inner hair cell (IHC) synapses. Mutations in the OTOF gene have been associated with two different clinical phenotypes: a prelingual severe-to-profound sensorineural hearing loss (ANSD-DFNB9); and the peculiar temperature-sensitive auditory neuropathy (TS-ANSD), characterized by a baseline mild-to-moderate hearing threshold that worsens to severe-to-profound when the body temperature rises that returns to a baseline a few hours after the temperature has fallen again. The latter clinical phenotype has been described only with a few OTOF variants with an autosomal recessive biallelic pattern of inheritance. Case report: A 7-year-old boy presented a picture compatible with TS-ANSD exacerbated by febrile states or physical exercise with mild-to-moderate hearing loss at low and medium frequencies and a decrease in speech discrimination that worsened with an unfavorable speech-to-noise ratio. Otoacoustic emissions (OAEs) were present whereas auditory brainstem responses (ABRs) evoked by a click or tone-burst were generally absent. No inner ear malformations were described from the CT scan or MRI. Next-generation sequencing (NGS) of the known deafness genes and multi-phasic bioinformatic analyses of the data detected in OTOF a c.2521G>A missense variant and the deletion of 7.4 Kb, which was confirmed by array-comparative genomic hybridization (array-CGH). The proband’s parents, who were asymptomatic, were tested by Sanger sequencing and the father presented the c.2521G>A missense variant. Conclusions: The picture presented by the patient was compatible with OTOF-induced TS-ANSD. OTOF has been generally associated with an autosomal recessive biallelic pattern of inheritance; in this clinical report, two pathogenic variants never previously associated with TS-ANSD were described. Full article
(This article belongs to the Special Issue Metabolic Disorders and Sensorineural Hearing Loss)
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