Sensorineural Research Articles

Compound Heterozygous Loss of Allele Coding for KCNJ16 in an Adult Presenting as Recurrent Hypokalemic Periodic Paralysis with Metabolic Acidosis and Hypokalemia

A 20-year-old male presented with fever and rapidly progressive muscle weakness. He had hypokalemia and metabolic acidosis, suggesting renal tubular acidosis (RTA). Further investigation revealed distal RTA with preserved acidification. A channelopathy was suspected. Whole exome sequencing identified a novel biallelic mutation in the KCNJ16 gene, which encodes the Kir5.1 protein. This is the first reported case of an Indian adult with biallelic KCNJ16 mutations presenting as pure renal phenotype without sensory neural hearing loss or cardiac manifestation.

Testing for genetic and viral etiologies in congenital hearing loss based on a survey of cochlear implant centers: proposed HEARRING group consensus and future directions

Background In cases of congenital sensorineural hearing loss, testing for genetic etiologies and congenital cytomegalovirus (cCMV) infection have become common practice. Aims/Objectives The purpose of this study is to determine which specific testing methodologies should be used and when. Material and methods We surveyed 20 practicing otolaryngologists across eighteen institutions in thirteen countries about their approach to cCMV, GJB2, and wider genetic testing. Results We found 90% of respondents employ all three tests, either in routine or special cases. cCMV testing is widely used, with 95% of respondents incorporating it into their clinical practice. GJB2 testing was employed by 90%. In cases with negative GJB2 test results, a majority of respondents proceeded to wider genetic screening. Test reimbursement was also examined for each test. For cCMV testing, 63.1% reported reimbursement, 68.4% reported reimbursement for GJB2 variant testing and 52.6% reported reimbursement for wider genetic screening. Conclusions and significance A common approach is to perform cCMV and GJB2 testing as the first tests, followed by wider genetic testing. This study offers insight into the prevalence, methodologies, and reimbursement status of these testing methodologies across multiple hearing centers and countries. Current consensus and future directions are described based on the current survey.

Evaluation of auditory system in vitiligo patients: A case-control study

BackgroundVitiligo is a common acquired pigmentary disorder characterized by well-defined areas of depigmentation due to loss of melanocytes. Vitiligo can affect melanocytes in the auditory system and potentially hearing. ObjectiveThe aim of this study was to investigate hearing changes in vitiligo patients. MethodsTwenty-six with vitiligo patients and 26 demographically matched healthy controls were included in this study. Audiological function was evaluated using pure tone audiometry (PTA), speech audiometry and tympanometry tests in all subjects. ResultsThe mean hearing threshold of the left ear at frequencies of 250 and 8000 Hz in vitiligo patients was significantly higher than the control group. Sensorineural hearing loss (SNHL) was seen in 14 (53.8 %) vitiligo patients and 5(19.2 %) control group (p < 0.05). Most affected patients (10/14; 71 %) had bilateral hearing loss especially in high frequency (8000 Hz). A significant association was identified between vitiligo and SNHL (OR 4.9; 95 % CI, 1.41–16.98, P = 0.01). There was a statistically significant correlation between hearing impairment and age in both groups. Gender, severity, activity and duration of vitiligo had no a significant effect on the results of audiometric tests. ConclusionThis study confirmed that the auditory system was affected in vitiligo patients. Vitiligo patients might be at a higher risk for auditory abnormalities at a younger age than control subjects. Auditory evaluation of vitiligo patients should be considered for early detection of hearing loss.

A Nationwide, Population-Based Study of Intelligence and Hearing Loss among 3,104,670 Adolescents

Purpose: Epidemiological studies have demonstrated a relationship between general intelligence (GI) in youth and hearing loss (HL). However, no large-scale study assessed the relations of GI in late adolescence with conductive HL (CHL) and sensorineural HL (SNHL), stratified by severity. This study examined the connection between HL and GI in late adolescence. Methods: Cross-sectional study on mandatory premilitary recruitment data recorded during 1967–2019 of patients aged 17–19. We compared GI between SNHL, CHL, and those with normal hearing. In addition, we used logistic regression to measure the associations between HL and GI after adjuring for age, sex, education, and socioeconomic status. Results: Among 3,104,851 adolescents assessed, 20,075 (0.6%) exhibited HL. We categorized GI into three levels for analysis: low (lowest category), medium, and high (reference category). Adjusted odds ratios (ORs) revealed that SNHL was associated with lower GI levels, with ORs ranging from 1.3 (95% confidence interval [CI] 1.4–1.2) for the lowest GI category to 1.1 (95% CI 1.15–0.04) for the medium category. CHL (CHL) also demonstrated significant associations, with ORs from 1.8 (95% CI 1.9–1.6) for the lowest GI level to 1.1 (95% CI 1.2–0.9) for medium. Further analysis revealed a statistically significant, severity-dependent relationship between SNHL and the odds of being in the lowest GI quartile (Q1). Specifically, the ORs for SNHL ranged from 1.2 (95% CI 1.1–1.3) to 1.3 (95% CI 1.1–1.5) as severity increased, indicating a strong link between greater SNHL severity and reduced cognitive performance. In contrast, CHL did not show a consistent correlation between its severity and GI outcomes, with an OR of 1.6 (95% CI 1.2–2.3) across severity levels. Conclusion: We report a strong relationship between HL and GI in late adolescence. SNHL, but not CHL, demonstrated a severity-based decline in GI. The results highlight the value of early, specifically targeted therapies for HL that consider its etiology and degree.

Sensorineural Hearing Loss Negatively Impacts Cognition in Older Subjects with Normal Lateral Semi-Circular Canal Function

Plain Language SummaryWithin the current study, the authors aimed to explore the effect of hearing loss on cognitive functioning in older adults, considering the effects of vestibular function, anxiety and depression on cognitive performance. There is increasing evidence that hearing loss is a modifiable risk factor for cognitive decline but also vestibular dysfunction, depression and anxiety symptoms may play a role in this association. Cognitive functioning was evaluated in a group with moderate-to-severe hearing loss and a matched control group based on sex, age, education level, vestibular function, anxiety and depression rates. The patients with a hearing impairment performed significantly worse on cognitive testing and the differences were most pronounced for the cognitive subdomains of Attention and Visuospatial/Constructional memory. Results from this study confirm that hearing loss negatively impacts cognitive functioning, even in older adults with normal vestibular function.

Automated volumetric analysis of the inner ear fluid space from hydrops magnetic resonance imaging using 3D neural networks

Due to the development of magnetic resonance (MR) imaging processing technology, image-based identification of endolymphatic hydrops (EH) has played an important role in understanding inner ear illnesses, such as Meniere’s disease or fluctuating sensorineural hearing loss. We segmented the inner ear, consisting of the cochlea, vestibule, and semicircular canals, using a 3D-based deep neural network model for accurate and automated EH volume ratio calculations. We built a dataset of MR cisternography (MRC) and HYDROPS-Mi2 stacks labeled with the segmentation of the perilymph fluid space and endolymph fluid space of the inner ear to devise a 3D segmentation deep neural network model. End-to-end learning was used to segment the perilymph fluid and the endolymph fluid spaces simultaneously using aligned pair data of the MRC and HYDROPS-Mi2 stacks. Consequently, the segmentation performance of the total fluid space and endolymph fluid space had Dice similarity coefficients of 0.9574 and 0.9186, respectively. In addition, the EH volume ratio calculated by experienced otologists and the EH volume ratio value predicted by the proposed deep learning model showed high agreement according to the interclass correlation coefficient (ICC) and Bland–Altman plot analysis.

A Frameshift Variant in ANKRD24 Implicates Its Role in Human Non-Syndromic Hearing Loss.

Hearing loss (HL) is the most prevalent sensorineural disorders, affecting about one in 1000 newborns. Over half of the cases are attributed to genetic factors; however, due to the extensive clinical and genetic heterogeneity, many cases remain without a conclusive genetic diagnosis. The advent of next-generation sequencing methodologies in recent years has greatly helped unravel the genetic etiology of HL by identifying numerous genes and causative variants. Despite this, much remains to be uncovered about the genetic basis of sensorineural hearing loss (SNHL). Here, we report an Iranian consanguineous family with postlingual, moderate-to-severe autosomal recessive SNHL. After first excluding plausible variants in known deafness-causing genes using whole exome sequencing, we reanalyzed the data, using a gene/variant prioritization pipeline established for novel gene discovery for HL. This approach identified a novel homozygous frameshift variant c.1934_1937del; (p.Thr645Lysfs*52) in ANKRD24, which segregated with the HL phenotype in the family. Recently, ANKRD24 has been shown to be a pivotal constituent of the stereocilia rootlet in cochlea hair cells and interacts with TRIOBP, a protein already implicated in human deafness. Our data implicate for the first time, ANKRD24 in human nonsyndromic HL (NSHL) and expands the genetic spectrum of HL.

Neurocognitive Evaluation of Patients With DiGeorge Syndrome

BackgroundDiGeorge syndrome (DGS), the most common microdeletion syndrome, affects multiple organs, including the heart, the nervous system, and the immune system. In this study, we aimed to evaluate the clinical, laboratory, brain magnetic resonance imaging (MRI), and neurocognitive findings of our patients with DGS. MethodsClinical and laboratory data of 52 patients with DGS between June 2000 and March 2022 were evaluated retrospectively. Brain MRI and neuropsychologic tests were performed to assess the neurocognitive status of the patients. ResultsFifty-two patients (28 males and 24 females) were included in our study. Fifteen of them died during the follow-up. All 37 patients who are alive had partial DGS. The median age of patients was 10 years and 7 months, and the median age at diagnosis was 5 years and 4 months. Bilateral conduction deceleration in the anterior visual pathways in six (20%) of 30 patients was determined by the visual evoked potentials. The auditory brainstem evoked potential test showed sensorineural hearing loss in 11 of 30 (36.6%) patients. Brain MRI disclosed brain parenchymal abnormalities in 18 of 25 (72%) patients. Impairments in executive functions, expressive language, and verbal memory were noted in 18 patients who were neuropsychologically assessed. ConclusionsIt is important to keep in mind that patients with DGS may be accompanied by neurocognitive findings. Awareness of the potential for underlying psychiatric and neurodevelopment disorders is key to anticipatory guidance, optimization of therapies, and maximizing life quality.

Incidental Findings on MRIs for Asymmetric Sensorineural Hearing Loss: A Clinical and Economic Analysis.

The gold-standard assessment of asymmetric sensorineural hearing loss (ASNHL) is contrast-enhanced MRI. Although rates of identifying a vestibular schwannoma are low (<5%), it is generally accepted as cost-effective. Yet, the impact of incidentalomas is rarely considered. This study aims to characterize the incidence of incidentalomas in the workup of ASNHL and quantify the associated socioeconomic burden. Retrospective cohort study. Single academic institution in a midsized city in the United States. Radiology records were queried for MRI's ordered for ASNHL between January 2012 and November 2022. Results were characterized as "group 1: normal," "group 2: abnormal read/normal variant," "group 3: abnormal-likely cause of ASNHL," or "group 4: abnormal-follow-up needed." Subsequent costs of workup for group 4 were estimated using Medicare Physician Fee Schedule for Medicare costs, US Congressional Budgeting Office data for private insurer costs, and USC-Brookings Schaeffer Initiative for Health Policy estimates for uninsured individuals. Six hundred patients met the inclusion criteria. Eighteen (3.0%) were categorized in group 3, whereas 40 (6.7%) were categorized in group 4. Of these patients, 7.5% (n = 3) had interventions to manage their incidental findings. Estimated per patient cost for further workup of incidental findings amounted to approximately $744, $1,534, and $2,260 for Medicare, private insurance, and uninsured costs, respectively. Incidentalomas occur over twice as often as retrocochlear pathologies responsible for ASNHL. Although the number of patients requiring treatment for incidentaloma is low, the economic impact is not insubstantial and should be considered for both individual patients and health system payers.

Association of hearing loss and risk of depression: a systematic review and meta-analysis.

Previous studies have suggested a possible link between hearing loss and the risk of depression. However, the strength of this association remains uncertain across varying follow-up periods, age groups, cohort studies, and longitudinal study designs. We conducted an extensive literature search across PubMed, Embase, and the Cochrane Library databases to retrieve pertinent studies. The quality of observational studies was assessed using the Newcastle-Ottawa Scale. Synthesis and meta-analysis of odds ratios (ORs) along with corresponding 95% confidence intervals (CIs) were performed using Stata 14.0. Funnel plot analysis and Egger's regression test were utilized to assess potential publication bias. This meta-analysis comprised 24 cohort studies conducted between 2007 and 2024, with sample sizes ranging from 548 to 254,466 participants. Among these participants, 24,304 had experienced depression events. The pooling analysis shows that hearing loss is associated with an increased risk of depression (OR = 1.35; 95%CI: 1.27-1.44). In the subgroup analysis, the retrospective cohort exhibited a slightly higher risk of depression compared to the prospective cohort (OR = 1.43; 95% CI: 1.30-1.58). There are differences in the risk of depression among young, middle-aged, and older individuals, with older adults facing a higher risk (OR = 1.33, 95% CI: 1.21-1.45). Additionally, the risk of depression was slightly higher in the sudden sensorineural hearing loss (SSNHL) group compared to the non-SSNHL group (OR = 1.62; 95% CI: 1.27-2.07). Furthermore, in cohorts with a follow-up time ≥ 5 years, the risk of depression was higher compared to those with <5 years of follow-up (OR = 1.39; 95% CI: 1.26-1.54). Our meta-analysis shows that hearing loss increases the risk of depression. These findings provide evidence that hearing loss should be recognized as an independent risk factor for depression. https://www.crd.york.ac.uk/PROSPERO/, identifier: CRD42024502043.

Reply on: "Comment on Intratympanic Lidocaine as a Potent Remedy for Tinnitus in Sudden Sensorineural Hearing Loss: A Double-Blind, Randomized Clinical Trial".

Reply on: "Comment on Intratympanic Lidocaine as a Potent Remedy for Tinnitus in Sudden Sensorineural Hearing Loss: A Double-Blind, Randomized Clinical Trial".

Comment on: "Intratympanic Lidocaine as a Potent Remedy for Tinnitus in Sudden Sensorineural Hearing Loss: A Double-Blind, Randomized Clinical Trial".

Comment on: "Intratympanic Lidocaine as a Potent Remedy for Tinnitus in Sudden Sensorineural Hearing Loss: A Double-Blind, Randomized Clinical Trial".

Mitochondrial-derived peptides, HNG and SHLP3, protect cochlear hair cells against gentamicin.

Preservation of hair cells is critical for maintaining hearing function, as damage to sensory cells potentially leads to irreparable sensorineural hearing loss. Hair cell loss is often associated with inflammation and oxidative stress. One promising class of bioactive peptides is mitochondrial-derived peptides (MDPs), which have already been proven to protect various tissues from cellular stresses and delay aging processes. Humanin (HN) is one of the best-known members of this family, and recently, we have shown its protective effect in hair cells. The synthetic derivate HN S14G (HNG) has a more potent protective effect than natural HN making it a more useful peptide candidate to promote cytoprotection. A less-known MDP is small humanin-like peptide 3 (SHLP3), which has cytoprotective effects similar to HN, but likely acts through different signaling pathways. Therefore, we examined the effect of exogenous HNG and SHLP3 in auditory hair cells and investigated the molecular mechanisms involved. For this purpose, explants of the organ of Corti (OC) were treated with gentamicin in the presence and absence of HNG or SHLP3. Administration of HNG and SHLP3 reduced gentamicin-induced hair cell loss. The protective mechanisms of HNG and SHLP3 in OC explants included, in part, modulation of AKT and AMPKα. In addition, treatment with HNG and SHLP3 reduced gentamicin-induced oxidative stress and inflammatory gene overexpression. Overall, our data show that HNG and SHLP3 protect hair cells from gentamicin-induced toxicity. This offers new perspectives for the development of therapeutic strategies with MDPs against hearing loss.

Brain networks in newborns and infants with and without sensorineural hearing loss: A functional near-infrared spectroscopy study.

Understanding the impact of early sensory deficits on brain development is essential for understanding developmental processes and developing potential interventions. While previous studies have looked into the impact of prenatal experiences on language development, there is a lack of research on how these experiences affect early language and brain function development in individuals with sensorineural hearing loss (SNHL). To investigate SNHL effects on early brain development and connectivity in 4-month-olds vs healthy newborns and controls. The research involved analyzing the functional brain networks of 65 infants, categorized into three groups: 28 healthy newborns, 22 4-month-old participants with SNHL, and 15 age-matched healthy participants. The resting-state functional connectivity was measured and compared between the groups using functional near-infrared spectroscopy and graph theory to assess the brain network properties. Significant differences were found in resting-state functional connectivity between participants with SNHL and age-matched controls, indicating a developmental lag in brain connectivity for those with SNHL. Surprisingly, SNHL participants showed better connectivity development compared to healthy newborns, with connectivity strengths of 0.13 ± 0.04 for SNHL, 0.16 ± 0.08 for controls, and 0.098 ± 0.04 for newborns. Graph theory analysis revealed enhanced global brain network properties for the SNHL group, suggesting higher communication efficiency at 4 months. No significant differences were noted in network properties between 4-month-old SNHL participants and neonates. A unique pattern of central hubs was observed in the SNHL group, with 2 hubs in the left hemisphere compared to 6 in controls. 4-month-old infants with SNHL have a distinct brain network pattern with efficient long-distance information transmission but less effective local communication compared to age-matched controls.

Clinical and genetic characterisation of childhood-onset sensorineural hearing loss reveal associated phenotypes and enrichment of pathogenic founder mutations in the Finnish population

Objective To examine the clinical and genetic characteristics of childhood-onset bilateral sensorineural hearing loss (SNHL) in Finland. Design Retrospective analysis. Study sample A total of 249 children younger than 18 years were diagnosed with bilateral SNHL in Oulu University Hospital, Finland, from 2017 to 2022. Results Pathogenic or likely pathogenic gene variants or chromosome abnormalities explaining SNHL were identified in 41% (N = 101/249) of children. Likely causative variants were more commonly identified in patients with severe SNHL than in those with moderate or mild SNHL. Our study identified likely causative gene variants in 24 different genes and six different likely causative chromosome abnormalities, demonstrating the genetic heterogeneity of SNHL. Population-enriched founder mutations were identified in the CABP2, CLRN1, MYO7A, SUCLA2, TMC1, and TWNK genes. A significant number of patients had associated phenotypes, including global developmental delay or intellectual disability (16%), language disorder (20%), ophthalmological abnormalities (16%), or malformations other than those involving the ear (10%). Conclusions SNHL is genetically and clinically heterogeneous. Pathogenic variants in GJB2 were the most common. Several population-enriched variants were identified as causing SNHL in the northern Finnish population. Associated medical phenotypes are common and should be taken into account in patients’ follow-up and treatment.

Identification of a novel FGF3 variant and a new phenotype in three LAMM syndrome families

Over 700 syndromes associated with hearing loss (HL) have been identified. Labyrinthine aplasia, microtia, and microdontia (LAMM syndrome, OMIM: 610706) is a rare HL syndrome characterized by congenital sensorineural HL, labyrinthine aplasia, type I microtia and microdontia, which is caused by biallelic variants in the FGF3 gene. Using Whole-exome sequencing (WES), we identified a novel missense FGF3 variant (c.137G > C, p. Arg46Pro (NM_005247.4) in three unrelated Uyghur ethnic families. This variant is classified as a variant of uncertain significance according to ACMG guidelines, with the applied criteria of PM3, PM2_Supporting, PP3 and PP4. Patients from the three families revealed variable clinical features. We found a novel phenotype, sparse hair, in one of the proband. Our findings expanded the variant and phenotype spectrum of LAMM syndrome and provided new insights to the diagnose and pathogenesis investigation of the disease.

A Variable-Stimulus Distortion Product Otoacoustic Emission Screening Method to Match Cochlear Place-Specific Properties.

Distortion product otoacoustic emissions (DPOAEs) are a popular screening tool for hearing loss in specific populations (e.g., newborns). Current screening protocols use stimulus conditions that are agnostic to local mechanical properties of the cochlea and are also limited to a narrow frequency range. We have recently reported locally optimized stimulus frequency ratio and level combinations for recording DPOAEs up to stimulus frequencies of 19 kHz. In normally functioning cochlea, optimized stimuli improved the signal to noise ratios and allowed the registration of higher DPOAE levels, especially at higher frequencies. The purpose of this study was to evaluate the clinical performance of these physiologically motivated, locally appropriate, stimulus parameters for a screening application to identify the presence of hearing loss. Subjects were 24 adults with sensorineural hearing loss and 31 adults with normal hearing. The cubic DPOAE was measured and analyzed up to frequencies of 16 kHz using a range of stimulus conditions. Receiver operating characteristic curves were used to identify stimulus combinations most sensitive to screening for hearing loss. Receiver operating characteristic curves demonstrated improved test efficacy for hearing loss detection when using stimulus frequency ratios and levels that are frequency-dependent and consistent with known mechanical properties of the cochlea. We propose a new DPOAE recording paradigm (variable-stimuli DP) using stimuli aligned to local cochlear properties which may improve early and accurate detection of decline in cochlear function.

POLD3 haploinsufficiency is linked to non-syndromic sensorineural adult-onset progressive hearing and balance impairments.

Hearing impairment (HI) is a significant health concern globally, influenced by genetic and environmental factors. We had identified a homozygous pathogenic variant in POLD3 in a Lebanese patient with an autosomal congenital recessive syndromic hearing loss (MIM#620869). This variant was found at heterozygous state in the parents, who developed progressive hearing impairment around age 40. We conducted a thorough clinical and genetic assessment of sixteen family members, including physical exams, audiometry and vestibular function evaluations. Additionally, gene expression analysis of the Pold3 gene was performed in mice using RNAscope. Twelve individuals were heterozygous for the variant in POLD3, of whom eight showed bilateral adult-onset HI, typically starting around ages 40-50, and two older patients displaying unilateral vestibular weakness. Additionally, two carriers of the variant developed cancer at an early age. RNAscope confirmed Pold3 expression in auditory and vestibular neurons. Exome sequencing analysis excluded the presence of pathogenic variants in any known hearing impairment or cancer predisposition genes. We present herein, for the first time, evidence of a heterozygous pathogenic POLD3 variant associated with a novel form of autosomal dominant progressive adult-onset hearing and vestibular impairments. We also highlight the necessity for further exploration of the role of POLD3 in cancer predisposition.

Good cochlear implantation outcomes in subjects with mono-allelic WFS1-associated sensorineural hearing loss – a case series

Objective This study aimed to evaluate long-term cochlear implant (CI) outcomes in individuals with mono-allelic pathogenic variants in WFS1, which is associated with both Wolfram-like syndrome and DFNA6/14/38. Design Retrospective case series. Study sample Seven CI recipients, ranging from eight months to 58 years of age, were included in the study, including four with Wolfram-like syndrome and three with DFNA6/14/38. A total of ten cochlear implantations were performed among these subjects. Results At one-year post-implantation, a mean phoneme score of 90 ± 9% at 65 dB SPL in quiet was found, which remained stable up to ten years post-implantation with a mean phoneme score of 94 ± 6%. Despite these excellent outcomes, one subject achieved no speech recognition with CI and eventually became a non-user. This individual had a prolonged absence of auditory stimulation prior to implantation and encountered multiple challenges during rehabilitation. Conclusion Individuals with Wolfram-like syndrome or DFNA6/14/38 demonstrate consistently good outcomes following implantation, which remain stable over time. These findings affirm cochlear implantation as an effective rehabilitation option for these individuals. Furthermore, the stable and good CI outcomes contradict the suggested link between WFS1-associated sensorineural hearing loss and auditory neuropathy.

Sensorineural hearing loss in post-COVID-19 patients

&lt;b&gt;Background: &lt;/b&gt;Hearing loss (HL) is one of the most common chronic conditions after hypertension and arthritis. Mounting indices suggest that sensorineural hearing loss (SNHL) may be one of the adverse effects of severe acute respiratory syndrome coronavirus 2.&lt;br /&gt; &lt;b&gt;Objective: &lt;/b&gt;This work aimed at studying SNHL in post-COVID-19 patients, alongside with exploring the relationship between severity of the disease and degree of hearing disability.&lt;br /&gt; &lt;b&gt;Materials and methods: &lt;/b&gt;This prospective cross-sectional study was conducted at Al-Azhar University Hospitals, Cairo, Egypt. It included 100 post-COVID-19 patients selected according to the study inclusion and exclusion criteria. Pure tone audiometry was done to confirm the presence of HL and to determine its degree.&lt;br /&gt; &lt;b&gt;Results: &lt;/b&gt;Hundred patients with recent oncent hearing symptoms after documented COVID-19 infection were included. Males (71%) were more affected than females (29%). The most of our cases had bilateral moderate HL (45%). Tinnitus was the most frequently associated otological symptom (96.96%). The time of HL onset varied from days to weeks (55% had time onset between 11-30 days post-confirmed infection). Higher thresholds were detected in all frequencies of affected ears with more deterioration of hearing as frequencies increase. All degrees of HL were detected; mild, moderate, severe and profound. There was no significant correlation between severity of COVID-19 infection and severity of HL.&lt;br /&gt; &lt;b&gt;Conclusions: &lt;/b&gt;SNHL is one of the extrapulmonary complications of COVID-19. COVID-19 patients may develop hearing affection regardless the severity of the infection. It is recommended to assess hearing functions in patients after COVID-19 for early detection and proper management.