Sudden Hearing Loss Research Articles

Access to Sudden Hearing Loss Care at Urgent Care Centers.

To compare patient access to urgent care centers (UCCs) with a diagnosis of sudden hearing loss based on insurance. One hundred twenty-five random UCCs in states with Medicaid expansion and 125 random UCCs in states without Medicaid expansion were contacted by a research assistant posing as a family member seeking care on behalf of a patient with a one-week history of sudden, unilateral hearing loss. Each clinic was called once as a Medicaid patient and once as a private insurance (PI) patient for 500 total calls. Each phone encounter was evaluated for insurance acceptance and self-pay price. Secondary outcomes included other measures of timely/accessible care. Chi-square/McNemar's tests and independent/paired sample t-tests were performed to determine whether there were statistically significant differences between expansion status and insurance type. Calls ended before answering questions were not included in the analysis. Medicaid acceptance rate was significantly lower than PI (68.1% vs. 98.4%, p < 0.001). UCCs in Medicaid expansion states were significantly more likely to accept Medicaid (76.8% vs. 59.2%, p = 0.003). The mean wage-adjusted self-pay price was significantly greater in states with Medicaid expansion at $169.84 than in states without at $145.34 when called as a Medicaid patient (mean difference: $24.50, 95% Confidence Interval: $0.45-$48.54, p = 0.046). The rates of referral to an emergency department and self-pay price nondisclosure rates were greater for Medicaid calls than for private insurance calls (8.2% vs. 0.4% and 17.4% vs. 5.8%; p < 0.001 for both). Medicaid patients with otologic emergencies face reduced access to care at UCCs. NA Laryngoscope, 2024.

When It Rains It Pours...: Wernicke Encephalopathy with Sudden Blindness after Bariatric Surgery in a Patient with Congenital Deafness.

Malabsorption syndromes are known chronic complications of bariatric surgery. Therefore, it is recommended to take oral supplementation with multivitamins. Wernicke's encephalopathy represents an acute neuropsychiatric syndrome associated with alcoholism or severe malnutrition; sporadic cases of this potential complication related to bariatric surgery are described in the literature. We present a case of Wernicke's encephalopathy due to severe vitamin B1 deficiency after bariatric surgery. A 31-year-old woman with deaf-mutism from the age of 3 years old, operated 3 months before with a mini-gastric bypass for severe obesity, was transferred to our unit after accessing the emergency room. In the immediate medical history, there was the sudden and rapid decline in vision, leading to complete loss of vision, marked asthenia, and paresthesia in the four limbs. Considering the previous bariatric surgery, the diagnosis of non-alcoholic Wernicke's syndrome was suspected, for which IV therapy with Vitamin B1 was started at a dosage of 5 vials of 200 mg in 100 cc of saline solution (three times a day for the first 72 hours, subsequently 1 once/day). After 12 hours, there was an improvement in visual acuity, and the symptoms completely resolved within 48 hours. She was discharged with complete resolution of all symptoms after 1 month. Initial vision loss without confusion or encephalopathy is one atypical presentation of Wernicke syndrome. Clinical suspicion must be high in case of alcoholism or post-bariatric surgery. Early recognition of atypical symptoms, including vision loss, and timely administration of therapy improves the prognosis of this potentially reversible but time-dependent neurological emergency.

Posterior semicircular canal involvement in sudden hearing loss

Posterior semicircular canal involvement in sudden hearing loss

Low-intensity noise exposure takes an essential part in the mechanism of late-onset hereditary hearing loss caused by Abcc1 mutation.

With the development of the social economy, we are exposed to increasing noise in our daily lives. Our previous work found an ABCC1(NM_004996.3:c.A1769G, NP_004987.2:p.N590S) variant which cosegregated with the patients in an autosomal dominant non-syndromic hearing loss family. At present, the specific mechanism of deafness caused by ABCC1 mutation is still not clear. Using the knock-in mouse model simulating human ABCC1 mutation, we found that the occurrence of family-related phenotypes was likely attributed to the combination of the mouse genotype and low-intensity noise. GSH and GSSG are important physiological substrates of ABCC1. The destruction of GSH-GSSG balance in the cochleae of both Abcc1N591S/+ mice and Abcc1N591S/N591S mice during low-intensity noise exposure may result in irreversible damage to the hair cells of the cochleae, consequently leading to hearing loss in mice. The findings offered a potential novel idea for the prevention and management of hereditary hearing loss within this family.

Comprehensive overview of the current state and impact of cohort studies in newborn screening

Abstract Inborn errors of metabolism (IEM) are a rare and complex group of inherited disorders, typically observed in infants as autosomal recessive or, less commonly, as X-linked recessive conditions. Newborn screening (NBS) for IEMs can significantly improve prognosis and quality of life through early diagnosis and intervention, marking a significant public health achievement. NBS programs vary by country, but generally include tests for a range of metabolic errors, endocrine disorders, primary immunodeficiency disorders, congenital deafness, congenital heart defects, and cystic fibrosis. Integrating mass spectrometry (MS/MS), genetic sequencing, and emerging technologies such as next-generation sequencing into NBS programs represents a substantial advancement in the field of healthcare. These technologies enable early detection, intervention, and the possibility of personalized treatments through gene therapies. Such advancements are poised to shape the future of NBS, potentially enhancing healthcare outcomes for newborns on a global scale. Neonatal screening is the most well-known and widely implemented proactive and preventative pediatric public health initiative worldwide. It encompasses a comprehensive education system, screening, diagnostic confirmation, management, follow-up, and counseling. This article reviews recent advances in newborn metabolic screening, drawing on the results of various clinical trials and pilot studies.

Transient Evoked and Distortion-Product Otoacoustic Emissions in Normal Ear of Patients with Sudden Unilateral Sensory Neural Hearing Loss

Transient Evoked and Distortion-Product Otoacoustic Emissions in Normal Ear of Patients with Sudden Unilateral Sensory Neural Hearing Loss

Advances in vestibular function and rehabilitation of large vestibular aqueduct syndrome

Large vestibular aqueduct syndrome（LVAS） is a common recessive hereditary hearing loss disease, and some patients may also experience vestibular dysfunction. With the wide application of cochlear implant（CI） and the development of vestibular medicine, the pathophysiological mechanism of LVAS and the influence mechanism of CI on vestibular function are gradually elucidated. Consequently, the evaluation and rehabilitation of vestibular dysfunction function have also become research hotspots. This article reviews studies on vestibular function and related rehabilitation in patients with large vestibular aqueduct syndrome.

Ototoxicity in Immune Checkpoint Inhibitors Therapy

&lt;b&gt;&lt;br&gt;Introduction:&lt;/b&gt; Immune checkpoint inhibitors (ICIs) and T-cell therapies are a modern, well-established cancer treatment. The priority of oncological treatment is to cure cancer. However, treatment-related toxicities, i.e. immune-related adverse events (irAEs), continue to emerge and are not that well understood yet. ICIs can cause profound, multiple, and diverse irAEs – the sequelae of unknown mechanisms. One of the organs susceptible to collateral damage is the hearing organ. Complications related to hearing, tinnitus, and balance disorders are extremely burdensome and significantly impair many aspects of the quality of life of patients and survivors.&lt;/br&gt; &lt;b&gt;&lt;br&gt;Aim:&lt;/b&gt; The aim of the work is to review the literature in the area of ototoxicity of ICIs.&lt;/br&gt; &lt;b&gt;&lt;br&gt;Materials and method:&lt;/b&gt; A systematic search of the Web of Science, PubMed, and Embase databases for studies published until 1 March 2022 was conducted.&lt;/br&gt; &lt;b&gt;&lt;br&gt;Results:&lt;/b&gt; Reported clinical symptoms ranged from sudden bilateral hearing loss and imbalance to mild hearing loss or tinnitus with preserved hearing. It was found that the median time from ICI initiation to hearing loss development was 3 months. The hearing impairment was secondary to bilateral sensorineural hearing loss in the majority of patients (&gt;60%), and at least one other irAE accompanied the hearing loss in 2/3 of patients. Hearing loss significantly improved in 45.7% of the patients.&lt;/br&gt; &lt;b&gt;&lt;br&gt;Conclusions:&lt;/b&gt; The majority of cases of ICI-related hearing loss presented in the literature were reversible. Therefore, it is important to develop and implement routine therapeutic algorithms. Further research is needed to define the true prevalence of ICI-related hearing loss, optimal diagnostics, and management.&lt;/br&gt;

Clinical Genetic Testing for Hearing Loss: Implications for Genetic Counseling and Gene-Based Therapies.

Genetic factors contribute significantly to congenital hearing loss, with non-syndromic cases being more prevalent and genetically heterogeneous. Currently, 150 genes have been associated with non-syndromic hearing loss, and their identification has improved our understanding of auditory physiology and potential therapeutic targets. Hearing loss gene panels offer comprehensive genetic testing for hereditary hearing loss, and advancements in sequencing technology have made genetic testing more accessible and affordable. Currently, genetic panel tests available at a relatively lower cost are offered to patients who face financial barriers. In this study, clinical and audiometric data were collected from six pediatric patients who underwent genetic panel testing. Known pathogenic variants in MYO15A, GJB2, and USH2A were most likely to be causal of hearing loss. Novel pathogenic variants in the MYO7A and TECTA genes were also identified. Variable hearing phenotypes and inheritance patterns were observed amongst individuals with different pathogenic variants. The identification of these variants contributes to the continually expanding knowledge base on genetic hearing loss and lays the groundwork for personalized treatment options in the future.

Effect of acupuncture intervention time on the therapeutic effect in patients with sudden hearing loss.

To observe the clinical efficacy of acupuncture intervention at different time for patients with sudden hearing loss. According to the timing of acupuncture intervention, 86 patients were divided into early exposure group (n=43) and late exposure group (n=43) . The early exposure group was given acupuncture treatment within 14 days of onset, and the late exposure group was given acupuncture treatment after 14 days of onset. After propensity score matching (PSM, a statistical matching technique for observational data) processing by using SPSS26.0 software, outcomes of 30 cases in the early exposure group and 30 cases in the late exposure group were analyzed. In addition to receiving basic treatment with drugs for vascular dilatation, thrombolysis, nourishing nerve, etc., all patients of the two groups were treated with neck acupuncture ("Neck Seven Meridian Lines" acupuncture), once every other day except Sundays, for a total of 12 time. Before, after the treatment and 3 months after the treatment, the total score of the Tinnitus Handicap Inventory (THI, 0, 2 and 4 points for each of the 25 items, total scores = 100 points) scale was used to evaluate the improvement of tinnitus symptoms caused by hearing loss. The clinical therapeutic effect was evaluated according to the efficacy grading criteria in the Guidelines for Diagnosis and Treatment of Sudden Deafness (2015) and the changes of pure tone audiometry curve. Multivariate Logistic regression was used to analyze the effect of factors that might influence efficacy before propensity score matching. The THI scores of patients in both groups decreased strikingly after the treatment and 3 months' follow-up (P<0.05). Compared with the same time-points of the late exposure group, the total THI scores of post-treatment and 3 months' follow-up were evidently lower in the early exposure group (P<0.05). The effective rate of the early exposure group (22/30, 80.00%) was significantly higher (P<0.05) than that of the late exposure group (16/30, 53.33%). The classification of sudden deafness and the application of traditional Chinese medicine in this study were not independent factors affecting the total effective rate. The time point of acupuncture intervention is an important factor affecting the effect on hearing and tinnitus disability of patients with sudden deafness. The earlier acupuncture treatment is accepted, the better the therapeutic effect is.

Sudden Sensorineural Hearing Loss Before COVID-19, During COVID-19, and After Inactivated Vaccination: Experience From a Tertiary Referral Center in China.

The purpose of this study was to investigate the incidence rate and audiological characteristics of sudden sensorineural hearing loss (SSHL) before the COVID-19 pandemic, during the COVID-19 pandemic, and after inactivated vaccination, and to analyze whether the novel coronavirus or inactivated vaccination have an impact on hearing loss. The clinical data of all patients who visited the hospital in July 2019 (before pandemic), July 2020 (during pandemic) and July 2021 (after the inactivated vaccination at least the first basic dose of COVID-19 vaccination) were retrospective analyzed. All patients diagnosed with sudden sensorineural hearing loss in these three periods were screened, and their incidence rate, hearing characteristics, and prognosis were compared. Overall, the incidence rates of sudden hearing loss in July 2019, July 2020, and July 2021 were 0.59, 0.60, and 0.52% (25 of 4225, 20 of 3322, 28 of 5432), respectively, without significant difference ( χ2 = 0.372, p = 0.830). A significant difference was noted in the incidence of hypertension ( p = 0.02), whereas no significant difference was noted in age ( p = 0.591, p = 0.66), sex ( p = 0.19, p = 0.08), main symptoms ( p = 0.18, p = 0.15), side ( p = 0.483, p = 0.89), audiogram shape ( p = 0.56, p = 0.989), average hearing threshold of affected frequency ( p = 0.81, p = 0.89), average hearing threshold of normal ear affected frequency ( p = 0.65, p = 0.68), average hearing threshold of affected frequency after treatment ( p = 0.49, p = 0.38), days between symptoms onset and hospital visit ( p = 0.62, p = 0.85), treatment plan ( p = 0.551, p = 0.474), and effectiveness ( p = 0.104, p = 0.050). Our study failed to find a correlation between the novel coronavirus pandemic and inactivated vaccination and SSHL, and there was no direct evidence supporting that COVID-19 or inactivated vaccination had an impact on the incidence rate and prognosis of SSHL patients.

Clinical characteristics of patients diagnosed with bilateral sudden sensorineural hearing loss.

This study investigated the etiology, clinical features, and prognosis of patients diagnosed with bilateral sudden sensorineural hearing loss (BSSNHL). The clinical data of 100 patients with bilateral sudden hearing loss as a chief complaint treated at Xiangya Second Hospital of Central South University between January 2010 and August 2022, including clinical characteristics, audiometric data, and prognosis, were retrospectively analyzed. These 100 cases accounted for 8.09% (100/1235) of all patients admitted for sudden sensorineural hearing loss (SSNHL) during the same period. Of these, 71 were simultaneous cases and 29 were sequential cases of BSSNHL. Among the 200 ears analyzed in this study, 13, 36, 57, and 94 had mild, moderate, severe, and profound sensorineural hearing loss, respectively. The overall effective rate after comprehensive treatment was 32%, with significant differences in efficacy and prognosis among different degrees of hearing loss (p < 0.05). Comorbidities of hypertension (24 cases), diabetes (14 cases), and coronary heart disease (9 cases) significantly impacted therapeutic efficacy and prognosis in patients with BSSNHL (p < 0.05). Compared to unilateral SSNHL, BSSNHL exhibits distinctive characteristics.

Case Report - Sudden sensorineural hearing loss and vertigo due to inner ear hemorrhage

Sudden deafness is a common cause of emergency consultation in otology. Usually, despite investigations, no etiology is known. Intracochlear hemorrhage is a rare cause of sudden sensorineural hearing loss (SSNHL) and may be associated with various labyrinthine disorders. In such cases, magnetic resonance imaging (MRI) is the clef of the diagnosis. We report the case of a 70-year-old patient who was referred to our department for sudden hearing loss, tinnitus, and vertigo. Otoscopic and neurological examinations were normal, and pure-tone audiometry revealed left profound sensorineural hearing loss. Videonystagmography (VNG) revealed left vestibular deficit. MRI demonstrated a high signal intensity inside the cochlea on unenhanced T1-weighted images, and no other abnormalities were found; in particular, no enhancement after intravenous administration of gadolinium. No etiology was identified. Vertigo disappeared rapidly with corticosteroid treatment and hyperbaric oxygen therapy, but hearing did not improve. Intra-labyrinthine hemorrhage causing SSNHL is rare, and the hearing prognosis is poor.

Sudden Hearing Loss before, during, and after the Pandemic: Investigating COVID-19 Illness and Vaccine-Related Symptoms.

Accumulating reports suggest an increase in sudden sensorineural hearing loss during the COVID-19 pandemic and vaccination periods. However, clear evidence is lacking. The goal of this study was to determine if sudden sensorineural hearing loss is associated with COVID-19 illness or its vaccine. Retrospective chart review of 50 randomly selected patients from three, 6-month time periods: "pre-pandemic," "early pandemic," and "late pandemic." Group comparisons were performed for demographics, comorbid conditions, audiologic history, audiometric data, speech reception thresholds, and word recognition. One hundred 50 patients were included in this study. A mean difference was observed in that the relative percentage of sensorineural hearing loss (SNHL) cases increased over time, corresponding to a relative decrease in conductive hearing loss cases. However, this change was not explained by proportional changes in sudden SNHL. Patients in the early pandemic time period were more likely to report tinnitus. Otherwise, the patient groups did not differ on demographic variables, hearing health history, hearing loss presentation, pure tone averages, speech reception thresholds, or word recognition performance. Proportion of patients with sudden sensorineural hearing loss did not change over time from the pre-pandemic period to the early or late pandemic phases. Despite a randomized sample, these findings do not support the hypothesis that COVID-19 illness or vaccine is associated with sudden sensorineural hearing loss.

Generation of an induced pluripotent stem cell line from a late-onset, progressive high frequency hearing loss patient due to mutation in CDH23

Cadherin 23 (CDH23) is one of the most common genes responsible for hereditary hearing loss; a mutation of CDH23 can cause a wide range of symptoms depending on the variant. In this study, an iPSC line was generated from a patient with late-onset, progressive high frequency hearing loss caused by c.[719C > T];[6085C > T]:p.[P240L];[R2029W] compound heterozygous variants of CDH23. The cells were confirmed to have a normal karyotype, express markers of pluripotency, and have tri-embryonic differentiation potential. This disease-specific iPSC line will further the construction of disease models and the elucidation of the pathophysiology of CDH23 mutations.

Human Organoids for Rapid Validation of Gene Variants Linked to Cochlear Malformations.

Developmental anomalies of the hearing organ, the cochlea, are diagnosed in approximately one-fourth of individuals with congenital deafness. Most patients with cochlear malformations remain etiologically undiagnosed due to insufficient knowledge about underlying genes or the inability to make conclusive interpretations of identified genetic variants. We used exome sequencing for genetic evaluation of hearing loss associated with cochlear malformations in three probands from unrelated families. We subsequently generated monoclonal induced pluripotent stem cell (iPSC) lines, bearing patient-specific knockins and knockouts using CRISPR/Cas9 to assess pathogenicity of candidate variants. We detected FGF3 (p.Arg165Gly) and GREB1L (p.Cys186Arg), variants of uncertain significance in two recognized genes for deafness, and PBXIP1(p.Trp574*) in a candidate gene. Upon differentiation of iPSCs towards inner ear organoids, we observed significant developmental aberrations in knockout lines compared to their isogenic controls. Patient-specific single nucleotide variants (SNVs) showed similar abnormalities as the knockout lines, functionally supporting their causality in the observed phenotype. Therefore, we present human inner ear organoids as a tool to rapidly validate the pathogenicity of DNA variants associated with cochlear malformations.

Genome-wide association study for age-related hearing loss in CFW mice.

Age-related hearing impairment is the most common cause of hearing loss and is one of the most prevalent conditions affecting the elderly globally. It is influenced by a combination of environmental and genetic factors. The mouse and human inner ears are functionally and genetically homologous. Investigating the genetic basis of age-related hearing loss (ARHL) in an outbred mouse model may lead to a better understanding of the molecular mechanisms of this condition. We used Carworth Farms White (CFW) outbred mice, because they are genetically diverse and exhibit variation in the onset and severity of ARHL. The goal of this study was to identify genetic loci involved in regulating ARHL. Hearing at a range of frequencies was measured using Auditory Brainstem Response (ABR) thresholds in 946 male and female CFW mice at the age of 1, 6, and 10 months. We obtained genotypes at 4.18 million single nucleotide polymorphisms (SNP) using low-coverage (mean coverage 0.27x) whole-genome sequencing followed by imputation using STITCH. To determine the accuracy of the genotypes we sequenced 8 samples at >30x coverage and used calls from those samples to estimate the discordance rate, which was 0.45%. We performed genetic analysis for the ABR thresholds for each frequency at each age, and for the time of onset of deafness for each frequency. The SNP heritability ranged from 0 to 42% for different traits. Genome-wide association analysis identified several regions associated with ARHL that contained potential candidate genes, including Dnah11, Rapgef5, Cpne4, Prkag2, and Nek11. We confirmed, using functional study, that Prkag2 deficiency causes age-related hearing loss at high frequency in mice; this makes Prkag2 a candidate gene for further studies. This work helps to identify genetic risk factors for ARHL and to define novel therapeutic targets for the treatment and prevention of ARHL.

Gene therapy for hereditary deafness.

Gene therapy for hereditary deafness.

Bilateral gene therapy in children with autosomal recessive deafness 9: single-arm trial results

Gene therapy is a promising approach for hereditary deafness. We recently showed that unilateral AAV1-hOTOF gene therapy with dual adeno-associated virus (AAV) serotype 1 carrying human OTOF transgene is safe and associated with functional improvements in patients with autosomal recessive deafness 9 (DFNB9). The protocol was subsequently amended and approved to allow bilateral gene therapy administration. Here we report an interim analysis of the single-arm trial investigating the safety and efficacy of binaural therapy in five pediatric patients with DFNB9. The primary endpoint was dose-limiting toxicity at 6 weeks, and the secondary endpoint included safety (adverse events) and efficacy (auditory function and speech perception). No dose-limiting toxicity or serious adverse event occurred. A total of 36 adverse events occurred. The most common adverse events were increased lymphocyte counts (6 out of 36) and increased cholesterol levels (6 out of 36). All patients had bilateral hearing restoration. The average auditory brainstem response threshold in the right (left) ear was >95 dB (>95 dB) in all patients at baseline, and the average auditory brainstem response threshold in the right (left) ear was restored to 58 dB (58 dB) in patient 1, 75 dB (85 dB) in patient 2, 55 dB (50 dB) in patient 3 at 26 weeks, and 75 dB (78 dB) in patient 4 and 63 dB (63 dB) in patient 5 at 13 weeks. The speech perception and the capability of sound source localization were restored in all five patients. These results provide preliminary insights on the safety and efficacy of binaural AAV gene therapy for hereditary deafness. The trial is ongoing with longer follow-up to confirm the safety and efficacy findings. Chinese Clinical Trial Registry registration: ChiCTR2200063181.

Investigation of Distance Education Processes of Students with Hard of Hearing in Inclusive Education Practices

The aim of this study is to examine the experiences of students with hard of hearing (HH) in inclusive education practices, their teachers and parents regarding the distance education process. This research was designed with the phenomenological approach from qualitative research methods. The participants consisted of five secondary school students with HH studying at general education schools, their five parents, and ten teachers teaching students with HH. The data was collected in the 2021-2022 academic year through semi-structured interviews. In the study, it was determined that students with HH show reluctant and irregular participation in online courses. On the other hand, it was concluded that online courses were not adapted to the special needs of students and they did not get support special education services. All parents of students with HH were unable to participate in the distance education process. Distance education could be used in only support education or not at all unless it is necessary. The anxiety disorder, anger control problems and behavioural disorders brought on by adolescence increased with the decrease in socialisation in COVID-19-related closures.