A novel heterozygous variant of the SALL1 gene with atypical Townes-Brocks syndrome phenotypes in Chinese family.

Townes-Brocks syndrome (TBS) is an autosomal dominant disorder characterised by the triad of anorectal, thumb, and ear malformations. It may also be accompanied by defects in kidney, heart, eyes, hearing, and feet. TBS has been demonstrated to result from heterozygous variants in the SALL1 gene, which encodes zinc finger protein believed to function as a transcriptional repressor. The clinical characteristics of an atypical TBS phenotype patient from a Chinese family are described, with predominant manifestations including external ear dysplasia, unilateral renal hypoplasia with mild renal dysfunction, and hearing impairment. A novel heterozygous variant c.3060T>A (p.Tyr1020*) in exon 2 of the SALL1 gene was identified in this proband. Pyrosequencing of the complementary DNA of the proband revealed that the variant transcript accounted for 48% of the total transcripts in peripheral leukocytes, indicating that this variant transcript has not undergone nonsense-mediated mRNA decay. This variant c.3060T > A is located at the terminal end of exon 2, proximal to the 3' end of the SALL1 gene, and exerts a relatively minor impact on protein function. We suggest that the atypical TBS phenotype observed in the proband may be attributed to the truncated protein retaining partial SALL1 function.

Hearing loss in beta-thalassaemia: An Italian multicentre case-control study.

BACKGROUND: Despite numerous studies, the true scenario of hearing loss in beta-thalassaemia remains rather nebulous. MATERIALS AND METHODS: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non-transfusion-dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. RESULTS: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non-transfusion-dependent vs. 87/310 transfusion-dependent patients; p = 0.16). Sensorineural-notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non-significantly lower compared to chelation-treated patients while it was significantly higher compared to controls (p = 0.003). CONCLUSIONS: Sensorineural hypoacusis rate is high in beta-thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural-notch in beta-thalassaemia appears questionable.

The therapeutic effect of hyperbaric oxygen therapy in patients with severe to profound idiopathic sudden sensorineural hearing loss.

The optimal treatment for sudden sensorineural hearing loss (SSNHL) is unclear. Hyperbaric oxygen therapy (HBOT) has been suggested as a viable option for treatment of SSNHL as it improves vascular dysfunction. In this study, we evaluated the therapeutic effects of HBOT by retrospectively reviewing the records of 2206 patients with SSNHL. 54 who had received HBOT were selected for the HBOT groups, while 59 age-matched controls who had not were selected for the control groups. The HBOT and control groups were divided into subgroups according to intratympanic steroid (ITS) use. Groups A-D had received oral steroids + HBOT, oral steroids only, oral steroids + ITS + HBOT, and oral steroids + ITS, respectively. Of the 113 SSNHL patients, 21 had diabetes mellitus (DM) (2, 0, 9, and 10 patients in Groups A-D, respectively). There was no notable difference in hearing improvement between patients receiving HBOT and those in the control group. However, among diabetic patients, those who underwent HBOT demonstrated a significant improvement in hearing when compared to the control group. The combination of HBOT and steroids could potentially be beneficial for treating severe to profound SSNHL patients with DM.

The psychological intervention and clinical efficacy of Rosenthal effect and acupuncture bleeding on middle-aged and young patients with sudden hearing loss.

Combining traditional Chinese medicine theory and modern medical knowledge, this study explores the pathogenesis of sudden hearing loss in middle-aged and young people. Sixty-four young and middle-aged patients with sudden hearing loss who visited a public tertiary hospital in China are chosen as experimental objects. All experimental patients are broken into an experimental group (n = 32) and a control group (n = 32). The control group receive conventional Western medicine treatment regimen. The experimental group receive select acupoint acupuncture and bloodletting combined with Rosenthal effect for psychological intervention, and both groups have a treatment course of 14 days. The changes in the patient's condition before and after treatment are observed, and the differences in hearing threshold values, tinnitus, and dizziness clinical efficacy before and after treatment are observed and recorded. It evaluates the efficacy using the Anxiety, Depression Scale, and Hope Scale and statistically analyzes the data. The dizziness score of the experimental group decreased rapidly, the treatment onset time was shorter, and the improvement effect on dizziness symptoms was better (P < .05). After 1 month of intervention treatment, the intervention of the experimental group was better (P < .05). The hope level and self-efficacy of both groups of patients were raised in contrast with before treatment (P < .05). After 1 month, the intervention effect of the experimental group was more significant (P < .01). Both groups could improve patient ear blood circulation, but the experimental group had lower plasma viscosity, hematocrit, and red blood cell aggregation index, higher red blood cell deformation index, and more significant improvement effect (P < .05). The effective rates of improving hearing and tinnitus in the experimental group reached 87.5% and 81.5%, and the clinical treatment efficacy was better than that in the control group (P < .05). The level of depression and anxiety in the experimental group remained relatively stable, while that in the control group showed a significant rebound (P < .05). In conclusion, both groups had a certain effect in treating sudden deafness, both of which could effectively improve the patient's hearing. But in contrast with the control group, the experimental group had better clinical efficacy, higher safety, and better psychological intervention results, which is worthy of clinical promotion.

The Effectiveness of Salvage Hyperbaric Oxygen Therapy Following Combined Steroid Therapy for Refractory Sudden Sensorineural Hearing Loss.

OBJECTIVE: Hyperbaric oxygen therapy (HBOT) is an accepted treatment option for sudden sensorineural hearing loss (SSNHL), but it is still recommended in combination with corticosteroids. We investigated the efficacy of salvage HBOT in refractory SSNHL that does not respond to corticosteroid combination therapy. METHODS: Eighty-four patients were included, who had unilateral SSNHL with an improvement of pure-tone average (PTA) less than 10 dB after using intratympanic plus systemic corticosteroids (combined therapy) as the initial therapy. The control group (n = 66) received no further treatment, and the HBOT group (n = 18) received additional treatment with HBOT (10 sessions in total with 2.5 atmospheres absolute for 1 hour). RESULTS: No differences in PTA or WDS were found between the 2 groups. However, the mean hearing gain in the HBOT group (16.8 ± 4.49 dB) was significantly higher than that in the control group (4.45 ± 1.03 dB) (P = .015). The proportion of patients with hearing recovery (hearing gain of 10 dB or more) after treatment was significantly higher in HBOT group (38.9%) than in the control group (10.6%). CONCLUSIONS: In patients with refractory SSNHL after steroid combined therapy, salvage HBOT showed a significant effect on hearing gain and recovery rate.

Deep Learning Models for Predicting Hearing Thresholds Based on Swept-Tone Stimulus-Frequency Otoacoustic Emissions.

OBJECTIVES: This study aims to develop deep learning (DL) models for the quantitative prediction of hearing thresholds based on stimulus-frequency otoacoustic emissions (SFOAEs) evoked by swept tones. DESIGN: A total of 174 ears with normal hearing and 388 ears with sensorineural hearing loss were studied. SFOAEs in the 0.3 to 4.3 kHz frequency range were recorded using linearly swept tones at a rate of 2 Hz/msec, with stimulus level changing from 40 to 60 dB SPL in 10 dB steps. Four DL models were used to predict hearing thresholds at octave frequencies from 0.5 to 4 kHz. The models-a conventional convolutional neural network (CNN), a hybrid CNN-k-nearest neighbor (KNN), a hybrid CNN-support vector machine (SVM), and a hybrid CNN-random forest (RF)-were individually built for each frequency. The input to the DL models was the measured raw SFOAE amplitude spectra and their corresponding signal to noise ratio spectra. All DL models shared a CNN-based feature self-extractor. They differed in that the conventional CNN utilized a fully connected layer to make the final regression decision, whereas the hybrid CNN-KNN, CNN-SVM, and CNN-RF models were designed by replacing the last fully connected layer of CNN model with a traditional machine learning (ML) regressor, that is, KNN, SVM, and RF, respectively. The model performance was evaluated using mean absolute error and SE averaged over 20 repetitions of 5 × 5 fold nested cross-validation. The performance of the proposed DL models was compared with two types of traditional ML models. RESULTS: The proposed SFOAE-based DL models resulted in an optimal mean absolute error of 5.98, 5.22, 5.51, and 6.06 dB at 0.5, 1, 2, and 4 kHz, respectively, superior to that obtained by the traditional ML models. The produced SEs were 8.55, 7.27, 7.58, and 7.95 dB at 0.5, 1, 2, and 4 kHz, respectively. All the DL models outperformed any of the traditional ML models. CONCLUSIONS: The proposed swept-tone SFOAE-based DL models were capable of quantitatively predicting hearing thresholds with satisfactory performance. With DL techniques, the underlying relationship between SFOAEs and hearing thresholds at disparate frequencies was explored and captured, potentially improving the diagnostic value of SFOAEs.

An Italian case series' description of thiamine responsive megaloblastic anemia syndrome: importance of early diagnosis and treatment.

BACKGROUND: Individuals with thiamine-responsive megaloblastic anemia (TRMA) mainly manifest macrocytic anemia, sensorineural deafness, ocular complications, and nonautoimmune diabetes. Macrocytic anemia and diabetes may be responsive to high-dosage thiamine treatment, in contrast to sensorineural deafness. Little is known about the efficacy of thiamine treatment on ocular manifestations. CASES PRESENTATION: Our objective is to report data from four Italian TRMA patients: in Cases 1, 2 and 3, the diagnosis of TRMA was made at 9, 14 and 27 months. In 3 out of 4 subjects, thiamine therapy allowed both normalization of hyperglycemia, with consequent insulin suspension, and macrocytic anemia. In all Cases, thiamine therapy did not resolve the clinical manifestation of deafness. In Cases 2 and 3, follow-up showed no blindness, unlike Case 4, in which treatment was started for megaloblastic anemia at age 7 but was increased to high doses only at age 25, when the genetic diagnosis of TRMA was performed. CONCLUSIONS: Early institution of high-dose thiamine supplementation seems to prevent the development of retinal changes and optic atrophy in TRMA patients. The spectrum of clinical manifestations is broad, and it is important to describe known Cases to gain a better understanding of this rare disease.

Auditory enhancement in younger and older listeners with normal and impaired hearinga).

Auditory enhancement is a spectral contrast aftereffect that can facilitate the detection of novel events in an ongoing background. A single-interval paradigm combined with roved frequency content between trials can yield as much as 20 dB enhancement in young normal-hearing listeners. This study compared such enhancement in 15 listeners with sensorineural hearing loss with that in 15 age-matched adults and 15 young adults with normal audiograms. All groups were presented with stimulus levels of 70 dB sound pressure level (SPL) per component. The two groups with normal hearing were also tested at 45 dB SPL per component. The hearing-impaired listeners showed very little enhancement overall. However, when tested at the same high (70-dB) level, both young and age-matched normal-hearing listeners also showed substantially reduced enhancement, relative to that found at 45 dB SPL. Some differences in enhancement emerged between young and older normal-hearing listeners at the lower sound level. The results suggest that enhancement is highly level-dependent and may also decrease somewhat with age or slight hearing loss. Implications for hearing-impaired listeners may include a poorer ability to adapt to real-world acoustic variability, due in part to the higher levels at which sound must be presented to be audible.

Case Report: Leptospirosis Complicated by Persistent, Bilateral Sensorineural Hearing Loss.

The clinical manifestations of leptospirosis range from mild to life-threatening and can impact on multiple organ systems. A wide array of neurological manifestations of leptospirosis have been reported, although the pathophysiology of neuroleptospirosis remains incompletely understood. We present a case of leptospirosis complicated by bilateral sensorineural deafness, with nodular meningitis demonstrated in the internal auditory meatus on magnetic resonance imaging. The patient was treated with doxycycline, ceftriaxone, systemic and topical steroids, and hyperbaric oxygen therapy, with modest, but incomplete, improvement.

Sensorineural Hearing Loss due to Acute Carbon Monoxide Poisoning.

Carbon monoxide (CO) can cause "irreversible" severe-to-profound sensorineural hearing loss. However, there are few reports of detailed hearing test results. Here, we report a case of acute sensorineural hearing loss caused by acute CO poisoning with partial hearing recovery, evaluated by a detailed hearing examination. A 25-year-old woman was brought to the emergency department for attempted suicide. On admission, her consciousness was impaired, and she was treated for severe CO poisoning, including using hyperbaric-oxygen therapy. After regaining consciousness, symptoms of hearing loss and tinnitus were discovered, and a detailed audiological examination revealed bilateral hearing loss, suggesting cochlear damage. Steroids were systemically administered, and her hearing impairment was partially resolved. Sensorineural hearing loss caused by acute CO poisoning includes cochlear pathology and may be partially treatable. The early evaluation of hearing in patients with severe CO poisoning is advisable for early treatment.

A Case Report of Riboflavin Treatment and Cochlear Implants in a 4-Year-Old Girl with Progressive Hearing Loss and Delayed Speech Development: Brown-Vialetto-Van Laere Syndrome.

BACKGROUND Brown-Vialetto-Van Laere (BVVL) syndrome is a rare autosomal recessive disorder caused by mutations in intestinal riboflavin transporter genes, resulting in a motor neuron disorder of childhood, which can be associated with sensorineural deafness. This report describes a 4-year-old Polish girl with progressive hearing loss and delayed speech development diagnosed with Brown-Vialetto-Van Laere syndrome who was treated with riboflavin (vitamin B2) and cochlear implants. CASE REPORT The case report concerns a girl from Poland who, at the age of 2 years 10 months, developed progressive atypical neurological symptoms of unknown etiology: ataxia of the upper and lower limbs, gait abnormalities, generalized muscle weakness, visual and hearing problems, and regression of speech development. A karyotype study (whole-exome sequencing) revealed alterations within SLC52A2, leading to the diagnosis of Brown-Vialetto-Van Laere syndrome and initiation of high-dose riboflavin treatment. As a 4-year-old child, she presented to the Institute of Physiology and Pathology of Hearing - World Hearing Center in Poland with progressive hearing loss and speech regression. Hearing tests revealed bilateral profound sensorineural hearing loss with auditory neuropathy. Surgical treatment was applied in the form of bilateral cochlear implantation. CONCLUSIONS This report shows the importance of genetic testing in infants who present with atypical symptoms or signs. In this case, the diagnosis of Brown-Vialetto-Van Laere syndrome resulted in timely correction of the genetic riboflavin (vitamin B2) deficiency and improved hearing following the use of cochlear implants.

Indications of Effective Hyperbaric Oxygen Therapy Combined With Steroid Therapy for Sudden Hearing Loss.

OBJECTIVE: This study evaluated the therapeutic effect of hyperbaric oxygen therapy (HBOT) combined with steroid therapy to treat sudden hearing loss and examined the index associated with excellent therapeutic effect. METHODS: We included 109 patients with sudden hearing loss. Patients were divided into the HBOT combination group (59 sides) treated with HBOT and steroid therapy and HBOT noncombination group (50 sides) involving steroid therapy only. The recovery rate of each group was compared according to the severity of hearing loss. Blood samples were evaluated and inflammatory markers, such as neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR), were calculated and compared by severity. We evaluated the usefulness of inflammatory markers for predicting recovery rate, and calculated cutoff values were also evaluated. RESULTS: The HBOT combination group exhibited a higher overall recovery rate than the HBOT noncombination group, particularly in severe cases. However, there was no significant difference in the severity of hearing loss based on various inflammatory markers. NLR and PLR are useful for predicting the effect in patients treated with concomitant HBOT. By setting 2.43 and 146.67 as cutoff values for NLR and PLR, respectively, we observed that lower values resulted in better recovery rates. CONCLUSION: The use of HBOT is effective for severe cases and early blood flow disorders with low NLR and PLR and less inflammation. When determining treatment, not only should the severity of hearing loss be considered, but also the NLR and PLR should be evaluated and examined based on the cutoff values.

Analysis of Clinical Prognosis of Totally Deaf Idiopathic Sudden Sensorineural Hearing Loss in Different Ages.

Objective: The purpose of this study is to analyze the clinical prognosis of idiopathic sudden sensorineural hearing loss in patients with total deafness, and to check the differences in different age groups and hearing loss degrees. Methods: From February 2021 to January 2022, 75 patients with complete deafness and idiopathic sudden sensorineural hearing loss were analyzed retrospectively. According to different age stages, they were divided into five groups: children's group, youth group, middle-aged group, pre-senile group and elderly group, with 15 cases in each group. According to the degree of initial hearing loss, the patients were divided into three groups: 81-89dbhl (81dB Hearing Loss Group), 90-99 dbhl (90 dB group) and ≥100 dbhl (100 dB group), with 25 cases in each group. All patients were given routine comprehensive treatment. Analyze the general situation of each group, the relationship between each age group and the degree of hearing loss, the relationship between each age group and the curative effect, the relationship between each hearing loss group and the curative effect, and the relationship between each age group and the vertigo. To analyze the relationship between hearing loss group and curative effect. Results: (1) Patients with idiopathic sudden sensorineural hearing loss had more unilateral hearing loss than bilateral hearing loss, P < .05. (2) The clinical therapeutic effect of (3) 81 dB group was the highest, followed by 90 dB group. Compared with the total effective rate data of 100 dB group, P < .05. (4) The clinical efficacy of patients with complete deafness and idiopathic sudden sensorineural hearing loss without vertigo in all ages was higher than that of patients with vertigo (P < .05). The clinical effect of hearing loss in patients without vertigo was higher than that in patients with vertigo (P < .05). Conclusion: The patients with total deafness and Idiopathic sudden sensorineural hearing loss are mainly young and middle-aged people aged 14-59, with 100 dB as the boundary, and important information for predicting clinical prognosis and curative effect can be obtained.It is also suggested that for young and middle-aged patients aged 14-59, the focus of treatment may be more on restoring hearing function so that they can continue their work, study and social activities. For patients with mild hearing loss, the goal of treatment may be to maintain the existing hearing level or slow down the process of hearing loss. For patients with severe hearing loss, the focus of treatment may be to restore hearing function to improve their quality of life.

Benefit of high-dose oral riboflavin therapy in riboflavin transporter deficiency.

Riboflavin transporter deficiency (RTD) is a progressive inherited neuropathy of childhood onset, characterised by pontobulbar palsy, sensorineural deafness, sensory ataxia, muscle weakness, optic atrophy and respiratory failure. Riboflavin supplementation is beneficial in short-term reports, but the quantum of benefit in various clinical domains is not well understood. A PubMed search was conducted, which identified 94 genetically confirmed cases of RTD who received riboflavin supplementation and had follow-up assessments. Information on the clinical and functional status before and after riboflavin supplementation was collected and analysed. Seventy-six of the 94 patients (80.9%) showed an overall improvement after riboflavin supplementation, and the remaining (19.1%) were stable, though some patients had deteriorations in individual domains with no reported deaths. The domains that had the highest rates of response to riboflavin supplementation were gross motor function (93.3% improved), bulbar palsy (91.3%) and ataxia (90.0%). Improvements were also seen in limb muscle weakness, audiology, facial nerve palsy and respiratory function. Despite treatment, many patients required assistance to ambulate and had severe or profound hearing loss and some remained gastrostomy or tracheostomy dependent. Riboflavin supplementation is a lifesaving intervention for patients with RTD and results in a profound improvement in several functional domains, with early diagnosis and treatment further improving outcomes. Despite treatment, patients are left with residual disability. There is a need to accurately measure functional outcomes in children with RTD and develop additional disease-modifying therapies.

Space Station-like Composite Nanoparticles for Co-Delivery of Multiple Natural Compounds from Chinese Medicine and Hydrogen in Combating Sensorineural Hearing Loss.

Ototoxic drugs such as aminoglycoside antibiotics and cisplatin (CDDP) can cause sensorineural hearing loss (SNHL), which is closely related to oxidative stress and the acidification of the inner ear microenvironment. Effective treatment of SNHL often requires multifaceted approach due to the complex pathology, and drug combination therapy is expected to be at the forefront of modern hearing loss treatment. Here, space-station-like composite nanoparticles (CCC@mPP NPs) with pH/oxidation dual responsiveness and multidrug simultaneous delivery capability were constructed and then loaded with various drugs including panax notoginseng saponins (PNS), tanshinone IIA (TSIIA), and ammonia borane (AB) to provide robust protection against SNHL. Molecular dynamics simulation revealed that carboxymethyl chitosan/calcium carbonate-chitosan (CCC) NPs and monomethoxy poly(ethylene glycol)-PLGA (mPP) NPs can rendezvous and dock primarily by hydrogen bonding, and electrostatic forces may be involved. Moreover, CCC@mPP NPs crossed the round window membrane (RWM) and entered the inner ear through endocytosis and paracellular pathway. The docking state was basically maintained during this process, which created favorable conditions for multidrug delivery. This nanosystem was highly sensitive to pH and reactive oxygen species (ROS) changes, as evidenced by the restricted release of payload at alkaline condition (pH 7.4) without ROS, while significantly promoting the release in acidic condition (pH 5.0 and 6.0) with ROS. TSIIA/PNS/AB-loaded CCC@mPP NPs almost completely preserved the hair cells and remained the hearing threshold shift within normal limits in aminoglycoside- or CDDP-treated guinea pigs. Further experiments demonstrated that the protective mechanisms of TSIIA/PNS/AB-loaded CCC@mPP NPs involved direct and indirect scavenging of excessive ROS, and reduced release of pro-inflammatory cytokines. Both in vitro and in vivo experiments showed the high biocompatibility of the composite NPs, even after long-term administration. Collectively, this work suggests that composite NPs is an ideal multi-drug-delivery vehicle and open new avenues for inner ear disease therapies.

Clinical role of vitamin D, vitamin B12, folate levels and hematological parameters in patients with sudden sensorineural hearing loss.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL), which is frequently observed in otolaryngology clinics, is characterized by sudden onset hearing loss that can develop within hours or days. Its etiology is still not fully understood. AIMS/OBJECTIVES: This research aims to identify prognostic biomarkers that can be utilized to assess the progress of SSNHL as well as circumstances that may predispose individuals to the disease. MATERIALS AND METHODS: Between 1 January 2019 and 1 October 2020, patients diagnosed with SSNHL in our clinic and a control group consisting of healthy people were examined retrospectively. The files of the groups were examined and the levels of hemoglobin, Neutrophil-Lymphocyte Ratio (NLR), ferritin, iron, iron binding capacity (UIBC), vitamin D, vitamin B12, folate were statistically compared with the control group. In addition, the effect of the parameters studied in the patient group on the degree of recovery was examined. RESULTS: There was no statistically significant difference between the groups in terms of mean age and gender distribution (p > .05). NLR level of the patient group was higher than the control group (p < .05). Vitamin D level of the patient group was lower than the control group (p < .05). NLR and vitamin D levels had no effect on the degree of recovery (p > .05). There was no significant difference between the groups in terms of other parameters (p > .05). CONCLUSIONS AND SIGNIFICANCE: We think that our study can be a guide for adding vitamin D as a routine laboratory test in patients with SSNHL. In addition, we think that NLR value can be used as a marker in patients with SSNHL. It is recommended to investigate the role of vitamin D supplementation in better and faster response in these patients.

The reasons that predispose to SSNHL are not fully elucidated. However, there is no laboratory marker that can be used in this disease.In our study, the prevalence of vitamin D deficiency was found to be higher than in healthy people. These results suggest that vitamin D deficiency may be associated with sudden sensorineural hearing loss.The neutrophil-lymphocyte ratio was found to be higher in the patient group. We think that the NLR value obtained from the complete blood count, which is a simple and inexpensive laboratory test, can be used as a marker in patients with SSNHL.

Steroid Efficacy on Audiologic Recovery in Patients With Sudden Sensorineural Hearing Loss and Vestibular Schwannoma: A Retrospective Review.

OBJECTIVES: The objectives of this study are to (i) estimate the incidence of vestibular schwannoma (VS) among patients in an integrated healthcare system who present for evaluation of sudden sensorineural hearing loss (SSNHL) and (ii) evaluate the efficacy of empiric steroid therapy on audiologic recovery among SSNHL patients ultimately diagnosed with VS. METHODS: A retrospective chart review was performed on patients presenting with SSNHL in 2021 at a multicenter integrated healthcare system serving over 4 million members. Patient demographics, audiometric data, VS diagnosis, therapeutic steroid intervention, and data regarding treatment response were recorded. A clinically significant audiometric improvement was defined as (i) an increase of 15% in word recognition score, (ii) a decrease of 15 dB in four-frequency pure-tone average (PTA) using frequencies of 500, 1000, 2000, and 4000 Hz, or (iii) a PTA of <20 dB on follow-up audiogram. RESULTS: Six hundred fifty-eight patients were reviewed, of which 309 (56.0% male; mean, 57.5 years) met the inclusion criteria with audiometric data and magnetic resonance imaging data. Ten patients (70.0% male; mean, 51.3 years) were found to have VS. Of these, five patients received oral steroid therapy alone, and five had combination therapy (oral + intratympanic steroid injections). No patients received intratympanic steroid therapy alone. Median PTA improvement with steroid therapy was 3.1-dB hearing loss, and median word recognition score improvement was 16.5%. Six of 10 patients demonstrated clinically significant audiometric improvement with steroid therapy. CONCLUSION: This study represents the largest US-based study showcasing the prevalence of VS in patients originally presenting with SSNHL. It also reinforces previous findings that VS does not preclude trials of steroid therapy.