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Objective: To analyze the results of auditory assessment in standard (SA) and extended high-frequency (EHF) audiometry, associating the findings with sudden tinnitus and mental health of patients with unilateral sudden sensorineural hearing loss (SSNHL)., Study Design: Prospective, cohort study., Setting: Outpatient otology clinic in a tertiary care hospital., Subjects and Methods: Patients experiencing unilateral SSNHL were evaluated with pure-tone audiometry performed at frequencies of 250 to16,000 Hz, tinnitus pitch and loudness matching tests, Tinnitus Handicap Inventory (THI), Analog and Visual Scale (AVS) for bothersome tinnitus, and the Hospital Anxiety and Depression Scale (HADS)., Results: Eighteen patients with unilateral SSNHL were assessed. After starting treatment, there was a significant improvement in the SA (71.1 dB to 50 dB; p < 0.001*) and EHF audiometry (64.5 dB to 54.4 dB; p < 0.001*) thresholds at 15 days, and this persisted at 30 days of follow-up. Significant improvements were seen for tinnitus in loudness, VAS, and THI and for mental health in the realms of anxiety and depression by HADS. Despite improvements in SA, persistent EHF hearing loss was accompanied by persistent tinnitus, but it was of diminished loudness., Conclusion: Despite improvement in pure-tone thresholds by SA, a subset of unilateral SSNHL patients did not experience hearing recovery in EHF thresholds and reported persistent tinnitus. We postulate that their diminished anxiety and better mental health may be related to both hearing improvement in standard audiometry and reduction in tinnitus loudness. This pilot prospective study investigates the utility of performing EHF audiometry to better understand outcomes in patients with SSNHL., Competing Interests: Conflicts of interest and source of funding: This work was supported by Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP process number 2021/07346-6). Andreza Tomaz received funding from the Coordination for the Improvement of Higher Education Personnel (CAPES). Norma de Oliveira Penido received funding from National Council for Scientific and Technological Development (CNPq process number 302910/2020-4). For the remaining authors, none were declared., (Copyright © 2024, Otology & Neurotology, Inc.)

We present a case study of a 24-year-old man who reported mild balance and walking difficulties for 2 years. He had a history of recurrent fever, skin lesions, headache, and elbow pain, but most of these events resolved spontaneously. There was no significant family history. On examination, we observed frontal bossing, sensorineural hearing loss, and gait ataxia. This case underscores the significance of identifying clinical indicators in patients with neurologic symptoms, particularly recurrent fever, to establish a precise and thorough differential diagnosis.

Objective: Vascular disease like small-vessel disease (SVD) is the most likely cause among the potential causes of Sudden sensorineural hearing loss (SSNHL). Understanding the relationship between SVD and SSNHL is crucial for developing effective prevention and treatment strategies. To confirm the relationship between SVD and SSNHL, the effect of SVD is confirmed by focusing on the duration and recurrence of SSNHL., Methods: This article reports a retrospective observational study that investigated the relationship between SVD and SSNHL using the South Korea Health Insurance Review and Assessment Service (HIRA) database from 2010 to 2020. This retrospective observational study included 319,569 SSNHL patients between 2010 and 2020., Results: Participant demographics were controlled using Propensity Score Matching. The hazard ratios (HR) for the effect of SVD on the duration of SSNHL were 1.045 for the group with SVD before the onset of SSNHL and 1.234 for the group with SVD after the onset of SSNHL. SVD was statistically significant for the recurrence of SSNHL, with an odds ratio of 1.312 in the group with SVD compared to the group without SVD. The HR for the period until a recurrence in the group with SVD was 1.062., Conclusions: The study identified SVD as a possible cause of SSNHL and found that the duration of SSNHL increased only in the presence of SVD. SVD also affected the recurrence of SSNHL, with the recurrence rate being 1.312 times higher in the group with SVD., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Yoon et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Objectives: This study investigated the association between sudden sensorineural hearing loss (SSNHL) and the risk of cardio-cerebrovascular disease (CCVD) among older adults in South Korea., Methods: Data from 38,426 patients in the Korean National Health Insurance Service-Senior Cohort from 2002 to 2019 were collected. The risk of CCVD includes both stroke and acute myocardial infarction. Propensity score matching (1:1) was used to identify pairs of individuals with and without SSNHL (n = 19,213 for cases and controls). Cox proportional hazards regression models were used to analyze the associations between variables., Results: Patients with SSNHL had a higher risk of CCVD (hazard ratio [HR] = 1.17, 95% confidence interval [CI] = 1.11-1.24) compared to those without. The risk of CCVD was higher among those who experienced a stroke than those who did not (HR = 1.17 95% CI = 1.10-1.25). Compared to their matched controls, patients with SSNHL were 1.69 times (HR = 1.69 CI = 1.46-1.94) more likely to have CCVD during the first 12 months of the follow-up period., Conclusion: Older patients with SSNHL are at an increased risk of CCVD. Hence, a more attentive approach featuring aggressive monitoring of patients with SSNHL is required to lessen their risk of CCVD., Level of Evidence: 3 Laryngoscope, 134:2372-2376, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Sensorineural hearing loss (SNHL) induced by noise has increased in recent years due to personal headphone use and noisy urban environments. The study shows a novel model of gradually progressive SNHL induced by repeated exposure to moderate noise (8-kHz octave band noise, 90-dB sound pressure level) for 1 hr exposure per day in BALB/cCr mice. The results showed that the repeated exposure led to gradually progressive SNHL, which was dependent on the number of exposures, and resulted in permanent hearing loss after 5 exposures. Repeated exposure to noise causes a loss of synapses between the inner hair cells and the peripheral terminals of the auditory nerve fibers. Additionally, there is a reduction in the expression levels of c-fos and Arc, both of which are indicators of cochlear nerve responses to noise exposure. Oral administration of resveratrol (RSV, 50 mg/kg/day) during the noise exposure period significantly prevented the noise exposure-induced synapse loss and SNHL. Furthermore, the study found that RSV treatment prevented the noise-induced increase in the gene expression levels of the proinflammatory cytokine interleukin-1β in the cochlea. These results demonstrated the potential usefulness of RSV in preventing noise-induced SNHL in the animal model established as gradually progressive SNHL.

Objectives: The aim of this study is to assess the association between atrial fibrillation (AF) and sudden sensorineural hearing loss (SSNHL)., Methods: This study was conducted by searching the longitudinal health insurance database of the Taiwan National Health Insurance Program for relevant information from January 1, 2000, to December 31, 2011. Patients with AF were matched with non-AF controls with a 1:1 strategy according to propensity scores. Multiple logistic regression analyses were performed to determine the risk of SSNHL., Results: In total, 14 698 patients with AF were matched with the same number of non-AF patients as controls. After propensity score matching, the use of antiplatelet or anticoagulation medications and the occurrence of SSNHL were found to have a significant difference between AF and non-AF patients. The occurrence of SSNHL was found to be higher in men, those of 45 to 74 years old, and patients with hypertension in both AF and non-AF groups. Multiple logistic regression analyses revealed that male gender, age between 45 and 74 years, hyperlipidemia, and hypertension are risk factors for SSNHL. The use of aspirin was found to reduce the rate of SSNHL (odds ratio [OR]: 0.67, 95% CI: 0.49-0.94, P = .019), but AF was not found to be a risk factor for SSNHL (OR: 0.89, 95% CI: 0.64-1.23, P = .467)., Conclusion: The association between AF and SSNHL is not significant., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Tinnitus is a frequent condition that indicates the sensation of sound in the absence of a corresponding external stimulus and can significantly impair the quality of life. The main risk factor for developing tinnitus is hearing loss. The diagnosis of tinnitus is based on history, assessment of tinnitus severity, clinical examination, and audiological tests. The main purpose of this research was to examine the relationship between the presence and level of hearing loss and the characteristics of tinnitus in patients with bilateral subjective tinnitus., Methods: Total number of 50 participants, 20 men, and 30 women were included in the research. Demographic data, data on hearing impairment obtained by tone audiometry, and data on difficulties caused by tinnitus obtained in two questionnaires - Tinnitus Handicap Inventory (THI) and Tinnitus Functional Index (TFI) were used., Results: Age above 30 years is significantly associated with tinnitus with hearing loss. Hearing impairment is also significantly more often associated with an auditory TFI index >6.7, a total THI index >20, and an emotional THI index >3. Hearing loss was noted in 76% of patients., Conclusion: Tinnitus represents a significant burden for patients, therefore it is important to assess the impact of tinnitus on daily activities and quality of life.

Rationale: Maternally inherited diabetes and deafness (MIDD) is a rare genetic disorder arising from mitochondrial DNA mutations, characterized by a combination of diabetes mellitus and sensorineural deafness. It is known that MIDD patients with cardiomyopathy have a poor prognosis, but there are no established guidelines for the diagnosis and follow-up of cardiomyopathy in MIDD patients., Patient Concerns: Patient 1 was a 48-year-old woman who visited the hospital with cardiomegaly and had been taking oral hypoglycemic agents for 8 years. Patient 2 was a 21-year-old man, the son of patient 1, who visited the hospital for genetic screening. Patient 2 was also diagnosed diabetes mellitus 2 years ago., Diagnosis: Patient 1 was found to have restrictive cardiomyopathy on echocardiography and underwent endomyocardial biopsy and genetic testing to determine the etiology. The m.3243A>G mutation was confirmed and she was diagnosed with MIDD accompanied with diabetes and hearing loss. Additionally, patient 2 had m.3243 A>G mutation and was diagnosed with MIDD due to diabetes and hearing loss., Interventions: Because MIDD does not have a specific treatment, patient 1 took ubidecarenone (coenzyme Q10), acetylcarnitine, and multivitamin along with the treatment for diabetes control and heart failure. Patient 2 was taking ubidecarenone (coenzyme Q10), acetylcarnitine, and multivitamin along with treatment for diabetes., Outcomes: She subsequently underwent routine transthoracic echocardiography, and a progressive decline in global longitudinal strain (GLS) was first observed, followed by a worsening of the patient's clinical situation. Patient 2 had concentric remodeling and decreased GLS. On periodic echocardiography, GLS decreased at a very slow rate, and the patient's clinical course was stable., Lessons: The findings of this report contribute to the understanding of the clinical course of MIDD-associated cardiomyopathy and highlight the potential of GLS as a sensitive marker for disease progression., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Vascular causes are most commonly associated with sudden sensorineural hearing loss (SSHL). This study was performed to determine the relationship between serum endothelin-1 (ET-1), high-density lipoprotein cholesterol (HDL-C), soluble vascular cell adhesion molecule-1 (sVCAM-1) levels, and the degree of hearing loss in patients with SSHL. Firstly, 60 SSHL patients were admitted to The First Hospital of Shanxi Medical University. In the same period, 60 healthy subjects matching the age and gender of SSHL patients were selected as the control group. Then, serum levels of ET-1, HDL-C, and sVCAM-1 were measured by enzyme-linked immunosorbent assay (ELISA). Next, the relationship between serum levels of ET-1, HDL-C, and sVCAM-1 with clinicopathological factors and their diagnostic and prognostic values were analyzed and evaluated. Serum ET-1 and sVCAM-1 were increased, and HDL-C was decreased in patients with SSHL. Serum ET-1 and sVCAM-1 were higher and HDL-C was lower in patients aged ≥ 45 years, or severe hearing loss patients (P < 0.05). ROC analysis determined that ET-1 (AUC = 0.839), HDL-C (AUC = 0.830), and sVCAM-1 (AUC = 0.865) had excellent diagnostic values. In addition, patients with low levels of ET-1 and sVCAM-1 and high levels of HDL-C had better hearing prognosis (P < 0.05). Abnormal serum ET-1, HDL-C, and sVCAM-1 in patients with SSHL are closely related to age, and degree of hearing loss, and perform diagnostic and prognostic values., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: The aims of this study were to investigate the cumulative recurrence rate of SSNHL and to determine association between comorbidities and recurrence of SSNHL by comparing patients with and without recurrence., Methods: Using the National Health Insurance Claims Database (NHICD) from 2009 to 2020, we conducted a population-based study. We only enrolled patients whose records showed a prescription for steroid and audiometry findings as well as an appropriate diagnostic code. Recurrence of SSNHL was defined as an episode of SSNHL greater than or equal to 3 months after the first episode of SSNHL. We compared the recurrence rate of SSNHL according to age and number of SSNHL recurrences. We also explored comorbidities including autoimmune, metabolic, chronic renal diseases, cancer, and migraine associated with recurrent SSNHL., Results: A total of 257,123 patients were identified. We found that 6.7% (17,270/257,123) of the patients had at least one recurrence of SSNHL. The recurrence rate increases with the number of recurrences and over time. The incidence per 100,000 people tended to increase with age, and the recurrence rate appeared to decrease with age. We found an increase in the incidence of ankylosing spondylitis (AS) and a decrease in the incidence of type 2 diabetes mellitus (T2DM), myocardial infarction (MI), and hemorrhagic stroke in patients with recurrence., Conclusion: For patients with recurrence or AS, considerable efforts should be made to prevent recurrence. As SSNHL is an emergent otologic condition, when symptoms occur, they should receive immediate treatment. Additional well-designed population-based studies are required to generalize our results., Level of Evidence: 3 Laryngoscope, 134:1417-1425, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Whether cervical spondylosis (CS) is a risk factor for sudden sensorineural hearing loss (SSNHL) remains unclear. This study used national population-based data to investigate the risk of SSNHL in patients with CS in Taiwan of different ages and sexes. This study used data covering 2 million people in Taiwan, which were obtained from the National Health Insurance Research Database. The data that support the findings of this study are available from National Health Insurance Research Database but restrictions apply to the availability of these data, which were used under license for the current study, and so are not publicly available. Data are however available from the corresponding authors upon reasonable request and with permission of National Health Insurance Research Database. This retrospective cohort study enrolled 91,587 patients with a newly diagnosed CS between January 2000 and December 2018. Case and control cohorts were matched 1:1 according to age, sex, and comorbidities. SSNHL incidence rate and risk were compared between the groups. Cox regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). The mean follow-up period was 8.80 (SD = 4.12) and 8.24 (SD = 4.09) years in the CS and control cohorts, respectively. The incidence rate of SSNHL in the CS cohort (85.28 per 100 000 person-years) was 1.49-fold significantly higher than that in the non-CS cohort (57.13 per 100,000 person-years) (95% CI 1.32-1.68, P < .001). After age, sex, and selected comorbidities were adjusted for, CS exhibited an independent risk factor for SSNHL (adjusted HR = 1.52; 95% CI   1.34-1.71, P < .001). An age-stratified analysis in this study demonstrated a strong and highly significant association between CS and SSNHL in patients aged < 35 years (IRR = 2.28, 95% CI   1.18-4.39, P = .013). This large-scale Taiwanese-population-based retrospective study found that CS was associated with an increased risk of SSNHL. Acute hearing loss in patients with CS, particularly at a young age, should be carefully evaluated, and prompt treatment for SSNHL should be initiated., (© 2024. The Author(s).)

Background: Hypertrophic cranial pachymeningitis (HCP) is uncommon but a poorly understood complication of granulomatosis with polyangiitis (GPA)., Objectives: We conducted this retrospective study to elucidate the clinical characteristics and factors independently associated with granulomatosis with polyangiitis (GPA) complicated by hypertrophic cranial pachymeningitis (HCP) in China., Methods: We collected the medical records of 78 patients diagnosed with GPA who were admitted to the inpatient department of Peking Union Medical College Hospital between January 2003 and September 2021. Clinical features, laboratory and radiological findings, and Birmingham Vasculitis Activity Scores (excluding meningitis score) were recorded. A binary logistic regression analysis was performed to analyze factors independently associated with GPA-related HCP., Results: Headache (100%) and cranial nerve palsy (61.5%) were common manifestations of HCP. Compared to 52 GPA patients without HCP, 26 patients with HCP required more time from initial symptoms to diagnosis, with a lower ratio of pulmonary and renal involvement, a higher ratio of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, conductive or sensorineural hearing loss, mastoiditis, and decreased vision or sudden visual loss. Binary logistic regression analysis indicated that proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) negativity (OR 10.698, p = 0.001), conductive or sensorineural hearing loss (OR 10.855, p = 0.005), and decreased vision or sudden visual loss (OR 8.647, p = 0.015) were significantly associated with GPA-related HCP. Of the 26 patients, 18 received methylprednisolone pulse treatment, and 18 received intrathecal injections of dexamethasone and methotrexate., Conclusions: HCP was a severe manifestation of GPA in our study. Independent factors associated with the occurrence of HCP in patients with GPA included PR3-ANCA negativity, conductive or sensorineural hearing loss, and decreased vision or sudden visual loss. Furthermore, GPA-related HCP was associated with higher disease activity, requiring more intensive treatments., (© 2023. The Author(s).)

Background: Postmeningitic hearing loss from Haemophilus influenzae (H. influenzae) is increasingly due to encapsulated serotypes other than type b (Hib) and nontypeable strains (collectively, nHiB H. influenzae). Pediatric hearing loss after nHib H. influenzae meningitis remains poorly described., Methods: Retrospecive case series of nHiB H. influenzae meningitis cases identified from a microbiologic database at Children's Hospital Colorado from 2000 to 2020. Literature regarding nHiB H. influenzae and H. influenzae postmeningitic hearing loss was also reviewed., Results: Eleven cases of nHib H. influenzae meningitis (median age 15.9 months) were identified due to serotype f (36 %), serotype a (27 %), and nontypable strains (36 %). Seven (64 %) patients were male, 55 % were white and 18 % were Hispanic or Latino. Hearing loss was initially identified in 4 children (40 %), with two patients with moderate conductive hearing loss (CHL) and one child with unilateral moderate sensorineural (SNHL) hearing loss patients recovering normal hearing. One patient with bilateral profound sensorineural hearing loss and associated labyrinthitis ossificans required cochlear implantation. All children (4) with identified hearing loss were noted to have additional intracranial sequelae, which included empyema (2), sinus thrombosis (2), and seizures (2). Of patients receiving steroids, 25 % had hearing loss on initial testing, compared to 66 % of those who did not receive steroids., Conclusions: nHib H. influenzae can cause both transient and permanent postmeningitic hearing loss. Steroids may offer otoprotection in nHib H. influenzae meningitis similar to Hib meningitis. Given the limited literature, further study is needed to better characterize hearing outcomes after nHib H. influenzae meningitis., Competing Interests: Declaration of competing interest The authors have no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Importance: Congenital cytomegalovirus (cCMV) is the major cause of congenital nonhereditary sensorineural hearing loss in children. Currently, criteria to identify infants at increased risk for unfavorable hearing outcome are lacking., Objective: To identify risk factors associated with cCMV-related hearing improvement, hearing deterioration, and late-onset hearing loss., Design, Setting, and Participants: This multicenter cohort study included patients from 6 secondary and tertiary hospitals enrolled in the Flemish CMV registry (Belgium). Newborns with untreated cCMV infection with at least 4-year audiological follow-up were included. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Data were collected for 15 years (January 1, 2007, to February 7, 2022) and analyzed from September 26, 2022, to January 16, 2023., Main Outcomes and Measures: Primary outcome was hearing evolution (per-ear analysis; described as stable hearing, improvement, or deterioration). The association of gestational characteristics, clinical findings, timing of seroconversion, viral load, and hearing status at birth with hearing evolution was investigated using effect sizes (Cramer V, odds ratio [OR], or Hedges g)., Results: Of the 387 children, 205 of 385 with nonmissing data were male (53.2%), 113 (29.2%) had a symptomatic infection, and 274 (70.8%) had an asymptomatic infection. Every child was 4 years or older at final hearing evaluation. A total of 701 of 774 ears (90%) showed stable hearing (normal hearing or stable hearing loss since birth) over time. Late-onset hearing loss (normal hearing at birth followed by hearing loss) was present in 43 of 683 ears (6.3%). Among children with hearing loss present at birth, 24 of 34 ears (70.6%) had hearing deterioration, and 6 of 91 ears (6.6%) had hearing improvement. Prematurity was associated with a higher chance of hearing improvement (OR, 12.80; 95% CI, 2.03-80.68). Late-onset hearing loss was more prevalent in a first trimester infection (OR, 10.10; 95% CI, 2.90-34.48). None of the 104 ears of children with a third trimester seroconversion developed late-onset hearing loss., Conclusions and Relevance: Findings of this cohort study support that ongoing audiological follow-up for untreated children with congenital hearing loss is important, as the majority of patients had hearing deterioration. The timing of seroconversion was associated with the risk of developing late-onset hearing loss. These insights can aid in parental counseling, patient stratification, and follow-up. Future research should focus on the effect of treatment, the influence of determined risk factors, and the study of eventual new risk factors in patients at high risk to develop hearing loss.

Objectives/hypothesis: The co-occurrence of sensorineural hearing loss (SNHL) and congenital heart disease (CHD) is a rare condition with complex etiologies. The purpose of this study is to assess the etiologies, clinical features, and outcomes of cochlear implant (CI) in this patient population., Study Design: Case series and literature review., Methods: Clinical data of children who were diagnosed with SNHL and CHD and received CIs at a tertiary hospital from 2016 to 2021 were retrospectively analyzed. A literature review was performed to identify patients with SNHL and CHD., Findings: Of the 382 children who underwent cochlear implantation at our center, eight (2.1%) were diagnosed with SNHL and CHD. A literature review identified 1525 patients from 254 studies; the database therefore consisted of 1533 patients. The most common genetic etiologies of co-occurring SNHL and CHD were CHARGE syndrome (36.3%), Turner syndrome (8.4%), 22q11.2 deletion (3.0%), Noonan syndrome (2.9%), and Down syndrome (2.5%), whereas the most common non-genetic etiologies were congenital rubella syndrome (22.9%) and SNHL after early cardiac surgery (5.5%). Most of the patients presented with congenital, bilateral, severe-profound SNHL requiring early rehabilitation. Of the 126 children who received CIs at a median age of 2.5 years, half showed delayed speech development at last follow-up., Conclusions: Co-occurring SNHL and CHD is a rare condition with complex etiologies. Timely hearing intervention with long-term follow-up and proper timing of heart surgery is essential for these children., Level of Evidence: 4, case series Laryngoscope, 134:400-409, 2024., (© 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Introduction: The etiology of idiopathic sudden sensorineural hearing loss (ISSNHL) remains elusive, with vascular compromise as a proposed cause. This study aimed to explore the correlation between the vertebrobasilar vascular system laterality (VBVSL) and ISSNHL laterality., Methods: We conducted a retrospective analysis of consecutive patients diagnosed with ISSNHL from 2015 to 2020. The VBVSL pattern was established via magnetic resonance imaging scans by a neuroradiologist. ISSNHL occurring contralaterally to the basilar artery (BA) curvature or ipsilaterally to the dominant vertebral artery (VA) was designated as a "positive match," with all other scenarios classified as a "negative match.", Results: Our study included 191 ISSNHL patients (median age 57 years, 89 males, 93 right ears). The majority of patients did not exhibit a positive match between ISSNHL laterality and the sides of BA curvature or dominant VA (28.8% and 36.6% for BA and VA, respectively). Notably, VA-positive match patients were significantly older than VA-negative match patients (59 vs. 53 years, p = 0.043), with a similar trend observed in BA-positive match compared to BA-negative match (59 vs. 54.5 years, p = 0.057). However, there was no significant difference in any other clinical, audiometric, or outcome factors between the positive and negative match groups., Conclusion: The findings suggest no association between VBVSL and ISSNHL laterality. Furthermore, patients in the positive match group did not exhibit distinct clinical or audiometric features compared to those without a match., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Objective: Cochlear implants (CIs) for single-sided deafness (SSD) have only been approved for patients 5 years and older despite data supporting that younger children can also benefit from implantation. This study describes our institution's experience with CI for SSD in children 5 years and younger., Study Design: Case series with chart review., Setting: Tertiary referral center., Methods: A case series with chart review identified 19 patients up to age 5 years who underwent CI for SSD between 2014 and 2022. Baseline characteristics, perioperative complications, device usage, and speech outcomes were collected., Results: The median age at CI was 2.8 (range, 1.0-5.4) years, with 15 (79%) patients being below age 5 at implantation. Etiologies of hearing loss were idiopathic (n = 8), cytomegalovirus (n = 4), enlarged vestibular aqueduct (n = 3), hypoplastic cochlear nerve (n = 3), and meningitis (n = 1). The median preoperative pure-tone average was 90 (range, 75-120) and 20 (range, 5-35) dB eHL in the poor and better hearing ears, respectively. No patients had postoperative complications. Twelve patients achieved consistent device use (average, 9 h/d). Three of the seven who were not consistent users had hypoplastic cochlear nerves and/or developmental delays. The three patients with available preoperative and postoperative speech testing showed significant benefits, and five patients with available postoperative testing demonstrated speech recognition in the implanted ear when isolated from the better ear., Conclusion: CI can safely be performed in younger children with SSD. Patients and families accept early implantation, as evidenced by consistent device use, and derive notable benefits in speech recognition. Candidacy can be broadened to include SSD patients under age five years, particularly individuals without hypoplastic cochlear nerves or developmental delay., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Objective: To investigate the impairment patterns in peripheral vestibular organs in sudden sensorineural hearing loss (SSNHL) with and without vertigo., Study Design: Retrospective study., Setting: Single tertiary medical center., Methods: Data from 165 SSNHL patients in a tertiary referral center from January 2017 to December 2022 were retrospectively analyzed. All patients underwent a video head impulse test, vestibular evoked myogenic potential test, and pure-tone audiometry. Hierarchical cluster analysis was performed to investigate vestibular impairment patterns. The prognosis of the hearing was determined using American Academy of Otolaryngology-Head and Neck Surgery recommendations., Results: After excluding patients with vestibular schwannoma and Meniere's disease, 152 patients were included in this study. A total of 73 of 152 patients were categorized as SSNHL with vertigo (SSNHL&#95;V) and showed an independent merge of the posterior semicircular canal (PSCC) in cluster analysis. A total of 79 of 152 patients were categorized as SSNHL without vertigo (SSNHL&#95;N) and showed an independent merge of saccule in cluster analysis. The PSCC (56.2%) and saccule (20.3%) were the most frequently impaired vestibular organs in SSNHL&#95;V and SSNHL&#95;N, respectively. In terms of prognosis, 106 of 152 patients had partial/no recovery and showed an independent merge of the PSCC in cluster analysis. A total of 46 of 152 patients had a complete recovery and showed an independent merge of the saccule in cluster analysis., Conclusion: A tendency of isolated PSCC dysfunction was seen in SSNHL&#95;V and partial/no recovery. A tendency of isolated saccular dysfunction was seen in SSNHL&#95;N and complete recovery. Different treatments might be needed in SSNHL depending on the presence of vertigo., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Rationale: Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used., Patient Concerns: The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa., Diagnoses: A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss., Interventions and Outcomes: A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively., Lessons: This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Congenital cytomegalovirus (cCMV) infection can have a broad range of manifestations. This study aimed to assess cCMV-associated sequelae and healthcare resource utilization (HCRU) in infants during the first year of life in Germany., Methods: A retrospective, controlled cohort study using German claims data from the Institute for Applied Health Research Berlin (InGef) database was conducted. cCMV-associated sequelae and HCRU during the first year of life were assessed by matching (1:60) infants with at least one inpatient/outpatient cCMV diagnosis (ICD-10-GM: P35.1) ≤90 days after birth (cCMV90 cohort) and infants with at least one inpatient cCMV diagnosis plus specific sequelae ≤21 days after birth (cCMV21-S) to infants without cCMV or CMV (ICD-10-GM: B25) diagnosis (control group), respectively. Outcomes were analyzed during the first 365 days of life., Results: Between 2014-2018, we identified 54 newborns for cCMV90 and 24 newborns for cCMV21-S cohort. Compared to the 3,240 and 1,440 controls, respectively, more cCMV90 infants (83.3% vs. 41.9%, p<0.01) presented with at least one sequela during the first year of life, including intrauterine growth retardation (42.6% vs. 5.3%, p<0.01), sensorineural hearing loss (SNHL) to deafness (38.9% vs. 2.2%, p<0.01), and motor development disorders (33.3% vs. 10.9%, p<0.01). Further, 13.0% of cCMV90 infants (vs. 2.3%, p<0.01) suffered from visual impairment. In cCMV21-S cohort, intrauterine growth retardation (79.2% vs. 6.0%, p<0.01), prematurity (54.2% vs. 7.3%, p<0.01), and motor development disorders (50.0% vs. 11.0%, p<0.01) were the most frequent sequelae. Infants in the cCMV90 and cCMV21-S cohort had, on average, 7.3 times and 9.5 times more hospitalizations and 2.0 times and 2.1 times more outpatient physician visits than their respective controls (p<0.01). Hospitalized infants with cCMV stayed, on average, significantly longer in hospital compared to their controls (cCMV90 cohort: 30.3 days vs. 9.0 days, p<0.01; cCMV21-S cohort: 46.5 days vs. 9.3 days, p<0.01)., Conclusions: cCMV-infection shows a considerable disease and healthcare burden during the first year of life. More than 80% of the identified newborns with cCMV suffered from at least one associated sequela during the first year of life, including long-term sequelae such as SNHL (40%) and visual impairment (13%). Additional steps for prevention of cCMV infection and associated sequelae as well as a comprehensive monitoring of disease burden are needed., Competing Interests: ML, RW, AL, and MR are full-time employees of MSD Sharp & Dohme GmbH. AJS was a full-time employee of MSD Sharp & Dohme GmbH at the time the study was conducted. WW is a full-time employee of Merck Sharp & Dohme LLC, a subsidiary of Merck & Co., Inc., Rahway, NJ, USA. KMS and CJ are full-time employees of Xcenda GmbH, acting as contractors of MSD Sharp & Dohme GmbH for the execution of this study. WG received personal fees from Xcenda GmbH during the conduct of the study. RG, KH, PK, and SM received honoraria as scientific advisor from MSD Sharp & Dohme GmbH. HB received research grants (third-party funding), reimbursement of travel expenses, consulting fees or reimbursement of conference fees from the following companies related to the topic of ’cytomegalovirus infections’: Biotest, MSD Sharp & Dohme GmbH, Merck Sharp & Dohme LLC, a subsidiary of Merck & Co., Inc., Rahway, NJ, USA, and Hoffmann-La Roche Ltd. This does not alter our adherence to PLOS ONE policies on sharing data and materials., (Copyright: © 2023 de Lepper et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background and Purpose: Sudden sensorineural hearing loss (SSNHL) is a neurological and otolaryngological emergency during which rapid diagnosis and early treatment are of great importance. Clinical experience indicates that a considerable number of patients with SSNHL have concurrent right-to-left shunt (RLS). With limited reports, the association between SSNHL and RLS is yet unclear and there is a need for large observational studies to explore their latent relationship., Methods and Analysis: This proposed study is a prospective, observational case-control study. A total of 194 eligible participants matched in age and sex will be divided equally into two groups: 97 patients with SSNHL included in the case group and 97 individuals without SSNHL in the control group. Medical evaluations, including clinical characteristics, laboratory examination, audiological examination and ultrasonography examination, will be performed in all subjects. The primary outcome of the study is the difference in RLS rates between the groups. Differences in patent foramen ovale rates and other measured variables will be further assessed. A conditional logistic regression as a correlation analysis will be used to evaluate the relationship between RLS and SSNHL., Discussion: This study may provide evidence on the correlation between RLS and SSNHL in order to enrich the aetiology of SSNHL., Ethics and Dissemination: The study protocol has been approved by the Ethics Committee of Peking University Shenzhen Hospital. A written informed consent form will be signed and dated by the participants and the researchers before the study begins. The results will be disseminated in peer-reviewed publications., Trial Registration Number: ChiCTR2200064067., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The prevalence of sudden sensorineural hearing loss and facial palsy in patients with vestibular schwannoma and the association of sudden sensorineural hearing loss or facial palsy with vestibular schwannoma were investigated based on the population data of Korea., Methods: This retrospective study used the Korean National Health Insurance Service data. Patients with vestibular schwannoma and those with a previous history of sudden sensorineural hearing loss or facial palsy were identified based on diagnostic, medication, magnetic resonance imaging, or audiometric codes from 2005 to 2020. The control group was established with propensity score matching. The risk for vestibular schwannoma in patients with a previous history of sudden sensorineural hearing loss or facial palsy was analyzed., Results: There were 5751 patients in the vestibular schwannoma group and 23004 in the control group. The rate of patients with a previous history of sudden sensorineural hearing loss in the vestibular schwannoma group (25.8%) was significantly higher than in the control group (P -lt; .0001), as was the rate of patients with a previous history of facial palsy in the vestibular schwannoma group (4.7%) (P -lt; .0001). Previous history of sudden sensorineural hearing loss was a significant risk factor for vestibular schwannoma (hazard ratio=7.109, 95% confidence interval=6.696-7.547). Previous history of facial palsy was also a significant risk factor for vestibular schwannoma (hazard ratio=3.048, 95% confidence interval=2.695-3.447)., Conclusion: The prevalence of sudden sensorineural hearing loss or facial palsy was significantly higher in patients with vestibular schwannoma than in those without vestibular schwannoma. Based on the population data of Korea, sudden sensorineural hearing loss and facial palsy were significant risk factors for vestibular schwannoma.

Although peripheral facial paralysis and sudden sensorineural hearing loss are not as common as anosmia, they are reported neurological manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We present a 62-year-old, serologically COVID-19 positive woman with seventh and eighth nerve involvement showed electrophysiologically with Auditory Brainstem Response and electroneurography and radiologically with internal acoustic canal magnetic resonance imaging. This single case report suggests a possible association between the SARS-CoV-2 infection with simultaneous sudden sensorineural hearing loss and isolated facial paralysis. However, further studies are needed to determine whether this relationship is coincidental or occasional., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: To investigate the bidirectional association between sudden sensorineural hearing loss (SSNHL) and open-angle glaucoma (OAG) over a 12-year follow-up period using nationwide, population-based data., Methods: The study was conducted using the National Health Information Database of the National Health Insurance Service (NHIS-NHID), which covered 3.5 million individuals from 2008 to 2019. In Study 1, we evaluated the effect of OAG on SSNHL, and in Study 2, we evaluated the effect of SSNHL on OAG. Participants of the control group were enrolled through "greedy nearest-neighbor" 1:1 propensity score matching., Results: In Study 1, 26,777 people were included in each group. The hazard ratio (HR) for SSNHL of the OAG group was 1.27 (95% confidence interval [CI], 1.15-1.39). In subgroup analysis, there was significant HR value regarding (old age: 1.17, hyperlipidemia: 1.19). In Study 2, 15,433 people were included in each group. The HR for OAG of the SSNHL group was 1.18 (95% CI, 1.07-1.30). In subgroup analysis, the HRs were significant for old age (2.31), hypertension (1.17), diabetes (1.39), and hyperlipidemia (1.26)., Conclusion: Over the 12-year follow-up, we found a bidirectional association between SSNHL and OAG, suggesting a shared pathogenesis., Level of Evidence: N/A. Laryngoscope, 133:3169-3177, 2023., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

MYH9-related disease is an autosomal dominant disorder characterized by macrothrombocytopenia, nephropathy, inclusion bodies in leukocytes, sensorineural hearing loss, and cataract. Severe cases require kidney replacement therapy in the patient's second decade of life; thrombocytopenia constitutes a major risk factor for hemorrhagic complications during dialysis initiation or kidney transplantation. Prophylactic platelet transfusion prior to surgery is commonly administered to affected patients in these cases. However, transfusion in such patients has limitations other than the general risk of allergic reactions and blood-borne infections; it may also trigger alloimmunization, leading to platelet transfusion resistance or the development of anti-donor antibodies in potential kidney transplant recipients. Here, we describe prophylactic administration of eltrombopag, an oral thrombopoietin receptor agonist, prior to laparoscopic peritoneal dialysis catheter placement in a 15-year-old girl with MYH9-related disease. Her platelet count was approximately 30 × 10 3 /μL at baseline; it increased to 61 × 10 3 /μL on the day before surgery, thereby avoiding the need for platelet transfusions. There were no major bleeding or adverse events associated with eltrombopag administration. Thus, eltrombopag may be a safe and effective alternative to prophylactic platelet transfusions in patients with MYH9-related disease., (© 2023. The Author(s) under exclusive licence to The Japan Society of Nephrology.)

Background: Approximately 28%-57% of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) have an acute vertigo attack and probable vestibular dysfunction; however, the prognosis of vestibular function in these patients remains unclear., Methods: A prospective cohort study of patients with ISSNHL and vertigo was conducted to evaluate the prognosis of vestibular function, especially the roles of peripheral vestibular restoration and central compensation, in patients with ISSNHL and vertigo. Clinical data were recorded at baseline and at 60 days from onset in participants with unilateral ISSNHL with vertigo. Enrolment occurred from May 1, 2019 to May 1, 2022 in the outpatient clinics and inpatient departments of the Eye and ENT Hospital of Fudan University in Shanghai. The primary outcome measure was the recovery rate of vestibular function 60 days after onset as assessed by vestibular function tests, including caloric tests, cervical and ocular vestibular-evoked myogenic potential tests (cVEMP, oVEMP), video head impulse tests (vHIT), and sensory organization tests (SOT). The secondary outcome measure was the recovery of subjective evaluations in vestibular dysfunction (the dizziness handicap inventory [DHI], and the visual analogue scale for vertigo [VAS-V]) and hearing assessments (pure-tone audiometry [PTA])., Results: Overall, 86 patients were recruited, with an average duration of disease of 11.7 days and follow-up time of 60.7 days. Vestibular function was significantly improved (p < 0.05) after 60 days. The recovery rates were 100% for anterior semicircular canal (ASC), 56% for posterior semicircular canal (PSC), 41% for horizontal semicircular canal (HSC), 28% for saccule, and 23% for utricle. The recovery of vestibular function was not significantly related to changes in DHI (p = 0.245), VAS-V score (p = 0.509), or hearing outcome (p = 0.390)., Conclusions: Restoration of peripheral vestibular sensory input and central vestibular compensation can occur during the course of ISSNHL with vertigo. The otolith organs are at a higher risk of being affected and have worse recovery than the semicircular canals. Incomplete and in-process restoration of vestibular dysfunction may perturb and delay the establishment of central compensation for balance. Neither hearing outcomes nor subjective vestibular symptoms are related to recovery from vestibular dysfunction., Trial Registration: ClinicalTrials.gov (identifier NCT03951584)., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Background and Objectives : Hearing loss after septicemia has been found in mice; the long-term risk increased 50-fold in young adults in a previous study. Hearing loss after septicemia has not received much attention. The aim of this study was to assess the relationship between septicemia and subsequent hearing loss. Materials and Methods : Inpatient data were obtained from the Taiwan Insurance Database. We defined patients with sensorineural hearing loss and excluded patients under 18 years of age. Patients without hearing loss were selected as controls at a frequency of 1:5. The date of admission was defined as the date of diagnosis. Comorbidities in the 3 years preceding the date of diagnosis were retrieved retrospectively. Associations with hearing loss were established by multiple logistic regression and forward stepwise selection. Results : The odds ratio (OR) for the association between sepsis and hearing loss was 3.052 (95% CI: 1.583-5.884). Autoimmune disease (OR: 5.828 (95% CI: 1.906-17.816)), brain injury (OR: 2.264 (95% CI: 1.212-4.229)) and ischemic stroke (OR: 1.47 (95% CI: 1.087-1.988)) were associated with hearing loss. Conclusions : Our study shows that hearing loss occurred after septicemia. Apoptosis caused by sepsis and ischemia can lead to hair cell damage, leading to hearing loss. Clinicians should be aware of possible subsequent complications of septicemia and provide appropriate treatment and prevention strategies for complications.

Sudden sensorineural hearing loss (SSNHL) accompanied by benign paroxysmal positional vertigo (BPPV) is relatively common in the clinic. There are unified standards for the treatment of primary BPPV with good reduction effect, while there are few studies on the treatment of BPPV secondary to SSNHL within 1 week of onset. The study was to investigate the treatment of BPPV secondary to SSNHL and compare its manual reduction with that of primary BPPV. We selected 90 patients with BPPV accompanied by SSNHL within a week of onset and 210 primary BPPV patients at Hebei Provincial Eye Hospital from June 2020 to December 2022. The former group was divided into the medicine group and manual reduction plus medicine group. The medicines used were extract of Ginkgo biloba leaves injection, betahistine hydrochloride injection and oral prednisone. We contrasted the efficacy respectively for posterior semicircular canal BPPV (psc-BPPV), horizontal semicircular canal BPPV (hsc-BPPV) and multiple semicircular canal BPPV (msc-BPPV). In addition, we compared the manual reduction effect for primary BPPV and manual reduction group, and the evaluation of efficacy are the intensity of nystagmus and the clinical symptoms. In the secondary BPPV group, there was no difference in efficacy between the medicine group and manual reduction group at the 7th-day after reduction for psc-BPPV, hsc-BPPV, and msc-BPPV (P > .05). The immediate effect of reduction was significantly different between the primary BPPV group and the group with SSNHL and BPPV for both psc-BPPV and hsc-BPPV (P < .05), and the effect of the primary BPPV group was better, but it was no difference for msc-BPPV (P > .05). For the treatment of BPPV accompanied by SSNHL within 1 week of onset, the additional reduction therapy showed no benefit, so we need to apply medication for SSNHL., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: Enlarged vestibular aqueduct (EVA) is the most common anatomic abnormality contributing to permanent hearing loss (HL) in children. Although the association between EVA and HL is well-documented, the pass rate for the newborn hearing screening (NBHS) for patients with EVA-related HL is not. Our objective was to investigate the association between NBHS results and audiologic and clinical outcomes in a large cohort of pediatric patients with EVA., Methods: This was a retrospective chart review of patients seen in the Boston Children's Hospital (BCH) Department of Otolaryngology and Communication Enhancement with confirmed HL, known NBHS results, and confirmed EVA. Demographic, clinical, audiologic, and imaging data were collected from the medical record. Frequency-specific data points from pure-tone audiograms and/or automated auditory brainstem response tests were recorded, and four-frequency pure tone average was calculated using air conduction thresholds at 500, 1000, 2000, and 4000 Hz., Results: Of the 183 patients included in the study, 84 (45.9%) passed their NBHS, whereas 99 (54.1%) did not pass. Compared with patients who did not pass, patients who passed were more likely to have unilateral EVA and unilateral HL, whereas they were less likely to undergo cochlear implantation and to have causative SLC26A4 variants., Conclusions: EVA-associated HL may be identified at birth or during childhood, with nearly half the patients in this cohort passing their NBHS. Our results provide prognostic information for patients with EVA who pass their NBHS and highlight the importance of regular hearing monitoring for children not initially suspected of having HL., Level of Evidence: 4 Laryngoscope, 133:2786-2791, 2023., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

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.

Objective: Cochlear implantation (CI) has been reported as a treatment modality for radiation-induced sensorineural hearing loss (SNHL). However, its efficacy is based on individual reports with no cumulative supporting evidence. Therefore, we conducted the current systematic review to provide cumulative evidence regarding the feasibility and safety of CI in this context., Databases Reviewed: An online bibliographic search was conducted in PubMed, ProQuest, Scopus, Google Scholar, and Web of Science using MeSH-based terms., Methods: A systematic review was conducted to retrieve both observational and interventional studies that reported the outcomes of CI for patients suffering from radiation-induced SNHL., Results: We included 12 studies that recruited 88 patients who underwent CI because of radiation-induced SNHL. All included studies reported satisfactory hearing/speech perception outcomes. No serious complications were reported, whereas some manageable adverse events were reported, such as paroxysmal facial spams (n = 1), postauricular wound dehiscence with mastoid cutaneous fistula (n = 1), dehiscence in blind sac closure (n = 1), and electrode exposure (n = 1). Four studies assessed the postoperative quality of life/patient satisfaction, showing improved outcomes., Conclusion: CI could be considered to be feasible and safe in patients with irradiation-induced SNHL. The adverse events of CI in such cases are manageable. Future studies are needed to be strengthened this context., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

Objective: This study evaluates the natural course of hearing loss (HL) prior to treatment in patients with progressive tumors and an indication for active intervention. Evaluating this patient group specifically can put hearing outcomes after vestibular schwannoma therapy into an adequate context., Study Design: Retrospective cohort study., Setting: Tertiary referral center., Methods: Inclusion criteria comprised unilateral vestibular schwannomas prior to active treatment, with ≥2 mm extracanalicular (EC) tumor growth and ≥2 audiograms. We performed a comprehensive assessment of hearing using multiple outcome parameters including (the annual decrease in) pure-tone averages (PTAs; an average of 0.5, 1, 2, and 3 kHz). Predictors for HL were evaluated (patient age, tumor size/progression, follow-up duration, baseline hearing)., Results: At presentation, 86% of patients suffered from sensorineural HL on the affected side (≥20 dB PTA) with a median of 39 dB (interquartile rate [IQR]: 27-51 dB). The median follow-up duration was 21 months (IQR: 13-34 months), after which 58% (187/322) of patients experienced progressive HL (≥10 dB), with a median increase of 6.4 dB/year. At the last follow-up, the median PTA was 56 dB (IQR: 37-73). Median speech discrimination scores deteriorated from 90% (IQR: 70%-100%) to 65% (IQR: 35%-100%). Tumor progression (maximal EC diameter) was significantly correlated to the progression of sensorineural HL, corrected for follow-up (F(2,228) = 10.4, p < .001, R 2  = 8%)., Conclusion: The majority of patients (58%) with radiologically confirmed progressive vestibular schwannomas experience progressive sensorineural HL during observation. Tumor progression rate, EC tumor extension, and longer follow-up are factors associated with more sensorineural HL., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Objective: To analyze the clinical and imaging features of patients with sudden sensorineural deafness and acute cerebral infarction in order to provide evidence for early recognition of such diseases. Methods: This was a case series reporting study. A retrospective analysis was performed on the clinical and imaging data of 29 patients with sudden hearing loss (SHL) who admitted to the Otolaryngology Head and Neck Surgery Department of Beijing Tiantan Hospital from January 2017 to December 2021 and diagnosed with acute cerebral infarction using MRI-DWI. Results: The patients were aged 31-71 years, with an average age of 56±12 years, and 82.8% (24/29) were men. In total, 82.8% (24/29) of the patients had three or more atherosclerotic risk factors, and 24.1% (7/29) had a history of SHL. The hearing types were flat and total deafness: 86.2% (25/29) of the patients had severe hearing loss, 27.6% (8/29) had bilateral SHL, 17.2% (5/29) had further hearing loss during hospitalization, and 82.8% (24/29) had dizziness or vertigo at the onset. The signs of central nervous system involvement mainly included speech impairment, diplopia, dysphagia, central facial paralysis, facial and limb hypoesthesia, ataxia, and decreased muscle strength. Imaging evaluation showed that 21 cases were located in the posterior circulation supply area and 8 cases in the anterior circulation supply area. Additionally, 82.8% (24/29) patients had vertebrobasilar artery stenosis, and 58.6% (17/29) patients had severe vertebrobasilar artery stenosis or occlusion. Conclusions: Patients with SHL who progress to cerebral infarction often have multiple atherosclerotic risk factors and SHL. Most of the patients are middle-aged and older men who often complain of dizziness or dizziness accompanied by severe flat and total deafness with unilateral or bilateral SHL. Imaging findings suggest that most patients have posterior circulation infarction, often accompanied by severe stenosis or occlusion of the vertebrobasilar artery.

Objectives: To evaluate the associations between obstructive sleep apnea (OSA) and sensorineural hearing loss (SNHL) and the effects of continuous positive airway pressure (CPAP) therapy on SNHL., Data Sources: Ovid Medline, Embase, and Scopus databases., Review Methods: A systematic search was done for studies investigating relationships between OSA and SNHL in adults, with manual searches for additional references. The final update was done on December 22, 2021. The Risk of Bias Assessment Tool for Nonrandomized Studies was applied for quality assessments., Results: The 20 included studies had a total of 34,442 participants (66% male; mean age, 46.6 years). The OSA group had a significantly worse mean hearing threshold level (HTL) than the control group for midfrequency ranges (500, 1000, 2000 Hz; mean difference, 4.00 dB; 95% CI, 2.40-5.61) and high-frequency ranges (4000, 8000 Hz; mean difference, 6.24 dB; 95% CI, 2.99-9.49). An association between OSA and SNHL was found. When compared with controls, patients with OSA had an odds ratio of 1.52 (95% CI, 1.12-2.06) for midfrequency hearing impairment and 1.19 (95% CI, 1.05-1.34) for high-frequency hearing impairment. However, we did not find significant improvements in midfrequency HTL after CPAP therapy., Conclusions: HTL was significantly poorer among participants with OSA (especially in severe cases) than non-OSA controls. Studies on patients with OSA with SNHL treated with CPAP did not show significant improvements in midfrequency HTL. Further studies are warranted on these issues., (© 2022 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Background: The World Health Organization recently described sudden sensorineural hearing loss (SSNHL) as a possible adverse effect of COVID-19 vaccines. Recent discordant pharmacoepidemiologic studies invite robust clinical investigations of SSNHL after COVID-19 messenger RNA (mRNA) vaccines. This postmarketing surveillance study, overseen by French public health authorities, is the first to clinically document postvaccination SSNHL and examine the role of potential risk factors., Objective: This nationwide study aimed to assess the relationship between SSNHL and exposure to mRNA COVID-19 vaccines and estimate the reporting rate (Rr) of SSNHL after mRNA vaccination per 1 million doses (primary outcome)., Methods: We performed a retrospective review of all suspected cases of SSNHL after mRNA COVID-19 vaccination spontaneously reported in France between January 2021 and February 2022 based on a comprehensive medical evaluation, including the evaluation of patient medical history, side and range of hearing loss, and hearing recovery outcomes after a minimum period of 3 months. The quantification of hearing loss and assessment of hearing recovery outcomes were performed according to a grading system modified from the Siegel criteria. A cutoff of 21 days was used for the delay onset of SSNHL. The primary outcome was estimated using the total number of doses of each vaccine administered during the study period in France as the denominator., Results: From 400 extracted cases for tozinameran and elasomeran, 345 (86.3%) spontaneous reports were selected. After reviewing complementary data, 49.6% (171/345) of documented cases of SSNHL were identified. Of these, 83% (142/171) of SSNHL cases occurred after tozinameran vaccination: Rr=1.45/1,000,000 injections; no difference for the rank of injections; complete recovery in 22.5% (32/142) of cases; median delay onset before day 21=4 days (median age 51, IQR 13-83 years); and no effects of sex. A total of 16.9% (29/171) of SSNHL cases occurred after elasomeran vaccination: Rr=1.67/1,000,000 injections; rank effect in favor of the first injection (P=.03); complete recovery in 24% (7/29) of cases; median delay onset before day 21=8 days (median age 47, IQR 33-81 years); and no effects of sex. Autoimmune, cardiovascular, or audiovestibular risk factors were present in approximately 29.8% (51/171) of the cases. SSNHL was more often unilateral than bilateral for both mRNA vaccines (P<.001 for tozinameran; P<.003 for elasomeran). There were 13.5% (23/142) of cases of profound hearing loss, among which 74% (17/23) did not recover a serviceable ear. A positive rechallenge was documented for 8 cases., Conclusions: SSNHL after COVID-19 mRNA vaccines are very rare adverse events that do not call into question the benefits of mRNA vaccines but deserve to be known given the potentially disabling impact of sudden deafness. Therefore, it is essential to properly characterize postinjection SSNHL, especially in the case of a positive rechallenge, to provide appropriate individualized recommendations., (©Hung Thai-Van, Marie-Blanche Valnet-Rabier, Maëva Anciaux, Aude Lambert, Anaïs Maurier, Judith Cottin, Tessa Pietri, Alexandre Destère, Marlène Damin-Pernik, Fanny Perrouin, Haleh Bagheri. Originally published in JMIR Public Health and Surveillance (https://publichealth.jmir.org), 14.07.2023.)

Background and Objectives: Tinnitus is one of the most common symptoms of sudden sensorineural hearing loss (SSNHL), with the incidence of tinnitus in patients with SSNHL ranging from 60% to 90%. Little is known, however, about the specific audiologic and hematologic factors that may be associated with the development of tinnitus. To better understand the relationship between tinnitus and SSNHL, the present study compared audiologic and hematologic factors in SSNHL patients with tinnitus and without tinnitus., Subjects and Method: The present study compared 120 patients with SSNHL with tinnitus and 59 patients with SSNHL without tinnitus at their initial examination. Their audiology and hematologic test results were analyzed, and hearing recovery was determined by comparing the hearing thresholds before and after treatment., Results: 120 patients with tinnitus showed longer III and V latency in auditory brainstem response (ABR) tests, lower signal-to-noise ratios (SNR) at 2 kHz in transient evoked otoacoustic emissions (TEOAE) tests, and lower response rates at 2 kHz in distortion product otoacoustic emissions (DPOAE) tests of the affected ear ( p  < 0.05 each) than the 59 patients without tinnitus. However, there were no significant between-group differences in the mean hearing threshold and hearing recovery rate of the affected ear. Patients with tinnitus had significantly worse mean hearing thresholds and hearing thresholds at 4 kHz in the nonaffected ear. The percentages of monocytes and large unstained cells (%LUCs) were higher in the group without tinnitus ( p  < 0.05), although there were no significant between-group differences in inflammatory markers, such as neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR) and platelet-to-lymphocyte ratio (PLR)., Conclusion: Tinnitus accompanying SSNHL may be associated with baseline hearing level, as well as being an indicator of damage to outer hair cells and auditory nerves. Additional studies are needed to evaluate hematologic data in SSNHL patients with and without tinnitus.

Background: Meniere Disease (MD) is an inner ear syndrome, characterized by episodes of vertigo, tinnitus and fluctuating sensorineural hearing loss. The pathological mechanism leading to sporadic MD is still poorly understood, however an allergic inflammatory response seems to be involved in some patients with MD., Objective: Decipher an immune signature associated with the syndrome., Methods: We performed mass cytometry immune profiling on peripheral blood from MD patients and controls. We analyzed differences in state and differences in abundance of the different cellular subsets. IgE levels were quantified through ELISA on supernatant of cultured whole blood., Results: We have identified two clusters of individuals according to the single cell cytokine profile. These clusters presented differences in IgE levels, immune cell population abundance, including a reduction of CD56 dim NK-cells, and changes in cytokine expression with a different response to bacterial and fungal antigens., Conclusion: Our results support a systemic inflammatory response in some MD patients that show a type 2 response with allergic phenotype, which could benefit from personalized IL-4 blockers., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interests., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Auditory implants are classified into bone conduction (BAHA and Bonebridge; BB) and active middle ear implants (Vibrant Soundbridge; VSB) that stimulate cochlear hair cells, and cochlear implants (CIs) that stimulate neural structures. CIs should be performed as early as possible, and bilateral CIs have become popular because sound localization and speech recognition can be improved. CI is also considered a desirable treatment option for patients with single-sided deafness. VSB provides a safe and effective option for patients with conductive or mixed hearing loss and moderate to severe sensorineural hearing loss (SNHL); however, it use in patients with conductive or mixed hearing loss have only been approved in Japan. BAHA and BB implants have been approved by national insurance in Japan as bone conduction implants for patients with conductive or mixed hearing loss. Two fully implantable devices (Cochlear Carina and Envoy Esteem) are provided for patients with SNHL., Competing Interests: Declaration of Competing Interest The author declares no financial relationships or conflict of interest., (Copyright © 2022. Published by Elsevier B.V.)

Background: In the otology clinic, we often receive some sudden sensorineural hearing loss (SSNHL) patients accompanied by annoying tinnitus, who usually visited over three weeks after the onset. Nevertheless, due to the high treatment cost and relatively low cure rate, there are still great disputes about hospitalization or not for these patients. Aim: This study aimed to perform a retrospective analysis for analyzing the efficacy of treatment with oral steroids combined with postauricular steroid injection in patients with delaying effective treatment., Material/methods: A total of 157 eligible SSNHL patients with delaying effective treatment over three weeks were enrolled in this study. According to different treatment methods of oral steroids with or without postauricular steroid injection, these patients were divided into three groups: PO (prednisone oral) group, PSI (prednisone oral and postauricular steroid injection) group, and PII (prednisone oral and postauricular lidocaine injection) group. The changes in level of hearing, mean subjective tinnitus loudness, and side effects were analyzed in the three groups., Results: Hearing improvement and tinnitus remission were all observed in three groups after treatment. Compared with PO and PII groups, those patients in PSI groups had more improvement in level of hearing and mean subjective tinnitus. The level of tinnitus loudness was statistically significantly correlated with the level of PTA both before treatment and after treatment., Conclusion: Oral steroids combined with postauricular steroid injection should be employed for treatment of SSNHL patients with delaying effective treatment over three weeks., Competing Interests: None

Background: This study aims to determine and assess prognostic variables that might affect the hearing result in patients with idiopathic sudden sensorineural hearing loss following intratympanic steroid injection., Methods: In total, 190 patients with idiopathic sudden sensorineural hearing loss received intratympanic steroid injection. Two hearing indices (recovery and nonrecovery) will be analyzed as dependent variables; patient's age, time period between the onset of hearing loss and treatment, initial level of hearing (hearing loss pre), type of audiogram curve (upsloping, downsloping, and flat), presence of vertigo, presence of tinnitus, and diabetes) will be analyzed as prognostic factor variables., Results: Recovery was seen in 72% of the patients. Different preinjection audiogram curves and hearing grades had a significant effect on recovery, absence of vestibular symptoms and no diabetic history were noted to have a good prognosis. Delay in treatment by more than 30 days from the onset of hearing loss was associated with a worse prognosis., Conclusion: Idiopathic sudden sensorineural hearing loss associated with late treatment plan more than 1 month, presence of vertigo, diabetes, and profound prehearing loss were negative prognostic factors. Whereas age, gender, and presence of tinnitus did not affect prognosis. More stable response was obtained when intratympanic steroids were added within 1 month after diagnosis, and the patient presented with mild or moderate hearing loss grade, flat or downsloping pure tone audiometery curve, and absence of vertigo and nondiabetic with significantly good results.

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center., Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results., Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years., Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

Background: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menière-like cochlear vestibular symptoms, which may also have systemic effects. Corticosteroids are first-line treatment. DMARDs and biologics have been used to treat ocular and systemic symptoms of CS., Case Presentation: This is a case of a 35-year-old female who reported hearing loss, eye redness and photophobia. Her condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. CS was diagnosed after excluding other diseases. The patient still developed bilateral sensorineural hearing loss after receiving hormone, methotrexate, cyclophosphamide, and a variety of biological agents. Joint symptoms were relieved after treatment with a JAK inhibitor (tofacitinib), and hearing did not deteriorate further., Conclusions: CS should be involved in the differential diagnosis of keratitis. Early identification and intervention of this autoimmune disease can minimize disability and irreversible damage., (© 2023. The Author(s).)

Objective: The interruption of vascular supply to the inner ear is one of several proposed etiologies of sudden sensorineural hearing loss (SSNHL). The increased presence of cardiovascular risk factors may predispose patients to SSNHL through this pathway. This systematic review and meta-analysis studies the presence of cardiovascular risk factors in patients diagnosed with SSNHL., Data Sources: Databases included PubMed/Medline, OVID, EMBASE, Cochrane, and Web of Science., Review Methods: Inclusion criteria included studies featuring SSNHL patients presenting with 1+ cardiovascular risk factors. Exclusion criteria included case reports and studies without outcome measures. Two investigators independently reviewed all manuscripts and performed quality assessments using validated tools., Results: Of 532 identified abstracts, 27 studies met inclusion criteria (19 case-control, 4 cohorts, 4 case series). Of these, 24 underwent meta-analysis encompassing a total of 77,566 patients (22,620 SSNHL patients, 54,946 matched controls). The mean age was 50.43 years. SSNHL patients were more likely to have concomitant diabetes (odds ratio [OR] 1.61 [95% confidence interval [CI]: 1.31, 1.99; p < .00001]) and hypertension (OR 1.5 [95% CI: 1.16, 1.94; p = .002]). An increased standard mean difference of total cholesterol of 11.09 mg/dL (95% CI; 3.51, 18.67; p = .004) was noted in the SSNHL group compared with the controls. No significant differences in smoking, high-density lipoprotein, triglycerides, or body mass index were detected., Conclusion: Patients presenting with SSNHL have a significantly higher risk of concomitant diabetes, hypertension, and higher total cholesterol in comparison to matched controls. This may indicate a higher cardiovascular risk profile in this population. More prospective and matched cohort studies are needed to understand the role of cardiovascular risk factors in SSNHL., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Objective: To determine if exfoliation syndrome (XFS) is associated with hearing loss (HL) or vestibular dysfunction., Data Sources: PubMed, Scopus, CINAHL, and Cochrane Library through April 1, 2022., Review Methods: Two reviewers independently screened abstracts, selected articles for inclusion, and extracted data. Studies included for qualitative analysis conducted audiometric, tympanometric, or vestibular evaluations on all subjects., Results: Twenty-one publications (1148 patients with XFS and 1212 controls) were included in the systematic review, and 16 publications (968 patients with XFS and 1147 controls) in the meta-analysis. Greater severity of HL was seen for patients with XFS compared to controls across all frequencies (odds ratio [OR] 8.8 [7.3-10.2]). Patients with XFS were more likely to have moderate to profound sensorineural HL (OR 1.8 [1.3-2.5]), and less likely to have none to mild HL (OR 0.34 [0.17-0.67]) or no HL (OR 0.37 [0.28-0.50]). Three studies found patients with XFS had lower tympanometric peaks. Two studies found that abnormal vestibular testing results could be more common for patients with XFS., Conclusions: HL is associated with XFS. A sensorineural component to HL is confirmed, and mixed HL is possible. Given the high prevalence and infrequent diagnosis of XFS, the authors hypothesize that the current understandings of presbycusis might be improved by further investigation in XFS. Laryngoscope, 133:1025-1035, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: To assess hearing function in chronic glaucoma patients in comparison to healthy individuals., Materials and Methods: This cross-sectional study included 24 primary open-angle glaucoma (POAG) patients (24 ears) and 22 pseudoexfoliative glaucoma (PEG) patients (22 ears) who were followed for at least 5 years in the Afyonkarahisar Health Sciences University Ophthalmology Department, as well as 21 age- and gender-matched healthy individuals (21 ears, control group). Following a thorough ophthalmological examination that included visual acuity and intraocular pressure measurements, as well as anterior and posterior slit-lamp biomicroscopy, audiometry was performed in all participants to determine hearing function., Results: Mean ages in the POAG, PEG, and control groups were 64.50±7, 66.90±4.51, and 64.38±4.36 years, respectively. The mean deviation in standard automated perimetry was -14.47±2.89 in the POAG group and -15.02±2.87 in the PEG group (p=0.306). When compared with the control group, the POAG group had significantly higher hearing thresholds at 500 (p=0.011) and 1,000 Hz (p=0.003), while the PEG group had significantly higher hearing thresholds at 250 (p=0.009), 500 (p=0.009), 1,000 (p=0.001), 2,000 (p=0.005), 4,000 (p=0.001), 8000 (p=0.010), and 10,000 Hz (p=0.009)., Conclusion: Both glaucoma and hearing loss are common chronic diseases that have an impact on the well-being of older people. Potential hearing problems in chronic glaucoma patients make routine ocular and otolaryngology examinations in older patients critical for prompt diagnosis and treatment., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors., (©Copyright 2023 by Turkish Ophthalmological Association | Turkish Journal of Ophthalmology, published by Galenos Publishing House.)