OBJECTIVE: We report the second case of bilateral sudden sensorineural hearing loss with intracochlear fibrosis due to chronic myeloid leukemia.
METHODS: A 44-year-old man presented to the emergency department with rapidly progressive bilateral hearing loss, tinnitus and vertigo, associated with dyspnea. Chronic myeloid leukemia complicated by pulmonary and cochleovestibular leukostasis was diagnosed, and cytoreductive treatment was started. Despite this treatment, bilateral total hearing loss and complete vestibular deficit persisted. MRI showed bilateral labyrinthitis, and emergency cochlear implantation was indicated. During surgery, inflammatory intracochlear tissue made electrode array insertion possible only against resistance. One year after implantation, there was significant improvement in speech recognition and communication scores.
CONCLUSIONS: In case of sudden sensorineural hearing loss induced by chronic myeloid leukemia, treatment should be as fast as possible, with prompt cochlear implantation in case of definitive profound hearing loss, because of the risk of cochlear fibrosis and ossification.

OBJECTIVE: To corroborate the vascular etiology of sudden sensorineural hearing loss (SNHL) utilizing magnetic resonance imaging (MRI).
METHODS: A 24-year-old male with a history of sickle cell disease experienced sudden SNHL and right horizontal nystagmus, without accompanying vertigo.
METHODS: Audiometric evaluation revealed left-sided SNHL, predominantly affecting high frequencies. Video head impulse testing demonstrated isolated dysfunction of the left posterior semicircular canal. An urgent brain MRI identified a recent punctiform ischemic stroke in the frontal region. A subsequent MRI, conducted with a 4-hour delay and post-contrast enhancement, highlighted a hyperintense signal within the left cochlear region and the left posterior semicircular canal.
CONCLUSIONS: The investigative results substantiate an infarction in the territory of the cochlear artery, precipitated by a vaso-occlusive event, thereby reinforcing the vascular hypothesis of cochleovestibular artery syndrome. This case underscores the congruence between clinical observations and delayed post-contrast MRI findings.

UNASSIGNED: The aim of present study was to evaluate the clinical efficacy of hyperbaric oxygen therapy (HBOT) as a primary therapy combined with standard systemic corticosteroid treatment for sudden sensorineural hearing loss (SSNHL) compared to treatment without the use of HBOT (non-HBOT) through clinical data and advanced analytical approaches.
UNASSIGNED: Case-control study.
UNASSIGNED: Conducted across three Japanese medical centers involving 298 SSNHL patients diagnosed between 2020 and 2023. Inclusion criteria encompassed first onset and treatment, WHO grade 3 or 4 initial hearing impairment, receipt of systemic corticosteroid therapy within 14 days of symptom onset, and initiation of HBOT within the same timeframe for the case group. The primary outcome measure was the difference in hearing improvement (mean hearing level in decibels, dB) between the two groups, assessed by pure-tone audiometry at baseline and 3 months post-treatment, using the inverse probability of treatment weighting (IPTW) method adjusted for covariate differences.
UNASSIGNED: The study included 67 patients in the HBOT group and 68 in the non-HBOT group. The HBOT group exhibited significantly greater hearing improvement (IPTW-adjusted difference: 7.6 dB, 95% CI 0.4-14.7; p = 0.038). Patients without vertigo in the HBOT group demonstrated substantial hearing improvement (11.5 dB, 95% CI 2.3-20.6; p = 0.014), whereas those with vertigo showed no significant improvement (-1.8 dB, 95% CI -11.8-8.3; p = 0.729). The HBOT group also had a significantly higher association with complete recovery (IPTW-adjusted odds ratio: 2.57, 95% CI 1.13-5.85; p = 0.025).
UNASSIGNED: In SSHNL, HBOT combination therapy yielded slightly but significantly improved hearing outcomes compared to non-HBOT treatment.
UNASSIGNED: 4.

OBJECTIVE: Hearing loss is a frequently observed comorbidity in patients with nasopharyngeal carcinoma (NPC). Accumulating evidence demonstrated that acupuncture can safely manage cancer and its treatment-related symptoms, but its effect in minimizing the likelihood of experiencing sudden sensorineural hearing loss (SSHL) has not been established. So this work aimed to determine the risk of SSHL among NPC persons with or without acupuncture use.
METHODS: One population-level, nested case-control design within a cohort study is employed. Relevant information on persons aged 20-80 years who were afflicted with NPC between 2000 and 2010 was extracted from a nationwide health claims database. From them, we identified the cases who had the first SSHL diagnosis occurring after NPC, and all of them were randomly matched to two controls without SSHL. Conditional logistic regression was employed to calculate odds ratios (OR) and its respective 95% confidence intervals (CI) for incident SSHL in relation to acupuncture treatment.
RESULTS: Eight hundred eleven SSHL cases were randomly matched to 1452 controls. Those receiving conventional care plus acupuncture use had a reduced adjusted OR of 0.39 (95% CI, 0.25-0.60) for SSHL. We further discovered that the longer usage of acupuncture remarkably correlated with reduction of SSHL risk in a dose-dependent manner.
CONCLUSIONS: Delineation of the benefit from integration of acupuncture into conventional care may be a reference in instituting more appropriate care for NPC subjects.
CONCLUSIONS: Patients living with NPC may benefit from a timely integration of acupuncture into routine care to lessen SSHL risk.

In multiple sclerosis (MS), the occurrence of sudden sensorineural hearing loss (SSNHL) is considered rare, with reported cases predominantly being unilateral. Bilateral cases are even rarer. Here, we report a case of bilateral SSNHL in a 20-year-old male diagnosed with MS. The patient, undergoing corticosteroid therapy for the management of MS, additionally received an intratympanic dexamethasone injection; however, it could not achieve significant improvement. Subsequently, the systemic dosage was increased for one week, resulting in substantial hearing improvement in both ears after three months. A review of MS-related SSNHL cases from 1987 to 2022 revealed 39 ears in the literature, with only five ears showing no hearing recovery. A remarkable 87.2% exhibited restored hearing, presenting a more favorable prognosis compared with idiopathic SSNHL. Although there were slight variations in administration methods and duration, all documented treatment approaches involve systemic corticosteroids. In some instances, SSNHL manifested as the initial symptom of MS. When SSNHL occurs in MS, auditory brainstem response (ABR) tests may reveal prolonged abnormalities, making ABR testing effective in cases where MS is suspected following SSNHL. In conclusion, the treatment of MS-related SSNHL appears appropriate with systemic corticosteroids, showing a significantly superior prognosis compared with idiopathic SSNHL.

Lyme neuroborreliosis (LNB) is a rare but potentially serious manifestation of Lyme disease, caused by the spirochete Borrelia burgdorferi. Although LNB can affect various neurological systems, neurosensory hearing loss as the sole presentation is uncommon. We report a case of a 23-year-old woman who presented with a 2-month history of temporal headache, tinnitus, and instability, which was followed by sudden bilateral hearing loss without any other associated symptoms. Pure-tone audiometry revealed profound bilateral hearing loss. Serological testing for various pathogens was negative, except for B. burgdorferi IgM, which was confirmed using Western blot analysis. The patient received doxycycline treatment; unfortunately, no recovery of hearing was observed. This case report highlights the importance of considering LNB as a potential cause of neurosensory hearing loss, particularly in areas where Lyme disease is endemic, as well as the need for timely diagnosis and treatment to prevent potential complications.

UNASSIGNED: The study aimed to evaluate the clinical characteristics and prognostic factors associated with unilateral sudden sensorineural hearing loss (SSNHL) related to environmental noise exposure before its onset.
UNASSIGNED: A total of 50 unilateral SSNHL patients exposed to environmental noise before onset (case group) and 924 unilateral SSNHL patients without any exposure to obvious inducing factors before onset (control group) were enrolled between January 2018 and October 2022. We retrospectively analyzed differences between both groups using the chi-square test, Fisher\'s exact tests, independent t-tests, and Mann-Whitney U-tests as appropriate before and after propensity score matching (PSM) based on sex, age, and initial pure-tone average (PTA). Prognostic factors for the case group were analyzed using univariate and multivariate logistic analyses between the effective and ineffective groups.
UNASSIGNED: Before PSM, significant differences were noted in age, sex, time to treatment, the proportion of combined diabetes mellitus, initial PTA, hearing gain, the incidence of vertigo or aural fulness, the rate of vestibular dysfunction or inner ear MRI abnormalities, the effective rate, the glucose and homocysteine levels, and the proportion of audiogram curve types (P < 0.05) between both groups. After PSM, compared to the control group, a longer time to treatment (Z= -3.02, P < 0.05), higher final PTA (Z= -2.39, P < 0.05), lower hearing gain (Z= -3.46, P < 0.05), lower rate of vestibular dysfunction (χ2 = 55.1, P < 0.001), and lower effective rate (χ2 = 4.87, P < 0.05) were observed in the case group. There was a significant difference between the audiogram curve types in both groups (χ2 = 14.9, P < 0.05). Time to treatment (95% confidence interval: 0.692-0.965, P < 0.05) and final PTA (95% confidence interval: 0.921-0.998, P < 0.05) were associated with the clinical outcomes for the case group.
UNASSIGNED: Unilateral SSNHL patients exposed to environmental noise triggers before onset showed a poorer effective rate and a lower rate of vestibular dysfunction than those who were not. The time to treatment and final PTA were associated with the prognosis of these patients.

We present an uncommon case of a pediatric patient with sudden-onset sensorineural hearing loss (SSNHL), a medical condition in which a person experiences a rapid loss of 30 or more decibels within a matter of hours or days. The patient is a nine-year-old female who, two years prior, suddenly lost hearing in her left ear after a 24-hour episode of nausea, vomiting, and left ear pain. She presented to our clinic two years after the episode, long after the window for evidence-based treatment for acute SSNHL, such as corticosteroid therapy or antivirals, had passed. However, she remembered the moment of her hearing loss vividly, an uncommon occurrence in pediatric patients. CT, MRI, family history, and physical exam were unremarkable. The patient had a brief hearing aid trial where she described being able to hear the sound but did not have any clarity in understanding the sound. The patient was ultimately treated with a unilateral cochlear implant and showed excellent subjective and audiogram responses. Continued research on the management of SSNHL in pediatric patients who present outside of the acute therapeutic window is needed.

Viral infection serves as the crucial etiology for the development of sudden sensorineural hearing loss (SSNHL). We aimed to investigate whether there is an association between concurrent Epstein-Barr virus (EBV) infection and SSNHL in an East Asian population. Patients who were older than 18 years of age and met the criteria of sudden hearing loss without an identifiable etiology were enrolled from July 2021 until June 2022, followed by the serological testing of IgA antibody responses against EBV-specific early antigen (EA) and viral capsid antigen (VCA) with an indirect hemagglutination assay (IHA) and real-time quantitative polymerase chain reaction (qPCR) of EBV DNA in serum before the treatment was initiated. After the treatment for SSNHL, post-treatment audiometry was performed to record the treatment response and degree of recovery. Among the 29 patients included during enrollment, 3 (10.3%) had a positive qPCR result for EBV. In addition, a trend of poor recovery of hearing thresholds was noted for those patients with a higher viral PCR titer. This is the first study to use real-time PCR to detect possible concurrent EBV infection in SSNHL. Our study demonstrated that approximately one-tenth of the enrolled SSNHL patients had evidence of concurrent EBV infection, as reflected by the positive qPCR test results, and a negative trend between hearing gain and the viral DNA PCR level was found within the affected cohort after steroid therapy. These findings indicate a possible role for EBV infection in East Asian patients with SSNHL. Further larger-scale research is needed to better understand the potential role and underlying mechanism of viral infection in the etiology of SSNHL.

UNASSIGNED: To assess the prevalence of cardiovascular risk factors (CVRFs) and their impact on acute unilateral inner ear hypofunction (AUIEH), including acute unilateral peripheral vestibulopathy (AUPVP), sudden sensorineural hearing loss (SSNHL) and acute unilateral audiovestibular hypofunction (AUAVH).
UNASSIGNED: One hundred and twenty-five patients consecutively diagnosed with AUPVP, SSNHL or AUAVH and 250 sex- and age-matched controls were included. Cases presented a mean age of 58.6 ± 14.7 years and included 59 women and 66 men. The correlation between CVRFs (high blood pressure [HBP], diabetes mellitus [DM], dyslipidemia [DLP], cardiocerebrovascular disease [CCVD]) and AUIEH was assessed by multivariate conditional logistic regression analysis.
UNASSIGNED: A higher prevalence of CVRFs was identified in patients than in controls (30 individuals with DM, 53 with HBP, 45 with DLP and 14 with a previous history of CCVD, p < .05). A significantly elevated risk of AUIEH was found in patients with two or more CVRFs (adjusted odds ratio [OR] 5.11; 95% CI 2.23-11.70). Previous CCVD individually predicted AUIEH (OR 8.41; 95% CI 2.36-29.88). Subgroup analysis showed the same tendency for AUPVP and SSNHL.
UNASSIGNED: Acute unilateral inner ear hypofunction patients presented significantly more CVRFs than controls, and the presence of two or more CVRFs was associated with AUIEH. Future studies evaluating vascular risk in AUIEH may include AUPVP and SSNHL patients from the same source population to better characterize risk profiles that can indicate a vascular origin.
UNASSIGNED: 3b.