Search Results (15)

Background: Sudden, non-traumatic hearing loss has been associated with vascular or inflammatory disorders. Hearing loss in Neuromyelitis optica spectrum disorder (NMOSD) is a very rare presentation. Methods: In this paper, we describe the case of a 58-year-old female patient with aquaporin-4-positive NMOSD exhibiting bilateral tinnitus and right-sided deafness in the context of a relapse. The auditory brainstem responses pointed to a lesion of the right peripheral auditory pathway (cochlea and/or auditory nerve). The patient’s hearing failed to improve after high-dose intravenous steroids; however, it showed slight improvement after plasmapheresis. We also conducted a systematic literature review in databases MEDLINE and Scopus in English, searching for all reported cases of hearing loss in NMOSD. Results: We included 10 studies reporting 15 cases of NMOSD with hearing loss. The vast majority of patients were female (11 out of 15, 73.3%), with an age range of 26 to 70 years. Hearing loss, ranging from mild to severe, seems more frequent in AQP4-positive cases, and it can even be the presenting symptom. It can present isolated or in combination with tinnitus, ataxia, and/or intractable vomiting. The auditory pathway impairment in NMOSD seems to be localized either centrally, i.e., cochlear nuclei or higher brainstem levels, or peripherally, i.e., in the cochlea or cochlear nerve itself. Intravenous methylprednisolone in high doses, followed by oral tapering, was the most common treatment option, resulting in a gradual improvement. Conclusions: This paper describes a rare case of peripheral auditory pathway affection in NMOSD, which is an inflammatory astrocytopathy mainly affecting the central nervous system. Early recognition of hearing loss in the context of an NMOSD relapse and subsequent treatment have a crucial impact on the hearing outcome of NMOSD patients. This expands our knowledge of NMOSD as an autoimmune aquaporin-4 channelopathy. Full article

Background/Objectives: Single-sided deafness (SSD) treatment options include Contralateral Routing of Signal (CROS) or Bilateral Routing of Signal (BiCROS) systems, bone conduction devices, cochlear implants (CIs) and no intervention. Aligning treatment recommendations with patient motivations is fundamental for satisfaction and successful outcomes. At our institution, a structured telehealth consultation precedes formal testing and includes treatment motivation exploration and comprehensive review of all interventions. This study examined SSD treatment motivations and their association with pursuing cochlear implantation. Methods: Adults who completed a pre-treatment SSD telehealth consultation over a four-year period were identified. Charts were retrospectively reviewed for demographics, SSD characteristics, treatment motivations, treatment choice, and CI outcomes. Results: A total of 122 adults were evaluated. Mean age was 56.3 (±13.0) years, and 59.8% were male. Mean SSD duration was 10.8 (±15.8) years. The most common etiology was sudden sensorineural hearing loss. The top primary motivations were improving overall hearing (23.0%), restoring hearing to the deaf ear (22.1%), and improving hearing in noise (21.3%). Most patients (45.1%) opted for a hearing aid, CROS or BiCROS system; 38.5% chose CI; and 14.8% declined treatment. Only 57.4% of those who selected CI had the implant, primarily due to surgery avoidance (31.5%) and insurance limitations (10.5%). Motivation did not predict treatment choice or CI receipt. Among CI recipients (n = 27), those motivated by hearing restoration demonstrated poorer speech outcomes and datalogging. Conclusions: Improving overall hearing and restoring hearing to the deaf ear were the most common motivations for seeking SSD treatment. Adult CI recipients had similar motivations to those who chose non-surgical options. Full article

Introduction: Hyperbaric oxygen therapy (HBOT) is one of the treatment methods in patients with sudden sensorineural hearing loss (SSNHL). It is recommended as an elective treatment in patients undergoing steroid therapy. According to current scientific reports, HBOT should be implemented within two weeks after the first symptoms. However, as far as the profile of HBOT is concerned, there are no straightforward recommendations. Methods: The data obtained from the medical records of 218 patients undergoing HBOT for SSNHL at the Military Institute of Medicine—National Research Institute were analyzed statistically for the impact of the duration and the delay in implementing HBOT on the end results of pure-tone audiometry (PTA). Results: A statistically significant hearing improvement in patients undergoing more than 15 cycles of HBOT was detected at all frequencies except for 1500 Hz; in the group reporting for treatment with a delay of more than 10 days, hearing improvement was statistically unsignificant at frequencies of 1500, 3000, and 4000 Hz. Conclusions: The statistical analysis showed that the urgent onset of HBOT could be a significant factor in the therapy of SSNHL. Full article

Hearing loss and the related otologic manifestations are receiving increased scrutiny as significant causes of morbidity in Fabry disease. However, the relative risks of auditory deficits among patients with Fabry disease relative to the general population without a diagnosis of Fabry disease have not been studied. This study aims to explore the associations between Fabry disease and hearing-related manifestations using a nationwide population-based dataset. We identified study patients for this cross-sectional study from the 2015–2017 claims databases of the Taiwan Longitudinal Health Insurance Database 2005. We first identified 2312 patients aged over 20 years with a diagnosis of Fabry disease. We used propensity score matching to select five comparison patients per patient with Fabry disease and 11,560 comparison patients without Fabry disease. We used multivariable logistic regressions to estimate the odds ratios (ORs) and corresponding 95% confidence intervals (CIs) for tinnitus, hearing loss, and sudden deafness among Fabry disease patients vs. comparison patients. Chi-square tests showed statistically significant differences between patients with and without Fabry disease in the prevalence rates of tinnitus (16.7% vs. 11.7%, p < 0.001), hearing loss (7.5% vs. 6.2%, p = 0.014) and sudden deafness (1.7% vs. 1.0%, p = 0.005). Multiple logistic regression revealed that patients with Fabry disease were more likely to suffer from tinnitus, hearing loss and sudden deafness, with adjusted odds ratios of 1.513 (95% CI = 1.336–1.713), 1.246 (95% CI = 1.047–1.483), and 1.681 (95% CI = 1.166–2.423), respectively. We found that Fabry disease is significantly associated with certain auditory manifestations, including hearing loss, sudden deafness, and tinnitus. Full article

Oral, intravenous, or intratympanic steroid therapy (ST) are commonly applied methods of pharmacotherapy in Sudden Sensorineural Hearing Loss (SSNHL). There are vast discrepancies on the recommended initial dose and the duration of ST in medical reports. The aim of the research is a retrospective comparison of patients’ examination results with different therapeutical strategies. The medical records of 218 patients treated for SSNHL at the Military Institute of Medicine were subjected to retrospective analysis by comparison of the results of pure tone audiometry prior to and after treatment with steroid therapy (first-dose delay, mass of first dose, duration of treatment). Postponement of implementation of ST to 5 days resulted in a significant improvement of hearing across all frequencies. The implementation of ST sequentially in periods (5th–10th day; >10th day) resulted in a statistically insignificant improvement of hearing in the following frequencies: 250 Hz, 1000 Hz, 1500 Hz, 2000 Hz, 3000 Hz, 4000 Hz. There was a statistical improvement of hearing within all frequencies analyzed for the initial dose of prednisone above 50 mg. For an initial dose below 50 mg, in 4000 Hz, the improvement of hearing was statistically insignificant. The research demonstrated a significant influence of steroid therapy on treatment results in patients with Sudden Sensorineural Hearing Loss. Full article

Background and Objectives: Several treatments are available for sudden sensorineural hearing loss (SSNHL), but no studies have compared the different treatments based on the delay from the onset of the disease. Our study aims to compare the effect of hyperbaric oxygen therapy (HBOT), oral steroids (OS) and combination of both therapies (HBOT + OS) for treating SSNHL. Materials and Methods: This randomized study analyzed 171 patients with SSNHL. Patients were evaluated by pure tone audiometry test (PTA) at baseline (T0) and 20 days after treatment (T1). Three groups were available HBOT-A-, OS-B- and HBOT + OS-C-. After baseline PTA, patients were randomly assigned to each group. Statistical analysis was performed by one-way ANOVA and Chi-square. Results: Patients in the HBOT + OS and HBOT groups improved their auditory function (p < 0.05). HBOT was the best choice for treatment when started by 7 days from SSNHL onset, while HBOT + OS in case of late treatment. Profound SNHL recovered equally by HBOT and HBOT + OS (p < 0.05). Upsloping SNHL obtained better auditory results by HBOT compared to HBOT + OS (p < 0.05). Downsloping and flat SSNHL had the most improvement with HBOT + OS compared to HBOT only (p < 0.05). Conclusions: Combination of HBOT and OS is a valid treatment for SSNHL both in case of early and late treatment. Combination of HBOT and OS was the choice with the best results in case of treatment started >14 days from symptom onset. Full article

Various prognostic factors for idiopathic sudden sensorineural hearing loss (SSNHL) have been reported. Hearing loss directly derived from idiopathic SSNHL is important for understanding underlying pathogenesis and outcomes. We assessed the usefulness of evaluating hearing loss and recovery of idiopathic SSNHL on the basis of estimated hearing loss. The study included 115 patients whose characteristics and outcomes of hearing loss were investigated. The effects of vertigo/dizziness and age on hearing thresholds before/after treatment, nonaffected ear threshold, estimated hearing loss, improvement of hearing loss, and estimated remaining hearing loss were investigated. Vertigo/dizziness was a significant prognostic factor for hearing. In vertigo/dizziness patients, significantly more severe hearing loss and poorer improvement of hearing loss were observed above 500 Hz and below 1000 Hz, respectively. Severe hearing disorder remained at all frequencies. Conversely, post-treatment thresholds were significantly higher in the older population (≥65 years), although no differences in pretreatment thresholds were observed between the younger (≤64 years) and older populations. However, on the basis of nonaffected ear thresholds, previously existing hearing loss could have influenced the outcome. Thus, comparison of hearing outcomes between affected and nonaffected ears is essential for understanding hearing loss and outcomes in idiopathic SSNHL cases with existing hearing disorder. Full article

A 13-year-old girl with Jervell and Lange-Nielsen syndrome associated congenital long QT syndrome (LQTS) and central deafness was admitted for generalized seizures. LQTS had been diagnosed after birth and confirmed at genetic testing. β-blocker treatment was immediately started. Despite this, since the age of 12 months, recurrent cerebral seizures occurred leading to the diagnosis of epilepsy. Anti-convulsive therapy was initiated but without success. At the last admission, nadolol dosage seemed infratherapeutic. Considering malignant ventricular arrhythmias as the cause of seizures, the β-blocker dosage was adjusted to weight and levels of magnesium and potassium optimized. Furthermore, the patient received an implantable Medtronic Reveal LINQ Recorder^®. Since then, the adolescent has been asymptomatic with no arrhythmia documented. LQTS is due to one or more mutations of genes coding for ion channels. It may induce malignant ventricular arrhythmias and is a major cause of sudden cardiac death in children. Generalized cerebral seizures are extra-cardiac manifestations caused by decreased cerebral perfusion during ventricular arrhythmia. They are commonly misinterpreted as manifestations of epilepsy. For any patient with known or unknown LQTS who presents seizures with resistance to anti-convulsive therapy, a cardiac electrophysiological investigation should be performed promptly to ensure etiological diagnosis and optimize treatment. Full article

(1) Background: Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells. When they originate from neural elements within the vestibule or cochlea, they are defined as intralabyrinthine schwannomas (ILSs). Cochlear implant (CI) has been reported as a feasible solution for hearing restoration in these patients. (2) Methods: Two patients with single-sided deafness (SSD) due to sudden sensorineural hearing loss and ipsilateral tinnitus were the cases. MRI detected an ILS. CI was positioned using a standard round window approach without tumor removal. (3) Results: The hearing threshold was 35 dB in one case and 30 dB in the other 6 mo after activation. Speech audiometry with bisillables in quiet was 21% and 27% at 65 dB, and the tinnitus was completely resolved or reduced. In the localization test, a 25.9° error azimuth was obtained with CI on, compared to 43.2° without CI. The data log reported a daily use of 11 h and 14 h. In order to not decrease the CI’s performance, we decided not to perform tumor exeresis, but only CI surgery to restore functional binaural hearing. (4) Conclusions: These are the sixth and seventh cases in the literature of CI in patients with ILS without any tumor treatment and the first with SSD. Cochlear implant without tumor removal can be a feasible option for restoring binaural hearing without worsening the CI’s performance. Full article

The COVID-19 pandemic brought about many challenges to course delivery methods, which have forced institutions to rapidly change and adopt innovative approaches to provide remote instruction as effectively as possible. Creating and preparing content that ensures the success of all students, including those who are deaf and hard-of-hearing has certainly been an all-around challenge. This study aims to investigate the e-learning experiences of deaf students, focusing on the college of the Technical and Vocational Training Corporation (TVTC) in the Kingdom of Saudi Arabia (KSA). Particularly, we study the challenges and concerns faced by deaf students during the sudden shift to online learning. We used a mixed-methods approach by conducting a survey as well as interviews to obtain the information we needed. Our study delivers several important findings. Our results report problems with internet access, inadequate support, inaccessibility of content from learning systems, among other issues. Considering our findings, we argue that institutions should consider a procedure to create more accessible technology that is adaptable during the pandemic to serve individuals with diverse needs. Full article

Objectives: Sudden sensorineural hearing loss (SSNHL) is defined as sensorineural hearing loss of 30 dB or more over at least three adjacent audiometric frequencies occurring within a 72-h period of time. One of the causes of SSNHL could be the progressive inflammatory state caused by an infection. The aim of this study was to assess the prevalence of SSNHL caused by various factors, most importantly those potentially related to Lyme disease. Material and Methods: The study includes a group of 86 patients between the ages of 20 and 70 who were hospitalized due to SSNHL between 2017 and 2018. All of these patients underwent a detailed medical interview and an otolaryngological examination, including audiological and diagnostic tests. Additionally, ELISA and Western blot tests were performed to confirm the diagnosis of Lyme disease. Results: In this group of 86 patients, nine patients presented with positive antibodies toward Borrelia burgdorferi sensu lato. This group was treated with antibiotics and experienced partial or complete regression of their deafness. This may suggest a relationship between SSNHL and Lyme disease. Conclusion: Infections caused by Borrelia burgdorferi may contribute to the development of inflammatory and angiopathic lesions, which are a possible cause of SSNHL. The longer the duration of the infection, the greater the likelihood of permanent and irreversible changes in the vessels of the cochlea or auditory nerve. Therefore, serological tests for Borrelia burgdorferi should be performed during the diagnosis of SSNHL as a possible cause of this illness. Full article

Sudden sensorineural hearing loss seems to become a serious social health problem in modern societies. According to the World Health Organization (WHO) reports, adult-onset sensorineural hearing loss is found to be one of the leading diseases at the global level, especially in high-income countries, and is foreseen to move up from the 14th to 7th leading cause of the global burden of diseases by the year 2030. Although the direct mortality rate of this disease is very low, its influence on quality of life is huge; that is the reason why the implementation of the most effective and the safest therapies for the patient is crucial for minimizing the risk of complications and adverse reactions to treatment. The aim of this paper is to present hyperbaric oxygen therapy (HBOT) as a medical procedure useful in the treatment of sudden sensorineural hearing loss as adjunctive therapy of high efficacy. This paper focuses on the molecular mechanisms of action and clinical effectiveness of HBOT in the treatment of idiopathic sudden deafness, taking into consideration both the benefits and potential risks of its implementation. Full article

The inner ear contains many types of cell, including sensory hair cells and neurons. If these cells are damaged, they do not regenerate. Inner ear disorders have various etiologies. Some are related to aging or are idiopathic, as in sudden deafness. Others occur due to acoustic trauma, exposure to ototoxic drugs, viral infections, immune responses, or endolymphatic hydrops (Meniere’s disease). For these disorders, inner ear regeneration therapy is expected to be a feasible alternative to cochlear implants for hearing recovery. Recently, the mechanisms underlying inner ear regeneration have been gradually clarified. Inner ear cell progenitors or stem cells have been identified. Factors necessary for regeneration have also been elucidated from the mechanism of hair cell generation. Inducing differentiation of endogenous stem cells or inner ear stem cell transplantation is expected. In this paper, we discuss recent approaches to hair cell proliferation and differentiation for inner ear regeneration. We discuss the future road map for clinical application. The therapies mentioned above require topical administration of transgenes or drug onto progenitors of sensory cells. Developing efficient and safe modes of administration is clinically important. In this regard, we also discuss our development of an inner ear endoscope to facilitate topical administration. Full article

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan’s syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented. Full article

The purpose of this study is to test the effectiveness of intratympanic dexamethasone injections as a treatment for severe tinnitus in idiopathic sudden sensorineural hearing loss (SNHL). We studied 37 patients who received intratympanic dexamethasone injections and 14 control patients who did not receive it, with severe tinnitus after onset of unilateral sudden SNHL. Hearing level did not change during this study in any patient. The relationship between the duration of tinnitus and effectiveness of treatment was investigated in sudden SNHL. We used a visual analogue scale to evaluate 51 patients with severe tinnitus at the stage of stable hearing level after idiopathic sudden sensorineural hearing loss. Forty-one per cent of patients showed significant improvement after treatment. The average period between onset of sudden sensorineural hearing loss and initiation of intratympanic dexamethasone injection was significantly shorter (207 days) in the improved group than in the unchanged group (482 days) (P<0.001). In control group, one of 14 patients presented significant improvement spontaneously. Intratympanic dexamethasone treatment may be effective in treatment of severe tinnitus after sudden SNHL at the stage of stable hearing level, and the shorter the period from onset of sudden deafness to the start of intratympanic dexamethasone treatment, the greater the improvement in tinnitus that can be expected. Full article