Bilateral Sudden Hearing Loss Research Articles

Ototoxicity in Immune Checkpoint Inhibitors Therapy

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

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

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

Mitochondrial DNA 13513G>A Mutation Causing Leber Hereditary Optic Neuropathy Associated With Adult-Onset Renal Failure.

Leber hereditary optic neuropathy (LHON) is one of the more common mitochondrial diseases and is rarely associated with mitochondrial renal disease. We report 3 unrelated patients with a background of adult-onset renal failure who presented to us with LHON and were shown to have a heteroplasmic mitochondrial DNA mutation (m.13513G>A). Retrospective chart review. All 3 patients had a background of chronic renal failure and presented to us with bilateral optic neuropathy (sequential in 2) and were found to have heteroplasmic m.13513G>A mutations in the MT-ND5 gene. Two of the patients were females (aged 30 and 45 years) with chronic kidney disease from their 20s, attributed to pre-eclampsia, one of whom also had diabetes and sudden bilateral hearing loss. One patient was a male (aged 54 years) with chronic kidney disease from his 20s attributed to IgA nephropathy. His mother had diabetes and apparently sudden bilateral blindness in her 70s. Renal biopsy findings were variable and included interstitial fibrosis, acute tubular necrosis, focal segmental glomerulosclerosis, and IgA/C3 tubular casts on immunofluorescence. Mild improvements in vision followed treatment with either idebenone or a combination supplement including coenzyme Q10, alpha-lipoic acid, and B vitamins. Our cases expand the clinical syndromes associated with m.13513G>A to include bilateral optic neuropathy and adult-onset renal disease. This highlights that in patients with bilateral, especially sequential, optic neuropathy a broad approach to mitochondrial testing is more useful than a limited LHON panel. Mitochondrial diseases present a diagnostic challenge because of their clinical and genetic variability.

Clinical characteristics of sudden sensorineural hearing loss with acute cerebral infarction

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. Conclusion: 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..

Sudden Bilateral Sensorineural Hearing Loss due to Basilar Artery Occlusion (P3-5.027)

<h3>Objective:</h3> We describe a case of a patient with occlusion of the basilar artery and bilateral anterior inferior cerebellar arteries (AICAs) who presented with a chief complaint of sudden bilateral sensorineural hearing loss (SNHL). <h3>Background:</h3> Sudden bilateral SNHL, defined as SNHL developing in less than 72 hours, is a rare phenomenon representing less than 5% of all acute SNHL cases. Whereas more frequent unilateral presentation is relatively benign, sudden bilateral SNHL represents a medical emergency and warrants immediate evaluation for life-threatening and/or reversible causes. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 56-year-old right-handed African American woman with personal medical history of hypertension, hyperlipidemia, chronic obstructive pulmonary disease, stroke, tobacco abuse, and obesity presented with sudden onset bilateral hearing loss preceded by one week of persistent vertigo and nausea. On admission she was hypertensive to 240/122 mm Hg, physical examination was significant for saccadic pursuit with spontaneous bilateral horizontal and vertical down-beating nystagmus, truncal ataxia, and bilateral SNHL confirmed by audiogram. CT of head alongside CT angiography showed occlusion of V4 segment of right vertebral artery extending through most of the basilar artery, and near-occlusion of bilateral AICAs. MRI showed late acute/early subacute ischemic infarcts in bilateral anterior cerebellum, cerebellar peduncles, and pons. Patient was out of time window for thrombolysis with rt-PA, mechanical thrombectomy was deferred due to &gt;24h from symptom onset and stable neurological examination throughout hospital admission. Blood pressure was gradually lowered and antiplatelet therapy with aspirin was initiated prior to discharge to rehabilitation facility. <h3>Conclusions:</h3> Sudden bilateral SNHL in isolation or accompanied by vestibular, cerebellar and/or brainstem signs particularly in patients with significant risk factors for cerebrovascular disease should alarm a clinician of a possibility of vertebrobasilar occlusion and prompt appropriate and timely medical management as delay in optimal medical treatment is associated with increased morbidity and mortality in this patient population. <b>Disclosure:</b> Dr. Buciuc has nothing to disclose. Dr. Gonzalez has nothing to disclose. Dr. Ali has nothing to disclose. Dr. Nankee has nothing to disclose. Dr. Holmstedt has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astrazeneca. The institution of Dr. Holmstedt has received research support from NIH. The institution of Dr. Holmstedt has received research support from Patient-Centered Outcomes Research Institute. The institution of Dr. Holmstedt has received research support from CSPC Pharmaceuticals . Dr. Holmstedt has received personal compensation in the range of $5,000-$9,999 for serving as a Study Adjudicator with Ischemia Care.

Sudden bilateral hearing loss due to vertebral artery dissection

AbstractA 69‐year‐old man suddenly developed bilateral hearing impairment and dizziness. Magnetic resonance imaging showed multiple infarctions in the area of the bilateral posterior circulation, and magnetic resonance angiography showed a completely occluded right vertebral artery (VA) and severe stenosis of the left VA. Pearl and string sign was detected in the left VA by cerebral angiography, leading to the diagnosis of arterial dissection. The infarctions in the anterior inferior cerebellar artery area were considered a result of artery‐to‐artery embolism. He regained his hearing gradually and was discharged in 2 weeks. Acute ischemic stroke with bilateral hearing loss is generally accompanied by proximal severe vascular legions, with poor outcome. Prognosis may depend on the underlying vascular pathology.

Bilateral Sudden Hearing Loss Caused by Basilar Artery Dissection: A Case Report

Bilateral sudden sensorineural hearing loss (SNHL) is rare, but a possible symptom of vertebrobasilar ischemia. A 69-year-old female patient with hypertension and atrial fibrillation presented with bilateral sudden hearing loss and vertigo without other neurological symptoms. On examination, she had left-beating horizontal nystagmus with positive head impulse on the left side. Pure tone audiometry revealed severe SNHL on both sides. Brain computed tomography angiography showed a dissection in the proximal portion of the basilar artery (BA) with occlusion of the mid-BA and bilateral anterior inferior cerebellar arteries (AICA), which confirmed on transfemoral cerebral angiography (TFCA). Left common carotid angiography demonstrated retrograde blood flow into the BA and right AICA via the left posterior communicating artery. During TFCA, her right hearing loss dramatically improved. Nine days later, follow-up TFCA showed an improvement of antegrade flow of the BA and AICA. We suggest that vertebrobasilar ischemia can be suspected in patients with bilateral sudden SNHL who present with risk factors for stroke.

Symptoms: Sudden Hearing Loss and Facial Paralysis

Symptoms: Sudden Hearing Loss and Facial Paralysis

Basilar artery occlusion presenting as sudden bilateral deafness: a case report

BackgroundMost sudden-onset hearing loss is due to otolaryngologic- and very rarely to cerebrovascular disease. We report a woman with sudden bilateral sensorineural hearing loss. This case suggests that even in the absence of brainstem or cerebellar signs, magnetic resonance imaging (MRI) and MR angiography (MRA) should be performed since such studies may reveal signs of life-threatening vertebrobasilar artery occlusion.Case presentationA 73-year-old Japanese woman with a history of hypertension, hyperlipidemia, and atrial fibrillation who suffered bilateral deafness with vertigo and vomiting was transferred from a local hospital to our department. On admission her consciousness was clear and vertigo was absent. Neurological examination revealed only bilateral sensorineural hearing loss. Head computed tomography (CT) returned no significant findings. The next morning she gradually developed severe drowsiness. Diffusion-weighted MRI demonstrated acute cerebral infarction in the brainstem and bilateral cerebellum; MRA showed basilar artery occlusion due to a cardioembolic thrombus. Revascularization was obtained by endovascular treatment. However, her condition worsened progressively during the following hours. CT revealed new brainstem lesions, massive cerebellar swelling, and obstructive hydrocephalus. She died on the second day after her admission.ConclusionsWhen hearing loss is due to vertebrobasilar occlusive disease, the prognosis is very poor. We suggest that vertebrobasilar stroke be suspected in patients with bilateral sensorineural hearing loss who present with risk factors for stroke such as atrial fibrillation and other neurologic signs.

Acute onset of tinnitus in patients with sudden deafness.

Objective:We made hypotheses that tinnitus will appear more likely in patients with sudden deafness with superior hearing in unaffected ear or with more severe acute hearing loss.Methods:A retrospective cohort study was performed. Five hundred forty-one patients were identified with idiopathic sudden sensorineural hearing loss (ISSHL) from January 1995 to August 2006. The exclusion criteria for this study were as follows: bilateral sudden hearing loss and Meniere disease, previous tinnitus or bilateral tinnitus at initial evaluation, and onset of hearing loss less than 7 days. The cohort enrolled 454 patients. The enrolled patients were classified into two groups: patient with acute onset tinnitus in the affected ear and patients without tinnitus at initial visit. Main outcome measures were patient age, the presence or absence of vertigo and tinnitus, audiometric patterns, the severity of hearing loss, and hearing in the unaffected ear.Results:Better contralateral hearing (n = 220 versus n = 72, P < 0.001) and younger age (48 versus 55 years, P < 0.001) were independently associated with the acute onset of tinnitus in patients with ISSHL. The degree of asymmetry between the ears did not differ significantly between patients with and without tinnitus. The sex, presence of vertigo, shape of audiogram, and severity of hearing loss were not correlated with tinnitus occurrence.Conclusions:Tinnitus triggered by ISSHL was more frequent in patients with better contralateral hearing and of a younger age, irrespective of the severity of hearing loss on the affected side or the asymmetry between the ears.

Valproic Acid-induced Bilateral Irreversible Hearing Loss

Valproic acid (VPA) is a widely used drug in the field of neuro-pschiatry for the treatment of various types of epilepsy, as prophylaxis for migraine headaches, and in the treatment and prevention of manic attacks related to bipolar disorder. Ototoxicity related to VPA is very rare and only a few cases have been reported in the literature. In this case report, a 21-year old male patient admitted to our clinic with the complaint of bilateral sudden total hearing loss associated with VPA is presented. The patient had a history of moderate hearing loss before initiation of VPA and on the second day of his VPA treatment, a further reduction in his hearing levels occurred. At the end of the third day, the total hearing loss had developed which did not respond to treatment and became permanent.

Bilateral Hearing Loss in Wernicke Encephalopathy

Wernicke encephalopathy is a syndrome caused by thiamine deficiency whose three typical symptoms are ophthalomoplegia, ataxia, and confusion. There are also rare reports of bilateral hearing loss, which can be caused by damage to the bilateral inferior colliculus or thalamic lesions, or by energy failure of the cochlea. This case suggests that thiamine should be administered based on the possibility of Wernicke encephalopathy occurring in malnourished or alcoholic patients with sudden bilateral hearing loss. Key Words: Wernicke encephalopathy, Thiamine, Hearing loss, bilateral

Clinical Practice Guideline: Sudden Hearing Loss (Update).

ObjectiveSudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently but not universally accompanied by tinnitus and/or v...

A sudden bilateral hearing loss caused by inner ear hemorrhage

IntroductionLabyrinthine hemorrhage is a rare cause of sudden deafness and generally concerns only on one side. Case summaryAn 84-year-old man with a past medical history of myelomonocytic chronic leukemia (CMML) suffered from sudden bilateral hearing loss associated with vertigo. The audiogram revealed a left cophosis and a right profound deafness. Videonystagmography showed a left vestibular deficit. The MRI showed a spontaneous strong T1 weighted signal in the left and right labyrinths, corresponding to a bilateral inner ear hemorrhage (IEH). Dizziness resolved rapidly following vestibular physiotherapy, in contrast to hearing which did not improve at all and let the patient isolated in his environment. The patient successfully underwent cochlear implantation so that he could communicate. DiscussionMost IEHs are unilateral and due to anticoagulants treatments and hematological diseases. Only rare cases have described bilateral labyrinth hemorrhage. This is the first case reported of bilateral labyrinth hemorrhage due to CMML.

Sudden hearing loss due to bee sting: A rare case

Sudden hearing loss (SHl); Define as, at least 30 dB sensorineural hearing loss in 3 consecutive frequencies. In general, idiopathic, viral and vascular causes are at the forefront. Local or systemic hypersensitivity reactions due to insect bites such as bee sting may occur. Sudden hearing loss is a more unknown finding in the literature due to bee sting. The first choice in treatment is systemic or local corticosteroid uses. In this case report, the patient who had bilateral sudden hearing loss following bee sting and treatment approaches have been presented.

Hereditary bilateral sudden sensorineural hearing loss

The aim of our study is to demonstrate acausal link between two distinct diagnoses, the hereditary hearing loss, and the sudden sensorineural hearing loss. Sudden sensorineural hearing loss is an emergency condition in otolaryngology and a rare diagnosis in childhood. Most often it only affects one ear and its cause remains unknown. We present a clinical study of a 10-year-old female patient presenting with bilateral sudden sensorineural hearing loss analyzed by Sanger sequencing of the GJB2 gene. The subject was referred to the hospital for bilateral sudden hearing loss which developed 3 days before the admission. Audiometric testing confirmed bilateral asymmetric sensorineural hearing loss. All routine diagnostic procedures including MRI and CT imaging showed normal results. She was treated with intravenous and intratympanic corticosteroids followed by hyperbaric oxygen therapy with partial hearing recovery in one ear. DNA analysis of the GJB2 gene identified biallelic c.35delG deletion. The subject had no other affected family members and her auditory development to that time was normal. Our finding extends the knowledge on phenotype variability in GJB2 variants. We suggest considering genetic testing in pediatric cases of bilateral sudden sensorineural hearing loss (Tab. 1, Fig. 4, Ref. 24).

Successful Treatment of Sudden Bilateral Sensorineural Hearing Loss due to Atherosclerotic Vertebral Artery Occlusion: A Case Report

Successful Treatment of Sudden Bilateral Sensorineural Hearing Loss due to Atherosclerotic Vertebral Artery Occlusion: A Case Report

Sudden bilateral deep hearing loss in a child – case study

Sudden bilateral deep hearing loss in a child – case study

Simultaneous idiopathic bilateral sudden hearing loss – characteristics and response to treatment

IntroductionThe aetiology of sudden hearing loss is poorly defined; however, infectious, vascular and neoplastic aetiologies are presumed to be responsible. In addition, the aetiology of bilateral sudden hearing loss is also unknown. ObjectiveThe objective of this study is identify the characteristics and treatment response of simultaneous bilateral sudden hearing loss. MethodsThis is a case–control study that practised in tertiary care academic centre. 132 patients with sudden hearing loss who were treated with systemic steroid and hyperbaric oxygen together were included. 26 patients had bilateral sudden hearing loss and 106 patients had unilateral sudden hearing loss. Patients were evaluated with clinical, audiological and radiological examinations and laboratory tests were done. Findings and response to treatment of the patients were compared. ResultsThe mean ages of patients with unilateral and bilateral sudden hearing loss were 42.0 years and 24.5 years respectively with a statistically significant difference (p<0.001). Immune response markers were more prevalent in bilateral sudden hearing loss. Pre-treatment audiologic thresholds were 69.1dB for unilateral sudden hearing loss and 63.3dB for the left ears and 67.6dB for the right ears for bilateral sudden hearing loss without significant difference. Post-treatment average hearing threshold in unilateral sudden hearing loss was 47.0dB and 55.4dB for the left ears and 59.0 for the right ears in bilateral sudden hearing loss. Average hearing improvement in unilateral sudden hearing loss group was significant (p<0.001) in spite of it was not significant in bilateral sudden hearing loss group for both ears. Between the groups; there was a significant difference for hearing improvement favouring unilateral sudden hearing loss (p<0.001). Tinnitus scores decreased significantly in both groups of patients (p<0.001) in spite of there was no significant difference between the groups of patients. ConclusionPatients with bilateral sudden hearing loss showed lower age, worse prognosis and higher rate of positive immune response markers. Cardiovascular risk factors seem to have an important role in the aetiology of unilateral cases whereas this importance was not present in bilateral ones.

Management of sudden sensorineural hearing loss.

Sudden sensorineural hearing loss (SNHL) is an otologic emergency and should be managed quickly and effectively. This review focuses on the management of sudden SNHL, primarily idiopathic sudden SNHL as it is the most common cause. Management options include observation, oral steroids, intratympanic steroids, or combined oral/intratympanic steroids. One-third to two-thirds of patients will achieve spontaneous recovery, most likely within the first 2 weeks. Despite the lack of randomized controlled trials on steroid therapy efficacy, all patients should be offered steroid treatment given low risk and possible significant benefits. All patients should undergo MRI with gadolinium to rule out retrocochlear disorder. Bilateral sudden hearing loss should alert the clinician to possible systemic disease. Sudden hearing loss is an otologic emergency. Appropriate counseling of patients is necessary to allow physician and patient to make a joint, educated decision. It is paramount to rule out retrocochlear disorder and to follow patients closely for improvement or need for future auditory rehabilitation.