Sensorineural hearing loss in neurobrucellosis

1993 ◽  
Vol 107 (11) ◽  
pp. 1034-1036 ◽  
Author(s):  
Ratna Thomas ◽  
Mohan Kameswaran ◽  
Vel Murugan ◽  
B. C. Okafor
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Combination Treatment ◽  
Incidental Finding ◽  
Treatment Options ◽  
Sensorineural Hearing ◽  
Clinical Feature ◽  
Diagnostic Features ◽  
Diagnostic Screening

AbstractA case of neurobrucellosis presenting to the otologist with sensorineural hearing loss (SNHL) as the predominant clinical feature is reported. The diagnostic features and treatment options are discussed and the need for prolonged combination treatment to prevent relapse and further deterioration of hearing stressed. SNHL in neurobrucellosis has hitherto been reported principally in neurology literature as something of an incidental finding and so escapes the attention of otologists. It is hoped that this report will alert otologists in areas where brucellosis is endemic to the need to include tests for brucellosis in the routine diagnostic screening for SNHL. Practitioners in other locations should also consider this possibility when dealing with patients who have visited or lived in endemic regions.

Sudden Sensorineural Hearing Loss in Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Clinical Review

2020 ◽  
Vol 16 (2) ◽  
pp. 84-91
Author(s):  
Julia L. Riera ◽  
María del R. Maliandi ◽  
Jorge L. Musuruana ◽  
Javier A. Cavallasca
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Clinical Feature ◽  
Systemic Lupus ◽  
Bilateral Involvement

Background: Sudden sensorineural hearing loss (SSNHL) is defined as a sudden loss of hearing, usually unilateral, of more than 30 dB in 3 contiguous frequencies of the tonal audiometry. SSNHL estimates an incidence ranging from 5 to 20 per 100.000 people per year. In approximately 75% of cases, a cause cannot be identified. However, it could be a clinical manifestation of Systemic lupus erythematosus (SLE) and Antiphospholipid Syndrome (APS). Objective: This review will focus on the clinical presentation, diagnosis, and management of the SLE and APS associated SSNHL. Methods: We searched in PubMed, Scopus, Lilacs, and Cochrane reviewing reports of Sudden sensorineural hearing loss in SLE and/or APS. Articles written in English and Spanish, and were available in full text, were included. Results: In patients with SLE, bilateral involvement was frequent. Antiphospholipid antibodies were positive in the majority of the patients. Corticosteroids were the mainstay of the treatment. The auditory prognosis was poor with total hearing loss recovery reached in only 22% of patients. : On the other hand, most of the patients with SSNHL and APS were males and presented associated symptoms such as vertigo, tinnitus and/or headache, 75% had bilateral disease. Lupus anticoagulant and aCL were found in equal proportions, all patients were anticoagulated, and aspirin was associated in 25% of the cases. Complete resolution or improvement of symptoms was observed in 25% of the patients. Conclusion: Sudden sensorineural hearing loss, can be a clinical feature of SLE and APS. Treating physicians should be aware of this devastating complication, especially when bilateral involvement occurs.

S210 – Asymmetric Sudden SNHL: Is All This Screening Necessary?

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P146-P146
Author(s):  
Selena E. Heman-Ackah ◽  
Dunn Ryan ◽  
Tina C. Huang
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Tertiary Care ◽  
Care Facility ◽  
Retrospective Chart Review ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Diagnostic Evaluation ◽  
Diagnostic Screening ◽  
Tertiary Care Facility

Objectives A number of factors have been identified that contribute to the presentation of asymmetric sudden sensorineural hearing loss (ASSNHL). Routinely patients presenting with ASSNHL undergo a battery of serologic testing and imaging in an attempt to determine a cause. The study will assess the utility of this diagnostic evaluation in elucidating a cause of an asymmetric sensorineural hearing loss. At the conclusion of this presentation, the participants should be able to: 1) Understand the utility of diagnostic screening in the management of ASSNHL. 2) Understand the cost-effectiveness of the diagnostic screening for ASSNHL. Methods The study design is a retrospective chart review. Charts from patients presenting to the otolaryngology clinic of a tertiary care facility between January 1, 2000, and November 30, 2007, with ASSNHL confirmed with audiometric evaluation were reviewed. Diagnostic tests included in the work-up of ASSNHL and test results were recorded. Percentage of positive tests was determined and compared to national data. Cost analysis of the diagnostic battery was performed. Results All patients presenting with sudden sensorineural hearing loss received 1 or more of the following serologic and radiographic tests: ANA, cholesterol, creatinine, DNA-ds, ESR, blood glucose, Lyme titer, rheumatoid factor, RPR, FTA-ABS, HSP, T3, TSH and MRI with gadolinium. The average cost associated with the full diagnostic evaluation is greater than $1,500. The positive rate for any of these tests was extremely low. Conclusions The utility of the comprehensive ASSNHL evaluation should be reconsidered. The choice of diagnostic evaluation should be directed by patient risk factors and exposures.

scholarly journals Hyperbaric Oxygen Therapy in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss

2015 ◽  
Vol 23 (3) ◽  
pp. 87-91
Author(s):  
Debashish Guha ◽  
Shaoni Sanyal ◽  
Chayan Bhattacharya ◽  
Abhijit Santra ◽  
Swagatam Banerjee
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hyperbaric Oxygen ◽  
Hyperbaric Oxygen Therapy ◽  
Oxygen Therapy ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Clinical Feature ◽  
Tertiary Referral Hospital ◽  
Before And After

Backgrounds and objectivesIdiopathic sudden sensorineural hearing loss (ISSNHL) is an entity whose diagnosis and treatment remain controversial to date. Various modalities of treatment have been tried with varying degrees of success. Hyperbaric oxygen therapy (HBOT) is a recent modality of treatment for this condition which acts by improving cochlear microcirculation. The objective of this study was to evaluate the effect of HBOT in improving hearing loss and secondary symptoms patients with ISSNHL.MethodsThis prospective study was undertaken over a 6 month period from the Department of Otorhinolaryngology in a tertiary referral hospital in eastern India. 48 consecutive newly diagnosed ISSNHL patients were included in this study. The patients were subjected to HBOT at 2 A.T.A (Atmosphere Absolute) for an hour over 45 days. Hearing status and secondary symptoms as assessed by clinical tests, pure tone audiogram were analysed before and after HBOT.ResultsMales comprised 62.5% (30/48) of our study population, mean age of the population was 49.3 ± 13.4 years. 45/48 patients (93.7%) had unilateral ISSNHL, while 3 (6.3%) had bilateral ISSNHL. Average hearing loss in the affected ear before and after treatment was 79.96dBHL and 62.27dBHL respectively. The most common secondary clinical feature was tinnitus (27/48, 56.3%), followed by vertigo (24/48, 50%), aural fullness (15/48, 31.25%) and nystagmus (5/48, 10.41%). After treatment tinnitus and vertigo were showed marked improvement; (22/27, 81.48%) and (18/24, 75%). 5 out of 15 (33.34%) patients showed reduced aural fullness. There was no improvement in patients suffering from nystagmus.

scholarly journals A Window of Opportunity: Perilymph Sampling from the Round Window Membrane Can Advance Inner Ear Diagnostics and Therapeutics

2022 ◽  
Vol 11 (2) ◽  
pp. 316
Author(s):  
Madeleine St. Peter ◽  
Athanasia Warnecke ◽  
Hinrich Staecker
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Hearing Aids ◽  
Biomarker Discovery ◽  
Treatment Options ◽  
Round Window ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Promising Source

In the clinical setting, the pathophysiology of sensorineural hearing loss is poorly defined and there are currently no diagnostic tests available to differentiate between subtypes. This often leaves patients with generalized treatment options such as steroids, hearing aids, or cochlear implantation. The gold standard for localizing disease is direct biopsy or imaging of the affected tissue; however, the inaccessibility and fragility of the cochlea make these techniques difficult. Thus, the establishment of an indirect biopsy, a sampling of inner fluids, is needed to advance inner ear diagnostics and allow for the development of novel therapeutics for inner ear disease. A promising source is perilymph, an inner ear liquid that bathes multiple structures critical to sound transduction. Intraoperative perilymph sampling via the round window membrane of the cochlea has been successfully used to profile the proteome, metabolome, and transcriptome of the inner ear and is a potential source of biomarker discovery. Despite its potential to provide insight into inner ear pathologies, human perilymph sampling continues to be controversial and is currently performed only in conjunction with a planned procedure where the inner ear is opened. Here, we review the safety of procedures in which the inner ear is opened, highlight studies where perilymph analysis has advanced our knowledge of inner ear diseases, and finally propose that perilymph sampling could be done as a stand-alone procedure, thereby advancing our ability to accurately classify sensorineural hearing loss.

Susac-Syndrom: ein diagnostisches Chamäleon

2019 ◽  
Vol 98 (04) ◽  
pp. 268-275
Author(s):  
M. I. Stefanou ◽  
D. Doycheva ◽  
A. Ebrahimi ◽  
J.-M. Dörr ◽  
U. Ziemann
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Interdisciplinary Collaboration ◽  
Immunosuppressive Treatment ◽  
Response To Therapy ◽  
Sensorineural Hearing ◽  
Neurological Deficits ◽  
Mri Findings ◽  
Diagnostic Features ◽  
Clinical Triad

AbstractSusac’s syndrome (SuS) is a rare, probably autoimmune endotheliopathy of the central nervous system, retina and inner ear. It is characterized by a clinical triad of encephalopathy, branch retinal artery occlusions (BRAOs) and sensorineural hearing loss. To date, more than 300 cases of SuS have been reported in the literature. However, SuS remains an under- and misdiagnosed entity in the clinical setting. This report presents an exemplary case of a patient, who was initially misdiagnosed with relapsing-remitting multiple sclerosis. At initial presentation, the patient did not demonstrate the complete clinical triad, and the interval between symptom onset and diagnosis was 4 months. Typical diagnostic features, which enabled the diagnosis of SuS were: a) MRI findings with T2-hyperintense snowball-like lesions of the corpus callosum and subcortical white matter and hyperintense lesions in diffusionweighted imaging with reduced apparent diffusion coefficient; b) BRAOs and vessel wall hyperfluorescence in fluorescein angiography and a significant thickness reduction of the inner retinal layers in optical coherence tomography; c) bilateral sensorineural hearing loss. The patient was aggressively treated with cyclophosphamide, rituximab, glucocorticoids and acetylsalicylic acid with a good response to therapy. This report draws attention to the need to take SuS into consideration in the differential diagnosis at the interface of neurological, psychiatric, ophthalmological and otorhinolaryngological disorders. As SuS may result in severe and persistent neurological deficits, an interdisciplinary collaboration is fundamental for the prompt diagnosis and initiation of adequate immunosuppressive treatment.

Novel TECTA Mutations Identified in Stable Sensorineural Hearing Loss and Their Clinical Implications

2014 ◽  
Vol 20 (1) ◽  
pp. 17-25 ◽  
Author(s):  
Ah Reum Kim ◽  
Mun Young Chang ◽  
Ja-Won Koo ◽  
Seung Ha Oh ◽  
Byung Yoon Choi
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
High Frequency ◽  
Autosomal Dominant ◽  
Sensorineural Hearing ◽  
Clinical Feature ◽  
Phenotype Correlation ◽  
Causative Gene ◽  
Bioinformatics Analyses ◽  
Genotype Phenotype Correlation

TECTA is a causative gene of autosomal dominant (DFNA8/A12) and autosomal recessive (DFNB 21) nonsyndromic sensorineural hearing loss (NSHL). Mutations in TECTA account for 4% of all autosomal dominant NSHL cases in some populations and are thus thought to be one of the major causes of autosomal dominant NSHL. A genotype-phenotype correlation for autosomal dominant mutations in the TECTA gene has been proposed. Two families (SB146 and SB149), which segregated moderate NSHL in an autosomal dominant fashion, were included in this study. We performed targeted resequencing of 134 known deafness genes (TRS-134) and bioinformatics analyses to find causative mutations for NSHL in these 2 families. Through TRS-134, we detected 2 novel mutations, i.e. c.3995G>T (p.C1332F) and c.5618C>T (p.T1873I), in the TECTA gene. These mutations cosegregated with NSHL in the studied families and were not detected in normal controls. The mutations c.3995G>T and c.5618C>T reside in the von Willebrand factor type D3-D4 (vWFD3-D4) interdomain of the zonadhesin (ZA) domain and the zona pellucida (ZP) domain, respectively. p.C1332F is the first mutation detected in the vWFD3-D4 interdomain of the ZA domain. The mutations p.C1332F and p.T1873I were associated with stable high-frequency and mid-frequency hearing loss, respectively. Notably, the cysteine residue mutated to phenylalanine in SB146 was not related to progression of sensorineural hearing loss, which argues against the previous hypothesis. Here we confirm a known genotype-phenotype correlation for the ZP domain and propose a hypothetical genotype-phenotype correlation which relates mutations in vWFD3-D4 to stable high-frequency NSHL in Koreans. This clinical feature makes subjects with the missense mutation in the vWFD3-D4 interdomain of TECTA potentially good candidates for middle ear implantation. i 2014 S. Karger AG, Basel

Idiopathic Sudden Sensorineural Hearing Loss in Children: A Systematic Review and Meta-analysis

2020 ◽  
pp. 019459982097657
Author(s):  
Leonardo Franz ◽  
Chiara Gallo ◽  
Gino Marioni ◽  
Cosimo de Filippis ◽  
Andrea Lovato
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Case Reports ◽  
Meta Analysis ◽  
Treatment Options ◽  
Case Series ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Systemic Steroid ◽  
Significant Difference

Objective Idiopathic sudden sensorineural hearing loss (ISSNHL) is uncommon in children, and its treatment and outcome are debated. We aimed to critically review evidence in the literature about treatment options and functional outcomes of pediatric ISSNHL. Additionally, we performed a meta-analysis of the results of combined systemic-intratympanic steroid therapy versus solely systemic treatment. Data Sources A search was run in the PubMed, Scopus, and Google Scholar databases. Review Methods Included articles were original ISSNHL case series, written in English, with a population age ranging from 2 to 19 years. Other study types (single case reports, editorials, and reviews) and case series with known etiology of sudden hearing loss were excluded. Descriptive data of patients, treatments, outcomes, and possible prognostic factors were extracted and recorded for every included study. Results Twelve articles (7 cohort and 5 case-control studies) met all the selection criteria. Based on only the studies that provided sufficient data about clinical outcome, the pooled overall recovery rate was 67.91% (95% CI, 58.34%-77.48%). No studies showed a significant difference between systemic steroid and combined systemic-intratympanic steroid. The pooled odds ratio for combined systemic-intratympanic steroid versus systemic steroid alone was 0.90 (95% CI, 0.36-2.27) based on a random effects model, ruling out any significant difference between these treatment options. Conclusions The results of our meta-analysis did not support combination therapy more than systemic steroid alone. Further prospective clinical trials are necessary to establish evidence-based therapies.

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