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'Inner Ear Autoimmune Disease' (830)


Mar
2018

This study aims to assess the frequency and the profile of hearing loss among patients with primary Sjögren's syndrome in a tertiary care hospital in India and to look for an association between hearing loss and immunological parameters (anti-SSA antibody, anti-SSB antibody, anticardiolipin antibodies, complements C3 and C4). This prospective observational study was done from January 2011 to October 2011 on consecutive patients diagnosed with primary Sjögren's syndrome in our tertiary care hospital. All patients underwent a puretone audiogram, tympanogram and acoustic reflex testing.

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Dec
1969

Meniere's disease (MD) is a rare disorder characterized by episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. It is associated with a fluid imbalance between the secretion of endolymph in the cochlear duct and its reabsorption into the subarachnoid space, leading to an accumulation of endolymph in the inner ear. Epidemiological evidence, including familial aggregation, indicates a genetic contribution and a consistent association with autoimmune diseases (AD).

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Dec
1969

Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism.

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Nov
2017

A rare subset of sarcoidosis, neurosarcoidosis, is reported to occur in 5-7% of sarcoid patients and can manifest in a variety of ways. The most common are facial paralysis and optic neuritis, less commonly causing cochleovestibulopathy, blindness, anosmia, and other cranial nerve (CN) palsies. The sensory deficit may be severe and psychiatric symptoms may result from the effects of the disease or steroid treatment.

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Sep
2017

Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by episodic inflammatory attacks on cartilaginous tissue. Elastic cartilage of the ears and nose, hyaline cartilage of the peripheral joints, vertebral fibrocartilage, tracheobronchial cartilage, and tissues rich in proteoglycan such as those in the eyes, heart, blood vessels, and inner ear are more likely to be affected. A 35-year-old male presented with complaints of hoarseness, tinnitus and dyspnea for 19 years, with a history of several diagnostic and therapeutic interventions for laryngeal and respiratory tract.

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Nov
2017

Autoimmune inner ear disease as a cause of sensorineural hearing loss is a poorly understood entity. Thus, the role of anticochlear antibodies (ACLAs) in clinical management is still not well established.
The aims of this study were to describe the use of ACLAs in our clinical setting and to understand the clinicians' therapeutic approach in these cases.

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Dec
2017

To investigate the relationship between sudden sensorineural hearing loss (SSNHL) and migraine, assess the prevalence of migraine in patients with idiopathic SSNHL, and determine a possible common vascular etiopathogenesis for migraine and SSNHL.
Prospective cohort study.
Tertiary referral center.

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Oct
2017

To describe the results in terms of audiometric relapse-free survival and relapse rate in immunomediated hearing loss patients treated exclusively with corticosteroids.
Retrospective study of patients with audiometric relapses, monitored from 1995 to 2014, in two centres of the Community of Madrid.
We evaluated 31 patients with a mean age of 48.

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Dec
1969

Among various forms of hearing loss, there are acute (within 72 hrs) or subacute (weeks to months) presentations that may be reversible with early pharmacological intervention. The workup of a patient presenting with hypoacusia includes the usual history and physical examination in conjunction with an audiometric assessment in order to categorize the hearing loss as conductive, sensorineural, or mixed. Sudden sensorineural hearing loss and autoimmune inner ear disease are acute and subacute forms of sensorineural hypoacusia most likely to be reversed with prompt pharmacological intervention.

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Dec
1969

Vitiligo is a chronic auto-immune skin disease, often associated or discovers other autoimmune pathologies. His association with Ophthalmological type pan uveitis and/or neurological type of meningitis and/or inner ear type of hearing loss determines the disease or Vogt -Koyanagi-Harada syndrome (VKH). We related the case of a young woman who consulted for recurrent uveitis for a year, and it was only with the onset of vitiligo lesions that VKH disease diagnosis was discussed and confirmed.

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Dec
1969

Although recent advances in magnetic resonance imaging (MRI) techniques have contributed to the detection of tiny lesions in the internal auditory canal (IAC) that may be responsible for sudden sensorineural hearing loss (SSNHL), there have been relatively few studies on the clinical characteristics of intra-labyrinthine hemorrhage (ILH) and labyrinthitis versus those regarding IAC tumors. Our purpose was to investigate the frequency of those IAC lesions on MRI and their clinical characteristics. Initial MRIs of 200 patients with SSNHL (93 men, 107 women; mean age = 48.

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Aug
2017

Mycoplasmas of humans and animals are usually associated with respiratory, autoimmune, genital and joint diseases. Human mycoplasmas have also been known to affect the brain. Severe central nervous system (CNS) diseases, such as encephalitis, have been linked to Mycoplasma pneumoniae and ureaplasma infections.

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Oct
2017

Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors.
Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers.

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Sep
2017

Methotrexate is a dihydrofolate reductase enzyme inhibitor with very high selectivity, and it is an antiproliferative folic acid antagonist used for the treatment of autoimmune diseases. In this study, our objective was to evaluate the effect of intratympanic Methotrexate application in the inner ear.
This study was planned as an animal study.

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Aug
2017

Tissue inhibitor of metalloproteinase-1 (TIMP-1) is a protein implicated in the control of inflammation in a number of autoimmune diseases. We hypothesized that the balance of TIMP-1 and matrix metalloproteinase-9 (MMP-9) may influence the control or perpetuation of inflammation in corticosteroid-responsive (RES) and corticosteroid-resistant (NR) autoimmune inner ear disease (AIED) patients. In the present study, we observed that plasma from AIED patients exhibited greater levels of TIMP-1 values compared with normal healthy controls.

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Dec
1969

Sudden sensorineural hearing loss (S-SNHL) is an inner ear disorder with an abrupt hearing loss occurring <3days. The pathologic mechanism of the disease remains unclear, although autoimmunity has been regarded as one of the suggested causes, especially in bilateral form. In this study, we aimed to provide evidence for the involvement of autoimmunity in bilateral S-SNHL using proteomic approaches such as ProtoArray®, western blotting, immunoprecipitation, and liquid column mass spectrometry for mass screening of candidate antigens and autoantibodies based on the hypothesis that multiple autoantibodies and target antigens must exist in order for autoimmune bilateral S-SNHL to develop.

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Jul
2017

Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course.

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May
2017

The aim of this study was to investigate the potential role of video head impulse test (vHIT) in the detection of brainstem lesions in patients with multiple sclerosis (MS).
Sixty-eight participants were enrolled and divided into two groups: 39 healthy subjects (HC) (78 ears, 20 females, mean age 25,3±6,3) and 29 MS patients (58 ears, 14 females, mean age 33,7±7,7). Both groups underwent vHIT, and in MS group MRI was analyzed for the presence of brainstem lesions.

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Aug
2017

Loss of neural structures (such as hair cells or neurones within the spiral ganglion) has been proposed to be involved in Menière's disease (MD) (Spoendlin et al. Acta oto-laryngologica Supplementum 499:1-21, 1; Merchant et al. Eur Arch Oto-Rhino-Laryngol Off J Eur Feder Oto-Rhino-Laryngol Soc (EUFOS) Affil German Soc Oto-Rhino-Laryngol Head Neck Surg 252(2):63-75, 2; Tsuji et al.

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Jun
2017

F-18 FDG PET Findings for Vogt-Koyanagi-Harada Disease.

Nucl Med Mol Imaging 2017 Jun 1;51(2):190-192. Epub 2015 Dec 1.
Hye Lim Park, Ie Ryung Yoo, Sonya Youngju Park
Vogt-Koyanagi-Harada disease is a rare multisystemic granulomatous autoimmune disorder affecting pigmented tissues such as the choroid, meninges, inner ear, and the skin. Neurologic symptoms are usually mild. Clinical manifestations include generalized muscle weakness, headache, meningismus, vertigo, decreased visual acuity, hearing loss and mental changes ranging from mild confusion to psychosis, hemiparesis, dysarthria, and aphasia.

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Feb
2017

The purpose of this study was to consider the possible role of autoimmune diseases and paraneoplastic syndrome in the genesis of tinnitus. The incidence of autoimmune inner ear disease (AIED) is rare, accounting for <1% of all cases of hearing impairment and dizziness. In presence of auditory and vestibular deficit in oncological patients, a paraneoplastic syndrome with cochleovestibulopathy should be considered.

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Feb
2017

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.

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Feb
2017

Susac syndrome, a rare disorder, is thought to be mediated by autoantibodies. One of the potential targets of these autoantibodies could be an antigen in the microvessels of the brain, the retina, and the inner ear leading to central nervous system (CNS) alterations, visual disturbances, and hearing deficits. Our aim is to expand clinicians' diagnostic options when facing psychosis due to medical conditions.

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Oct
2017

Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed.

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Mar
2017

Mobile tablet audiometry in fluctuating autoimmune ear disease.

J Otolaryngol Head Neck Surg 2017 Mar 7;46(1):18. Epub 2017 Mar 7.
Scott Kohlert, Matthew Bromwich
Autoimmune inner ear disease (AIED) is a rare condition characterized by bilateral fluctuating sensorineural hearing loss (SNHL). The labile nature of this hearing loss makes it difficult to accurately quantify with conventional methods, and therefore it is challenging to rehabilitate.
Over a 9-month period one pediatric patient with severe AIED was monitored and conducted home audiograms using a previously validated testing system (Shoebox Audiometry).

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Dec
1969

A New Theory for Ménière's Disease: Detached Saccular Otoconia

Otolaryngol Head Neck Surg 2017 02 14;156(2):350-352. Epub 2016 Nov 14.
Jeremy Hornibrook, Philip Bird
Ménière's disease is an inner ear disorder characterized by vertigo attacks, fluctuating and progressive hearing loss, tinnitus, and aural fullness in the affected ear. The pathophysiology of Ménière's disease remains elusive. Theories so far are anatomical variation in the size or position of the endolymphatic sac and duct, viral inflammation or autoimmune involvement of the sac, or a genetically determined abnormality of endolymph control.

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Dec
1969

. Prospective, controlled cohort study to investigate possible alterations in brain glucose metabolism (CMRglc) in patients with Cogan's syndrome (CS)..

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Dec
1969

To detect and graduate endolymphatic hydrops or endolymphatic space dilations in patients with suspected Meniere's disease or immune-mediated inner ear disease by magnetic resonance imaging.
A prospective study was performed including all the patients with clinical suspicion of Meniere's disease or immune-mediated inner ear disease treated at the Otolaryngology department during a one year period. In all cases, magnetic resonance imaging (MRI) was performed in a 3T scanner.

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Oct
2016

Autoinflammatory diseases are a family of immune-mediated, rare diseases, some of which, exhibit sensorineural hearing loss (SNHL), suggesting potentially similar mechanisms of molecular pathogenesis between autoinflammatory-mediated hearing loss and autoimmune inner ear disease (AIED) may exist. The purpose of this review is to compare the clinical features of autoimmune and autoinflammatory diseases that affect hearing, discuss the limitations of our knowledge, and highlight potential new disease mechanisms and therapeutics.
Pubmed Literature Review; Google Scholar Literature review.

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Jul
2017

Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein.

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Dec
1969

Relapsing polychondritis (RP) is a very rare autoimmune disease characterised by a relapsing inflammation of the cartilaginous tissues (joints, ears, nose, intervertebral discs, larynx, trachea and cartilaginous bronchi), which may progress to long-lasting atrophy and/or deformity of the cartilages. Non-cartilaginous tissues may also be affected, such as the eyes, heart, aorta, inner ear and skin. RP has a long and unpredictable course.

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Dec
1969

Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments.

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Dec
1969

Globally, a body of comparative case-control studies suggests that rheumatoid arthritis (RA) patients are more prone to developing hearing loss (HL). However, experimental evidence that supports this hypothesis is still lacking because the human auditory organ is not readily accessible. The aim of this study was to determine the association between bone damage to the ossicles of the middle ear and HL, using a widely accepted murine model of collagen-induced arthritis (RA mice).

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Dec
1969

This chapter will focus on vertigo/dizziness due to inner-ear malformations, labyrinthine fistula, otosclerosis, infectious processes, and autoimmune inner-ear disorders. Inner-ear malformation due to dehiscence of the superior semicircular canal is the most recently described inner-ear malformation. Vertigo/dizziness is typically induced by sound and pressure stimuli and can be associated with auditory symptoms (conductive or mixed hearing loss).

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Dec
1969

Bilateral vestibulopathy.

Handb Clin Neurol 2016 ;137:235-40
M Strupp, K Feil, M Dieterich, T Brandt
The leading symptoms of bilateral vestibulopathy (BVP) are postural imbalance and unsteadiness of gait that worsens in darkness and on uneven ground. There are typically no symptoms while sitting or lying under static conditions. A minority of patients also have movement-induced oscillopsia, in particular while walking.

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Sep
2016

Autoimmune sensorineural hearing loss, also known as autoimmune inner ear disease (AIED) is a rare clinical entity characterized by progressive and bilateral sensorineural hearing loss often accompanied by vestibular symptoms. Diagnosis is essential as a consistent number of patients show a positive response to steroids alone or in association with other immunosuppressive drugs. AIED is defined as primary when the disease is limited to the ear, whereas in up to a third of cases it is associated to other systemic autoimmune diseases such as Behçet disease (BD).

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Dec
2016

Cutaneous melanoma (CM) is an aggressive skin cancer entity, causing most skin cancer-related deaths. Autoimmune disorders have been described as potential paraneoplastic complications. The purpose of this study was to elucidate the possibility of a combinatory paraneoplastic affection of the retina and cochlea in patients with CM.

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Aug
2017

To report the treatment efficacy of continual intratympanic steroid injection (ITSI) therapy in a patient with refractory sensorineural hearing loss accompanied by relapsing polychondritis.
A 49-year-old female diagnosed with relapsing polychondritis at the age of 45 years and who had been treated with corticosteroids and immunosuppressants developed sensorineural hearing loss in the left ear.
Her unilateral hearing loss did not recover despite receiving one cyclophosphamide pulse treatment, one methylprednisolone pulse treatment, and weekly leukapheresis.

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Dec
1969

Relapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort.
We used the Hungarian Health Care Database to identify all persons with possible RP infection.

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Dec
2016

Ankylosing spondylitis (AS) is a chronic systemic inflammatory disease. Via autoimmune mediators, AS can damage the auditory system similar to other systems. Otoacoustic emission studies in AS patients showed that the damage that causes hearing loss was in the outer hair cells.

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Oct
2016

It was found that JIR children had potential sensory neural hearing loss and vestibular affection. Therefore, this study recommends: early complete audiologic evaluation of JIA child followed by regular follow-up, including TOAEs, extended high-frequency audiometry, and VNG. This follow-up is important for preliminary diagnosis and management in order to prevent the negative impact of hearing loss on a child's life.

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May
2016

Susac syndrome (retinocochleocerebral vasculopathy) is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. It presents with encephalopathy, branch retinal artery occlusions, and hearing loss. The condition is often under recognized because the clinical symptoms may present at different times and physicians may be unfamiliar with the syndrome.

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Feb
2016

Anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitides (AAVs) include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), the incidences of which are reported to be increasing in Japan. We reviewed the clinical records of 20 cases with systemic AAVs (five cases with MPA, nine cases with GPA, and six cases with EGPA), who visited our otolaryngology department with otological symptoms from 2004 to 2014, and compared the otological characteristics among the diseases. Otologic symptoms appeared as an initial symptom(s) in 40% of MPA cases, 56% of GPA cases, and 83% of EGPA cases.

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Oct
2016

Ossification of the cochlea was once considered to be a contraindication for cochlear implantation. Advances in cochlear implant technology and coding strategies have led to developments in different surgical procedures to manage cochlear ossification. The endoscopic technique allows a direct approach to the round window and the cochlea, especially in remodeled labyrinth, allowing a better vision of scala tympani.

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Dec
1969

We hypothesized that, in archived human temporal bone samples from patients with systemic lupus erythematosus (SLE), a pathologic condition exists in the stria vascularis and cochlear hair cells.
Sensorineural hearing loss is a common feature in SLE patients. However, the pathophysiologic mechanism of cochlear dysfunction is unclear.

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Mar
2016

Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1*0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis.

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Jan
2017

Carbon monoxide (CO) is well known as a highly toxic poison at high concentrations, yet in physiologic amounts it is an endogenous biological messenger in organs such as the internal ear and brain. In this study we tested the hypothesis that chronic very mild CO exposure at concentrations 25-ppm increases the expression of oxidative stress protecting enzymes within the cellular milieu of the developing inner ear (cochlea) of the normal CD-1 mouse. In addition we tested also the hypothesis that CO can decrease the pre-existing condition of oxidative stress in the mouse model for the human medical condition systemic lupus erythematosus by increasing two protective enzymes heme-oxygenase-1 (HO-1), and superoxide dismutase-2 (SOD-2).

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Feb
2016

microRNAs are a class of short non-coding RNAs containing about 19-22 nucleotides that regulate target gene expression through post-transcriptional repression or mRNA degradation, and involved in a variety of biological processes, such as cellular differentiation, proliferation, apoptosis and metabolism. microRNA-182 (miR-182), belonging to miR-183/96/182 cluster that consists of miR-182, -183, and -96, highly expresses in many cells and tissues, including osteoblasts, lymphocytes, adipocytes, retina, inner ear, etc. The recent studies of miR-182 highlighted its multiple important roles in differentiation, development, and functional maintenance in the cells and tissues.

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Dec
1969

Immune related adverse events affecting various organ systems are a recognized potential consequence of immune checkpoint inhibition. However, autoimmune inner ear disease is one complication not previously associated with the use of checkpoint inhibitors, though it has been reported after adoptive cell immunotherapy.
Here we present what we believe is the first case of autoimmune inner ear disease resulting from treatment with an immune checkpoint inhibitor in a patient with metastatic melanoma.

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