1,721,024 research outputs found

    The diagnosis of autoimmune inner ear disease: Evidence and critical pitfalls

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    The purpose of this paper is to review the current diagnostic work-up for patients with suspected Autoimmune Inner Ear Disease (AIED). AIED is a rare disease accounting for less than 1% of all cases of hearing impairment or dizziness, characterized by a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. While specific tests for autoimmunity to the inner ear would be valuable, at the time of writing, there are none that are both commercially available and proven to be useful. Thus far, most of the identified antigens lack a clear association with localized inner ear pathology and the diagnosis of AIED is based either on clinical criteria and/or on a positive response to steroids. For clinical practice, we recommend an antigen-non-specific test battery including blood test for autoimmune disorders and for conditions that resemble autoimmune disorders. Nevertheless, if financial resources are limited, a very restricted work-up study may have a similar efficiency

    New drugs, new tests: Can we trust them?

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    Very few drugs utilized frequently in audiological medicine have undergone rigorous methodological testing to prove their efficacy. The employment of drugs on patients with dizziness, tinnitus, Meniére's disease or sudden sensorineural hearing loss is determined by empirical not scientific premises. Recent progress in genetics, molecular biology and neurobiology raises expectations that a breakthrough will be made before long for the treatment of certain audiological pathologies. While we are keen to see this progress delivered, we need to proceed with caution in the face of the proposed advances

    Symmetric sensorineural progressive hearing loss from chronic idiopathic pachymeningitis

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    We present the case of a 68 year-old man with a diffused hypertrophic pachymeningitis (HP) involving both internal auditory canals. The clinical symptoms were headache, decreased vision in one eye, progressive bilateral and symmetrical sensory-neural hearing loss (PSNHL) responsive to steroid treatment. Although hearing loss is a frequent manifestation of HP, only few studies reported an adequate audiological assessment and follow-up. Mechanisms related to the auditory involvement are discussed on the basis of audiological data. Gadolinium enhanced MRI is the most adequate technique for HP detection and for the differential diagnosis. A delay in the diagnosis of HP seems to be quite common and the consequences may be severe, especially in cases of optic nerve involvement. For these reasons, a cerebral MRI should probably be included in the assessment of PSNHL, especially when neurological signs coexist or are reported in the medical history

    Bilateral sudden profound hearing loss and vertigo as a unique manifestation of bilateral symmetric inferior pontine infarctions

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    Objectives: We present a case of sudden bilateral profound deafness and vertigo, without any accompanying neurologic signs, secondary to bilateral infarctions of the cochlear and vestibular nuclei. Methods: Vertigo, vomiting, tinnitus, and bilateral profound deafness suddenly developed in a 65-year-old woman without any accompanying neurologic signs. In particular, she did not present dysarthria, numbness, cranial nerve palsies, or visual or cerebellar signs. Results: Magnetic resonance imaging of the brain revealed 2 fresh infarctions of 8 to 10 mm symmetrically localized in the posterolateral bulbopontine junction. Angiography revealed a complete occlusion of the basilar artery, with a well-represented backward flow of its distal portion from the carotid artery via posterior communicating arteries. Excluding a transient ischemic attack that occurred 16 days after the acute episode, the patient had had no other neurologic events at 8 months of follow-up. Conclusions: Acute vertigo and sudden deafness in a patient with known cerebrovascular occlusive disease may represent the warning signs of an impending brain stem or cerebellar infarction, even when other neurologic signs are absent. These events are fortunately very rare, but should be considered by clinicians who see patients with vertigo. © 2007 Annals Publishing Company. All rights reserved

    The McGurk phenomenon in Italian listeners

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    In the classic example of the McGurk effect, when subjects see a talker say /ga/ and hear a simultaneous /ba/, they typically perceive /da/, a syllable that was not presented either acoustically, either visually. This phenomenon, although non-natural and recreated in laboratory studies, has been studied in order to better understand how, where and when the central nervous system processes and integrates visual and auditory signals. Till now, it has been demonstrated for English, Spanish, German languages, while in Japanese and Chinese it seems weaker. Aim of this study was to evaluate the entity of the McGurk effect for the Italian language. Our results demonstrate a robust McGurk effect for the Italian language, which has never been described before. The phenomenon is highly significant when an auditory bilabial CV is dubbed with a visual apico-dental or velar CV. Results are discussed on the basis of recent hypothesis regarding the bimodal perception.In the classic example of the McGurk effect, when subjects see a speaker say /ga/ and hear a simultaneous /ba/, they typically perceive /da/, a syllable that was not presented either acoustically, or visually. This phenomenon, although non-natural and recreated in laboratory investigations, has been studied in order to better understand how, where and when the central nervous system processes and integrates visual and auditory signals. Till now, it has been demonstrated for English, Spanish and German languages, while in Japanese and Chinese it seems weaker. Aim of this study was to evaluate the entity of the McGurk effect for the Italian language. Results obtained demonstrate a robust McGurk effect for the Italian language, which has never been described before. The phenomenon is highly significant when an auditory bilabial Consonant-Vowel is dubbed with a visual apico-dental or velar Consonant-Vowel. Results are discussed on the basis of the recent hypothesis regarding the bimodal perception

    Vertigo and autoimmunity

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    Autoimmune inner ear disease probably accounts for less than 1% of all cases of balance disorders, but its incidence is often overlooked due to the absence of a specific diagnostic test. Furthermore, in several systemic autoimmune diseases the vestibulo-cochlear system may be affected. Clinical features comprise generalized imbalance, ataxia, motion intolerance, episodic vertigo and positional vertigo. An autoimmune mechanism seems to be responsible for 6% of unilateral and 16% of bilateral forms of Ménière's disease. Oscillopsia and disequilibrium secondary to a bilateral vestibular paresis are probably caused by an autoimmune response in 5% of cases. Balance disorders of central origin may be due to other immuno-mediated disorders such as multiple sclerosis, brainstem encephalitis and vasculitidis. Aim of this paper is to assess the clinical features of autoimmune vertigo disorders through a systematic literature review
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