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Robert W Baloh - One of the best experts on this subject based on the ideXlab platform.
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familial clustering of migraine Episodic Vertigo and meniere s disease
Otology & Neurotology, 2008Co-Authors: Michael J Kane, Robert W BalohAbstract:Epidemiological and family studies support an association between migraine, Episodic Vertigo, and Meniere’s disease, but reports on detailed pedigrees are limited (1–9). Several childhood periodic symptoms not temporally associated with headache such as cyclical vomiting, abdominal migraines, and benign paroxysmal Vertigo of childhood are recognized as precursors to the development of migraine headaches (10). This indicates that the susceptibility to migraine may manifest as these “migraine equivalents” during different periods of a person’s life. Episodic Vertigo, for example, has been shown to occur as commonly as visual auras in migraine patients, often without associated headache (11–13). Aural symptoms and even fluctuating hearing loss has been noted in migraine patients raising the possibility that Meniere’s disease, in some patients, might be directly related to their susceptibility to migraine. If Meniere’s disease could develop as part of a spectrum of migraine-associated symptoms (perhaps as a “complication”), then one would expect to see combinations of migraine, Meniere’s disease, and Meniere’s-like symptoms run within families because there has been growing evidence that migraine has a strong genetic basis. To show the association between migraine, Episodic Vertigo, and Meniere’s disease, we report 6 families in which these syndromes were highly associated, indicating that there may be a pathophysiological link between them.
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the relevance of migraine in patients with meniere s disease
Acta Oto-laryngologica, 2007Co-Authors: Y H Cha, Jae Brodsky, Gail Ishiyama, Chiara Sabatti, Robert W BalohAbstract:Conclusion. Coexistent migraine affects relevant clinical features of patients with Meniere's disease (MD). Objective. Epidemiological studies have shown an association between migraine and MD. We sought to determine whether the coexistence of migraine affects any clinical features in patients with MD. Patients and methods. In this retrospective case-control study of University Neurotology Clinic patients, 50 patients meeting 1995 AAO-HNS criteria for definite MD were compared to 18 patients meeting the same criteria in addition to the 2004 IHS criteria for migraine (MMD). All had typical low frequency sensorineural hearing loss and episodes of rotational Vertigo. Outcome measures included: sex, age of onset of Episodic Vertigo or fluctuating hearing loss, laterality of hearing loss, aural symptoms, caloric responses, severity of hearing loss, and family history of migraine, Episodic Vertigo or hearing loss. Results. Age of onset of Episodic Vertigo or fluctuating hearing loss was significantly lower in p...
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Migraine associated Vertigo.
Journal of Clinical Neurology, 2007Co-Authors: Robert W BalohAbstract:The interrelations of migraine and Vertigo are complex, eluding a simple localization either centrally or peripherally. Spontaneous Episodic Vertigo, benign paroxysmal positional Vertigo, and Meniere's disease all occur more frequently in patients with migraine than in those without. Family studies support a hereditary predisposition to migraine associated Vertigo. In this review, we discuss definitions, epidemiology, associated syndromes, neurootological abnormalities, genetics and treatment for patients with migraine and Vertigo.
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genetics of familial Episodic Vertigo and ataxia
Annals of the New York Academy of Sciences, 2002Co-Authors: Robert W Baloh, Joanna C JenAbstract:: The familial Episodic ataxias are prototypical inherited channelopathies that result in episodes of Vertigo and ataxia triggered by stress and exercise. Episodic ataxia type 1 (EA-1) is caused by missense mutations in the potassium channel gene KCNA1, whereas Episodic ataxia type 2 (EA-2) is caused by missense and nonsense mutations in the calcium channel gene CACNA1A. These ion channels are crucial for both central and peripheral neurotransmission. Within the last few years, the genetic mechanisms underlying these relatively rare familial Episodic ataxia syndromes have been worked out. They provide a model for understanding the mechanisms of more common recurrent Vertigo and ataxia syndromes, particularly those associated with migraine. Migraine affects as many as 15-20% of the general population, and it has been estimated that about 25% of patients with migraine experience spontaneous attacks of Vertigo and ataxia. We identified 24 families with migraine and benign recurrent Vertigo inherited in an autosomal dominant fashion. These families have numerous features in common with EA-1 and EA-2 (particularly EA-2), suggesting that benign recurrent Vertigo may be an inherited channelopathy. An ion channel mutation shared by brain and inner ear could explain the combined central and peripheral features of the syndrome.
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Episodic Vertigo: central nervous system causes.
Current opinion in neurology, 2002Co-Authors: Robert W BalohAbstract:Episodic ataxia type 2 is a prototypical Episodic Vertigo and ataxia syndrome that is caused by mutations in the calcium channel gene CACNA1A. Recent discoveries regarding the molecular mechanisms that underlie this syndrome provide a model for understanding the more common familial Episodic Vertigo syndromes, particularly those associated with migraine. Vertigo due to cerebrovascular disease can be of peripheral or central origin, and can mimic more benign peripheral vestibular disorders. Small infarcts in the cerebellum and lateral medulla can present with Vertigo without other localizing symptoms.
Niklas Dahl - One of the best experts on this subject based on the ideXlab platform.
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Stereocilin gene variants associated with Episodic Vertigo: expansion of the DFNB16 phenotype
European Journal of Human Genetics, 2018Co-Authors: Carina Frykholm, Joakim Klar, Tatjana Tomanovic, Adam Ameur, Niklas DahlAbstract:Vestibular disorders comprise a heterogeneous group of diseases with transient or permanent loss of vestibular function. Vestibulopathy is in most cases associated with migraine, Ménière disease, hereditary ataxias, or sensorineural hearing loss. We identified two brothers and their first cousin affected by hearing loss and Episodic Vertigo. The brothers were homozygous STRC nonsense variant [c.4027 C > T, p.(Q1343*)], whereas their first cousin was compound heterozygous for the STRC nonsense variant and a 97 kb deletion spanning the entire STRC gene. Clinical investigations confirmed pathological vestibular responses in addition to a characteristic DFNB16 hearing loss. The STRC gene encodes Stereocilin in the cochlea and in the vestibular organ where it ensheathes the kinocilium of the otolithic membranes. Stereocilin is associated with the gel overlaying the vestibular kinocilia, suggesting a role for the protein in sensing balance and spatial orientation. Our findings support such a function for Stereocilin in the vestibular organ and expand the phenotype associated with DFNB16.
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Stereocilin gene variants associated with Episodic Vertigo: expansion of the DFNB16 phenotype.
European journal of human genetics : EJHG, 2018Co-Authors: Carina Frykholm, Joakim Klar, Tatjana Tomanovic, Adam Ameur, Niklas DahlAbstract:Vestibular disorders comprise a heterogeneous group of diseases with transient or permanent loss of vestibular function. Vestibulopathy is in most cases associated with migraine, Meniere disease, hereditary ataxias, or sensorineural hearing loss. We identified two brothers and their first cousin affected by hearing loss and Episodic Vertigo. The brothers were homozygous STRC nonsense variant [c.4027 C > T, p.(Q1343*)], whereas their first cousin was compound heterozygous for the STRC nonsense variant and a 97 kb deletion spanning the entire STRC gene. Clinical investigations confirmed pathological vestibular responses in addition to a characteristic DFNB16 hearing loss. The STRC gene encodes Stereocilin in the cochlea and in the vestibular organ where it ensheathes the kinocilium of the otolithic membranes. Stereocilin is associated with the gel overlaying the vestibular kinocilia, suggesting a role for the protein in sensing balance and spatial orientation. Our findings support such a function for Stereocilin in the vestibular organ and expand the phenotype associated with DFNB16.
Thomas Brandt - One of the best experts on this subject based on the ideXlab platform.
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Chapter 62 - Vestibular migraine
Handbook of clinical neurology, 2010Co-Authors: Michael Strupp, Maurizio Versino, Thomas BrandtAbstract:Vestibular migraine is a chameleon among the Episodic Vertigo syndromes because considerable variation characterizes its clinical manifestation. The attacks may last from seconds to days. About one-third of patients presents with monosymptomatic attacks of Vertigo or dizziness without headache or other migrainous symptoms. During attacks most patients show spontaneous or positional nystagmus and in the attack‐free interval minor ocular motor and vestibular deficits. Women are significantly more often affected than men. Symptoms may begin at any time in life, with the highest prevalence in young adults and between the ages of 60 and 70. Over the last 10 years vestibular migraine has evolved into a medical entity in dizziness units. It is the most common cause of spontaneous recurrent Episodic Vertigo and accounts for approximately 10% of patients with Vertigo and dizziness. Its broad spectrum poses a diagnostic problem of how to rule out Meniere's disease or vestibular paroxysmia. Vestibular migraine should be included in the International Headache Classification of Headache Disorders (ICHD) as a subcategory of migraine. It should, however, be kept separate and distinct from basilar-type migraine and benign paroxysmal Vertigo of childhood. We prefer the term “vestibular migraine” to “migrainous Vertigo,” because the latter may also refer to various vestibular and non-vestibular symptoms. Antimigrainous medication to treat the single attack and to prevent recurring attacks appears to be effective, but the published evidence is weak. A randomized, double-blind, placebo-controlled study is required to evaluate medical treatment of this condition.
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Hyperviscosity syndrome and Vertigo
Vertigo, 2003Co-Authors: Thomas BrandtAbstract:Pathological hyperviscosity of the blood may be associated with polycythemia, hypergammaglobulinaemia or Waldenstrom’s macroglobulinaemia. Depending on the degree of hyperviscosity, Episodic Vertigo may also occur; it is most often caused by venous obstruction of the peripheral labyrinth. Symptomatic improvement is seen after blood hyperviscosity is reduced.
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Episodic Vertigo related to migraine 90 cases vestibular migraine
Journal of Neurology, 1999Co-Authors: Marianne Dieterich, Thomas BrandtAbstract:A retrospective study was conducted on 90 patients with Episodic Vertigo that could be related to migraine as the most probable pathomechanism. Since the majority of the patients did not fulfill the criteria of the International Headache Society (IHS) for basilar migraine, the diagnosis was substantiated by disease course, medical efficacy in treating (ergotamines) and preventing (metoprolol, flunarizine) attacks, ocular motor abnormalities in the symptom-free interval, and careful exclusion of the most relevant differential diagnoses, such as transient ischemic attacks, Meniere’s disease, and vestibular paroxysmia. The following clinical features were elaborated. The initial manifestation could occur at any time throughout life, with a peak in the fourth decade in men and a “plateau” between the third and fifth decades in women. The duration of rotational (78%) and/or to-and-fro Vertigo (38%) could last from a few seconds to several hours or, less frequently, even days; duration of a few minutes or of several hours was most frequent. Monosymptomatic audiovestibular attacks (78%) occurred as Vertigo associated with auditory symptoms in only 16%. Vertigo was not associated with headache in 32% of the patients. In the symptom-free interval 66% of the patients showed mild central ocular motor signs such as vertical (48%) and/or horizontal (22%) saccadic pursuit, gaze-evoked nystagmus (27%), moderate positional nystagmus (11%), and spontaneous nystagmus (11%). Combinations with other forms of migraine were found in 52%. Thus, migraine is a relevant differential diagnosis for Episodic Vertigo. According to the criteria of the IHS, only 7.8% of these patients would be diagnosed as having basilar migraine. However, to ensure that at least those presenting with monosymptomatic Episodic Vertigo (78% in our study) receive effective treatment, we propose the use of the more appropriate term “vestibular migraine.”
Roger C Lindeman - One of the best experts on this subject based on the ideXlab platform.
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surgical labyrinthectomy in the older patient
Otolaryngology-Head and Neck Surgery, 1998Co-Authors: Alan W. Langman, Roger C LindemanAbstract:OBJECTIVE: Ablation of vestibular function is a highly efficacious option in the treatment of disabling Vertigo arising from unilateral labyrinthine dysfunction. Regardless of the method used to ablate vestibular function, permanent posttreatment impairment of the balance function will develop in a number of treated patients. Many physicians who are involved in the care of the older patient with Episodic Vertigo are reluctant to recommend or perform a vestibular ablation procedure, because this treatment may result in permanent disequilibrium, which may be more detrimental to the older patient than the Episodic Vertigo. This study evaluates the outcome in older patients who underwent unilateral surgical labyrinthine ablation.METHODS: A retrospective analysis was done of the cases of 30 patients, all more than 60 years old, who underwent unilateral vestibular ablation because of disabling Episodic Vertigo by either transmastoid labyrinthectomy (n = 22) or transcanal labyrinthectomy (n = 8).RESULTS: Episodi...
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Sensorineural Hearing Loss with Delayed Onset of Vertigo
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 1995Co-Authors: Alan W. Langman, Roger C LindemanAbstract:Sensorineural hearing loss with delayed onset of Vertigo is a syndrome in which Episodic Vertigo arises in a person who has preexisting unilateral severe-to-profound sensorineural hearing loss. This syndrome has an ipsilateral form in which the Vertigo arises from the poorer hearing ear and a contralateral form in which the aural symptoms arise from the better hearing ear. The existence of this syndrome has only been noted within the past two decades. This report details our clinical experience with 17 persons with the ipsilateral form of this disorder. The onset of the Vertigo after the occurrence of the hearing loss was quite variable. It ranged from 1 to 60 years after the development of the hearing loss. The hearing loss occurred for several reasons. Most patients had hearing loss due to an unknown cause. The development of the Vertigo and the timing of the onset of the Vertigo were not related to the cause of the hearing loss. Bithermal caloric testing identified the offending labyrinth in most patients who underwent ablative vestibular surgery. Ablative vestibular surgery was performed in 13 of the 17 persons in this study because of disabling symptoms. In all 13 cases, the Episodic Vertigo was eliminated. Surgical treatment for sensorineural hearing loss with delayed-onset Vertigo, as with all surgery for Vertigo, should be based on the severity of the afflicted person's symptoms.
Carina Frykholm - One of the best experts on this subject based on the ideXlab platform.
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Stereocilin gene variants associated with Episodic Vertigo: expansion of the DFNB16 phenotype
European Journal of Human Genetics, 2018Co-Authors: Carina Frykholm, Joakim Klar, Tatjana Tomanovic, Adam Ameur, Niklas DahlAbstract:Vestibular disorders comprise a heterogeneous group of diseases with transient or permanent loss of vestibular function. Vestibulopathy is in most cases associated with migraine, Ménière disease, hereditary ataxias, or sensorineural hearing loss. We identified two brothers and their first cousin affected by hearing loss and Episodic Vertigo. The brothers were homozygous STRC nonsense variant [c.4027 C > T, p.(Q1343*)], whereas their first cousin was compound heterozygous for the STRC nonsense variant and a 97 kb deletion spanning the entire STRC gene. Clinical investigations confirmed pathological vestibular responses in addition to a characteristic DFNB16 hearing loss. The STRC gene encodes Stereocilin in the cochlea and in the vestibular organ where it ensheathes the kinocilium of the otolithic membranes. Stereocilin is associated with the gel overlaying the vestibular kinocilia, suggesting a role for the protein in sensing balance and spatial orientation. Our findings support such a function for Stereocilin in the vestibular organ and expand the phenotype associated with DFNB16.
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Stereocilin gene variants associated with Episodic Vertigo: expansion of the DFNB16 phenotype.
European journal of human genetics : EJHG, 2018Co-Authors: Carina Frykholm, Joakim Klar, Tatjana Tomanovic, Adam Ameur, Niklas DahlAbstract:Vestibular disorders comprise a heterogeneous group of diseases with transient or permanent loss of vestibular function. Vestibulopathy is in most cases associated with migraine, Meniere disease, hereditary ataxias, or sensorineural hearing loss. We identified two brothers and their first cousin affected by hearing loss and Episodic Vertigo. The brothers were homozygous STRC nonsense variant [c.4027 C > T, p.(Q1343*)], whereas their first cousin was compound heterozygous for the STRC nonsense variant and a 97 kb deletion spanning the entire STRC gene. Clinical investigations confirmed pathological vestibular responses in addition to a characteristic DFNB16 hearing loss. The STRC gene encodes Stereocilin in the cochlea and in the vestibular organ where it ensheathes the kinocilium of the otolithic membranes. Stereocilin is associated with the gel overlaying the vestibular kinocilia, suggesting a role for the protein in sensing balance and spatial orientation. Our findings support such a function for Stereocilin in the vestibular organ and expand the phenotype associated with DFNB16.