Central neurogenic vertigo


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  • Baillieres Clin Neurol 1994 Nov;3(3):453-65 Approach to the dizzy patient. Baloh RW UCLA Medical School, Reed Neurological Research Center 90024-1769. As dizziness can be caused by so many different pathophysiological mechanisms, it is crucial to determine the type of dizziness before proceeding with the diagnostic evaluation. Vertigo, defined as an illusion of movement, is an important subtype of dizziness that indicates a lesion somewhere within the vestibular system. Probably the most useful feature for differentiating between peripheral and central causes of vertigo is the associated symptoms. Vertigo of peripheral origin is typically associated with auditory symptoms such as hearing loss and tinnitus, while vertigo of central origin is nearly always associated with neurological symptoms such as diplopia, weakness, numbness and ataxia. Each of the common causes of vertigo has a characteristic clinical profile that should suggest a likely diagnosis after the history and examination are complete. Probably the most important treatment breakthrough is the positional manoeuvre that reliably cures benign positional vertigo (see Chapter 6). The treatment strategy for an acute peripheral vestibular lesion has evolved over the past few years. Patients are encouraged to return to normal physical activity as rapidly as possible. Repeated head, eye and body movements (vestibular rehabilitation) help the brain to recalibrate the relationship between visual, proprioceptive and vestibular signals (Chapter 9).
  • Ann Otol Rhinol Laryngol 2000 Apr;109(4):377-80 Migraine and benign positional vertigo. Ishiyama A, Jacobson KM, Baloh RW Division of Head and Neck Surgery, UCLA School of Medicine, Los Angeles, California, USA. Because inner ear symptoms are common in patients with migraine, we questioned whether benign positional vertigo (BPV) is more common in patients with migraine than in the general population. We reviewed the records of 247 patients seen in our neurotology clinic over the past 5 years with a confirmed diagnosis of BPV. Each patient had the typical history of BPV, and in each case the characteristic torsional vertical positioning nystagmus was identified. All were interviewed regarding migraine symptoms by means of standard International Headache Society criteria. Migraine was 3 times more common in patients with BPV of unknown cause than in those with BPV secondary to trauma or surgical procedures. Most patients were cured with the particle repositioning maneuver, regardless of the cause. Presumably, patients with migraine suffer recurrent damage to the inner ear (due to vasospasm or some other mechanism) that predisposes them to recurrent bouts of BPV. Int Tinnitus J 1999;5(1):50-52 The Migraine: Benign Paroxysmal Vertigo of Childhood Complex. Herraiz C, Calvin FJ, Tapia MC, de Lucas P, Arroyo R Unidad Funcional O.R.L.-Pabellon 8, Complejo Universitario San Carlos, Ciudad Universitaria, Madrid, Spain. [Record supplied by publisher] The migraine-a benign paroxysmal vertigo of childhood complex is the most frequent etiology of pediatric dizziness, with an incidence of 34.7%. We present a series of 34 children in whom this entity was diagnosed. We describe the most important characteristics and discuss the possible etiological factors. We review the theories about a common pathophysiological origin for migraine, benign paroxysmal vertigo of childhood, and paroxysmal torticollis. Evolution of these entities confirms the idea of a common origin and a different vestibular symptomatology, depending on the age of the child experiencing dizziness. J Neurol 1999 Oct;246(10):883-92 [Migraine: differential diagnosis in episodic vertigo]. [Article in German] Lempert T, Menzhausen L, Tiel-Wilck K Neurologische Klinik, Universitatsklinikums Rudolf Virchow, Berlin. Vestibular symptoms can be the predominant feature of migraine both in children and adults. Attacks of spontaneous or positional vertigo lasting from minutes to days may occur with or without concomitant headache. In the literature three syndromes of vestibular migraine have evolved: basilar artery migraine, benign recurrent vertigo and benign recurrent vertigo of childhood. In clinical practice, however, variants seem to be more frequent than the pure syndromes. Diagnosis is based on the individual constellation of typical precipitants and symptoms of migraine and the efficacy of pharmacological migraine prophylaxis. Nine cases are presented Am J Med Genet 1998 Sep 1;79(2):148-51 Familial migraine with vertigo: no mutations found in CACNA1A. Kim JS, Yue Q, Jen JC, Nelson SF, Baloh RW Department of Neurology, UCLA School of Medicine, Los Angeles, California 90095-1769, USA. We searched for mutations in the voltage-gated calcium channel gene, CACNA1A, in nine propositi of families with migraine headaches and episodic vertigo inherited in an autosomal dominant pattern. All 47 exons and flanking introns in CACNA1A were subjected to single-strand conformation polymorphism analysis of polymerase chain reaction-amplified genomic DNA. Exons with aberrantly migrating fragments were sequenced using standard techniques. We also determined the CAG repeat length at the 3' end of CACNA1A. Several polymorphisms were found but no mutations identified in any of the 47 exons of the 9 patients. No index-case had a CAG repeat length greater than 13 (normal <17). Mutations in CACNA1A are not common in families with migraine headaches and episodic vertigo. Other ion channel genes expressed in the brain and inner ear remain candidate genes. Laryngoscope 1998 Jan;108(1 Pt 2):1-28 Medical management of migraine-related dizziness and vertigo. Johnson GD Department of Otolaryngology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire 03756, USA. Historically, review of migraine-related vestibular symptoms has focused on the various clinical presentations that occur and the results of diagnostic studies of vestibular function. Treatment of vestibular symptoms related to migraine has been proposed similar to that used for headache control, but few examples of the effectiveness of this therapy have been published. The purpose of this study is to present the various approaches that can be used to manage vestibular symptoms related to migraine, and to evaluate the overall effectiveness of these treatment approaches. This was a retrospective review of 89 patients diagnosed with migraine-related dizziness and vertigo. The character of vestibular symptoms, pattern of cochlear symptoms, results of auditory and vestibular tests, and comorbidity factors are presented. Treatment was individualized according to symptoms and comorbidity factors, and analyzed regarding effectiveness in control of the major vestibular symptoms of episodic vertigo, positional vertigo, and nonvertiginous dizziness. Medical management included dietary changes, medication, physical therapy, lifestyle adaptations, and acupuncture. Complete or substantial control of vestibular symptoms was achieved in 68 (92%) of 74 patients complaining of episodic vertigo; in 56 (89%) of 63 patients with positional vertigo; and 56 (86%) of 65 patients with non-vertiginous dizziness. Similarly, aural fullness was completely resolved or substantially improved in 34 (85%) of 40 patients; ear pain in 10 (63%) of 16 patients; and phonophobia in 17 (89%) of 19 patients. No patient reported worsened symptoms following medical management. The conflicting concept of a central disorder (migraine) as the cause of cochlear and vestibular dysfunction that often has peripheral features is discussed. Ital J Neurol Sci 1997 Oct;18(5):283-8 Periodic syndrome and migraine in children and adolescents. Lanzi G, Zambrino CA, Balottin U, Tagliasacchi M, Vercelli P, Termine C Dipartimento di Neuropsichiatria Infantile, IRCCS, Istituto Neurologico C.Mondino, Universita di Pavia, Italy. Many reports in the literature seem to confirm the hypothesis that the symptoms of periodic syndrome are precursors or the equivalent of migraine: the aim of this study was to assess the prevalence of periodic syndrome in a group of children and adolescents suffering from migraine in comparison with that observed in various control groups. We considered seven symptoms: recurrent vomiting and abdominal pain, migrating limb pain, vertigo, recurrent hyperthermia with no visible cause, sleep disturbances and eating disorders. The study involved 171 children divided into four groups; 42 migraineurs; 37 subjects with chronic nervous pathologies but no psychosomatic symptoms; 46 subjects with a known psychosomatic disease, and 46 healthy subjects. The prevalence of the symptoms in the different control groups was different, although the pattern was more similar in the migraineurs and psychosomatic patients than in the other control groups. The development continuum of the syndrome may support the view that periodic syndrome is predictive of the subsequent development of a psychosomatic pathology. Cephalalgia 1997 Jun;17(4):505-10; discussion 487 Migraine-associated vertigo. Savundra PA, Carroll JD, Davies RA, Luxon LM Department of Neuro-otology, National Hospital for Neurology & Neurosurgery, London, UK. A retrospective analysis was performed on a consecutive series of 363 patients presenting with vertigo; 32% had migraine. Of the 224 patients with no pathology other than migraine or vestibular dysfunction, migraineurs had a significantly higher prevalence of normal, central, and combined central and peripheral vestibular dysfunction compared to non-migraineurs. The combination of central and peripheral vestibular signs was a feature of migraine with aura. The results support the hypothesis that migraine-associated vertigo is a diagnostic entity. Am J Otol 1997 May;18(3):350-4 Efficacy of antimigrainous therapy in the treatment of migraine-associated dizziness. Bikhazi P, Jackson C, Ruckenstein MJ Department of Otolaryngology, Head and Neck Surgery, University of California, San Francisco, USA. OBJECTIVE: To assess the effects of antimigrainous therapy on migraine-associated dizziness/vertigo. We hypothesized that a medication's ability to ameliorate dizziness/vertigo in this patient population would be directly correlated with its efficacy in improving headache symptoms. STUDY DESIGN: Patient survey. SETTING: Patients were entered into the study from the University of California, San Diego (UCSD) Headache Clinic, a tertiary care referral clinic. PATIENTS: All patients presenting to the UCSD headache clinic are entered into its comprehensive database. Patients who identified dizziness or vertigo as symptoms were entered into this study and were surveyed. MAIN OUTCOME MEASURES: Patients were surveyed as to the nature of their vestibular symptoms, and the therapeutic response of these symptoms and their headaches to various antimigrainous medications. Patients were asked to rank therapeutic efficacy utilizing a numeric scale. These results were then subjected to statistical analysis (Spearman rank correlation) to identify any correlation between the efficacies of the medications in improving headache and dizziness/vertigo. RESULTS: The efficacy of the medications in treating migraine-associated dizziness was directly correlated with their ability to alleviate headaches. CONCLUSION: We conclude that antimigrainous therapy may offer specific treatment to patients suffering from the spectrum of migraine-associated vestibular disorders. This would include the entity known alternately as vestibular Meniere's disease, benign recurrent vertigo, or recurrent vestibulopathy. Given the potential benefits that may be derived from this therapy, clinicians should be sensitive to a history of migraines in patients complaining of dizziness, particularly in those complaining of recurrent episodic vertigo. Eur J Emerg Med 1997 Mar;4(1):33-8 Migraine-related vestibulopathy. Cass SP, Furman JM, Ankerstjerne K, Balaban C, Yetiser S, Aydogan B Department of Otolaryngology, University of Pittsburgh School of Medicine, Pennsylvania 15213, USA. Migraine has been associated with specific vestibular disorders, including benign paroxysmal vertigo of childhood and benign recurrent vertigo in adults. Migraine may also play a role in chronic nonspecific vestibulopathy. Because scant data exist that describe the clinical findings and vestibular function abnormalities in suspected migraine-related vestibulopathy, we reviewed the history, physical examination, vestibular tests (electronystagmography, rotational chair, posturography), and response to treatment of 100 patients with diagnoses of migraine-related vestibulopathy. Dominant clinical features included chronic movement-associated dysequilibrium, unsteadiness, space and motion discomfort, and occasionally, episodic vertigo as an aura prior to headache, or true vertigo without headache. Common vestibular test abnormalities included a directional preponderance on rotational testing, unilateral reduced caloric responsiveness, and vestibular system dysfunction patterns on posturography. Treatment was usually directed at the underlying migraine condition by identifying and avoiding dietary triggers and prescribing prophylactic anti-migraine medications. Symptomatic relief was also provided using anti-motion sickness medications, vestibular rehabilitation, and pharmacotherapy directed at any associated anxiety or panic disorder. Cephalalgia 1995 Feb;15(1):22-5; discussion 4 [Vertigo and migraine]. [Article in German] Muri RM, Meienberg O Neurologische Universitatsklinik, Inselspital Bern. Vertigo consists of a variety of syndromes and can be due to many etiologies. One of these causes is migraine, which in our experience is often overlooked, although migrainous vertigo is well known in the literature. Vertigo in migraine can occur as aura or during the headache phase, or independent of the attacks as aura without headache. The aim of this retrospective study was to analyze cases with vertigo and migraine: 23 (8%) of 298 patients with migraine examined in a neurological outpatient department also had rotational vertigo. 48% of these patients had vertigo independent from typical migraine headache. Two types of vertigo were found: permanent vertigo, and vertigo with the characteristics of paroxysmal positional vertigo. 57% of the vertiginous attacks lasted hours, 26% even days, and 17% minutes. Most of the patients had several attacks of vertigo, some involving up to 30 episodes. To recognize migraine as a cause of vertigo has therapeutic implications. Most of our patients with vertigo and migraine showed a good response to antimigraine therapy. Headache 1993 Mar;33(3):125-8 Migraine: an alternative in the diagnosis of unclassified vertigo. Aragones JM, Fortes-Rego J, Fuste J, Cardozo A Service of Neurology, Hospital Clinic i Provincial de Barcelona, Spain. Vertigo and unsteadiness are frequent reasons for medical consultation. In some cases, these symptoms remain unclassified. The association of equilibrium disorders with migraine is often mentioned in literature. Seventy-two cases of unclassified vertigo were studied in order to ascertain the prevalence of migraine in patients affected by recurring episodes of vertigo from undetermined causes, and attempting to establish a possible relationship with migraine. Characteristics of the vertigo and the headache, were evaluated by clinical history and examination, electronystagmogram (ENG), electroencephalogram (EEG), computerized tomography scanning (CT) and/or magnetic resonance imaging (MRI). In the group of patients studied 50% suffered from headache, and 32.8% fulfilled the diagnostic criteria of migraine. Results suggest that only a thorough clinical history would be able to give enough information to establish the diagnosis of migraine in these patients. These observations imply an alternative diagnosis to be taken into account when a case of unclassified vertigo is evaluated. Nervenarzt 1993 Feb;64(2):121-6 [Migraine: differential diagnosis in episodic vertigo]. [Article in German] Lempert T, Menzhausen L, Tiel-Wilck K Neurologische Klinik, Universitatsklinikums Rudolf Virchow, Berlin. Vestibular symptoms can be the predominant feature of migraine both in children and adults. Attacks of spontaneous or positional vertigo lasting from minutes to days may occur with or without concomitant headache. In the literature three syndromes of vestibular migraine have evolved: basilar artery migraine, benign recurrent vertigo and benign recurrent vertigo of childhood. In clinical practice, however, variants seem to be more frequent than the pure syndromes. Diagnosis is based on the individual constellation of typical precipitants and symptoms of migraine and the efficacy of pharmacological migraine prophylaxis. Nine cases are presented. Headache 1992 Jun;32(6):300-4 Migraine-associated dizziness. Cutrer FM, Baloh RW Department of Neurology, Reed Neurological Research Center, UCLA School of Medicine 90024-1769. We reviewed the clinical histories, examinations and results of quantitative vestibular testing in 91 patients with migraine-associated dizziness. Nausea and vomiting, hypersensitivity to motion and postural instability accompanied the dizziness. In the majority of patients, the temporal profile of the dizziness was more typical of the headache phase of migraine than of the aura phase. Nineteen patients (20.9%) had unilateral hypoexcitability to caloric stimulation, which represents a modestly increased risk of damage to the peripheral vestibular apparatus. We propose two separate pathophysiologic mechanisms for the production of dizziness with migraine: Short-duration vertiginous attacks lasting minutes to 2 hours and temporally associated with headache are due to the same mechanism as other aura phenomena (spreading wave of depression and/or transient vasospasm). Longer-duration attacks of vertigo and motion sickness lasting days, with or without headache, result from the release of neuroactive peptides into peripheral and central vestibular structures, causing an increased baseline firing of primary afferent neurons and increased sensitivity to motion. Am J Otol 1991 Jan;12(1):25-34 Migraine and the vestibular system in adults. Parker W Cleveland Clinic Foundation, Ohio 44195. The purpose of this paper is to explore the relationships between migraine and the vestibular system in adults. A review of the literature on migraine reveals that paroxysmal vertigo is not uncommonly associated with migraine in various temporal relationships with headache and/or other neurologic symptoms. Vertigo (or nonvertiginous dizziness) may be the chief presenting complaint sometimes masking less dramatic symptoms causing patients to be directed to an otolaryngologist. Meticulous family, past personal, and present history are necessary to produce the information leading to suspicion of the association of paroxysmal vertigo with the migraine diathesis. A careful prospective study of a large neurotologic population seems indicated to further define this relationship leading to diagnostic and therapeutic benefits. Sixteen cases are presented, six of them in detail. Laryngoscope 1988 Feb;98(2):160-4 Migraine equivalent as a cause of episodic vertigo. Harker LA, Rassekh C Department of Otolaryngology-Head and Neck Surgery, University of Iowa, Iowa City 52242. There are many transient neurologic disturbances associated with various types of migraine. Visual symptoms, such as scintillating scotomata are most common, but somatosensory, motor, cranial nerve, and brain stem symptoms also occur. Among the brain stem symptoms, vestibular manifestations are quite common and include nonspecific dizziness, disequilibrium, vertigo, and motion intolerance. Auditory symptoms are less common. These transient neurologic symptoms can precede the headache as an aura, can occur during the headache, or, uncommonly, can immediately follow the headache. It is also well documented that the neurologic symptoms can occur in the period between headaches, a situation termed "migraine equivalent." Migraine equivalents usually occur in patients who have experienced typical migraine headaches earlier in life or who have migraine headaches at times other than when they experience equivalent symptoms. Rarely, typical migraine equivalent symptoms precede the development of the headaches by months or years, or occur in individuals who never develop headaches. Five patients with migraine equivalent symptoms that include vertigo are presented. The vertigo was the dominant symptom in some cases and was accompanied by nausea and vomiting. Differentiation from peripheral labyrinthine disorders is difficult, but a personal or family history of migraine, the temporal association of the neuro-otologic symptoms with other migraine equivalent symptoms, a characteristic pattern of occurrence of the symptoms, and a positive response to antimigrainous therapy are features that strengthen the diagnosis of a migraine equivalent phenomenon. Otolaryngol Head Neck Surg 1987 Mar;96(3):239-50 The periodic syndrome in pediatric migraine sufferers. Lanzi G, Balottin U, Fazzi E, Rosano FB The present study takes into consideration some of the symptoms comprised in the Periodic Syndrome. These include motion-sickness, cyclic vomiting, recurrent abdominal pains and paroxysmal vertigo. Particular consideration is given to the chronological and long-term aspects of such symptoms. Among 247 migraine sufferers in the pediatric age group, 173 subjects who complained of at least one of these symptoms were chosen. Results of the data show that motion-sickness is the first to manifest itself (mean age of onset 2 years), and has a tendency to continue into the headache period; cyclic vomiting appears in the third year of life, and terminates sooner than the other symptoms. At the mean age of five years abdominal pains start, and at seven, paroxysmal vertigo. Headache is the final symptom in this group. The sequentiality of such disturbances in each subject leads to the assumption that the Periodic Syndrome is the expression of a single disorder which manifests itself polymorphously as a rather precisely timed process. Pediatr Med Chir 1982 Nov-Dec;4(6):593-600 [Childhood periodic syndromes and their long-term development]. [Article in Italian] Cavazzuti GB, Ferrari P The long term development of periodic syndromes among children is little known. Our research has revealed that about one third of periodic headaches, two thirds of cyclic vomiting and half the cases of recurring abdominal pain disappear either before puberty or during adolescence. Other Authors have shown that this also happens in most cases of early-onset vertigo. The remaining headache cases develop into migraines in adults. When there is persistent cyclic vomiting, the collateral neurologic phenomena (headaches, vertigo, pallor, hypotonia, drowsiness) become more intense. This also happens in some cases of abdominal pain and paroxysmal vertigo which start in late childhood. Other sufferers from acute abdominal pain develop ulcers, gastroduodenitis and colitis as adults. Altogether, some infantile periodic syndromes (in particular the multi-symptomatic ones) have a common outcome, i.e. develop into more or less typical migraine syndromes. In these cases one can presume a common pathogenetic mechanism. In those cases where the outcome is favorable the pathogenesis may be different. These cases may often be spotted in early childhood on account of the monosymptomatic nature of the complaint or the absence of collateral neurologic symptoms as well as of the infrequency of critical episodes. Acta Otolaryngol 1982 Mar-Apr;93(3-4):283-9 Common migraine and vestibular function. Electronystagmographic study and pathogenesis. Toglia JU, Thomas D, Kuritzky A Even though "classic migraine" and "complicated migraine" may be diagnosed readily, "common migraine" may be easily confused with other types of vascular headaches. This differential diagnosis is of great importance for the appropriate choice of drug therapy. It is frequently stated that family history of migraine and history of motion sickness in childhood suggest that a periodic vascular headache is most likely of migrainous origin; although this statement applies to ophthalmoplegic and hemiplegic migraine, it is doubtful that it applies to common migraine. In fact, in a pilot study of patients with common migraine, we have observed that family history and history of motion sickness in childhood did not contribute to the diagnosis. Vestibular dysfunctions are frequently associated with migraine including the common type. Utilizing labyrinthine tests with the aid of electronystagmography, abnormalities of labyrinth function were demonstrated in 80% of patients with common migraine who had no history of vertigo or of other otological and neurological disorders. Ann Neurol 1981 Feb;9(2):126-30
  • [Basilar migraine]. [Article in French] Monday LA Basilar migraine has been studied by Bickerstaff who considers that there is a vasoconstriction in the basilar territory resulting in transient ischemia with the corresponding neurological symptoms including vertigo, and followed by the vasodilatation causing the headache. Three cases, treated in neurology, have had an audiovestibular investigation with an E.N.G. and an audiogram and are described herein. Nystagmus and hearing loss have been observed in one of these cases and it is suggested that the internal auditory artery participates in the basilar migrainous processes. The diagnosis of basilar migraine is impossible to prove and the investigation is very limited. Finally it is the evolution of the patient which helps in establishing the diagnosis of basilar migraine. Am J Dis Child 1976 Oct;130(10):1099-1100 Basilar migraine. Kuhn WF, Kuhn SC, Daylida L Department of Emergency Medicine, Medical College of Georgia, Augusta 30912, USA. Basilar migraine is a complicated headache which the International Headache Society describes as 'migraine with aura symptoms clearly originating from the brainstem or from both occipital lobes'. For years this headache was thought to originate from a transient disturbance in the vertebrobasilar circulation, but more recent studies suggest that a central neuronal disorder may be the source of migraine. Basilar migraines may have certain symptoms which are similar to other neurologic, vascular, psychiatric and metabolic diseases, yet there are specific criteria which can help differentiate it from other diagnoses. It is characterized by a throbbing occipital headache which may be preceded by an aura. The unusual symptoms of basilar migraine, which may precede and continue throughout the duration of the headache and even after it, include bilateral visual symptoms, altered mental status, vertigo, gait ataxia, bilateral paresthesia, bilateral paralysis and dysarthria. We describe a 29-year-old black female whose husband brought her to the emergency department complaining of confusion, headache, and left-sided weakness for 2 h prior to arrival. Ann Otol Rhinol Laryngol 1997 Mar;106(3):182-9 Episodic vertigo in basilar artery migraine. Harker LA, Rassekh CH Vestibular symptoms commonly occur in migraine, and episodic vertigo is most frequently seen. Auditory symptoms also occur, but are less common. When Bickerstaff described basilar artery migraine in 1961, he postulated that the many different symptoms were caused by basilar artery ischemia. He documented that neuro-otologic and other symptoms could occur before or during a migraine headache; others later established that these symptoms could also occur during the headache-free period. Case histories of eleven patients with basilar artery migraine are presented in detail. All met the diagnostic criteria for migraine and experienced vertigo before or during episodic headaches--sometimes with other symptoms of transient brainstem dysfunction. Cases represented both typical and unusual manifestations of migraine with vestibular symptoms: four patients were adolescents, three were more than 45 years old and had previously diagnosed migraine headaches, and four were young adults not previously known to have migraine. Many of the patients were thought to have disorders of the vestibular end organ (sometimes in addition to migraine) and three had undergone previous endolymphatic sac decompressions or perilymph fistula repairs. Diagnostic criteria are reviewed, in order that patients with basilar artery migraine can be distinguished from those with peripheral labyrinthine disease, to allow initiation of appropriate antimigraine therapy and avoidance of unnecessary medical and surgical therapy for end-organ disorders. ORL J Otorhinolaryngol Relat Spec 1984;46(2):97-104 Basilar artery migraine in young children. Golden GS, French JH Eight children with recurrent attacks of neurologic dysfunction referable to the brainstem and cerebellum are reported. The episodes occur suddenly, clear completely, and leave the patient without residua. The most frequent signs are ataxia, alternating hemipareses, and vertigo. The majority of patients are girls, and most have the onset of the condition prior to the age of 4 years. Headache was definitely present in three children, and possibly present in four. A striking history of migraine was found in seven families, accounting for 16 affected relatives. Fifteen of these were female and 14 were on the maternal side. Follow-up of the children has not provided any evidence for progressive neurologic disease. The patient followed for the longest period of time has developed classic migraine. Vestibular testing in basilar artery migraine. Eviatar L Vestibular function studies were performed in 20 children with symptoms suggesting basilar artery migraine. Sixteen children had abnormalities on bithermal caloric testing. Labyrinthine preponderance was found in 1 child with labyrinthine concussion and in 6 children with symptoms suggesting ischemia of the territory supplied by the basilar artery and the occipital branches of the posterior cerebral arteries. Directional preponderance was found in 4 children with symptoms suggesting ischemia of the posterior temporal branches of the posterior cerebral arteries. Five children had both labyrinthine and directional preponderance. normal electronystagmographic findings were present in 4 children and correlated clinically with a milder illness. Head trauma was the triggering factor for the migraine in 5 children. Five children developed complex partial seizures and had evidence of directional preponderance (2 children) and combined labyrinthine and directional preponderance (2 children). The presence of abnormalities on electronystagmography in children with basilar artery migraine correlates with the severity of the disease. Directional preponderance or labyrinthine and directional preponderance are more frequently correlated with ischemia in the posterior temporal branches of the posterior cerebral arteries and may suggest a higher risk of developing vertiginous seizures when ischemia is prolonged. Eur J Pediatr 1980 Aug;134(2):149-51
  • Benign paroxysmal vertigo in childhood. Eeg-Olofsson O, Odkvist L, Lindskog U, Andersson B Fifteen children with a syndrome called benign paroxysmal vertigo are presented. This syndrome is characterized by attacks of vertigo of sudden onset lasting a few minutes and in extreme cases several hours or even up to 2 days. Additional symptoms are pallor, sweating, vomiting and nystagmus. Consciousness is not impaired. The attacks usually occur in the first 4 years of life. They are recurrent, the appearance varying from several times a week to once a year, and they may cease spontaneously after only months but usually years. The most important differential diagnoses are epilepsy and posterior fossa tumour. The etiology is unknown, but there may be a vascular disturbance affecting the posterior cerebral circulation with secondary disturbances of the vestibular nuclei. Ann Otol Rhinol Laryngol 1981 May-Jun;90(3 Pt 1):267-71 Paroxysmal vertigo as a migraine equivalent in children: a population-based study. Abu-Arafeh I, Russell G Department of Child Health, University of Aberdeen, UK. We studied the prevalence, causes and clinical features of paroxysmal vertigo (PV) in the well-defined childhood population of the City of Aberdeen. We applied a screening questionnaire to 2165 children (10% random sample of all children 5-15 years of age) attending schools in Aberdeen. Children with a history of at least three episodes of vertigo over the past year due to unknown causes were invited for clinical interview and examination. Children with PV were compared with a group of children with migraine, and with a group of asymptomatic children matched for age and sex. Forty-five children fulfilled the diagnostic criteria for PV (prevalence rate 2.6%, 95% CI 1.9-3.4). They were noted to have clinical features in common with children with migraine, including trigger and relieving factors, associated gastrointestinal and sensory symptoms, vasomotor changes, and a similar pattern of associated recurrent disorders (such as headache, abdominal pain and cyclical vomiting), atopic diseases and travel sickness. Also, they had a twofold increase in the prevalence of migraine (24%) compared with the general childhood population (10.6%). The overlap in the clinical features of PV and migraine suggests that the two conditions are related and that it is reasonable to continue to regard PV as a migraine equivalent. Cephalalgia 1994 Dec;14(6):458-60 Benign paroxysmal vertigo of childhood: a long-term follow-up. Lanzi G, Balottin U, Fazzi E, Tagliasacchi M, Manfrin M, Mira E Department of Child Neuropsychiatry, C. Mondino Foundation, IRCCS, University of Pavia, Italy. We examined clinical aspects of Benign Paroxysmal Vertigo (BPV) in infancy and its most frequent differential diagnosis, in particular analogies and differences with forms of "migrainous vertigo" (MV) of later onset. During a long-term follow-up of 7 cases of BPV, diagnosed according to the Basser criteria, 5 of 7 BPV cases spontaneously resolved and 6 of 7 patients later developed migraine and other migraine-related symptoms. This course differs from that described for MV only in the age of onset of headache and in the chronological relationship with vertigo. The authors suggest that BPV can be interpreted as a migraine precursor and MV as a migraine equivalent. Schweiz Med Wochenschr 1993 Jul 3;123(26):1331-6 Benign paroxysmal vertigo in childhood: a migraine equivalent. Mira E, Piacentino G, Lanzi G, Balottin U, Fazzi E The two main problems in defining and classifying the syndrome of benign paroxysmal vertigo (BPV) in childhood are the vestibular function pattern and the relationship between BPV and migraine. 13 children suffering from this syndrome were submitted to complete otoneurological examination, including caloric and rotational labyrinthine stimulation with ENG recording, and to headache provocation tests with nitroglycerin, histamine and fenfluramine. Vestibular responses were normal in all except 2 cases which presented signs of central vestibular impairment at the level of the vestibulocerebellar pathways. Headache provocation tests were positive in 9 out of 10 children, and in 4 cases they induced a typical vertiginous attack instead of headache. In addition, several children had a positive family history for migraine, headache was frequently associated with the crisis and other signs of a 'periodic syndrome' (motion sickness, cyclic vomiting, abdominal pain) were present, unrelated to vertiginous attacks. During the follow-up period, some children responded positively to migraine treatment. BPV, like paroxysmal torticollis in infancy and the signs of the periodic syndrome, can be considered a migraine equivalent or a migraine precursor and could be due to the same vascular and/or biochemical mechanisms responsible for the migraine. In children, for anatomical or developmental reasons, these mechanisms could selectively affect parts of the brain stem, including the vestibular nuclei and vestibulocerebellar pathways. Cephalalgia 1983 Aug;3 Suppl 1:91-3 Benign paroxysmal vertigo of childhood: a migraine equivalent. Koehler B Eight patients with the syndrome of benign paroxysmal vertigo of childhood are presented. This symptom complex is characterized by attacks of vertigo in young children combined with nystagmus, ataxia and transiently decreased vestibular function, but without impaired consciousness. Diagnostic and follow-up studies revealed a close relationship to other forms of autonomic nervous system instability, particularly to the migraine symptom complex. J Otolaryngol 1977 Aug;6(4):320-6 Benign paroxysmal vertigo of childhood. Dunn DW, Snyder CH Thirty-three cases of benign paroxysmal vertigo in childhood have been seen at our institution since the disorder was recognized ten years ago. Progression from paroxysmal torticollis of infancy to paroxysmal vertigo of childhood is documented. Ear infections and allergy appeared causative in a few, but not most, of the cases. The most important consideration for the pediatrician is to rule out epilepsy and brain tumor. Parents should be reassured that the condition is benign, and that the attacks will cease in a few months or years. Pediatrics 1975 Nov;56(5):722-6
  • Episodic vertigo related to migraine (90 cases): vestibular migraine? Dieterich M, Brandt T Department of Neurology, Klinikum Grosshadern, Ludwig Maximilians University of Munich, Marchioninistrasse 15, D-81377 Munich, Germany. mdieterich@brain.nefo.med.uni-muenchen.de 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." Nervenarzt 1993 Feb;64(2):121-6
  • Schweiz Rundsch Med Prax 1997 Oct 1;86(40):1558-62 [Cortical vertigo]. [Article in German] Thier P, Haarmeier T Neurologische Universitatsklinik Tubingen, Sektion fur Visuelle Sensomotorik. Dizziness of cortical origin is the subjective correlate of a disturbance of spatial orientation resulting from cerebrocortical dysfunction. Cortical dizziness in the form of vertigo is rare. If present, it most probably reflects a dysfunction of a vestibular representation in the insula. It may be accompanied by tinnitus, sensory disturbance and possibly also spontaneous nystagmus. The dysfunction of this region may result either from a focal seizure or from a lesion, for instance due to ischemia. Nondirectional, visual dizziness is most probably much more common than vertigo. This latter type of dizziness results from a functional disturbance of those parts of parietooccipital cortex, contributing to the discrimination of self-induced and externally-induced retinal image slip. It is not accompanied by additional symptoms and should immediately cease upon closure of the eyes or avoidance of ego motion.
  • Cerebellopontine Angle Tumors {Back to Outline} Tumors of the cerebellopontine angle rarely present solely with episodic vertigo. The most common tumor in this location results from a proliferation of the Schwann cells, hence the name schwannoma. Most of these tumors arise on the vestibular portion of the VIII nerve within the internal auditory canal. They progressively enlarge, deforming the internal auditory meatus and compressing adjacent neural structures such as the acoustic portion of the eighth nerve, facial nerve, trigeminal nerve, brainstem and cerebellum. Other tumors occurring in the cerebellopontine angle include meningiomas, epidermoids and metastases. The most common symptoms associated with eighth nerve tumors are progressive hearing loss and tinnitus. Vertigo occurs in approximately 20%, but a symptom of imbalance or disequilibration is more common. Rarely a patient with a vestibular nerve tumor may present with subtle hearing loss, tinnitus and episodic vertigo. All those with progressive unilateral hearing loss, and particularly those with any vestibular symptoms should be carefully examined for additional neurological signs such as a depressed corneal reflex. Otolaryngol Head Neck Surg 1998 Apr;118(4):429-36 Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Matthies C, Samii M Department of Neurosurgery, Nordstadt Hospital, Hannover, Germany. OBJECTIVE: Despite good knowledge of the key symptoms of vestibular schwannomas and their significance for surgical results, the evolution of symptoms and signs and their relation to tumor extension still need thorough investigation. METHODS: From 1978 to 1993, operations were performed by the same surgeon (M.S.) on 1000 vestibular schwannomas at the Neurosurgical Department of Nordstadt Hospital. The vestibular schwannomas were diagnosed in 962 patients, including 522 female patients (54%) and 440 male patients (46%); the mean age was significantly higher in female patients (47.6 yr) than in men (45.2 yr). We focused our analysis on the incidence of subjective disturbances versus objective morbidity, on the sequence of symptom onset, and on symptom duration and symptomatology versus tumor size and extension. RESULTS: The most frequent clinical symptoms were disturbances of the acoustic (95%), vestibular (61%), trigeminal (9%), and facial (6%) nerves. Symptom duration was 3.7 years for hearing loss, 1.9 years for facial paresis, and 1.3 years for trigeminal disturbances. Symptom incidence and duration did not strictly correlate with tumor size. Key symptoms of various tumor extension classes precipitated the diagnosis, such as trigeminal disturbances in large tumors with brain stem compression or tinnitus in small neuromas. In cases of trigeminal or facial nerve symptoms, the overall duration of symptomatology was much shorter. According to the subjective perception of the patients, between only one- and two-thirds of nerve disturbances were noticed. Patients with preoperative deafness had become deaf either chronically (23%) or suddenly (3%); even in cases of moderate hearing deficit that lasts a long time, deafness can occur suddenly. The rate of tinnitus was higher in hearing than in deaf patients; however, deafness does not mean relief from tinnitus, because this symptom persists in 46% of preoperatively deaf patients. Vestibular disturbances most often occur as some unsteadiness while walking or as vertigo, and the symptoms frequently are fluctuating, not constant. CONCLUSION: Differences in tumor biology can be underestimated and are not visible on radiological scans. For example, intrameatal tumors, despite their small size, present with a duration of symptoms that is representative of the larger tumors and are most frequently associated with vestibular symptoms and with tinnitus. Large tumors with brain stem compression present with relatively shorter symptom durations and at a younger age; both factors are suggestive of especially fast tumor growth. The clinical findings presented in this study promote new consideration of the dynamics of tumor growth and of the affected neural tissues. Posterior Fossa Lesions {Back to Outline} Posterior fossa lesions in a variety of locations are unusual causes of isolated vertigo. The symptoms are usually positional vertigo of the central type (see Table 3). Magnetic resonance imaging (MRI) with coronal and sagittal reconstructions permits identification of small tumors close to the tissue-bone interface, a region often blurred by bone artifact in CT scans. Acquired disease of the brainstem and cerebellum produces a variety of types of nystagmus, which sometimes present as a complaint of oscillopsia, an illusion of environmental movement characterized by bouncing or jiggling of objects. Although oscillopsia is a common complaint with bilaterally reduced vestibular function as from ototoxicity, the presence of vertical oscillopsia should alert the physician to look for primary position upbeat or downbeat nystagmus. These nystagmus types are reliable indicators of CNS abnormality due to structural intrinsic midline cerebellar disease or drugs. An unusual cause of acute labyrinthine failure. Mehta JS, Sharr MM Department of Neurosurgery, King's College Hospital, London, UK. We report a case of a 40-year-old man presenting with acute vertigo and deafness. Computed tomography (CT) scanning at initial presentation was normal. However, one year later he developed numbness on the right side of his face and examination revealed fifth, seventh and eighth cranial nerve palsies as well as cerebellar dysfunction. Magnetic resonance imaging (MRI) demonstrated a cerebellopontine angle lesion. He underwent near total excision followed by neuro-axis irradiation. The main mass of the tumour projected into the cerebellopontine angle. Histology showed this to be a medulloblastoma. All features of this case are unusual; hence we discuss the pathogenesis and management of this very rare tumour. Otolaryngol Head Neck Surg 1998 Apr;118(4):429-36 Unusual presentations of acoustic tumours. Morrison GA, Sterkers JM Department of Otolaryngology, St Thomas' Hospital, London, UK. A series of 238 consecutive patients with acoustic neuromas, operated on in Paris has been studied to identify unusual presentations and varied symptomatology. The most common history was that of a progressive unilateral hearing loss (in 68.1%), with tinnitus (in 49.1%) or disequilibrium (in 49.1%) or both. Sudden hearing loss (in 14.7%) or fluctuating hearing loss (in 6.3%), and a single or repeated episodes of acute vertigo (in 8.8%) were seen less commonly. Headaches occurred as an associated symptom in 10.5%, tinnitus was the sole symptom in 2.8% and other uncommon symptoms included otalgia, facial nerve palsy, facial or ocular pain, altered sensation in the face or eye, or tingling of the tongue. Some 11.3% of patients presented with normal pure tone auditory thresholds and a 100% speech discrimination score and of these patients acoustic reflex thresholds were normal in 53% and brainstem auditory evoked responses were suggestive of the retro-cochlear abnormality in only 76.2%. Amongst the less common presentations, the initial symptoms mimicked such diagnoses as Meniere's disease, benign positional vertigo, vertebro-basilar migraine, vertebro-basilar insufficiency, Bell's palsy and Trigeminal neuralgia. Overall, 20.6% of patients had unusual initial presenting symptoms, 36.5% of the symptoms were unusual and these were found in isolation in 11.8% of patients. An awareness of the spectrum of more subtle symptoms of acoustic tumours may lead to the correct diagnosis at an earlier stage. Chung Hua Erh Pi Yen Hou Ko Tsa Chih 1995;30(6):335-7 [Vertigo in non-vascular diseases of the central nervous system]. [Article in French] Clanet M, Bonafe A, Esteve-Fraysse MJ, Fraysse B Service de neurologie, CHU Purpan, Toulouse. Excluding vascular involvement, vertigo due to a central vestibular syndrome reflects a median or paramedian lesion of the brain stem or the cerebellum. Recurrent attacks of vertigo usually occur with peripheral lesions. Persistent acute vertigo with peripheral destruction can reveal ischemia of the brain stem. Central positional vertigo is rare and has symptomatology that is different from that of benign positional vertigo. Persistent instability has a symptomatology that is more difficult to analyse and is usually associated with a central vestibular syndrome when it is organic. Diagnosis of a central vestibular syndrome is based on detection of well-defined clinical or electronystagmographic signs of which abnormal nystagmus is primordial. Some of them such as inferior vertical nystagmus or dissociated nystagmus can localise the site. MRI has become the diagnostic procedure which is best adapted for identifying the most frequent aetiologies such as tumors, congenital malformations and multiple sclerosis. Acta Otolaryngol Suppl 1994;513:15-27 CT and MR imaging of intralabyrinthine schwannoma: report of two cases and review of the literature. Mafee MF, Lachenauer CS, Kumar A, Arnold PM, Buckingham RA, Valvassori GE Department of Radiology, University of Illinois, Chicago 60680. Two cases of intralabyrinthine schwannoma were studied with computed tomography (CT) and magnetic resonance (MR) imaging. On CT scans, a soft-tissue mass was identified in the round window niche in both cases. Widening of the basilar turn of the cochlea and characteristic erosion of the promontory were noticed in one case. Preoperative MR imaging (performed in only one case) revealed a soft-tissue mass in the labyrnth, extending into the round window niche. Findings at CT and MR imaging are discussed, and a differential diagnosis is given. The literature is reviewed. The authors' findings suggest that CT and MR imaging may prove very valuable in the previously difficult preoperative diagnosis of these tumors. In the setting of progressive sensorineural hearing loss, atypical Meniere disease, or recurrent vertigo, the presence of a mass in the labyrinth or labyrinthine windows- delineated on CT or MR images, despite a normal internal auditory canal, cerebellopontine angle, or brain stem - is highly suggestive of intralabyrinthine schwannoma. Laryngoscope 1979 Mar;89(3):410-20 Intralabyrinthine schwannomas. Fitzgerald DC, Grundfast KM, Hecht DA, Mark AS Department of Otology/Neurotology, Washington Hospital Center, DC 20010, USA. OBJECTIVE: To describe characteristic features of intralabyrinthine schwannomas (ISs) that may be used to distinguish them from other otologic disorders with similar symptoms so that appropriate evaluation and management can be instituted. STUDY DESIGN: This study was a retrospective case review. SETTING: This study was conducted at a university-affiliated urban tertiary care medical center and a university medical center in the same city. PATIENTS: Seven patients with ISs were included in this study. INTERVENTIONS: Tumor removal versus observation and monitoring with periodic magnetic resonance imaging (MRI) scans was investigated. MAIN OUTCOME MEASURES: Hearing, vertigo, and tumor growth were measured. RESULTS: Four of seven patients with ISs underwent surgical excision with no evidence of tumor recurrence. The remaining three patients are being followed-up with repeat MRI that has demonstrated minimal or no tumor growth. CONCLUSIONS: ISs can be detected in early stages if MRI is performed in patients with unilateral sensorineural hearing loss without vertiginous symptoms typical of Meniere's disease. Although complete surgical excision can be achieved readily with labyrinthectomy, observation and monitoring with rep--MRI is an option for some patients. J Neurol 1998 Jun-Jul;245(6-7):314-21 [Early diagnosis of acoustic neuroma]. [Article in Chinese] Han D, Yang W, Jiang S PLA General Hospital, Beijing. The diagnostic findings in 31 patients (32 ears) with acoustic neuromas are reported. In patients suspicious of acoustic neuroma, case history and clinical examination are important. In our material of 31 patients with acoustic neuromas, hearing loss was presented in 94% (slow onset 87%, sudden onset 10% and fluctuating 3%), tinnitus in 91%, vestibular symptoms (dizziness or vertigo) in 62%. It should be stressed that in patients with unilateral hearing loss (or tinnitus) or vestibular symptoms, it is necessary to exclude the presence of an acoustic neuroma. In addition, the trigeminal never was affected in 34%, which was encountered in tumours larger than 26-40 mm. There was a positive correlation between duration of symptoms and size of the tumour, but there was no correlation between hearing loss and size of the tumour. Brainstem response audiometry (ABR) exhibited a very high sensitivity (100%), making it a suitable single test for screening patients suspected to have an acoustic neuroma. Laryngorhinootologie 1994 Jun;73(6):320-3 [Clinical presentation and diagnosis of small acoustic neurinomas]. [Article in German] Hohmann D, Dornhoffer JL Klinik und Poliklinik fur Hals-Nasen-Ohrenkranke, Universitat Wurzburg. With the invention of magnetic resonance imaging (MRI) and the auditory evoked brain stem responses (AEBR), it has become possible to diagnose acoustic tumours while they are still small. As a result, it has become obvious that the clinical presentation of smaller lesions can be some-what different from what is considered typical of an acoustic neuroma. Likewise, whereas the sensitivity of auditory brain stem responses for larger tumours is good, the sensitivity for smaller tumours has recently been in doubt, particularly if the patient presents early in the course of the disease with only mild otologic complaints. A retrospective study of patients treated for small acoustic neuromas, defined as less than 1 cm extension into the cerebellopontine angle, was conducted to assess ABR results as well as the clinical and audiological presentation in these patients. Of the 70 patients included in the study, auditory brain stem responses were abnormal in 65 (93%), based on wave V latency prolongation and intra-aural latency differences. These would indicate that auditory brain stem responses are a valid screening test for acoustic neuromas, even in the early stages of development. The clinical presentation of patients with small acoustic tumours was similar to the presentation reported for acoustic neuromas in general, but with vertigo occurring more often in patients with smaller tumours. Several atypical patterns of hearing loss were seen in this population. Rev Prat 1994 Feb 1;44(3):328-35 INTRALABYRINTHINE SCHWANNOMAS Dennis C. Fitzgerald, MD, Kenneth M. Grundfast, MD, David A. Hecht, MD, and Alexander S. Mark, MD Washington Hospital Center, Washington, D.C., U.S.A. Objective: To describe characteristic features of intralabyrinthine schwannomas that may be used to distinguish them from other otologic disorders with similar symptoms so that appropriate evaluation and management can be instituted. Study Design: Retrospective case review. Setting: A university affiliated urban tertiary care medical center and a university medical center in the same city. Patients: Seven patients with intralabyrinthine schwannoma. Interventions: Tumor removal vs. observation and monitoring with periodic magnetic resonance imaging seam. Main outcome measures: Hearing, vertigo and tumor growth. Results: Four of seven patients with intralabyrinthine schwannomas were managed by surgical excision with no evidence of tumor recurrence. The remaining three patients are being followed with repeat magnetic resonance imaging scans which have demonstrated minimal or no tumor growth. Conclusions: Intralabyrinthine schwannomas can be detected in early stages if magnetic resonance imaging (MRI) is requested for patients who have unilateral sensorineural hearing loss without vertiginous symptoms typical of Meniere's disease. Although complete surgical excision can be achieved readily with labyrinthectomy, observation and monitoring with repeat MRI is an option in some cases.
  • Arch Neurol 1996 Dec;53(12):1299-304 The diagnostic value of imaging the patient with dizziness. A Bayesian approach. Gizzi M, Riley E, Molinari S New Jersey Neuroscience Institute, Seton Hall University School of Graduate Medical Education, Edison, NJ, USA. BACKGROUND: Imaging studies are routinely used in the evaluation of patients with dizziness. A principal concern of the ordering physician is to rule out a cerebellopontine angle (CPA) mass. The incidence of such masses in patients presenting with dizziness is quite low, however, raising the question of the value of imaging this population. OBJECTIVE: To calculate the probability, using Bayes theorem, that a given patient with dizziness has a CPA mass. DESIGN: Meta-analysis of epidemiological data on CPA masses and of studies reporting the incidence of otologic symptoms in patients with these masses. We also conducted a study of consecutive patients with dizziness to determine the frequency of asymmetric hearing loss in this population. These data were combined in applications of Bayes theorem to calculate disease probabilities. RESULTS: The probability that a patient with dizziness has a CPA mass is 0.0004, indicating that 2500 imaging studies would have to be performed to identify 1 CPA mass. If patients with subjectively normal hearing are investigated (ie, those with isolated dizziness), the probability is 0.000107, indicating that 9307 scans would have to be performed to identify 1 CPA mass. If the search is restricted to those patients with dizziness and asymmetric hearing loss (the patients usually felt to be high risk), the probability is 0.00156, indicating that 638 scans would have to be performed to identify 1 CPA mass. CONCLUSIONS: Even when studying patients with dizziness and asymmetric hearing loss, the probability of identifying a CPA mass is sufficiently low that we do not feel imaging is generally warranted. When faced with a patient with dizziness, we recommend a careful neurologic and otologic examination. If abnormalities are detected on examination that suggest central nervous system disease or invasive otologic disease, imaging should be pursued as appropriate. In cases of acute vertigo, if the patient is at high risk for cerebrovascular disease by virtue of age and additional risk factors, imaging should probably be pursued. For the remainder of patients, if progression of hearing loss is not documented, we do not believe imaging is warranted. Progressive hearing loss with abnormal speech reception thresholds probably warrants a magnetic resonance imaging scan of the internal auditory canals. Clin Otolaryngol 1996 Feb;21(1):80-3 Cost-benefit ratio in our search for cerebellopontine angle tumors. Caparosa RJ There is agreement that the morbidity and mortality of CPA tumors are directly proportional to the size of the tumor. The diagnostic difficulty, however, is indirectly proportional to the size: the large tumors are diagnosed with little difficulty, the smaller ones with considerable challenge. In the search for these tumors, it is apparent that the incidence of all sizes is low. To arrive at the diagnosis, efforts must be pursued in any patient with unilateral sensorineural hearing loss, unilateral tinnitus, or vertigo. In the past 12 years, 19,000 new patients were seen in the office of the Pittsburgh Otological Association. In this new patient population, 120 tumors were proven by surgery. Six others (bilateral tumors) were diagnosed but not operated on, and four others were diagnosed but were operated on elsewhere for a total of 130 patients. An estimate of the cost of diagnosing these tumors is presented. Consideration is given to the cost-benefit ratio of diagnosing those diseases which must be considered also in the differential diagnosis when the above symptoms are present. These are: congenital changes, trauma, metabolic neuropathies, Meniere's disease, noise-induced hearing loss, lues, sudden hearing loss, and unilateral symptoms of undetermined etiology. The incidence of symptoms consistent with cerebellopontine angle lesions in a general ENT out-patient clinic. Harcourt JP, Vijaya-Sekaran S, Loney E, Lennox P Department of Otolaryngology, Northwick Park Hospital, Harrow, Middlesex, UK. To quantify the potential burden for screening for cerebellopontine angle lesions, all adult new patients attending the ENT outpatient department of a district general hospital were documented to see if their presenting symptom(s) could be consistent with the presence of a cerebellopontine angle lesion. Of the patients, 19.7 per cent were found to be potential candidates for screening. A 15 decibel asymmetry at one frequency was found in 11.8 per cent of patients. The burden of screening with a variety of audiological and symptomatic protocols was quantified. Effective age limits to reduce the burden for screening were found to be either 65 or 75 years of age. With such a large potential pool of patients for further investigation, the results of this study could be used to suggest protocols which are likely to produce a load of cases, which matches local resources for screening. Laryngoscope 1999 Nov;109(11):1803-5 Positional vertigo and nystagmus of central origin. Watson P, Barber HO, Deck J, Terbrugge K Four cases are described illustrating the clinical features of positional vertigo and nystagmus due to posterior fossa tumors and a case of obstructive hydrocephalus. In these cases positional vertigo was the first and only presenting symptom of central nervous system disease. One case of subependymoma of the fourth ventricle and one with hydrocephalus had characteristic symptoms of benign positional vertigo; each showed positional nystagmus of the benign paroxysmal type. J Am Acad Audiol 1995 Jul;6(4):346-9 When paroxysmal positioning vertigo isn't benign. Jacobson GP, Butcher JA, Newman CW, Monsell EM Department of Otolaryngology-Head and Neck Surgery, Henry Ford Hospital, Detroit, Michigan 48202, USA. The electronystagmographic auditory brainstem response and magnetic resonance imaging findings for a 33-year-old male with a 3.5 cm left vestibular schwannoma are presented. Of particular interest was the presence of an unusual positioning nystagmus following the Dix-Hallpike maneuver in the right head-hanging position. The patient demonstrated a nystagmus that was immediate in onset and not fatigueable upon repeated positioning. During positioning, the patient experienced a vertical bobbing sensation and dysphoria, but not rotational vertigo. Most importantly, the nystagmus had a predominant downbeating vertical component. the case illustrates the diagnostic significance of downbeating nystagmus elicited by the Dix-Hallpike maneuver. Laryngoscope 1985 Oct;95(10):1208-12 Intracranial tumors mimicking benign paroxysmal positional vertigo. Dunniway HM, Welling DB Department of Otolaryngology, The Ohio State University, Columbus 43210, USA. Benign paroxysmal positional vertigo is a common type of vertigo seen by the otolaryngologist; however, intracranial tumors can mimic benign paroxysmal positional vertigo in their presentation. A review of patients seen in the Department of Otolaryngology at The Ohio State University between July 1992 and August 1996 identified five patients with intracranial pathologic conditions mimicking benign paroxysmal positional vertigo. These patients were first seen with episodic vertigo associated with positional change. Failure of the symptoms to respond to the particle repositioning maneuver or the finding of associated auditory or neurologic symptoms prompted further evaluation by magnetic resonance imaging of the brain, which revealed intracranial pathologic conditions including two meningiomas, a vestibular schwannoma, a glioma, and a lipoma. These findings suggest that patients seen with symptoms like those of benign paroxysmal positional vertigo who do not show improvement after undergoing the particle repositioning maneuver or those who describe associated auditory or neurologic symptoms should have magnetic resonance imaging done to rule out intracranial pathologic conditions. Additionally, benign paroxysmal positional vertigo and intracranial tumors may coexist. Neurosurgery 1997 Jan;40(1):1-9; discussion 9-10 Pathophysiology of positional vertigo of the malignant paroxysmal type. Sakata E, Uchida Y, Nakano Y, Takahashi K It is well known that paroxysmal positional vertigo is induced by change of head position and subsides immediately when the head is returned to the original position. There are two types of paroxysmal positional vertigo. One is what DIX and HALLPIKE (1952) call the benign type which is thought to be caused by otolith lesions. Another is a malignant type, in contrast to the former and is related to a lesion in the central nervous system. Otologists are usually familiar with the former, but the latter is not known among neurologists and neurosurgeons. We experienced 37 cases of the malignant type and found that this type of paroxysmal vertigo is induced by various diseases. Furthermore, we believe that the mechanism of this paroxysmal vertigo is the lack of inhibitory function of the vestibular cerebellum, and not abrupt malfunction of communication of the cerebrospinal fluid in the ventricular system. We also believe that this symptom is a very useful sign for diagnosing lesions of the cerebellar vermis. Can J Neurol Sci 1981 May;8(2):133-7 Intracranial tumors mimicking benign paroxysmal positional vertigo. Dunniway HM, Welling DB Department of Otolaryngology, The Ohio State University, Columbus 43210, USA. Benign paroxysmal positional vertigo is a common type of vertigo seen by the otolaryngologist; however, intracranial tumors can mimic benign paroxysmal positional vertigo in their presentation. A review of patients seen in the Department of Otolaryngology at The Ohio State University between July 1992 and August 1996 identified five patients with intracranial pathologic conditions mimicking benign paroxysmal positional vertigo. These patients were first seen with episodic vertigo associated with positional change. Failure of the symptoms to respond to the particle repositioning maneuver or the finding of associated auditory or neurologic symptoms prompted further evaluation by magnetic resonance imaging of the brain, which revealed intracranial pathologic conditions including two meningiomas, a vestibular schwannoma, a glioma, and a lipoma. These findings suggest that patients seen with symptoms like those of benign paroxysmal positional vertigo who do not show improvement after undergoing the particle repositioning maneuver or those who describe associated auditory or neurologic symptoms should have magnetic resonance imaging done to rule out intracranial pathologic conditions. Additionally, benign paroxysmal positional vertigo and intracranial tumors may coexist. Auris Nasus Larynx 1984;11(2):79-90 The pitfalls of limiting ENG testing to patients with vertigo. Keim RJ A high incidence of patients who do not experience vertigo with unilateral vestibular disease has been observed. Recognizing that many clinicians perform electronystagmographic vestibular testing on only patients who describe vertigo, a retrospective study of 229 patients was designed to study the potential pitfalls of this practice. The results confirm a preliminary study and show that 66% of the patients described no vertigo. Included were 13 intracranial lesions of which only one had the classic symptom. Differences in cortical perception and adaptation are postulated as factors responsible for these and other observations relative to caloric stimulation, torsion swing testing, and the presence of spontaneous nystagmus. J Laryngol Otol 1999 Jun;113(6):518-22
  • Clinical significance of rebound nystagmus. Lin CY, Young YH Department of Otolaryngology, National Taiwan University, Taipei. OBJECTIVE/HYPOTHESIS: Magnetic resonance imaging (MRI) examination is performed in cases of rebound nystagmus to elucidate the responsible site for rebound nystagmus. METHODS: Patients with vertigo or tinnitus received a battery of audiological and neuro-otological tests. Those who had rebound nystagmus were rechecked by electronystagmography and examined by MRI. RESULTS: Ten patients had a second-degree nystagmus evoked by changing direction of fixation, from the lateral to the forward gaze. All patients displayed positive MRI findings in cerebellum or brainstem, including tumor in seven cases and stroke in three cases. Moreover, in seven of eight cases with unilateral lesions, rebound nystagmus was ipsilateral with respect to the side of the lesion. CONCLUSION: Rebound nystagmus implies a lesion in cerebellum or brainstem for which MRI examination is 100% sensitive. It has a certain lateralizing value, with its direction away from the lesioned side. Am J Otol 1999 May;20(3):381-5 Magnetic resonance examination of the inner ear and cerebellopontine angle in patients with vertigo and/or abnormal findings at vestibular testing. Casselman JW, Kuhweide R, Dehaene I, Ampe W, Devlies F Department of Radiology, A. Z. St-Jan Brugge, Belgium. The inner ears of 167 patients with vertigo and/or abnormal findings at vestibular testing were studied using magnetic resonance (MR). Pathology potentially explaining vertigo was found in 54 patients, and was detected in the posterior fossa (28%), the internal auditory canal (28%) and the membranous labyrinth (44%). The overall percentage of pathology and the percentage of pathology found in the membranous labyrinth was high and was probably influenced by the referral pattern in our hospital where high resolution MR of the inner ear (three dimensional Fourier transformation-constructive interference in steady state sequence; 3DFT-CISS sequence) is available. Unenhanced, and especially gadolinium (Gd)-enhanced T1-weighted spin-echo images, are needed to detect most of the pathology inside the internal auditory canal, and some of the lesions inside the membranous labyrinth. 3DFT-CISS images are the only images that can show fibrous obliteration of the intralabyrinthine fluid spaces, and are therefore necessary to recognise most of the intralabyrinthine pathology. Finally, T2-weighted spin-echo images are best suited to demonstrate cerebellar or brain stem infarction. An additional MR-angiography sequence (three dimensional Fourier transformation-fast imaging with steady precession; 3DFT-FISP) is used when vascular compression of the cochleovestibular nerve is suspected. MR is the method of choice to look for pathology in patients with vertigo, and allows detection of pathology that remains invisible with other imaging techniques. However, well adapted sequences are needed to detect these lesions. Radiology 1990 Feb;174(2):395-400
  • Bilateral loss of vestibular function: clinical findings in 53 patients. Rinne T, Bronstein AM, Rudge P, Gresty MA, Luxon LM MCR Human Movement and Balance Unit, Institute of Neurology, The National Hospital for Neurology and Neurosurgery, London, UK. The clinical presentations and aetiologies of a series of 53 cases of bilateral vestibular failure (BVF) seen by the authors over a decade were evaluated by retrospective review of the medical records. Thirty-nine per cent of patients had associated neurological disease; 13% had a progressive cerebellar syndrome with disabling gait ataxia, abnormal eye movements and cerebellar atrophy on neuro-imaging. BVF was usually unsuspected. Nine per cent had cranial or peripheral neuropathies and in this group there was no abnormality of brain stem/cerebellar oculomotor function, but hearing loss was common. Eleven per cent revealed BVF and hearing loss secondary to meningitis, and 6% had other neurological disorders. Idiopathic BVF was found in 21% of cases, characterised by paroxysmal vertigo and/or oscillopsia, but no abnormal clinical signs. Gentamicin ototoxicity accounted for a further 17%, while autoimmune disease was present in 9% of patients. Otological or neoplastic disease was diagnosed in the remaining 13% of patients. It was concluded that neurological, audiological and ocular motor assessments allow the probable cause of BVF to be defined in approximately 80% of cases. A group of BVF related to autoimmune pathologies is reported for the first time, indicating the need for immunological screening. Idiopathic BVF may present with only minor visual or vestibular symptoms, while in patients with cerebellar degeneration, BVF may be unsuspected and, thus, underdiagnosed. J Laryngol Otol 1998 Feb;112(2):193-5
  • The posterior circulation supplies blood to the brainstem, cerebellum, peripheral vestibular apparatus, in addition to other structures. It is not surprising that vertibrobasilar insufficiency may be accompanied by vertigo. In general, brainstem TIAs should be accompanied by neurologic symptoms or signs, in addition to vertigo or dizziness before a clear-cut diagnosis is entertained. However, it is clear that isolated episodes of vertigo lasting many minutes, may be due to posterior circulation dysfunction (Grad and Baloh, 1989, Oas and Baloh, 1992). Symptoms include transient clumsiness, weakness, loss of vision, diplopia, perioral numbness, ataxia, drop attack, and dysarthria (Amarenco, 1991; Caplan 1993). Common signs of vertebrobasilar ischemia include disorders of motor function such as weakness, clumsiness, or paralysis. A crossed defect (a motor or sensory deficit on one side of the face and the opposite side of the body) is good evidence of brainstem dysfunction. If the occipital lobes are the site of ischemia, transient visual loss in the form of complete or partial homonymous hemianopia will occur. Ataxia, imbalance, unsteadiness, or disequilibrium not necessarily associated with spinning vertigo may occur because of labyrinthine or cerebellar ischemia. However it is incorrect to believe that dizziness must be present before a TIA of the posterior circulation can be diagnosed. Isolated symptoms like those described may occur without dizziness. On the other hand, it has been overemphasized that such symptoms must always accompany dizziness, when the vertiginous symptoms are due to brainstem TIA. In elderly patients with no laboratory evidence of peripheral vestibulopathy or systemic disease, episodic disequilibration or dizziness may be due to vertebrobasilar disease (Grad and Baloh, 1989). Sudden hearing loss with moderate dizziness may be due to infarction in the distribution of the internal auditory artery. In isolation, this symptom complex is uncommon in elderly patient with atherosclerotic vertebrobasilar disease, and is more suggestive of diseases affecting small and intermediate-diameter arteries such as syphilis, systemic lupus erythematosus, or periarteritis nodosa. In the atherosclerotic patient, such symptoms are usually accompanied by other signs of brainstem or cerebellar dysfunction, which allow a more certain diagnosis. If actual brainstem infarction occurs, neurological signs are often present on examination. Such signs may not be obvious and should be carefully sought. They include nystagmus of the central type, hyperreflexia, internuclear ophthalmoplegia, homonymous visual field defects, dysarthria, vertebral bruits, and ataxia (Leigh and Zee, 1991). Symptoms of dizziness are also quite common in proximal extracranial occlusion of the vertebral arteries (Caplan 1993) and in the subclavian steal syndrome. Up to this point, the emphasis has been on the accompanying signs and symptoms that almost always occur with vertebrobasilar disease. It is noteworthy, however, that acute severe vertigo, mimicking labyrinthine disease, is an early symptom of acute cerebellar infarction in the distal territory of the posterior inferior cerebellar artery (Amarenco 1991). To differentiate this condition from labyrinthine disease, particular attention is directed to the type of nystagmus that is present. Acute peripheral vestibulopathy usually causes unidirectional nystagmus, with the fast phase in the opposite direction. This is similar to the mnemonic COWS (Cold, Opposite, Warm, Same) for remembering the direction of the nystagmus fast phase during thermal irrigation of the ear. The fast phase is away from the side of the cold water irrigation. Cold water mimics a peripheral destructive lesion of the labyrinth, and almost all lesions are destructive. Therefore, with a peripheral labyrinthine disturbance, the nystagmus fast phase is in the opposite direction or away from the involved ear. The nystagmus increases during gaze in the direction of the fast phase or contralateral to the peripheral vestibulopathy. Swaying or falling occurs toward the side of the lesion (opposite the nystagmus fast phase). The nystagmus direction is said to be fixed in that it tends to be uni-directional, away from the side of the peripheral vestibulopathy and tends to remain horizontal on upward gaze. Neurology 2000 Mar 28;54(6):1376-9 Vestibular and oculomotor abnormalities in vertebrobasilar insufficiency. Corvera J, Benitez LD, Lopez-Rios G, Rabiela MT The early diagnosis of vertebrobasilar insufficiency in patients with vertigo as their only symptom was attempted using a battery of vestibulo-oculomotor tests. With this testing procedure, we were able to find abnormal vestibulo-oculomotor mechanisms that could account for the vertigo in 41 of 42 patients. These abnormalities, however, did not fall into an easily recognizable pattern that could be considered characteristic of vertebrobasilar insufficiency. The large intersubject variability probably arises from the very different and widespread lesions that occur at the vestibular and neurological levels as a consequence of vertebrobasilar insufficiency. Rinsho Shinkeigaku 1994 Jan;34(1):32-7
  • Isolated vertiginous dysequilibrium due to bilateral caudal cerebellar infarction. Lin SH, Chang YY, Lan MY, Wang FM, Chen WH, Liu JS Department of Neurology, Kaohsiung Medical College, Taiwan, Republic of China. We present a 74-year-old woman who developed a sudden onset of vertigo, vomiting, and axial retropulsion without limb ataxia. A clinical diagnosis of acute peripheral labyrinthine lesion was made initially. However, magnetic resonance imaging demonstrated a small infarct area involving the caudal part of the cerebellar vermis and medial cerebellar hemispheres on both sides. We emphasize that a cautious attitude should be taken in managing patients with isolated vestibular dysfunction, especially in aged person or those associated with stroke risk factor. Ann Otol Rhinol Laryngol 1980 Jul-Aug;89(4 Pt 1):370-6 [Acute vertigo caused by cerebellar vascular accident]. [Article in French] Garin P, Deggouj N, Decat M, Gersdorff M Universite Catholique de Louvain, Clinique Universitaire St-Luc, Bruxelles, Belgique. At the beginning, small cerebellar strokes may present only with acute onset of vertigo, unsteadiness and unidirectional nystagmus, like a vestibular neuritis. In some cases, it is associated with tinnitus and hearing disturbance, like an endolymphatic hydrops. Other cases may mimic a benign cupulolithiasis, with only a paroxysmal positioning vertigo. Attention should be focused on transient associated symptoms: headache and blurred vision. One should not wait for classical cerebellar clinical signs: they are subtle and they appear late. Within a few days, the clinical picture will change: vertigo will disappear, while unsteadiness will progress. The electronystagmography confirms the integrity of the vestibular peripheric system. The cerebral CT Scan will show the ischaemic lesions only several days after the onset of the symptoms. A magnetic resonance imaging is far more efficient. Small cerebellar strokes have a good prognosis: complete recovery may be hoped with acetylsalicylic acid treatment and kinesitherapy. Isolated acute vertigo in the elderly; vestibular or vascular disease? Norrving B, Magnusson M, Holtas S Department of Neurology, University Hospital, Lund, Sweden. INTRODUCTION--Elderly patients with isolated acute vertigo are commonly encountered in clinical practice, but little is known about the underlying cause of the symptoms. MATERIAL & METHODS--We prospectively studied 24 patients aged 50-75 years with the acute onset of isolated vertigo lasting > 48 h and no abnormality on neurological examination other than nystagmus. The study protocol included neuro-imaging (MRI 22 patients, CT 2 patients), Doppler sonography, and electro-oculography. RESULTS--MRI/CT showed the presence of an infarction of the caudal cerebellum in six patients (25%), 3 of whom had a potential cardioembolic source and normal Doppler sonography findings, whereas 3 patients had ipsilateral vertebral artery occlusion and normal cardiac findings. MRI of the posterior fossa was normal in 18 patients. On electro-oculography, ataxic pursuit eye movements was a characteristic finding in patients with cerebellar infarction, whereas caloric test findings were not discriminative. CONCLUSION--A caudal cerebellar infarction may easily be misdiagnosed clinically as a labyrinthine disorder, and was found to be the cause in one fourth of patients presenting with isolated acute vertigo. Nippon Jibiinkoka Gakkai Kaiho 1999 Jul;102(7):925-31
  • Vertebrobasilar insufficiency and stroke. Baloh RW Department of Neurology, University of California, Los Angeles School of Medicine. Vertigo is the most common symptom of transient ischemia within the vertebrobasilar system. Episodes typically come on abruptly and last minutes. Although there are usually associated symptoms, isolated attacks of vertigo can occur. Vertigo is also a common symptom with brain stem and cerebellar strokes. Isolated cerebellar infarction can mimic peripheral vestibular disorders because vertigo and imbalance are the main symptoms with both disorders. Modern neuroimaging techniques have markedly improved diagnostic capabilities. Neurology 1996 Jul;47(1):94-7 Isolated vertigo disclosing infarction in the area of the posterior and inferior cerebellar arteries]. [Article in French] Masson C, Sterkers O, Chaigne P, Colombani JM, Masson M Clinique neurologique, Hopital Beaujon, Clichy. We report three cases of small cerebellar infarcts mimicking labyrinthine dysfunction. A sudden rotatory vertigo might be the only presenting symptom of a cerebellar infarct. In these cases, the clinical features may closely mimick an acute peripheral labyrinthine disorder. However, the absence of nystagmus or a direction changing nystagmus with different eye position and the normality of caloric responses may be suggestive of a cerebellar infarct. This syndrome may be explained by the involvement of the nodulus, part of the flocculo-nodular complex, that has primary vestibular connections. Cerebellar infarcts mimicking labyrinthine dysfunctions involved usually the cerebellar territory of the posterior inferior cerebellar artery (PICA). Infarcts may be limited to the territory of the medial branch of the PICA which supplies the nodulus. Rev Laryngol Otol Rhinol (Bord) 1997;118(2):119-24 [Acute vertigo caused by ischemia of the postero-inferior cerebellar artery or PICA. Apropos of 2 cases]. [Article in French] Duclos JY, Darrouzet V, Loiseau H, Chambrin A, Boussens J, Bebear JP C.H.U. Pellegrin, Clinique Universitaire O.R.L., Bordeaux, France. Based on two cases and a review of the recent literature, we would like to draw attention to the fact that a cerebellar infarction, localised to the territory of the PICA, can present with just a single clinical feature-acute rotary vertigo. Cerebellar vascular accidents remain a little known cause of acute vertigo, often masquerading as a peripheral vestibular disturbance. The main differential diagnosis is vestibular neuronitis. The prognosis is usually good, but the risk of a late recurrence or of a further cerebro-vascular episode is difficult to predict. This uncommon aetiology should not be ignored because of the risk of progression towards a sort of pseudo-tumoral infarct, in which the risk of a fatal outcome remains ever present. In most cases, however, sequelae are few or absent. Rinsho Shinkeigaku 1989 Sep;29(9):1156-62
  • However, it is incorrect to believe that dizziness must be present before a TIA of the posterior circulation can be diagnosed. Isolated symptoms like those described may occur without dizziness. On the other hand, it has been overemphasized that such symptoms must always accompany dizziness, when the vertiginous symptoms are due to brainstem TIA. In elderly patients with no laboratory evidence of peripheral vestibulopathy or systemic disease, episodic disequilibration or dizziness may be due to vertebrobasilar disease (Grad and Baloh, 1989). HNO 1998 Apr;46(4):296-300 [Isolated vertigo in vertebrobasilar ischemia--does it exist]? [Article in German] Berlit P Neurologische Klinik mit Klinischer Neurophysiologie, Alfried-Krupp-Krankenhaus Essen. An isolated vertigo may occur in vertebrobasilar ischemia, especially as the first symptom of cerebellar ischemia or basilar artery occlusive disease. Based on neuroanatomical findings, these short-lasting vertigo attacks result from transient insufficiency of the anterior inferior cerebellar artery (AICA), which supplies the inner ear and leads to ischemia of the upper parts of the vestibular labyrinth. Persisting vertigo with or without lateropulsion may occur in cerebellar infarctions with involvement of the medial inferior hemispheres due to occlusion of the medial branch of the posterior inferior cerebellar artery. Caloric testing with oculography can usually differentiate cerebellar infarction and peripheral vestibular disease. Since ischemic lesions with AICA insufficiency may cause pathological results in caloric testing, both clinical and neurophysiological analysis of associated oculomotor signs is essential for a correct diagnosis. Otolaryngol Head Neck Surg 1995 Jan;112(1):114-7 Isolated vertigo as a manifestation of vertebrobasilar ischemia. Gomez CR, Cruz-Flores S, Malkoff MD, Sauer CM, Burch CM Souers Stroke Institute, Saint Louis University Health Sciences Center, MO, USA. OBJECTIVE: We sought to demonstrate that isolated episodes of vertigo can be the only manifestation of vertebrobasilar ischemia. BACKGROUND: Isolated persistent vertigo is classically ascribed to labyrinthine disorders and is only rarely considered to reflect vertebrobasilar ischemia. METHODS: We retrospectively analyzed all of the records of the Saint Louis University Stroke Registry between January 1, 1992 and September 1, 1993. We set out to identify those patients discharged with a diagnosis of transient ischemic attack (TIA) in the vertebrobasilar system. We reviewed their clinical records and the results of their diagnostic studies. RESULTS: We screened 600 admissions and found 29 patients with vertebrobasilar circulation TIAs. Of these, five men and one woman had episodic vertigo for at least 4 weeks as their only presenting symptom. All six patients had one of two abnormal patterns on magnetic resonance angiography (MRA): focal basilar stenosis or widespread vertebrobasilar slow flow. In three patients, the MRA findings were confirmed by cerebral angiography. Five patients were treated with warfarin and one with aspirin. Two patients developed brainstem infarctions, one of them fatal. CONCLUSIONS: Isolated vertigo can be the only manifestation of vertebrobasilar ischemia. Its frequency may be underestimated in clinical practice. Noninvasive testing is helpful both for diagnosis and follow-up. Chung Hua Erh Pi Yen Hou Ko Tsa Chih 1993;28(3):138-41, 185-6 [Vertigo caused by vertebrobasilar insufficiency]. [Article in Chinese] Zhang SZ PLA General Hospital, Beijing. This paper deals with electronystagmographic changes in 81 patients suffered from recurrent vertigo considered to be caused by vertebro-basilar insufficiency. Electro-oculogram features showed that (1) In saccadic test, the latency prolonged, the peak velocity of larger amplitude decreased; (2) When the eye smooth pursuit system was tested, Benitez III Type curves appeared in 40% of patients, simultaneously digital computer showed that the total harmonic distortion was more than 15% in 41.9% of patients; (3) the velocity ratio of fast to slow phases of the optokinetic nystagmus decreased, as a result of decrease in fast phase velocity. (4) The spontaneous and positional nystagmus occurred in 58% of patients. (5) The intensity of caloric response was weaker than that in normal subjects, canal paresis increased, failure of fixation suppression occurred in 17.7% of patients. Ear Nose Throat J 1995 Jan;74(1):33-6 When is isolated vertigo a harbinger of stroke? Rao TH, Libman RB Department of Neurology, Long Island Jewish Medical Center, Albert Einstein College of Medicine, New Hyde Park, New York 11042. Isolated vertigo is usually a distressing though benign clinical entity. Recently, however, it has been recognized that in a subset of patients isolated vertigo may be a harbinger of vertebrobasilar stroke. Recognition of this "at risk" subgroup may be difficult. Brainstem auditory evoked potentials (BAEPs) are usually normal in patients with vertigo from labyrinthine disease. We describe a patient with isolated vertigo, in whom BAEPs were abnormal, and who subsequently developed an anterior inferior cerebellar artery territory infarct. BAEP testing might lead to early recognition of those patients at risk for catastrophic stroke, and prompt appropriate investigation and treatment to prevent this outcome. Diagnosis of vertebrobasilar insufficiency: time to rethink established dogma? Kumar A, Mafee M, Dobben G, Whipple M, Pieri A Department of Otolaryngology-Head and Neck Surgery, University of Illinois at Chicago, Illinois, USA. As isolated symptoms, vertigo, dizziness and imbalance are not regarded by neurologists as reflections of transient ischemia in the vertebrobasilar circulation. The purpose of this retrospective study was to demonstrate that these symptoms can and do occur in isolation. To this end, we analyzed the symptoms, stroke risk factors and diagnostic algorithms in 27 patients with a diagnosis of transient vertebrobasilar ischemia. None of the 27 patients included in the review complained of any associated neurologic symptoms. Against the reference standard of brain imaging, the site of the pathologic lesion was defined in the brainstem/cerebellum with the Torok monothermal caloric test, with a sensitivity greater than 86%. Vestibular decruitment and hyperactive caloric responses were of particular diagnostic value. Thus, we recommend that the neurologic dogma with regard to brainstem cerebellar ischemia be rethought. Kao Hsiung I Hsueh Ko Hsueh Tsa Chih 1995 Mar;11(3):186-90 [MRI findings in patients with vertigo and dizziness possibly arising from vertebrobasilar insufficiency]. [Article in Japanese] Watanabe M, Takahashi A, Arahata Y, Motegi Y, Inafuku S Department of Neurology, Nagoya University School of Medicine. In order to evaluate diagnostic usefulness of MRI in vertebrobasilar insufficiency (VBI), we performed magnetic resonance imaging (MRI) and MR angiography (MRA) in 90 patients presenting vertigo and dizziness as an initial and cardinal complaint. High signals observed by T2-weighted imaging in the basal ganglia (44.4%) or pontine base (48.9%) were more frequently seen in the possible VBI group than in the controls (p < 0.001). The electronystagmographical abnormalities were commonly observed in the patients with a high signal in the pontine base, reflecting diffuse ischemic lesion in the territory of the vertebrobasilar system. Vertebral artery asymmetry (45.6%) or basilar artery twisting (41.1%) as shown by MRA was also significantly more frequent in the patients than in the controls (p < 0.05). In conclusion, MRI and MRA were considered to be useful in making a clinical diagnosis of VBI in such patients. Acta Neurol Scand 1995 Jan;91(1):43-8 [The efficiency of magnetic resonance angiography (MRA) in the diagnosis and vertigo--prediction of vertebrobasilar insufficiency (VBI) and atherosclerosis]. [Article in Japanese] Arai M, Higuchi A, Umekawa J, Mochimatsu Y, Itoh K Department of Otolaryngology, Yokohama Red Cross Hospital. Major causes of vertigo in patients who attend Otolaryngology clinics are peripheral vestibular disorders (PVD) and vertebrobasilar insufficiency (VBI). The purpose of this study was to see whether MRA findings can distinguish VBI from PVD to evaluate the efficiency of MRA in the diagnosis of vertigo. A total of 185 vertigo patients were examined by MRA with the 3D-Phase Contrast method. Three kinds of abnormalities of vessels, that is, (a) disappearance, (b) meandering, and (c) stoppage were found in MRA imaging. Therefore, we classified the MRA patterns into four types with two subtypes: type I; normal, type II-R; right hypoform of the vertebral artery, II-L; left hypoform of the vertebral artery, III; meandering form of the vertebrobasilar artery, IV-1; stoppage form of the basilar artery, IV-2; hypoform of the bilateral vertebral and basilar arteries. Diagnosis of the MRA patterns in each patient was camed out by a radiologist and neurosurgeon with the cooperation of an otolaryngologist. In 185 vertigo patients, 139 patients were clinically diagnosed as having PVD, 41 patients as having VBI, and 5 patients as having cerebellar and brainstem infarctions. The numbers of patients in MRA patterns I, II-R, II-L, III and IV were 140, 17, 12, 8, and 8 cases, respectively. The total number of VBI patients who demonstrated type III or IV patterns in MRA was significantly higher than that of type I, II-R and II-L (P < 0.005). All of the 8 cases with cerebellar and brainstem infarction belonged to type IV. Cerebral angiography was performed in nine cases with type II-R, II-L, III or IV-1, and the number of patients in each group was 3, 3, 1 and 2 cases, respectively. All of the type II-R and II-L cases revealed hypoform of the vertebral artery. The appearance of type III was restricted to type III's view was only meandering and type IV-1 showed severe stenotic changes in the union area. These data show that MRA findings in the diagnosis of vertigo patients are very effective in distinguishing VBI and cerebellar and brainstem infarction from PVD and that some pattern of MRA may correspond to atherosclerosis of the vertebrobasilar artery. We think MRA examination of vertigo patients is useful not only to assist in the diagnosis of the etiology but also to predict the stage of atherosclerosis in each patient. Rev Prat 1994 Feb 1;44(3):336-41 [Vertigo of vascular origin]. [Article in French] Rancurel G, Timsit S Services des urgences cerebro-vasculaires, hopital de la Salpetriere, Paris. The concept of vascular origin in vertigo and equilibrium disorders is only a pragmatic clinical tool used to initiate and direct complex, complementary clinical investigation to determine the location (peripheral, central or mixed), the pathophysiological mechanisms and the multiple etiologies of the vestibular disorder. The concept includes focal and systemic ischemic processes and haemorrhagic processes of the posterior fossa. If the vertigo is associated with central neurological signs, the method can lead to certain diagnosis. If such signs are not detected by neurological screening of all the intra-axial signs of the vertebrobasilar regions, the diagnosis is uncertain clinical. Screening associated CT scan and MRI is more reliable than neuro-otological quantification. The major diagnostic difficulty involves TIA. Vertebrobasilar appears to be more heterogeneous than carotid ischemia. Schematic distinction is possible to differentiate thrombo-embolic from hemodynamic TIA. It is based on methodic clinical analysis and complementary investigations that are of unequal value. Rev Laryngol Otol Rhinol (Bord) 1992;113(2):87-90 [A case of ischemic disturbance of inner ear]. [Article in Japanese] Gotoh T, Hashimoto Y, Araki S, Nakagawa T, Nagata M We reported a 51-year-old male with ischemic disturbance of right inner ear resembling Meniere's disease. The patient had a sudden-onset episode of vertigo, right severe hearing disturbance, nausea, vomiting and gait disturbance. Two days after, he had hypersomnia, vertical gaze palsy, double vision, left Horner's sign, and sensory disturbance of pain and temperature of right half body involving face. Brain MRI disclosed high intensity area in T2-weighted image and proton density in bilateral paramedian thalamo-mesencephalic region and right cerebellum (area of the anterior inferior cerebellar artery). Cerebral angiography showed 90% or more stenosis of the right vertebral artery, 50% stenosis of the left vertebral artery before the posterior inferior cerebellar artery (PICA), and 60% stenosis of distal portion of the basilar artery. Furthermore, stem portion of the posterior cerebral artery, and the right anterior cerebellar artery and the left vertebral artery after the PICA were absent or occluded. Right deafness was evaluated to be Jerger type II, namely disturbance of inner ear. Caloric tests showed no response, and right auditory brainstem response showed no waves. Main cause of this vertigo and right deafness was considered to be disturbance of inner ear due to ischemia of right labyrinthine artery, though this patient was not a typical case of the anterior cerebellar artery syndrome. Ischemic disturbances of inner ear have been reported only in patients with the anterior cerebellar artery syndrome, therefore this patient who had only acute ischemic disturbance of inner ear and did not have disturbance of caudo-lateral portion of the pons was considered to be very rare. Ear Nose Throat J 1998 Dec;77(12):966-9, 972-4 Rotational vertebral artery occlusion syndrome with vertigo due to "labyrinthine excitation". Strupp M, Planck JH, Arbusow V, Steiger HJ, Bruckmann H, Brandt T Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians University, Munich, Germany. [Medline record in process] Leftward head rotations in a patient with a rotational vertebral artery occlusion syndrome elicited recurrent uniform attacks of severe rotatory vertigo and tinnitus in the right ear. These attacks were accompanied by a mixed clockwise torsional downbeat nystagmus with a horizontal component toward the right. A transient ischemia of the right labyrinth probably induced the attacks and led to a combined transient excitation of the right anterior and horizontal semicircular canals as well as the cochlea. Neurology 1992 Dec;42(12):2274-9 Vertigo and the anterior inferior cerebellar artery syndrome. Oas JG, Baloh RW Department of Neurology, UCLA Medical Center 90024-1769. We present two patients with clinical features of infarction in the distribution of the anterior inferior cerebellar artery (AICA) who had vertigo as an isolated symptom for several months prior to infarction. Both had risk factors for cerebrovascular disease and other episodes of transient neurologic symptoms not associated with vertigo. At the time of infarction they developed vertigo, unilateral hearing loss, tinnitus, facial numbness, and hemiataxia. MRI identified hyperintense lesions in the lateral pons and middle cerebellar peduncle on T2-weighted images. Audiometry and electronystagmography documented absent auditory and vestibular function on the affected side. Since the blood supply to the inner ear and the vestibulocochlear nerve arises from AICA, a combination of peripheral and central symptoms and signs is characteristic of the AICA infarction syndrome. The vertigo that preceded infarction may have resulted from transient ischemia to the inner ear or the vestibular nerve. Arch Neurol 1989 Mar;46(3):281-4 Vertigo of vascular origin. Clinical and electronystagmographic features in 84 cases. Grad A, Baloh RW Department of Neurology, Reed Neurological Research Center, UCLA School of Medicine 90024-1769. We reviewed the clinical and electronystagmographic findings of 84 patients who presented to our neuro-otology clinic with vertigo of presumed cerebrovascular origin. There was a surprisingly high incidence of isolated episodes of vertigo (abrupt in onset, lasting minutes). In some patients these episodes preceded other symptoms of vertebrobasilar insufficiency or infarction by months. Peripheral vestibular abnormalities were common on electronystagmographic testing; 42% had unilateral hypoexcitability to caloric stimulation. We conclude that the vestibular labyrinth is selectively vulnerable to ischemia within the vertebrobasilar system. Ann Otolaryngol Chir Cervicofac 1992;109(2):80-6
  • We performed a thorough neuro-otological evaluation and a radiological study of the cervical spine in 102 patients with vestibular symptoms. A group of 20 asymptomatic subjects served as control. Some degree of cervical spondylosis was found in 121 of 122 patients but also in the majority of control subjects. No relationship was observed between the degree of cervical spondylosis and vestibular abnormalities. Instead, the latter were strongly related to age. Thus, an age dependent microvascular damage may be more likely as an etiological factor for vestibular symptoms in the elderly.
  • Central neurogenic vertigo

    1. 1. Central Neurogenic Vertigo
    2. 2. Vertigo in Neuro Clinic <ul><li>June 99 to May 2000 </li></ul><ul><li>Total no of Neuro cases seen: 2456 </li></ul><ul><li>Total no of vertigo cases seen: 248 </li></ul><ul><li>Male : Female 150: 106 </li></ul><ul><li>< 10 years 6 </li></ul><ul><li>10 – 20 years 4 </li></ul><ul><li>20 – 40 years 112 </li></ul><ul><li>40 – 60 years 74 </li></ul><ul><li>> 60 years 48 </li></ul>
    3. 3. Is It Central Vertigo? <ul><li>Associated symptoms </li></ul><ul><li>Central : diplopia, dysarthria, dysphagia, perioral numbness, one sided weakness or ataxia, headache (occipital) </li></ul><ul><li>Peripheral: Hearing loss, tinnitus, </li></ul>
    4. 4. Vertigo Central/peripheral ?
    5. 5. Vertigo Central/peripheral?
    6. 6. Vertigo in Childhood Neurological deficit Isolated Tumor Migraine Epilepsy
    7. 7. Migraine and Vertigo <ul><li>Most common cause of vertigo in children 34.7% </li></ul><ul><li>Basilar migraine and Migraine equivalents as benign paroxysmal vertigo </li></ul><ul><li>Migraine and benign positional vertigo may coexist (migraine 3 times in BPV) 32% </li></ul><ul><li>Mi igraineurs had higher prevalence central and peripheral vestibular dysfunction compared to non-migraineurs </li></ul><ul><li>Respond to anti migraine medication </li></ul>
    8. 8. Basilar migraine <ul><li>Bickerstaff in 1961 </li></ul><ul><li>Vertigo with brain stem symptoms followed by headache </li></ul><ul><li>Due to basilar artery vasoconstriction followed by vasodilatation </li></ul><ul><li>Recently central mechanism postulated </li></ul>
    9. 9. Benign paroxysmal vertigo in childhood. <ul><li>V ertigo of sudden onset lasting a few minutes and in extreme cases several hours or even up to 2 days. </li></ul><ul><li>Autonomic symptoms </li></ul><ul><li>nystagmus </li></ul><ul><li>Consciousness is not impaired. </li></ul><ul><li>Frequency varies weeks to months </li></ul>
    10. 10. Episodic vertigo related to migraine vestibular migraine? <ul><li>Onset : at any age, peak fourth decade in men and a &quot;plateau&quot; between the third and fifth decades in women. </li></ul><ul><li>Duration: Few seconds to several hours </li></ul><ul><li>78% Mono - symptomatic Vertigo </li></ul><ul><li>16% audio-vestibular </li></ul><ul><li>32% not associated with headache </li></ul><ul><li>In the symptom-free interval </li></ul><ul><ul><li>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%). </li></ul></ul><ul><li>52% with other migraine </li></ul><ul><li>Nervenarzt 1993 Feb;64(2):121-6 </li></ul>
    11. 11. Epileptic vertigo <ul><li>Vertigo is common as aura of an epileptic attack </li></ul><ul><li>Isolated recurrent vertigo is rare </li></ul><ul><li>Usually last for few seconds </li></ul><ul><li>EEG shows temporal spikes </li></ul>
    12. 12. Vertigo in Adult Hearing loss Isolated Meniere's CA Mass Labyrinthitis Vestibular Neuronitis Benign Positional
    13. 13. CP angle tumor <ul><li>R arely present solely with episodic vertigo. </li></ul><ul><li>Vertigo occurs in approximately 20%, but a symptom of imbalance or dis - equilibration is more common </li></ul><ul><li>The most common symptoms associated progressive hearing loss and tinnitus. </li></ul><ul><li>Look for neurological signs such as a depressed corneal reflex. </li></ul>
    14. 14. The diagnostic value of imaging the patient with dizziness. A Bayesian approach. <ul><li>Dizziness : 0.0004 or 1 in 2500 </li></ul><ul><li>Isolated dizziness without hearing loss: .000107 or 1 in 9307 </li></ul><ul><li>Dizziness with asymmetric hearing loss: 0.00156 or 1 in 638 </li></ul><ul><ul><li>Clin Otolaryngol 1996 Feb;21(1):80-3 </li></ul></ul>
    15. 15. Imaging in Dizziness for CAM <ul><li>Progressive hearing loss with abnormal speech reception thresholds probably warrants a magnetic resonance imaging scan of the internal auditory canals . </li></ul>
    16. 16. Bilateral loss of vestibular function: clinical findings in 53 patients. <ul><li>39% had associated neurological disease </li></ul><ul><li>13% had a progressive cerebellar syndrome </li></ul><ul><li>9% cranial or peripheral neuropathies with hearing loss </li></ul><ul><li>11% had hearing loss secondary to meningitis </li></ul><ul><li>6% had other neurological disorders. </li></ul><ul><li>17% had Gentamicin ototoxicity </li></ul><ul><li>9% autoimmune disease </li></ul><ul><li>13% Otological or neoplastic disease </li></ul><ul><li>21% Idiopathic BVF - characterised by paroxysmal vertigo and/or oscillopsia, but no abnormal clinical signs. </li></ul><ul><li>J Laryngol Otol 1998 Feb;112(2):193-5 </li></ul>
    17. 17. Vertigo in Elderly Neurological deficit Isolated Vascular Cervicogenic
    18. 18. Vascular Vertigo <ul><ul><ul><li>Vertebrobasilar Insufficiency </li></ul></ul></ul><ul><ul><ul><li>Wallenberg’s syndrome </li></ul></ul></ul><ul><ul><ul><li>AICA syndrome </li></ul></ul></ul><ul><ul><ul><li>Cerebellar infarction </li></ul></ul></ul><ul><ul><ul><li>Cerebellar hemorrhage </li></ul></ul></ul>
    19. 19. Lateral medullary syndrome <ul><li>Complete syndrome: Vertigo with dysphagia, dysarthria, ipsilateral ataxia and facial anesthesia, Horner's syndrome and contralateral body anesthesia </li></ul><ul><li>Incomplete syndrome may present with vertigo followed by subtle Horner. Facial and body hypoesthesia. Needs careful examination. </li></ul>
    20. 20. Small Cerebellar infarction <ul><li>Vertigo, unsteadiness and unidirectional nystagmus – Vestibular neuritis </li></ul><ul><li>Vertigo with tinnitus and hearing disturbance – Meniere's disease </li></ul><ul><li>Paroxysmal positional vertigo – BPV </li></ul>
    21. 21. Clue to small cerebellar infarction <ul><li>Transient headache and blurred vision </li></ul><ul><li>Vertigo may disappear but ataxia may progress </li></ul><ul><li>Electronystagmography show normal peripheral apparatus </li></ul>
    22. 22. VBI and Vertigo <ul><li>Vertigo is common in VBI </li></ul><ul><li>Isolated vertigo was thought to rule out VBI in elderly </li></ul><ul><li>Recently many papers have emphasized isolated recurrent vertigo as symptom of VBI </li></ul><ul><li>Ischemia of vestibule by AICA and medial cerebellum by PICA </li></ul><ul><li>MRA, Angiography BAER may help </li></ul><ul><li>In elderly patients with no laboratory evidence of peripheral vestibulopathy or systemic disease, episodic disequilibration or dizziness may be due to vertebrobasilar disease </li></ul>
    23. 23. Cervicogenic Vertigo <ul><li>Abnormal cervical proprioceptive impulse to vestibular apparatus </li></ul><ul><li>Associated neck pain and tender points and limitation of neck movements </li></ul><ul><li>Improves with limitation of neck movements and aggravated by neck movements </li></ul><ul><li>Cervical spondylosis, cervical trauma </li></ul>
    24. 24. Clinical significance of cervical arthropathy in patients with vertigo . <ul><li>102 Patient examined with 20 Control with vestibular symptoms </li></ul><ul><li>No relationship was observed between the degree of cervical spondylosis and vestibular abnormalities </li></ul><ul><li>an age dependent microvascular damage may be more likely as an etiological factor for vestibular symptoms in the elderly. </li></ul><ul><li>Rev Med Chil 1990 Jun;118(6):657-61 </li></ul>
    25. 25. Isolated Vertigo 99-2000 <ul><li>Epilepsy 02 </li></ul><ul><li>Migraine 10 </li></ul><ul><li>Cervical spondylosis 30 </li></ul><ul><li>Peripheral unclassified 122 </li></ul><ul><li>Meniere's 01 </li></ul><ul><li>BPV 01 </li></ul><ul><li>CPA Mass 00 </li></ul><ul><li>VBI 00 </li></ul>
    26. 26. Thank you