1/47. An overlap syndrome with features of atypical cogan syndrome and Wegener's granulomatosis.A 48 year old women developed serous otitis, scleritis, myalgia, vertigo, polyneuropathy, crescentic glomerulonephritis, general cerebral dysrythmia, hilar adenopathy, and retroorbital granulomatous inflammation. Pulmonary manifestations were absent and antibodies against neutrophilic cytoplasmic antigens (ANCA) could not be detected. The clinical picture was classified as an overlap syndrome with features of both atypical cogan syndrome and Wegener's granulomatosis. The patient responded to treatment with high dose corticosteroids and pulse cyclophosphamide.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
2/47. Cogan's syndrome: an oculo-audiovestibular disease.Typical Cogan's syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Meniere-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogan's syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogan's syndrome.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
3/47. Superior canal dehiscence syndrome.OBJECTIVE: To present the symptoms, signs, and findings on diagnostic tests of patients with the superior canal dehiscence syndrome and to describe the surgical procedures used to treat the dehiscence in five patients. DESIGN AND SETTING: Prospective study of a series of patients identified as having this syndrome at a tertiary care referral center. patients AND RESULTS: Seventeen patients with vertigo, oscillopsia, or both evoked by intense sounds or stimuli that caused changes in middle ear and/or intracranial pressure were identified over a 4-year period. The evoked eye movements had vertical and torsional components, with the direction corresponding to the effect of the stimuli in causing excitation (Valsalva against pinched nostrils, tragal compression, sounds) or inhibition (Valsalva against a closed glottis or jugular venous compression) of the affected superior semicircular canal. Thirteen (76%) of these patients also experienced chronic dysequilibrium that was often the most debilitating symptom. Dehiscence of bone overlying the superior semicircular canal on the affected side was confirmed with computed tomographic scans in each case. Surgical procedures through the middle fossa approach to plug or resurface the superior canal were performed in five patients (canal plugging in three cases and resurfacing of the dehiscence without plugging in two). The debilitating symptoms resolved or improved after the procedures. Signs of vestibular hypofunction, without loss of hearing, were noted in one patient after plugging of the superior canal and in one other patient after resurfacing of the canal. CONCLUSIONS: The superior canal dehiscence syndrome is identified based on characteristic symptoms, signs, and computed tomographic findings. The clinical presentation and findings can be understood in terms of the effect of the dehiscence on the physiology of the labyrinth. The syndrome is a treatable cause of vestibular disturbance.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
4/47. Rotational vertebral artery occlusion syndrome with vertigo due to "labyrinthine excitation".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.- - - - - - - - - - ranking = 5keywords = vertigo (Clic here for more details about this article) |
5/47. Vestibular-evoked myogenic potentials in the diagnosis of superior canal dehiscence syndrome.patients with superior canal dehiscence (SCD) syndrome have vertigo and oscillopsia induced by loud noises and by stimuli that result in changes in middle ear or intracranial pressure. We recorded vestibular-evoked myogenic potentials (VEMP responses) in 10 patients with SCD syndrome. The diagnosis had been confirmed in each case by evoked eye movements and by high-resolution CT scans of the temporal bones that showed a dehiscence overlying the affected superior canal. For the 8 patients without prior middle ear disease, the VEMP threshold from the dehiscent ears measured 72 /- 8 dB NHL (normal hearing level) whereas the threshold from normal control subjects was 96 /- 5 dB NHL (p < 0.0001). The VEMP threshold measured from the contralateral ear in patients with unilateral dehiscence was 98 /- 4 dB NHL (p > 0.9 with respect to normal controls). Two patients with apparent conductive hearing loss from middle ear disease, and SCD, had VEMP responses from the affected ears. In the absence of dehiscence, VEMP responses would not have been expected in the setting of conductive hearing loss. These findings confirm earlier studies demonstrating that patients with SCD syndrome have lowered VEMP thresholds. Conditions other than SCD syndrome may also lead to lowered VEMP thresholds. Rather than being based upon a single test, the diagnosis of SCD syndrome is best established when the characteristic symptoms, signs, VEMP response, and CT imaging all indicate SCD.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
6/47. Fluctuant, progressive hearing loss associated with Meniere like vertigo in three patients with the Pendred syndrome.OBJECTIVE: To evaluate vestibular and long-term audiometric findings in patients with Pendred syndrome. Study design: Retrospective analysis of long-term clinical data. SETTING: University hospital department. patients: Three patients with Pendred syndrome caused by a mutation in the SLC26A4 gene. methods: Perchlorate discharge test, mutation analysis of the SLC26A4 gene, MR imaging of temporal bones, vestibular function test (in two cases) and serial audiometry. A saturation hyperbola with onset age was fitted to the audiometric threshold-on-age data using a nonlinear regression method. The residues remaining after regression were analyzed in a correlation analysis to detect significant ipsilateral or contralateral cofluctuation. RESULTS: All three patients had a mutation in the SLC26A4 gene and bilateral enlarged vestibular aqueduct; two of them had a positive perchlorate discharge test but in one of two siblings this test was negative. hearing loss was significantly progressive with significant ipsilateral and contralateral cofluctuation in all evaluable cases, combined with episodes of Meniere like vertigo in two cases. The episodes of vertigo are as seen in meniere disease. One case had unilateral caloric areflexia and one had bilateral vestibular hyporeflexia, proven to be progressive in a repeat examination. CONCLUSIONS: patients with Pendred syndrome may exhibit progressive and fluctuant hearing loss with episodes of vertigo.- - - - - - - - - - ranking = 7keywords = vertigo (Clic here for more details about this article) |
7/47. vertigo and the enlarged vestibular aqueduct syndrome.An enlarged vestibular aqueduct (EVA) is one of the most commonly identified inner ear bony malformations in children with sensorineural hearing loss of unknown cause. Most previous reports have focused on hearing loss, but individuals with EVA may also experience paroxysmal vertigo lasting minutes to hours. We report the clinical vestibular features and vestibular function testing of two children and one adult with EVA who had a history of sensorineural hearing loss and presented to our neurotology Clinic for the evaluation of episodic vertigo. All the patients had an antecedent history of profound bilateral sensorineural hearing loss that had been present since early childhood. The onset of vertigo was delayed into adulthood in one patient. Episodes of vertigo could be triggered by minor head trauma or vigorous physical activity. Despite recurrent episodes of vertigo, vestibular function was normal or moderately impaired compared with the severe auditory deficit. Careful analysis of temporal bone CT demonstrated EVA. Associated enlargement of the membranous endolymphatic sac was evident on brain MRI. While hearing loss is a prominent symptom in patients with EVA, vestibular symptoms may cause referral to a neurologist. Although hearing loss occurs early in childhood, vestibular symptoms can be delayed into adulthood, a finding not previously reported.- - - - - - - - - - ranking = 5keywords = vertigo (Clic here for more details about this article) |
8/47. basilar artery migraine in young children.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.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
9/47. Two cases of medically and surgically intractable SUNCT: a reason for caution and an argument for a central mechanism.We report two cases of SUNCT that demonstrate the medically and surgically refractory nature of this disorder and support the hypothesis that the causative 'lesion' lies within the central nervous system. After both patients had failed medical therapies, the first underwent a glycerol rhizotomy, gammaknife radiosurgery and microvascular decompression of the trigeminal nerve. The second patient underwent gammaknife radiosurgery of the trigeminal root exit zone and two microvascular decompression surgeries. Neither patient benefited from these procedures. Currently, the first patient suffers from anaesthesia dolorosa and the second patient from unilateral deafness, chronic vertigo and dysequilibrium as a result of surgical trauma. These cases of SUNCT highlight the uncertainty regarding the role of surgery given the potential for significant morbidity. These cases also suggest that SUNCT originates and may be maintained from within the CNS and this central locus explains why SUNCT is not typically amenable to interventions aimed at the peripheral portion of the trigeminal nerve.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
10/47. Recurrent abdominal pain: when should an epileptic seizure be suspected?Recurrent episodes of abdominal pain are common in childhood. Among the diagnostic possibilities are migraine and abdominal epilepsy (AE). AE is an infrequent syndrome with paroxystic episodes of abdominal pain, awareness disturbance, EEG abnormalities and positive results with the introduction of antiepileptic drugs. We present one 6 year-old girl who had short episodes of abdominal pain since the age of 4. The pain was followed by cry, fear and occasionally secondary generalization. MRI showed tumor in the left temporal region. As a differential diagnosis, we report a 10 year-old boy who had long episodes of abdominal pain accompanied by blurring of vision, vertigo, gait ataxia, dysarthria, acroparesthesias and vomiting. He received the diagnosis of basilar migraine. In our opinion, AE is part of a large group (partial epilepsies) and does not require a special classification. Pediatric neurologists must be aware of these two entities that may cause abdominal pain.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
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