1/14. Familial progressive vestibulocochlear dysfunction caused by a COCH mutation (DFNA9).OBJECTIVE: To describe the decline of vestibulocochlear function in a man with vestibulocochlear dysfunction caused by a Pro51Ser mutation within the COCH gene on chromosome 14q12-13 (DFNA9). methods: A follow-up of more than 15 years was performed in a single case. Clinical investigations were supplemented by oculomotor, vestibular, and auditory tests. RESULTS: A 50-year-old man had had progressive sensorineural hearing loss and dysequilibrium for 15 years; he had been asymptomatic at the age of 35 years. He suffered from instability in the dark, head movement-dependent oscillopsia, paroxysmal positional vertigo, and vertigo with and without nausea. Hearing impairment started unilaterally, predominantly in the high frequencies. He also reported tinnitus. Disease progressed to severe bilateral high-frequency hearing impairment and vestibular areflexia. Fluctuation of vestibulocochlear function was documented and mentioned by the patient. CONCLUSIONS: Our patient proved to suffer from an autosomal dominant vestibulocochlear disorder caused by a COCH gene mutation. The remarkable medical history has some features in common with meniere disease; however, there are also different clinical and neurophysiological features. In the family, phenotypic variability is present.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
2/14. An anatomic variant of the anterior inferior cerebellar artery in a patient with Meniere's disease.OBJECTIVE: To describe an anatomic variant of the anterior inferior cerebellar artery in a patient with Meniere's disease. STUDY DESIGN: Retrospective case review and review of the literature. SETTING: Tertiary referral clinic. INTERVENTION: vestibular nerve section and microvascular decompression. MAIN OUTCOME MEASURES: Audiometric testing and control of vertigo. RESULTS: The eighth nerve was identified via a retromastoid approach. The anterior inferior cerebellar artery was observed bisecting the eighth nerve. The vestibular nerve was sectioned, and microvascular decompression was performed on the cochlear division. At last follow-up, the patient had not experienced any vertiginous attacks but was observed to have progressive hearing loss. CONCLUSIONS: The course of the anterior inferior cerebellar artery is highly variable and difficult to predict. Knowing the potential paths is a necessity in performing posterior fossa surgery. Although the patient's vertigo was controlled by the vestibular nerve section, microvascular decompression of the cochlear nerve did not result in hearing improvement or stabilization. This case report does not support a benefit of microvascular decompression in Meniere's disease. vestibular nerve section remains the authors' treatment of choice for controlling disabling vertigo caused by Meniere's disease.- - - - - - - - - - ranking = 1.5keywords = vertigo (Clic here for more details about this article) |
3/14. Familial idiopathic intracranial hypertension.AIM: To analyze the development and occurrence of the idiopathic intracranial hypertension and consequent visual loss in a family affected with idiopathic intracranial hypertension. methods: We studied 15 members of the same family and found six of them affected with idiopathic intracranial hypertension, which was accompanied with visual loss as a repercussion of the disease. Idiopathic intracranial hypertension was diagnosed on neurological and radiological examination. Visual examination to establish visual loss included fundoscopy, visual acuity, visual field testing, and ultrasonography of the optic nerve. RESULTS: The construction of a family tree and detailed examination of 15 family members revealed idiopathic intracranial hypertension with visual disturbances, even amaurosis, and different stages of visual field constriction in three members of the family: the mother and her two daughters. Due to the symptoms of idiopathic intracranial hypertension, such as headaches, nausea, vertigo, and the presence of transient visual obscuration and papilledema, in three other members of this family (aged 16, 17, and 25 years), we considered a presumptive diagnosis of idiopathic intracranial hypertension, and the need for thorough follow-up. Medical data on the family grandmother, who died 34 years ago, suggested that she also had symptoms of idiopathic intracranial hypertension. One of the patients underwent surgical treatment by a lumbo-peritoneal shunt operation worsening of the symptoms. CONCLUSION: It is very important to include idiopathic intracranial hypertension in differential diagnosis of papilledema and recognize it in early stages to prevent vision loss. Current successful therapeutic approaches and close follow-up of such patients require teamwork of neurologists, ophthalmologists, and neurosurgeons.- - - - - - - - - - ranking = 0.5keywords = vertigo (Clic here for more details about this article) |
4/14. Cerebral cavernous malformations with dynamic and progressive course: correlation study with vascular endothelial growth factor.BACKGROUND: Cerebral cavernous malformations (CCMs) are reported to exhibit a wide range of dynamic patterns including growth, regression, and de novo formation, which generally show slow and steady courses. Although the pathogenesis of CCMs is not well known, vascular endothelial growth factor (VEGF) has been suggested as a possible mediating factor. OBJECTIVES: To report CCMs showing rapid progression over a short period and to investigate these biological characteristics. DESIGN: Experimental study. SETTING: Tertiary referral center, neurology department.Patient A 40-year-old man was admitted because of a left-sided numbness, vertigo, and ataxia, which were attributed to a pontine hemorrhage. He had experienced a left-sided weakness 6 months before admission, and thereafter had complained of intermittent headache. Serial brain magnetic resonance images showed multiple intracerebral microhemorrhages throughout the cerebral hemispheres. A biopsy of the lesion confirmed the diagnosis of CCM. MAIN OUTCOME MEASURES: We investigated the expression of VEGF by immunohistochemistry of the biopsy specimen. Dynamic patterns of CCMs, obtained with spin-echo magnetic resonance images with gradient-echo sequences, were compared with serial serum VEGF concentrations, determined by enzyme-linked immunosorbent assay. RESULTS: immunohistochemistry of the specimen displayed increased VEGF expression. Serial magnetic resonance images during 7 months showed dynamic signal changes of the preexisting lesions and 15 de novo formations in many cortices. The VEGF level in serum increased during this dynamic period and became normal during the steady and resolving stages. CONCLUSIONS: Cerebral cavernous malformations can be progressively deteriorating. The endothelial proliferation induced by VEGF is likely to be an important aspect of the pathogenetic mechanisms of CCMs.- - - - - - - - - - ranking = 0.5keywords = vertigo (Clic here for more details about this article) |
5/14. Problems in diagnosing cranial base meningioma in patients with multiple sclerosis.A 49-year-old patient had been suffering from the relapsing-remitting form of multiple sclerosis since the age of 23. Attacks of the disease appeared every 2 years in the form of right-sided hemiparesis, vertigo, and problems in maintaining balance. The symptoms disappeared after treatment. At the age 32 retrobulbar inflammation of the second cranial nerve appeared with visual acuity weakness. The symptoms disappeared after treatment. At the age of 42, bilateral weakness of visual acuity appeared and then epileptic attacks occurred. After surgical treatment of meningioma the symptoms disappeared. Only the features of a psycho-organic syndrome remained. The following attack of MS appeared 2 years after surgical intervention. MRI of the head disclosed numerous demyelinating foci.- - - - - - - - - - ranking = 0.5keywords = vertigo (Clic here for more details about this article) |
6/14. Cogan's syndrome: clinical evolution of deafness and vertigo in three patients.The aim of this study was to evaluate the clinical symptoms, the otoneurological examinations, the treatment and the clinical course of three patients suffering from Cogan's syndrome, a rare disease based on the clinical association of a non-syphilitic interstitial keratitis with a cochleo-vestibular deficit. This case series involved three patients with follow up. The clinical course of the three patients (aged 30, 48 and 49 years) with Cogan's syndrome during a follow-up period of 2 to 6 years is reported. All patients underwent complete otoneurological, ophthalmologic and rheumatologic examinations and were treated with immunosuppressive therapy such as glucocorticoids and cyclophosphamide in two and glucocorticoids and methotrexate in one patient. Using immunosuppressive therapy, ophthalmologic symptoms disappeared rapidly in two patients. Hearing improved only in one and stabilized in a second patient. One patient died after 6 years of treatment because of complications of generalized vasculitis. early diagnosis and rapid initiation of a combined immunosuppressive therapy such as corticosteroids and cyclophosphamide seem to be important in controlling the disease and avoiding persistent deafness. Whether systemic complications and a fatal outcome also can be prevented is still questionable.- - - - - - - - - - ranking = 2keywords = vertigo (Clic here for more details about this article) |
7/14. Progressive calvarial and upper cervical pneumatization associated with habitual valsalva maneuver in a 70-year-old man.A 70-year old man with a 15-year-history of chronic daily Valsalva maneuvers for left ear congestion presented with worsening vertigo and calvarial (occipitoparietal) and upper cervical hyperpneumatization. With continued frequent Valsalva maneuvers, subsequent studies demonstrated increased pneumatization with extension of air into the epidural space, causing mass effect on the left parietal lobe. Four months after discontinuing the habitual Valsalva maneuvers, CT demonstrated resorption of the epidural air and partial regression of the calvarial pneumatization.- - - - - - - - - - ranking = 0.5keywords = vertigo (Clic here for more details about this article) |
8/14. Pauci-symptomatic large epidermoid cyst of cerebellopontine angle: case report.It is estimated that 10% of intra-cranial tumours are localized in the cerebellopontine angle and internal auditory canal and early symptoms of the different histological forms are almost identical. Acoustic neuroma account for 90% and meningioma for 5-10% of these tumours, while a small percentage of rare tumours exist, the most frequent being epidermoid cyst, also known as congenital cholesteatoma or keratoma. The case is reported here of a large epidermoid cyst of the right cerebellopontine angle, and the clinical-radiological course is reviewed. The patient, a 35-year-old, male, initially presented an episode of objective rotatory vertigo, and a history of right ear fullness, of a few months' duration, with normal otoscopy. Audiometric test was normal in left ear, while slight pantonal sensori-neural hypoacusia was observed in the right ear. The impedenzometric findings were normal as was the vestibular test. Auditory brainstem evoked response showed an increased latency of fifth wave. gadolinium-enhanced magnetic resonance imaging of brain revealed the presence of a voluminous epidermoid cyst occupying the extra-axial side of the right cerebellopontine cistern with superior extension into the cistern. Due to the low-grade of symptoms, we had chosen to wait and not perform surgery immediately, with otologic and vestibular test-controls every 6 months, with cerebral magnetic resonance imaging to control extension of the mass, without radiation exposure for the patient. One year after diagnosis, at the last control, otofunctional findings were not modified and repeat magnetic resonance imaging did not demonstrate important variations compared to the first. Thus, the choice not to proceed with surgery was justified since surgery is burdened by the risk of important complications. At magnetic resonance imaging, the epidermoid cyst, unlike the majority of intra-cranial tumours, such as acoustic neuroma and meningioma, does not show gadolinium-enhancement; this again supporting the important role of magnetic resonance imaging in the differential diagnosis of intra-cranial neoformations. It is, therefore, worthwhile stressing the validity of the approach, step by step, in the diagnosis of patients with otologic symptoms, together with the importance of magnetic resonance imaging that, in comparison with computed tomography, allowed us to exactly assess the growth rate of the mass and to "wait and see" without risks and without radiations for the patient.- - - - - - - - - - ranking = 0.5keywords = vertigo (Clic here for more details about this article) |
9/14. temporal bone histopathology in dominantly inherited audiovestibular syndrome.OBJECTIVE: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. methods: history, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. RESULTS: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. CONCLUSIONS: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging.- - - - - - - - - - ranking = 0.5keywords = vertigo (Clic here for more details about this article) |
10/14. Ankylosing spondylitis and multiple sclerosis in an HLA-B27 negative patient.A 41-year-old man presented with vertigo and gait disturbance. He gave a 10-year history of definite ankylosing spondylitis with low back pain, limitation of spinal mobility, decreased chest expansion and radiological evidence of bilateral sacroiliitis. The vertigo attacks started 3 years before and he had insidious evolution of bilateral leg weakness, increased muscle tension and walking disability during the past 2 years. The HLA haplotypes of the patient were A2, A33, B14, B49, Bw4, Bw6, Cw7 and he was HLA-B27 negative. The axial and sagittal cranial magnetic resonance imaging (MRI) showed multiple foci of increased signal intensity in the periventricular white matter and cerebellar hemispheres, suggesting a demyelinating disease process. The MRI of the spine showed centromedullar high intensity lesions at C7, Th7-8, Th9-10 levels. The diagnosis was definite MS (primary progressive MS) as the patient had insidious neurological progression, CSF evidence of inthrathecal production of oligoclonal bands, conduction defects at VEP, multiple brain and additional spinal cord lesions on MRI and continued progression for more than 1 year.- - - - - - - - - - ranking = 1keywords = vertigo (Clic here for more details about this article) |
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