1/19. Relationship between cochleovestibular disorders in hemifacial spasm and neurovascular compression.OBJECTIVE: To investigate the evolution of cochleovestibular symptoms before, during, and after microvascular decompression (MVD) of the facial nerve in hemifacial spasm. STUDY DESIGN: Prospective study in patients with hemifacial spasm. Among our 13 patients who underwent MVD of the facial nerve from 1995 to 1997, 6 had associated cochleovestibular disorders confirmed by neurotologic tests. RESULTS: In four of these patients, a concomitant compression of the eighth and facial nerves was found at surgery. Preoperative magnetic resonance angiography studies had shown three cases of this double neurovascular compression. Intraoperative auditory brainstem response monitoring showed that interposition of Teflon between vessel and facial nerve was highly critical to the auditory function. Auditory brainstem response monitoring was used to guide the surgeon during this critical phase. Surgery improved at least one cochleovestibular symptom in each patient. CONCLUSIONS: The authors propose two pathophysiologic hypotheses. First, the concomitant facial and cochleo-vestibular symptoms may be due to a hyperactivity of both the facial and vestibular nuclei. According to theories about cryptogenic hemifacial spasm, the origin of this hyperactivity could be an ectopic excitation focus. However, the two nerves may have different sites of ectopic excitation. According to the second hypothesis, a pulsatile compression of the facial nerve may be transmitted to the eighth nerve. This could take place even if only the facial nerve is in contact with a vascular loop.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/19. Audiovestibular phenotype associated with a COL11A1 mutation in Marshall syndrome.BACKGROUND: Marshall syndrome is a dominant disorder characterized by craniofacial and skeletal abnormalities, sensorineural hearing loss, myopia, and cataracts, and is associated with splicing mutations in COL11A1. OBJECTIVE: To determine the auditory and vestibular phenotypes associated with a COL11A1 splicing. DESIGN: Clinical otolaryngologic, audiologic, vestibular, and radiologic evaluations of the auditory and vestibular systems. SUBJECTS: Three affected individuals from a family cosegregating Marshall syndrome and a COL11A1 splice site mutation. RESULTS: The study subjects have progressive sensorineural hearing loss that is predominantly cochlear in origin and asymptomatic dysfunction of the central and peripheral vestibular systems. Computed tomography detected no malformations of temporal bone structures. CONCLUSIONS: The observed auditory and vestibular abnormalities are not caused by defective morphogenesis of the osseous labyrinth, but by more direct effects of the COL11A1 mutation on the membranous labyrinth and the central nervous system. The onset and degree of hearing loss associated with COL11A1 mutations are useful clinical features to differentiate Marshall syndrome from the phenotypically similar Stickler syndrome.- - - - - - - - - - ranking = 1.9823990800851keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/19. The relationship between Usher's syndrome and psychosis with capgras syndrome.Usher's syndrome is a genetic disorder that causes congenital sensorineural hearing loss, visual impairment due to progressive pigmentary retinopathy, and, often, vestibular dysfunction. The aim of this article is to illustrate a case that clearly demonstrates psychotic symptoms in Usher's syndrome Type III and serves to increase clinical awareness of this disorder and its possible link to psychotic symptoms. There is some evidence in the literature of concurrent psychiatric symptoms, particularly psychotic symptoms, associated with Usher's syndrome, and several theories around this association have been proposed. These theories of associations include a genetic link between the genes responsible for schizophrenia and the genes for Usher's syndrome; a neuropathological explanation as radiologic studies have revealed that patients with Usher's syndrome have CNS abnormalities in multiple brain structures; and a sensory deficit model which proposes that the stressors associated with sensory impairment and the brain's adaptation to changes in sensory inputs place an individual at increased risk for psychopathology.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/19. Inferior vestibular neuritis.Sudden, spontaneous, unilateral loss of vestibular function without simultaneous hearing loss or brain stem signs is generally attributed to a viral infection involving the vestibular nerve and is called acute vestibular neuritis. The clinical hallmarks of acute vestibular neuritis are vertigo, spontaneous nystagmus, and unilateral loss of lateral semicircular function as shown by impulsive and caloric testing. In some patients with vestibular neuritis the process appears to involve only anterior and lateral semicircular function, and these patients are considered to have selective superior vestibular neuritis. Here we report on two patients with acute vertigo, normal lateral semicircular canal function as shown by both impulsive and caloric testing, but selective loss of posterior semicircular canal function as shown by impulsive testing and of saccular function as shown by vestibular evoked myogenic potential testing. We suggest that these patients had selective inferior vestibular neuritis and that contrary to conventional teaching, in a patient with acute spontaneous vertigo, unilateral loss of lateral semicircular canal function is not essential for a diagnosis of acute vestibular neuritis.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
5/19. Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system.OBJECTIVE: To describe cochleovestibular aspects of superficial hemosiderosis of the central nervous system. BACKGROUND: Superficial hemosiderosis of the central nervous system is a rare disease in which cochleovestibular impairment, cerebellar ataxia, and myelopathy are the most frequent signs. Chronic recurrent subarachnoidal hemorrhage with bleeding into the cerebrospinal fluid is the cause of deposition of hemosiderin in leptomeningeal and subpial tissue, cranial nerves, and spinal cord. Removing the cause of bleeding can prevent irreversible damage to these structures. Because this is the only effective treatment, an early diagnosis is crucial. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENT: A 72-year-old woman with superficial hemosiderosis of the central nervous system that developed when she was age 39. methods: Neurologic and imaging diagnostic examinations and longitudinal evaluation of cochleovestibular features were performed. neurosurgery was not performed. RESULTS: Progressive bilateral sensorineural hearing loss and severe vestibular hyporeflexia developed within 15 years, which can be attributed to lesions in the cochleovestibular system. Additional pathology of the central nervous system developed later. CONCLUSION: The patient demonstrated cochlear and vestibular findings that are typical of this pathologic condition. It is the first documented case with extensive serial audiometry used to precisely outline the degree of hearing deterioration during the course of the disease.- - - - - - - - - - ranking = 14.362030338143keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/19. A case of Cronkhite-canada syndrome with vestibular disturbances.A 66-year-old Japanese man with Cronkhite-canada syndrome (CCS) presented with complaints of long-lasting dysequilibrium. On neuro-otological examination, he showed gaze-evoked nystagmus at the rightward and leftward gaze, and saccadic pursuit. On the caloric test, he showed no response in either side, and on the head-impulse test he showed bilateral loss of vestibule-ocular reflexes around the yaw axis, while he had bilateral normal responses on the vestibular-evoked myogenic potential testing. Neuro-otological findings suggested that he had lesions in the peripheral vestibular system as well as the central nervous system. Neurological disorders such as sensory neuropathy have been reported in patients with CCS. This patient's balance problems could be due to CCS itself.- - - - - - - - - - ranking = 1.9823990800851keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/19. Clinical significance of type 5 patterns in platform posturography.Several abnormal patterns have been identified on the sensory portion of the computerized dynamic posturography test. The vestibular deficit pattern, also known as the "5-6" pattern, is frequently seen in patients with either uncompensated unilateral vestibular lesions, severe bilateral peripheral vestibular loss, or dysfunction involving the vestibular pathways in the brain stem and/or cerebellum. In both sensory conditions 5 and 6, the patient's balance/equilibrium is determined primarily by the vestibular system. A subgroup of the vestibular deficit pattern has been identified, in which only sensory condition 5 is abnormal. This article presents findings in several cases identified with the 5 pattern. Implications for diagnosis and for monitoring the recovery phase after treatment are discussed.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
8/19. Pendular pseudonystagmus arising as a combination of head tremor and vestibular failure.We describe three patients with spontaneous pendular oscillation of the eye during funduscopy. All patients had blurred, shimmering vision or oscillopsia, exacerbated by concentration, reading, or trivial head movements, and had a history of unsteadiness. Examination revealed a fine head tremor, mild unsteadiness, absent vestibulo-ocular reflex (VOR), and otherwise normal neurologic and ocular motor findings. Rigid immobilization of the head abolished the retinal oscillations. Simultaneous precision recordings of head and eye movements showed that the eye movement was in the compensatory direction to the head tremor but that, in contrast to normal VOR, it was in phase error. We conclude that the essential head tremor was provoking oscillopsia and retinal oscillation because of the absence of VOR. Recognizing the association of head tremor with absent VOR is important since in all these patients the presence of this pendular pseudonystagmus on ophthalmoscopy raised the diagnostic possibility of brainstem disease.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
9/19. Aseptic meningoencephalitis presenting with bilateral vestibular ataxia: a case report.Bilateral vestibular dysfunction is a rare condition, of which peripheral disorders are most common, whereas central disorders are extremely rare. A 35-year-old woman developed fever, headache, dizziness, convulsion, and disturbance of consciousness at the same time. MRI findings were normal. Based on the neurological findings and laboratory abnormalities, she was diagnosed as having aseptic meningoencephalitis. Steroid pulse therapy successfully ameliorated her encephalitis sign. However, isolated bilateral vestibular ataxia and dizziness, together with severe decrease of Caloric nystagmus, became apparent and lasted for the following 3 months, without cerebellar/brainstem involvement.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
10/19. cochlear implantation in superficial siderosis.Superficial siderosis (SS) of the central nervous system has been thought to be a rare condition that generates progressive hearing loss, ataxia, pyramidal signs, and dementia. The main cause of hearing loss by SS is thought to be neuronal. Because there is no histopathologic report of the human temporal bone in SS, there is a debate about the possibility of cochlear involvement. We present a 25-year-old man who was investigated for bilateral progressive sensorineural hearing loss and vestibular failure after head trauma. On brain MRI, SS of the central nervous system was detected. Distortion product otoacoustic emission (DPOAE) and auditory brainstem response (ABR) showed no response on both sides. However, integrity of the eighth nerve was proved by the electrical ABR test on the right side and the patient benefited significantly from cochlear implantation. The sensorineural hearing loss in SS seems to be related to cochlear damage as well as neuronal damage. So, cochlear implantation would be a hearing rehabilitation modality for the sensorineural hearing loss caused by SS.- - - - - - - - - - ranking = 4.9647981601703keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
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