Meniere Disease
Endolymphatic Duct
Endolymphatic Hydrops
Endolymphatic Sac
Vestibular Evoked Myogenic Potentials
Ear, Inner
Click evoked myogenic potentials in the differential diagnosis of acute vertigo. (1/127)
OBJECTIVE: In response to loud clicks, a vestibular evoked potential can be recorded from sternocleidomastoid muscles, called "click evoked myogenic potential" (CEMP). This paper reports on the usefulness of CEMP in the differential diagnosis of acute vertigo of presumed vestibular origin. METHODS: CEMP was examined in 40 patients with acute vertigo of vestibular origin (26 with acute peripheral vestibulopathy, five with Meniere's disease, three with benign paroxysmal positioning vertigo (BPPV), six with psychogenic vertigo) and the results compared with standard caloric reaction (CR). For CEMPs, clicks were delivered unilaterally via a pair of headphones. EMG activity was collected by surface electrodes placed on the sternocleidomastoid belly and averaged. RESULTS: In 29 patients, CR was unilaterally abnormal, pointing to a peripheral vestibular lesion. Seventeen of them had a corresponding loss of CEMPs; the other 12 patients had a normal CEMP. The remaining 11 patients had normal results in both tests. In comparison with CR, CEMP showed a sensitivity of 59% and a specificity of 100% for peripheral vestibular disorders. CONCLUSION: CR is a test of the horizontal canal whereas CEMP is thought to be a sacculus test. Different results of CR and CEMP may be due to this difference between target organs stimulated and may be of prognostic value. (+info)High prevalence of symptoms of Meniere's disease in three families with a mutation in the COCH gene. (2/127)
We report the genetic analysis of one large Belgian and two small Dutch families with autosomal dominant non-syndromic progressive sensorineural hearing loss associated with vestibular dysfunction. Linkage studies in the Belgian family mapped the disease to the DFNA9 locus on chromosome 14. Mutation analysis of the COCH gene, which is responsible for DFNA9, revealed a missense mutation changing a highly conserved residue. One of the patients, who had an earlier age of onset in comparison with most of the affected family members, was shown to be homozygous for the mutation. After the mutation was found in the Belgian family, we discovered that the same missense mutation was also present in two Dutch families with similar cochleo-vestibular symptoms. In all three families with hearing loss and imbalance problems, >25% of the patients showed additional symptoms, including episodes of vertigo, tinnitus, aural fullness and hearing loss. Clinically, these symptoms are consistent with the criteria for Meniere's disease. The importance of genetic factors in Meniere's disease has been suggested on many occasions, but this study is the first report of a mutation in a gene leading to the symptoms of Meniere's disease in a significant portion of the carriers. The COCH gene may be one of the genetic factors contributing to Meniere's disease and the possibility of a COCH mutation should be considered in patients with Meniere's disease symptoms. (+info)Sound- and pressure-induced vertigo associated with dehiscence of the roof of the superior semicircular canal. (3/127)
In many types of peripheral vertigo, imaging is not part of the initial evaluation. We present a patient with sound- and pressure-induced vertigo associated with bony dehiscence of the roof of the superior semicircular canal. The diagnosis of this new entity can only be made by high-resolution coronal CT imaging of the temporal bones. In patients with this symptom complex, CT should be performed early in the diagnostic workup. (+info)Detection of antibody to sialyl-i, a possible antigen in patients with Meniere's disease. (4/127)
An autoimmune hypothesis for the etiology of Meniere's disease has been proposed. In this study, we focused on gangliosides as potential antigens for autoantibodies in Meniere's disease patients. In an attempt to investigate ganglioside antigens which respond to the serum of patients with Meniere's disease, we analyzed gangliosides of human acoustic neurinomas, and used them as antigens to broadly explore gangliosides that react to serum. All the acoustic neurinoma samples used in the present study showed a similar ganglioside profile on TLC (thin-layer chromatography). For the microscale ganglioside analysis, a newly developed TLC blotting/secondary ion mass spectrometry (SIMS) system together with TLC immunostaining method was employed. Most of the ganglioside bands could be analyzed, and they were identified as GM3, GM2, SPG, GM1a, GD3, S-i (sialyl-i ganglioside) and GD1a. GD1a was the predominant ganglioside and many neolactoseries gangliosides were recognized by immunological analysis. Next, the immune reactivity of serum samples, from patients with Meniere's disease, with the acoustic neurinoma gangliosides was studied by TLC immunostaining. The result showed that five of 11 patients with Meniere's disease and one of eight normal subjects reacted with a specific band, which was identified as S-i by the TLC blotting/SIMS system. The findings of the present study indicate that S-i ganglioside is an autoantigen and possibly involved in the pathogenesis of Meniere's disease. (+info)The cerebral angiographic findings in Cogan syndrome. (5/127)
SUMMARY: Cogan syndrome is an uncommon disorder of unknown etiology characterized by vestibuloauditory dysfunction and nonsyphilitic interstitial keratitis. To our knowledge, the case herein is the first report to demonstrate the cerebral angiographic findings of a patient with this syndrome. (+info)Open trial of methotrexate as treatment for autoimmune hearing loss. (6/127)
OBJECTIVE: To assess the efficacy of low-dose methotrexate (MTX) administered for the treatment of autoimmune hearing loss. METHODS: This was a prospective, 12-month, open-label study of 17 patients with refractory autoimmune hearing loss. All patients had ongoing episodic worsening of hearing in one or both ears prior to enrollment despite traditional medical therapy. The MTX dose was 7.5-25 mg/week. Hearing loss and vertigo were evaluated at baseline and at completion of the study. Hearing improvement was defined as an improvement in pure tone threshold (PT) average of >10 dB or an increase in speech discrimination (SD) of >15%; worsening was defined as a decrease of >10 dB in PT or a decrease of >15% in SD in at least one ear. RESULTS: MTX was well tolerated. Among patients with Meniere's disease, 5 of 9 had improvement or resolution of vertigo. Equilibrium improved in all 3 patients with Cogan's syndrome and improved in 2 out of 3 patients with idiopathic hearing loss and this symptom. According to the parameters defined above, hearing improved in 11 patients (65%), was unchanged in 4 patients (23%), and worsened in 2 patients (12%). CONCLUSION: Long-term low-dose MTX therapy may be a useful therapy for at least some patients who have hearing loss with a presumptively autoimmune-mediated component that is refractory to traditional therapies. (+info)A systematic review of vertigo in primary care. (7/127)
The symptom of vertigo is usually managed in primary care without further referral. This review examines the evidence on which general practitioners can base clinical diagnosis and management of this relatively common complaint. Research in this area has in the main been from secondary and tertiary centres and has been of variable quality. Indications are that the conditions that present in general practice are most likely to be benign positional vertigo, acute vestibular neuronitis, and Meniere's disease; however, vascular incidents and neurological causes, such as multiple sclerosis, must be kept in mind. An important practice point is that vestibular sedatives are not recommended on a prolonged basis for any type of vertigo. There is a need for basic epidemiological and clinical management research of vertigo in general practice. (+info)Prolactinoma in some Meniere's patients--is stress involved? (8/127)
Dizziness is a common complaint in primary care clinics and can enter the diagnostic profile of different pathologies spanning from psychiatric problems to vestibular dysfunction. Episodes of vertigo in Meniere's patients are often reported to be triggered by stress but no physiological data are available to account for the subjective link. The study involved 42 Meniere's patients hospitalized for neurectomy of the vestibular nerve for relief of incapacitating vertigo. In addition 18 patients with neurinoma of the vestibular nerve and 12 patients with facial spasm, who underwent surgery, served as controls. A blood sample was taken on the day of surgery in order to determine the level of battery of different stress hormones. The most striking observation was the presence of hyperprolactinemia (above 20 microg/l) in 14 Meniere's patients. The presence of prolactinoma was confirmed by MRI in six cases out of six investigated and the others have not yet been followed up in this retrospective study. These observations are clearly indicative for systematic determination of prolactin levels before opting for surgery in Meniere's patients. (+info)Menière disease is an inner ear disorder that is characterized by episodes of vertigo (a spinning sensation), tinnitus (ringing or buzzing in the ear), hearing loss, and aural fullness (a feeling of pressure or blockage in the ear). It is caused by an abnormal accumulation of endolymphatic fluid in the inner ear, which can lead to damage of the vestibular system and cochlea. The exact cause of this fluid buildup is not known, but it may be related to genetics, allergies, or autoimmune disorders. Menière disease is typically a chronic condition, with symptoms that can vary in frequency and severity over time. Treatment options include dietary modifications, diuretics, vestibular rehabilitation therapy, and, in some cases, surgery.
The endolymphatic duct is a narrow canal in the inner ear that is part of the membranous labyrinth. It connects the utricle and saccule (two sensory structures in the vestibular system responsible for detecting changes in head position and movement) to the endolymphatic sac (a dilated portion of the duct that helps regulate the volume and pressure of endolymph, a fluid found within the membranous labyrinth).
The endolymphatic duct plays a crucial role in maintaining the balance and homeostasis of the inner ear by allowing the absorption and circulation of endolymph. Disorders or abnormalities in this region can lead to various vestibular and hearing dysfunctions, such as Meniere's disease, endolymphatic hydrops, and other inner ear disorders.
Endolymphatic hydrops is a term used to describe the abnormal accumulation of fluid (endolymph) within the inner ear. This condition is most commonly associated with Meniere's disease, but can also be seen in other disorders that affect the inner ear.
The inner ear is made up of two main parts: the cochlea, which is responsible for hearing, and the vestibular system, which helps to control balance. Both of these systems are filled with fluid, including endolymph, which is a watery fluid that bathes the sensory hair cells in these structures.
In endolymphatic hydrops, there is an overproduction or decreased absorption of endolymph, leading to an abnormal buildup of fluid within the inner ear. This can cause a variety of symptoms, including vertigo (a spinning sensation), tinnitus (ringing in the ears), hearing loss, and a feeling of fullness or pressure in the affected ear.
The exact cause of endolymphatic hydrops is not fully understood, but it is thought to be related to changes in the inner ear's fluid balance. Treatment options may include medications to help control symptoms, as well as surgical procedures to relieve pressure on the inner ear.
The endolymphatic sac is a small, fluid-filled structure that is part of the inner ear. It is located near the vestibular aqueduct and is responsible for maintaining the balance of fluids in the inner ear. The endolymphatic sac also plays a role in the resorption of endolymph, which is the fluid that fills the membranous labyrinth of the inner ear. Disorders of the endolymphatic sac can lead to conditions such as Meniere's disease, which is characterized by vertigo, hearing loss, and tinnitus.
Vestibular Evoked Myogenic Potentials (VEMPs) are short-latency electromyographic responses recorded from the sternocleidomastoid or other neck muscles in response to intense, high-frequency bone conducted vibration or air-conducted sound. They reflect the activation of the vestibular afferents that innervate the otolithic organs (saccule and utricle) in response to linear acceleration and head tilt. VEMPs are used in clinical settings to assess the function of the vestibular system, particularly the sacculocollic reflex pathway, and can help diagnose various vestibular disorders such as superior canal dehiscence syndrome, vestibular neuritis, and Meniere's disease.
The inner ear is the innermost part of the ear that contains the sensory organs for hearing and balance. It consists of a complex system of fluid-filled tubes and sacs called the vestibular system, which is responsible for maintaining balance and spatial orientation, and the cochlea, a spiral-shaped organ that converts sound vibrations into electrical signals that are sent to the brain.
The inner ear is located deep within the temporal bone of the skull and is protected by a bony labyrinth. The vestibular system includes the semicircular canals, which detect rotational movements of the head, and the otolith organs (the saccule and utricle), which detect linear acceleration and gravity.
Damage to the inner ear can result in hearing loss, tinnitus (ringing in the ears), vertigo (a spinning sensation), and balance problems.