Meniere's Syndrome Research Articles

Ménière's Syndrome, Otosclerosis, and Insulin Resistance Syndrome

Ménière's Syndrome, Otosclerosis, and Insulin Resistance Syndrome

Psychiatric comorbidity in patients with bilateral vestibulopathy

Objectives: Patients with vertigo exhibit a high coincidence of organic vestibular disorders and psychiatric disorders. This is especially true for patients with Meniere's syndrome and migraine, but not for patients with vestibular neuritis [1]. The aim of this study was to determine if patients with bilateral vestibulopathy (BVP) suffer from somatoform or psychiatric distress and comorbidity.

Drop attacks in elderly patients secondary to otologic causes with Meniere's syndrome or non-Meniere peripheral vestibulopathy

Many neurologists are unaware of the drop attack that may occur from an inner ear dysfunction especially in elderly. We studied the clinical features and results of quantitative audiovestibular tests in six elderly patients (≥65 years of age) who presented with drop attacks attributable to an inner ear pathology. Group was divided into Meniere's syndrome (4) or non-Meniere peripheral vestibulopathy (2). Standard dizziness questionnaire and quantitative audiovestibular function testing were performed. Episodes were described as a sudden push to the ground in four or a violent illusionary movement of environment leading to a fall in two. All cases gave a history of prior vertiginous episodes and vestibular testing revealed unilateral caloric paresis. Ipsilateral hearing loss was documented in four cases. Our results suggest that otologic causes should be considered in the differential diagnosis of the drop attack in elderly, even if the symptoms and signs were not consistent with Meniere's syndrome.

Pathophysiology of M??ni??re's Syndrome: Are Symptoms Caused by Endolymphatic Hydrops?

The association of Meniere's syndrome with endolymphatic hydrops has led to the formation of a central hypothesis: many possible etiologic factors lead to hydrops, and hydrops in turn generates the symptoms. However, this hypothesis of hydrops as being the final common pathway has not been proven conclusively. To examine human temporal bones with respect to the role of hydrops in causing symptoms in Meniere's syndrome. If the central hypothesis were true, every case of Meniere's syndrome should have hydrops and every case of hydrops should show the typical symptoms. Review of archival temporal bone cases with a clinical diagnosis of Meniere's syndrome (28 cases) or a histopathologic diagnosis of hydrops (79 cases). All 28 cases with classical symptoms of Meniere's syndrome showed hydrops in at least one ear. However, the reverse was not true. There were 9 cases with idiopathic hydrops and 10 cases with secondary hydrops, but the patients did not exhibit the classic symptoms of Meniere's syndrome. A review of the literature revealed cases with asymptomatic hydrops (similar to the current study), as well as cases where symptoms of Meniere's syndrome existed during life but no hydrops was observed on histology. We also review recent experimental data where obstruction of the endolymphatic duct in guinea pigs resulted in cytochemical abnormalities within fibrocytes of the spiral ligament before development of hydrops. This result is consistent with the hypothesis that hydrops resulted from disordered fluid homeostasis caused by disruption of regulatory elements within the spiral ligament. Endolymphatic hydrops should be considered as a histologic marker for Meniere's syndrome rather than being directly responsible for its symptoms.

Diagnosis and management of post-traumatic vertigo.

Patients with post-traumatic vertigo can be difficult to treat secondary to the chronicity of their symptoms. Patients can have peripheral, central, and combined vestibular deficits. Furthermore, no comprehensive guidelines exist for returning these patients to work. The objectives of the study were to discuss diagnosis, management techniques, and guidelines for returning these patients to work. Retrospective analysis of a tertiary referral neurotology and balance clinic. Between July 1997 and July 2003, 2390 patients with chief complaints of vertigo and/or dizziness were analyzed. Of these, 16 patients met the requirements for inclusion in the study, including head trauma and/or concussion and residual vertigo. Their inpatient and outpatient charts, imaging studies, audiograms, vestibular tests, and physical therapy evaluations were reviewed. All patients had at least 6 months of follow-up. There were 5 women and 11 men, with an average age of 42 years. Five patients had symptoms consistent with traumatic perilymphatic fistulas, and two patients had symptoms consistent with post-traumatic Meniere's syndrome. Surgical therapy was not beneficial in relieving dysequilibrium. Balance testing results did not predict return-to-work status. Eleven patients were not allowed to return to work in any capacity, two patients were allowed to return to work with limited duties, and three patients were allowed to return to work with no restrictions. Post-traumatic vertigo can result in chronic symptoms. Balance testing did not predict the ability of patients to return to work. Surgical intervention might not control patient symptoms. Many patients were unable to return to work.

Selective microsurgical vestibular neurectomy: an option in the treatment of intractable vertigo and related microsurgical landmarks.

Selective microsurgical vestibular neurectomy (SMVN) is an accepted and effective means of treating patients with intractable vertigo, a resistant component of Meniere's syndrome. Meniere's syndrome is a condition characterized by fluctuating hearing loss, tinnitus and serious vertigo attacks. Vertigo is the most disabling symptom of the disease. There may be permanent hearing loss in untreated cases and vertigo may continue after deafness. Selective microsurgical vestibular neurectomy is an elite surgical procedure providing exact relief from vertigo while protecting the preoperative hearing level. In this report, clinical results of nine patients with intractable vertigo operated in the University of Ankara Medical Faculty, Department of Neurosurgery between 1999 and 2001 are discussed. Accordingly, we aimed to represent the landmarks often required for microsurgical exposure. From this study we conclude that SMVN is an effective neurosurgical procedure for those patients who are resistant for medical treatment and require hearing preservation.

Occurrence of familial Meniere's syndrome and migraine in Brasília.

Since 1941, many families with several members affected by Meniere's syndrome have been described. "Migraine-like" headache was always mentioned. In 1997, we described a large family with Meniere's syndrome and migraine, both transmitted in an autosomal dominant mode. Recently, a gene that could be responsible for Meniere's symptoms has been identified. However, the incidence of familial Meniere's syndrome is still unknown. A. W. Morrison et al estimated this incidence as between 2.9% and 12%. During the years 1997 and 1998, we saw 8 new patients with typical Meniere's syndrome in our outpatient department. They were referred to Dr Messias, who did a careful search for other affected family members. Six of them had relatives with Meniere's syndrome and migraine transmitted in an autosomal dominant mode. The familial incidence of Meniere's syndrome and migraine in Brasília seems to be higher than the figures of Morrison et al for the United Kingdom. The 6 families are described herein.

Vestibular histopathology of the human temporal bone. What can we learn?

Histologic sections of the human temporal bone display snapshots of the entire lifetime integrated into the moment the bone enters fixative. The bulk of the literature on vestibular histopathology is anecdotal and descriptive in nature, rather than quantitative. This is because the means of describing and measuring patients' vestibular symptoms are poorly developed, and the complex geometry of the vestibular labyrinth complicates efforts to study it in serial histologic sections. Histopathologic findings in the common peripheral vestibulopathies, including Meniere's syndrome, benign paroxysmal positional vertigo, viral labyrinthitis, vestibular neuronitis, and ototoxicity, have all been described. A new quantitative method for assessment of vestibular otopathology using Nomarski optics has recently been reported. It has been successfully applied to create a normative database of age-related changes in the vestibular hair cell populations which, in turn, has been used to study the effects of aminoglycoside ototoxicity and Meniere's syndrome. These data provide the first meaningful opportunity to make structure-function correlations between vestibular function testing and temporal bone pathology in humans. Wider clinical application of vestibular function testing and postmortem temporal bone donation should be promoted by all investigators interested in accumulating the resources necessary to gain a deeper understanding of the human vestibular system in health and disease.

Perilymphatic fistula and Meniere's disease. Clinical series and literature review.

There continues to be considerable confusion concerning the diagnosis and treatment of perilymphatic fistulas (PLFs) and Meniere's disease. This paper reports an 8-year experience with patients whose symptoms were compatible with Meniere's disease but who had some other element that raised the possibility of their having a PLF. This review is a retrospective report on 64 patients who had fluctuating hearing loss, vertigo, tinnitus, and aural fullness typical of Meniere's disease. However, they also had a positive fistula test and/or symptoms beginning immediately after head trauma. They all underwent at least a unilateral PLF repair, and some also underwent an endolymphatic sac-mastoid shunt operation. This report provides a literature review to help put this series' results into perspective. Of those 40 patients who underwent an initial PLF repair, 58% had a successful outcome. Of the 10 patients who underwent an initial PLF repair plus an endolymphatic sac-mastoid shunt, 70% had a successful outcome. Since it is often difficult to distinguish patients with PLFs and secondary endolymphatic hydrops (Meniere's syndrome) from patients with Meniere's disease (idiopathic endolymphatic hydrops), PLF repair is a reasonable first operation, reserving an endolymphatic sac-mastoid shunt operation as a secondary procedure if needed. The decision to consider a PLF repair is guided by a positive fistula test or an immediate onset of Meniere's disease symptom complex after head or ear trauma.

“Hyperacusis” and Origins of Lowered Sound Tolerance

“Hyperacusis” and Origins of Lowered Sound Tolerance

遅発性内リンパ水腫について 特にメニエール病との関連

Delayed endolymphatic hydrops (DEH), a disease entity that can be differentiated from Meniere's disease, typically develops in patients who have experienced a profound, long-term hearing loss in one ear. This condition was first reported by Kamei et al. ('71), who indicated that it occurred in about 20% of patients who had had unilateral profound deafness since early childhood (UPDC), and it appeared at various stages of adulthood, during or after puberty. Wolfson and Leiberman ('75) and Nadol et al. ('75) reported other types of unilateral profound deafness causing this condition that were due to viral and bacterial labyrinthitis, head trauma, or sudden deafness. Schuknecht ('78) defined DEH and classified it into two types: the ipsilateral type, in which vestibular symptoms identical to the vestibular symptoms of Meniere's disease develop in a previously deafened ear, and the contralateral type, in which a fluctuating hearing loss and/or vestibular symptoms develop in the hearing ear. The underlying pathophysiologic mechanism for the development of DEH has been explained as progressive endolymphatic hydrops in the inner ear due to delayed atrophy or fibrous obliteration of the endolymphatic resorption system, resulting from a previous inner-ear injury. In the ipsilateral type, episodic vertigo is not usually accompanied by fluctuating hearing levels and tinnitus because the hearing loss is profound. The period between the onset of pre-existent deafness in one ear and the onset of DEH ranges from several months to 74 years. The cause of the pre-existent deafness is UPDC in more than half of the cases of both types of DEH. Almost all kinds of other degenerative disorders of the inner ear are mostly due to inflammation (viral and bacterial) and trauma (physical and acoustic). The ipsilateral type of DEH is more frequently seen than the contralateral type. The onset age of the contralateral type is higher, in general, than that of the ipsilateral type. Medical treatment may be effective for both types of DEH. Complete relief from episodic recurrent vertigo may be expected in 65% of cases within 5 years after the onset of vertigo and in 90% of cases within 10 years. Labyrinthectomy or vestibular nerve section in the deaf ear is curative in the ipsilateral type, but no satisfactory surgical therapy is available for the contralateral type of the disease. The audiological definition of DEH by Schuknecht seems, however, to be somewhat arbitrary, as the patients' symptoms form a continuous spectrum with other Meniere's syndrome cases, occurring in association with less-marked degrees of sensorineural hearing loss. The existence of the contralateral type of DEH due to UPDC, which often shows typical symptoms of Meniere's disease, seems to suggest that Meniere's disease may occur as a delayed sequela of subclinical damage of the inner ear, especially damage sustained in childhood.

Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients.

Chiari malformations are regarded as a pathological continuum of hindbrain maldevelopments characterized by downward herniation of the cerebellar tonsils. The Chiari I malformation (CMI) is defined as tonsillar herniation of at least 3 to 5 mm below the foramen magnum. Increased detection of CMI has emphasized the need for more information regarding the clinical features of the disorder. We examined a prospective cohort of 364 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine, and some were evaluated using CINE-magnetic resonance imaging and other neurodiagnostic tests. For 50 patients and 50 age- and gender-matched control subjects, the volume of the posterior cranial fossa was calculated by the Cavalieri method. The families of 21 patients participated in a study of familial aggregation. There were 275 female and 89 male patients. The age of onset was 24.9+/-15.8 years (mean +/- standard deviation), and 89 patients (24%) cited trauma as the precipitating event. Common associated problems included syringomyelia (65%), scoliosis (42%), and basilar invagination (12%). Forty-three patients (12%) reported positive family histories of CMI or syringomyelia. Pedigrees for 21 families showed patterns consistent with autosomal dominant or recessive inheritance. The clinical syndrome of CMI was found to consist of the following: 1) headaches, 2) pseudotumor-like episodes, 3) a Meniere's disease-like syndrome, 4) lower cranial nerve signs, and 5) spinal cord disturbances in the absence of syringomyelia. The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (364 patients), tonsillar herniation of at least 5 mm (332 patients), and varying degrees of cranial base dysplasia. Volumetric calculations for the posterior cranial fossa revealed a significant reduction of total volume (mean, 13.4 ml) and a 40% reduction of cerebrospinal fluid volume (mean, 10.8 ml), with normal brain volume. These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterized by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct compression of nervous tissue. The demonstration of familial aggregation suggests a genetic component of transmission.

Variable presentations in meniere's syndrome.

Variable presentations in meniere's syndrome.

Sir Charles Ballance: pioneer British neurological surgeon.

Sir Charles A. Ballance (1856-1936) began his medical career at St. Thomas's Hospital the University College, London, England, in 1875, receiving honors in every subject and a gold medal in surgery. Victor Horsley (1857-1916) and Ballance were classmates at the University and in the later 1880s began work together at the Brown Institute and the National Hospital, Queen Square. In addition to important studies on vascular surgery, Ballance was involved in primate work on cerebral localization with lifelong friends Charles Beevor, Charles Sherrington, David Ferrier, and others. In June of 1887, Ballance assisted Horsley at Queen Square in the successful removal of an extramedullary spinal cord tumor. Horsley was about to abandon the operation, but his friend urged the removal of one lamina higher and the tumor was discovered. Ballance, a demonstrator in anatomy, realized the spinal cord segments lay higher in relation to the vertebral bodies than was generally appreciated. Ballance popularized the operation of radical mastoidectomy for advanced middle ear infection (1890), standardized an approach to drain or excise temporal brain abscesses, and was the first to clearly understand the neurological signs of cerebellar abscess (1894). Ballance also devised cranial base approaches to attack infectious thrombophlebitis of the lateral, petrosal, and cavernous sinuses. He was the first to completely remove an acoustic tumor (1894); 18 years later, the patient remained well. Ballance also drained a posterior fossa subdural hematoma (1906) and successfully sectioned the auditory nerve for Meniere's syndrome (1908). Ballance's operative experience with both supra- and infratentorial brain lesions included approximately 400 cases, which are detailed in his 1907 book, Some Points in the Surgery of the Brain and Its Membranes. His two-volume set, Essays on the Surgery of the Temporal Bone (1919), remains a brilliantly written and illustrated classic. Ballance was an expert on nerve regeneration and nerve grafting, and after many years of devoted research, he devised successful operations for facial nerve paralysis. For this and early vascular work, he is often credited as the first English surgeon to reintroduce classical Hunterian methods of experiment into surgery. He was honored as the founder and President of The Society of British Neurological Surgeons in 1926. Perhaps best known as a general and aural surgeon, Ballance was second only to Horsley in reputation as a pioneer British neurological surgeon. Described as a painstakingly slow but delicate and meticulous operator, Ballance made a contribution to neurosurgery and temporal bone surgery that was immense.

Meniere's Syndrome.

The primary goals of treatment in Meniere's syndrome are to control attacks of vertigo and, if possible, to arrest the progression of hearing loss. Initial treatment consists of rigid dietary sodium restriction, possibly with the addition of a thiazide diuretic. If disabling attacks of vertigo persist despite sodium restriction, surgical intervention should be considered. Endolymphatic shunting is an option for patients who desire a less invasive surgical remedy, but recurrence of vertigo occurs in about 40% of cases. Vestibular nerve ablative surgery is the definitive surgical treatment for patients with intractable attacks of vertigo who still have useful hearing on the affected side. Labyrinthectomy is a surgical option when there is no serviceable hearing in the damaged ear. Intra-aural gentamicin instillation is an option in carefully selected cases.

Transtympanic gentamicin for Meniere's syndrome.

Study the impact of transtympanic gentamicin on patients with unilateral Meniere's syndrome. Partial chemical labyrinthectomy is a relatively recent concept for the treatment of Meniere's syndrome. It uses the ototoxic effect of gentamicin to reduce the symptom of vertigo and maintain cochlear function. A prospective study using transtympanic gentamicin was begun in January 1994. Patients selected had failed medical therapy, but were not incapacitated. Patients had preinjection audiometric and electronystagmography data. Most had an imaging study. All had one injection, about half had more than one. Patients were seen 1 month after therapy and repeat studies were obtained. Repeat injection was performed if indicated. Follow-up from the chart or by telephone was obtained. Data were tabulated using the 1995 American Academy of Otologaryngology-Head and Neck Surgery guidelines. Through December 1996 43 patients with unilateral Meniere's syndrome were treated. The pretherapy function level was 3 through 5. After therapy the function level was 1 or 2. There was almost no change in cochlear function and no patient became deaf. Many patients had mild ataxia or dysequilibrium during the first 2 weeks following therapy. Most patients showed some decrease in labyrinthine function measured on electronystagmography. No attempt was made to ablate labyrinthine function. Seventeen of 18 patients had a vertigo index in the class A or B category after 2 years. Transtympanic gentamicin has become the treatment of choice for patients who fail medical therapy for Meniere's syndrome at the authors' institution.

Use of a Screening RAST in a Large Neuro‐Otologic Practice

Evidence in the literature emphasizes the role of the immune system in disorders of the inner ear and eustachian tube. We initially investigated the presence of inhalant allergy in selected patients seen for otologic problems by means of a screening radioallergosorbent test (RAST), using either a microscreen or a limited antigen panel. This study analyzed the results of tests performed over a 2-year period on 186 patients seen by one of us (WLM) for treatment of vertigo (66%), tinnitus (63%), hearing loss (49%), aural fullness (48%), Meniere's quadrad (27%), balance disturbance other than true vertigo (21%), and eustachian tube dysfunction (4%). We found an incidence of immunoglobulin E-mediated hypersensitivity of nearly 40% in a patient population selected solely for neuro-otologic symptoms and not for sinonasal symptoms. This figure is more than double that quoted for the general population. We also found a surprisingly high incidence of mold antigen atopy in this selected population. Allergy can contribute to a number of otologic symptoms, including eustachian tube dysfunction, vertigo, tinnitus, hearing loss, aural fullness, and nonspecific balance disturbance. Allergy also has been emphasized as an etiologic factor in a portion of patients diagnosed with Meniere's syndrome. A screening RAST, combined with clinical evaluation, appears to be an excellent tool for evaluating these patients for inhalant allergy as part of a comprehensive workup.

Vestibular nerve section: long-term follow-up.

Vestibular nerve section is considered an effective modality in the treatment of refractory and incapacitating vertigo. Typically nerve section results are described on the basis of short-term follow-up. We have reviewed 41 cases of vestibular nerve section spanning an 18-year period. Although the majority of cases involved classic Meniere's disease, delayed endolymphatic hydrops, vestibular neuritis, and Meniere's syndrome secondary to head trauma were also included. Surgical approaches included translabyrinthine (20 cases), retrolabyrinthine (14 cases), retrosigmoid (six cases), and middle fossa (one case) procedures. Postoperative follow-up time averaged 102 months, with 46% of patients followed for a minimum of 9 years. Results are reported according to standards set forth by the American Academy of Otolaryngology--Head and Neck Surgery. Vertigo was cured or markedly improved in 88% of cases (90% in patients with Meniere's disease) at 18 to 24 months postoperatively. These vertigo results were sustained at the time of latest follow-up. Functional level was also preserved over time despite the development of bilateral symptoms in several cases. The rate of bilateral disease reached 22% of cases. Although vertigo results remained stable, long-term follow-up of successful hearing preservation cases demonstrated deterioration over time. Postoperative continuation of medical treatment is urged to optimize and sustain the vestibular neurectomy result.

Etidronate for the Neurotologic Symptoms of Otosclerosis: Preliminary Study

The efficacy of etidronate, a bisphosphonate, was assessed as a treatment for the inner ear symptoms of otosclerosis in a retrospective case review of 896 patients diagnosed with otosclerosis, with primary complaints of dizziness, hearing loss, tinnitus or Meniere's syndrome. The diagnosis of otosclerosis was based on small-pixel computed tomography of the temporal bones. Of the 896 patients placed on an etidronate protocol, 545 were followed for more than six months and were analyzed. The symptomatic response to etidronate, as well as audiologic and computerized rotary chair results were used in the assessment. Patients who were previously on sodium fluoride were separately analyzed. In this preliminary study etidronate appeared to be an effective treatment for the neurotologic symptoms of otosclerosis. Prospective blinded efficacy studies of the bisphosphonates in the treatment of otosclerosis should be undertaken.

A case-control study of prosthetic implants and selected chronic diseases

We examined the association between prosthetic nonbreast implants and selected malignant neoplasms, connective tissue disorders, and neurologic diseases. We conducted a case-control study from an insurance claims database. We selected controls who had diseases for which no association with implants have been claimed or reported. Data were analyzed using both tabular and polytomous regression analysis methods, including methods to account for the large number of comparisons. All analyses exhibited positive associations between implants (both silicone and metal) and neurologic conditions, especially idiopathic progressive neuropathy and Meniere syndrome, as well as the expected associations with arthritic conditions. There also was an unexpected negative association between metal implants and brain tumors. In conclusion, further studies of prosthetic implants and neurologic diseases appear warranted. These studies should obtain medical histories to control for possible confounding effects of drug treatments associated with implant surgery.