Obstructing lesions of the endolymphatic sac and duct mimicking Ménière's disease

In this retrospective case series and literature review, we demonstrate that temporal bone lesions that obstruct the endolymphatic sac or duct can cause symptoms of Ménière's disease. This finding is likely attributable to endolymphatic hydrops; initially, such cases typically masquerade as Ménière's disease. Between July 1995 and April 2002, a total of 379 patients were treated for an initial diagnosis of Ménière's disease at our institution. Among this group, 3 patients were found to have an obstructing lesion of the endolymphatic sac or duct that we felt was causally related to their Ménière's-like symptomatology. We reviewed these cases and noted the similarities in each patient's presentation, including a common pathophysiology. On imaging studies, each patient had a different pathologic lesion that involved the endolymphatic sac or duct: patient 1 had a jugular megabulb, and she was ultimately treated with vestibular nerve section; patient 2 had a cholesterol granuloma, which was treated with surgical excision; patient 3 had an endolymphatic sac tumor that was treated with surgical excision. As has been suggested in previous reports, not all cases of Ménière's disease are idiopathic. We conclude that obstruction of the endolymphatic sac or vestibular aqueduct by a mass lesion or vascular anomaly can lead to vestibulocochlear pathology that mimics Ménière's disease.     

Detection of viral DNA in the endolymphatic sac in Ménière's disease by in situ hybridization

The main purpose of this study is to search for a viral etiology in Ménière's disease by examining the presence or absence of herpes family virus DNA in the endolymphatic sac (ES) using the in situ hybridization method. This was a prospective study with the ES from 10 patients with Ménière's disease and from 7 control cases without any pre-mortem ear diseases except a case of acoustic tumor. These 10 patients underwent the ES surgery. The presence of herpes family virus DNA, such as herpes simplex virus types 1 and 2 (HSV1&2), varicella-zoster virus (VZV), Epstein-Barr virus (EBV) and human cytomegalovirus (CMV), was examined using the in situ hybridization method. Serum antibody titers against these viruses just before the ES surgery were studied in these patients. Of the 10 specimens from the patients with Ménière's disease, 7 were positive for VZV, 4 for EBV, 1 for CMV and none for HSV1&2, although the serum antibody titers against these viruses did not show any significant elevation in these patients just before the ES surgery. This result suggests that the viral DNA in the ES is inactive and is present in a latent form. From the statistical analysis, it can be postulated that VZV infection in early childhood may reach the ES and play a role in the pathogenesis of Ménière's disease (p = 0.0235). The double infection with both VZV and EBV tended to be another candidate for the pathogenesis of Ménière's disease (p = 0.0557).     

Outcome-based assessment of endolymphatic sac decompression for Ménière's disease using the Ménière's disease outcome questionnaire: a review of 90 patients.

OBJECTIVE: To evaluate the quality of life (QOL) of patients treated by endolymphatic sac decompression (ELSD) for Ménière's disease (MD) with a dedicated questionnaire, while recording the results of this surgery on vertigo attacks using the American Academy of Otolaryngology-Head and Neck Surgery criteria. STUDY DESIGN: Retrospective case review in a tertiary referral care center. MATERIAL AND METHODS: Ninety patients who have MD were treated by ELSD at our institution between 1986 and 2004. They were sent a 40-question Ménière's Disease QOL dedicated questionnaire (Ménière's Disease Outcome Questionnaire) concerning physical, mental, and social well-being. Five scores out of 100 were calculated (S1-S5): S3 for preoperative QOL score, S4 for postoperative QOL score, S5 for change in QOL score (S5 = S4 - S3), and S1 and S2 representing the answers to two other paired questions about the "overall" feeling of the patients. In parallel, results of ELSD on vertigo attacks and hearing were evaluated using the Academy of Otolaryngology-Head and Neck Surgery criteria. RESULTS: Fifty-nine interpretable answers (65.6%) to the Ménière's Disease Outcome Questionnaire were obtained from 28 men and 31 women (mean age, 56 yr and 9 mo), all suffering from "definite" MD. The mean S3 was 32.5 (range, 9.7-84.7), and the mean S4 was 60.8 (range, 0-98.6). Consecutively, S5 score was 28.3 (range, -34.7 to 77.4). QOL improved in 81.4% of cases with an average duration of follow-up of 57.5 months (range, 4-156 mo) (p < 0.001), worsened in 11.9% and was stable in 6.8%. There was no statistically significant difference as a function of sex (p = 0.7) or length of follow-up (p = 0.6). There was a significant correlation between S1 and S3 (p < 0.0001), and S2 and S4 (p < 0.0001), validating the whole questionnaire. In addition, 71% of satisfactory control of vertigo at 2 years postoperatively was noted. Hearing was improved or unchanged in 79% of cases at 3 months postoperatively. CONCLUSION: These results show that ELSD significantly improves the health of patients. It represents a first-line procedure that preserves the vestibular and afferent structures, unlike vestibular neurotomy and chemical labyrinthectomy, which may be indicated as a second line.     

Demyelination of vestibular nerve axons in unilateral Ménière's disease

We conducted a study to determine whether vestibular nerves in patients with unilateral Ménière's disease whose symptoms are refractory to medical management exhibit neuropathologic changes. We also endeavored to determine whether retrocochlear abnormalities are primary or secondary factors in the disease process. To these ends, we obtained vestibular nerve segments from five patients during retrosigmoid (posterior fossa) neurectomy, immediately fixed them, and processed them for light and electron microscopy. We found that all five segments exhibited moderate to severe demyelination with axonal sparing. Moreover, we noted that reactive astrocytes produced an extensive proliferation of fibrous processes and that the microglia assumed a phagocytic role. We conclude that the possible etiologies of demyelination include viral and/or immune-mediated factors similar to those seen in other demyelinating diseases, such as multiple sclerosis and Guillain-Barré syndrome. Our findings suggest that some forms of Ménière's disease that are refractory to traditional medical management might be the result of retrocochlear pathology that affects the neuroglial portion of the vestibular nerve.     

Results of serial vestibular testing in unilateral Ménière's disease

OBJECTIVE: To determine the prevalence and character of vestibular abnormalities and the changes in vestibular function that occur in unilateral Ménière's disease. STUDY DESIGN: Retrospective case review. SETTING: Ambulatory patients at a tertiary care facility. PATIENTS: Entry criteria included a diagnosis of unilateral Ménière's disease and test results from at least two vestibular test sessions at the Johns Hopkins Otologic Vestibular Laboratory. One hundred twenty-two cases were evaluated. MAIN OUTCOME MEASURES: Electronystagmographic evaluation, including caloric testing; audiometric tests; and medical records. RESULTS: Caloric weakness was demonstrated in 58% of patients on the involved side and in 19% on the normal side. Complete paralysis was found in 7%. Directional preponderance was seen in 33% of patients and completely normal scores in 27%. During the course of the disease, responses become weaker in 26% of patients and stronger in 11%. Of 39 patients tested more than twice, 26% showed both increases and decreases in caloric responses. After an acute attack, only one of eight patients showed a depressed response on the diseased side, and three showed an increased response. Spontaneous nystagmus, seen within 24 hours of an attack in 54 cases, was directed away from the diseased ear in only about one half of the cases. Benign paroxysmal positional vertigo was found in 44% of these patients. CONCLUSIONS: Possible pathophysiologic explanations for the various test results in Ménière's disease are discussed. Interpretation of caloric test results should take into account the absolute value of the slow phase eye speed scores, in addition to the right-left difference score. Also, established standards for the normal range of fluctuation in both absolute and comparative scores should be used when more than one test session has been undertaken.     

The endolymphatic sac in patients with Ménière's disease: correlation between the MRI and the surgical findings

The endolymphatic sac (ES) is thought to have close relations to pathogenesis of endolymphatic hydrops. Here is reported a retrospective study of 41 patients (42 ears) with Meniere's disease who underwent MRI prior to endolymphatic sac surgery. Based on proton-density weighted image (PDI) and T2-weighted image (T2), the ES including an endolymphatic duct (ED) were estimated whether it was detectable or not. Fourteen ESs were detected on both images (Group A), 14 ESs were detected only on PDI (Group B), and the remaining 14 ESs were not detected on either image (Group D). The actual shape of the sac, obtained from surgical findings, was classified into three (normoplastic, atrophic, invisible). Seventeen ears showed normoplastic ESs and 14 showed atrophic ESs. ES was not detected in 11 ears during surgery, and these findings were compared with image classification. From the study data, normoplastic ESs tend to be observed on both images whereas atrophic or invisible sacs were hardly observed on T2. This classification of ES on MRI was thought to correlate with surgical findings and this correlation was statistically significant (Spearman's rank correlation coefficient. r(s) = 0.58, p < 0.01).     

Ménière's disease and migraine

IntroductionThe possible link between vertigo and migraine has been known for a long time as well as its association with Ménière’s diseaseObjectiveTo determine the lifetime prevalence of migraine in EM patients and to compare it with the prevalence of migraine in general population according to the International Headache Society (IHS) criteriaMethodsWe interviewed 65 EM patients. Our results have been compared with a sex- and age-matched control group with no otological antecedentResultsThe lifetime prevalence of migraine was significantly higher in Ménière group compared to the age- and sex-matched control group ( 35,4% versus 15,4%, p £ 0,05). Nine patients with EM and migraine related an evident relationship between their vertigo episodes and the migraineConclusionsThe results obtained in our study suggest the possibility of common pathophysiological mechanisms to both pathologies and it may lead to new therapeutic options for treatment of EM and migraine patients     

Outcomes in the use of intra-tympanic gentamicin in the treatment of Ménière's disease

The pathogenesis of Ménière's disease is associated with a disorder of ionic homeostasis, with the pathologic correlate being endolymphatic hydrops. Despite uncertainty as to its particular mode of action, it is accepted wisdom that intra-tympanic gentamicin has a definite therapeutic role in the control of symptoms in patients who fail to respond to medical therapy. This study reports an evaluation of the efficacy of intra-tympanic gentamicin in the treatment of Ménière's disease and also presents a simple, reliable, safe method of administering gentamicin for this purpose. A retrospective review of 56 patients undergoing intra-tympanic gentamicin treatment for Ménière's disease was conducted. Response to treatment was analysed using a patient survey and examination of pure-tone averages. An overall significant improvement in vertigo symptoms of 81.3 per cent was found. There was a 21.4 per cent rate of significant hearing loss, defined as greater than 10 dB, with an average loss in this group of 18.5 dB. A single dose of gentamicin applied directly to the round window resulted in a high rate of control of vertigo, with acceptably low rates of hearing loss.     

Relationship of vestibular aqueduct and inner ear pressure in Ménière's disease and the normal population

OBJECTIVE: Etiopathogenesis of Ménière's disease has not been resolved. The principal histopathologic finding in this disease is endolymphatic hydrops. The majority of radiologic and histopathologic studies demonstrated a narrow vestibular aqueduct in Ménière's disease. There is no study in the literature investigating the relationship between inner ear pressure and vestibular aqueduct dimensions. Static acoustic compliance is a noninvasive procedure that is thought to measure perilymphatic pressure at the footplate. An increase in mechanical fluid pressure in the inner ear is transmitted to the footplate of the stapes. This causes a reduction in the compliance at the drum. The aim of this study is to investigate the relationship between vestibular aqueduct dimensions and static acoustic compliance in Ménière's disease and the normal population. STUDY DESIGN: Prospective study. METHODS: Forty patients with Ménière's disease and 40 healthy individuals with no otolaryngologic disorders were the subjects of this study. Each group was further divided into two according to static compliance value (normal and low static compliance). In these four groups dimensions of vestibular aqueduct were determined radiologically by high-resolution computerized tomography and correlated with normal and low static acoustic compliance values (normal and high perilymphatic pressure). RESULTS: The results demonstrated that vestibular aqueduct is narrower in patients with Ménière's disease than the normal population. However, there is no relationship between vestibular aqueduct dimensions and inner ear pressure obtained by static acoustic compliance measurements.     

Personality and psychopathology in Ménière's disease

Introduction and ObjectivesPsychological factors in vertigo patients have been extensively studied but the role of anxiety and personality traits in the clinical course of Ménière's disease (MD) is unknown. The objectives of this study are to identify and characterize psychopathology in MD and to find risk factors for an increased rate and intensity of crisis and chronic symptoms.Materials and MethodsWe performed a transversal study in all patients diagnosed with definite MD in our department during a 5-year period. Sample subjects were interviewed in 3 steps: first, an otorhinolaryngologist collected information about clinical and pharmacological background of MD; second, a psychiatrist screened for mood, anxiety and personality disorders; in a third stage, the patient completed the DHI (Dizziness Handicap Inventory), STAI-Y (State Trait Anxiety Inventory), NEO-PI-R (Neo Personality Inventory Reviewed) and VAS (Visual Analogue Scale) for vertigo and dizziness. Statistical analysis was performed to search for risk factors for multiple and intense crisis and chronic symptoms.ResultsThirty-four patients completed all 3 phases of the study. A predominant dysfunctional personality trait was identified in 80% of patients (predominantly cluster C type), 35% were being treated with psychiatric medication and 34.4% had a considerable mood or anxiety disorder. All patients scored high (>7) in VAS during crisis. There was a statistically significant positive correlation between crisis rate and STAI, anxiety-subscale (N1) in NEO-PI-R, VAS and DHI scores (p<.044). Crises were more common in bilateral MD (p=.041). DHI scores were higher with higher STAI and N1 (p=.001). Disease duration and pure tone average were found to have a positive moderate correlation (p=.017).ConclusionsThe positive correlations between crisis rate, chronic dizziness and anxiety-related personality traits reveal a bidirectional and intimate relationship between personality, anxiety and MD, affecting these patients’ quality of life. These results support the relevance of prospecting adjuvant psychological and psychiatric approaches to these patients.