Noninvasive perilymphatic pressure measurement in patients with Ménière's disease and patients with idiopathic sudden sensorineural hearing loss

OBJECTIVE: Inner ear disorders are proposed to be affected by changes in inner ear hydrostatic pressure. In humans, the perilymphatic hydrostatic pressure can be assessed in a noninvasive way with the MMS-10 Tympanic Displacement Analyser. In this study, measurements were performed in patients with Ménière's disease and patients with idiopathic sudden sensorineural hearing loss (ISSHL), and the results were compared with those of normal hearing ears. The measurement technique has been evaluated as a possible additional diagnostic tool for Ménière's disease or ISSHL. STUDY DESIGN: The hydrostatic perilymphatic pressure was measured noninvasively with the MMS-10 Tympanic Displacement Analyser. SETTING: Department of Otorhinolaryngology at the University Hospital. PATIENTS: Seventy-seven patients with Ménière's disease, 28 patients with ISSHL, and 50 normal hearing subjects. Intervention: Diagnostic. MAIN OUTCOME MEASURE: Significant differences of perilymphatic pressure measurement results, represented by the measurement parameters Vi and Vm, in affected, nonaffected, and normal hearing ears. RESULTS: On average, the measurement results showed no significant differences between affected (by Ménière's disease or ISSHL), nonaffected, and normal hearing ears. CONCLUSIONS: In affected ears, perilymphatic pressure measured noninvasively did not differ significantly from the pressure in nonaffected and normal hearing ears. The tympanic membrane displacement technique cannot be considered an additional tool to confirm or reject the diagnosis of Ménière's disease or ISSHL.     

Intratympanic dexamethasone and hyaluronic acid in patients with low-frequency and Ménière's-associated sudden sensorineural hearing loss.

BACKGROUND: Steroids are widely used for the treatment of cochleovestibular disorders. Direct steroid application in the middle ear cavity, when combined with a round window membrane permeability-modulating substance, increases the level of the steroid reaching the target cells. We measured hearing in patients with idiopathic isolated low-frequency sensorineural hearing loss and in patients with sudden sensorineural hearing loss and a history of Ménière's disease. Contradictory reports about effectiveness of intratympanic steroid therapy on vertigo control and hearing improvement in patients with Ménière's disease exist in the literature. METHODS: Eighteen patients with isolated low-frequency idiopathic sudden sensorineural hearing loss and 21 patients with sudden sensorineural hearing loss and a history of Ménière's disease were prospectively evaluated. The acute effect of the intratympanic application of dexamethasone with hyaluronic acid on hearing outcome after failure of an initial standard treatment with intravenous steroid and vasoactive substances was assessed. Evaluation was based on standard pure-tone audiometry findings. RESULTS: After intratympanic injection of dexamethasone and hyaluronic acid, 14 of the 18 patients with isolated low-frequency sensorineural hearing loss showed a significant improvement in hearing. After intratympanic therapy, 15 patients with a previous history of Ménière's disease and idiopathic isolated low-frequency sensorineural hearing loss showed an improvement in hearing on pure-tone audiometry, four remained unchanged, and two showed a tendency toward a slight deterioration. CONCLUSION: Intratympanic combined dexamethasone/hyaluronic acid application provides a reliable and safe therapeutic option for improvement of hearing in patients with isolated low-frequency idiopathic sudden sensorineural hearing loss or sensorineural hearing loss resulting from Ménière's disease who have failed intravenous steroid and vasoactive treatments.     

Menière's disease in children

Fourteen children (aged 14 years or younger) with typical Ménière's triad with cochlear sensorineural hearing loss, tinnitus, and intermittent vertigo attacks lasting from minutes to hours were investigated in four different neuro-otologic centers. Nine children, labeled as having "idiopathic Ménière's disease," developed the auditory and vestibular symptoms without any detectable causative factor. Five children, labeled as suffering from "secondary Ménière's syndrome," had histories of an initial hearing loss following mumps, hemophilus influenza meningitis, temporal bone fracture, or congenital or embryopathic complications in the ear that developed into the full Ménière's triad 5 to 11 years later. The 14 children represent 1% of all cases affected with idiopathic or secondary Ménière's disease (or syndrome) that have been detected during the past five years in the four collaborating centers.     

Hearing loss fluctuating with blood sugar levels in Ménière's disease

An unusual case of a patient with bilateral Ménière's disease is described whose disease presented in the second ear as a sensorineural hearing loss which fluctuated with the patient's level of blood glucose. The literature concerning the role of abnormal glucose metabolism in Ménière's disease is reviewed and the investigation and management of this patient's condition is discussed.     

Secondary Menière's disease

Among 93 patients presenting the typical symptoms of a Ménière's disease associating an unilateral fluctuating hearing loss of sensorineural type, tinnitus and vertiginous attacks lasting minutes to hours, 40 patients (43%) presented in their personal history a particular otologic insult in the ear which later on developed into the full Ménière's symptomatology, or a particular systemic disease with otologic manifestations. The Ménière's triad appeared in these patients six months to twenty nine years after the initial otologic or systemic lesion. Among these initial lesions were 16 cases of sudden partial or complete deafness related to viral or bacterial infection, 3 cases of sudden cochleo-vestibular deficit and 1 case of vestibular neuritis, 5 cases of temporal bone fractures and 4 cases of significant acoustic trauma, 2 cases of otosclerosis, 1 case of chronicotitis media and 1 case of severe hearing loss after otologic surgery, 5 cases of meningo-encephalitis and 2 cases of acquired syphilis. These particular lesion could be, in our opinion, the releasing factor of the inner ear dysfonction leading eventually to a secondary Ménière's syndrome.     

Audiological findings of prolonged Menière's disease

As Ménière's disease progresses, the fluctuations becomes less marked and the hearing level gradually declines. In the more established and advanced stages of the disease, a permanent and progressive hearing loss develops. It has been reported, however, that hearing acuity in some patients with Ménière's disease significantly improved after having had the disorder for many years. Such observation suggests that the inner ear pathology in late stages of Ménière's disease may not be necessarily irreversible. The purpose of this investigation was to assess whether or not various audiological evaluations including EcochG may be of value in predicting the irreversibility of hearing deficit in patients suffering from advanced stages of Ménière's disease. The audiological studies included pure tone audiogram, SISI test, Bekesy audiometry, glycerol (or urea) test, speech discrimination test, and electrocochleography. A combination of the recruiting type of response in the EcochG and the narrowing of the tracing width of continuous sound in the Bekesy pattern seemed to be indicative for irreversible hair cell damages. Patients who had a low score in the speech discrimination test as well as an absence of ABR response were suspected of having retrocochlear lesions. Our investigations suggest that the irreversibility of hearing impairment in patients with later stages of Ménière's disease may be predicted to some extent in the use of various audiological evaluations including the EcochG.     

Selective vestibular ablation by intratympanic gentamicin in patients with unilateral active Ménière's disease: a prospective, double-blind, placebo-controlled, randomized clinical trial

OBJECTIVE: To establish the efficacy of intratympanic gentamicin treatment in patients with unilateral Ménière's disease. MATERIAL AND METHODS: This was a prospective, double-blind, randomized clinical trial of intratympanic gentamicin versus intratympanic buffer solution (placebo) in patients with established active Ménière's disease in the affected ear. Outcome measures included the number of vertiginous spells, degree of sensorineural hearing loss, labyrinthine function and labyrinthine asymmetry. RESULTS: Topical gentamicin provided a significant reduction in the number of vertiginous spells, although a "placebo effect" was also observed. Sensorineural hearing loss did not occur in the gentamicin group, although some deterioration occurred in the placebo group. CONCLUSIONS: Intratympanic gentamicin is a safe and efficient treatment for the vertiginous spells associated with Ménière's disease. When applied early in the course of the disease, it may prevent some of the sensorineural hearing deterioration associated with it.     

Clinical observations on the on-off effect of the acoustic stapedius reflex.

The on-off effect of the acoustic stapedius reflex was found in association with several kinds of ear disease: otosclerotic stapes fixation, incudostapedial dislocation and sensorineural hearing loss. One fifth (8/40) of the patient group with an on-off effect and sensorineural hearing loss showed the typical symptoms of Ménière's syndrome.     

Use of electrocochleography for assessing endolymphatic hydrops in patients with Lyme disease and Ménière's disease

From an otological standpoint, Lyme disease can manifest itself as Ménière's disease both clinically and electrophysiologically. The aim of this study was to describe the findings of routine clinical, auditory and vestibular tests in patients with Ménière's and Lyme disease and to use electrocochleography (ECoG) to assess the presence of endolymphatic hydrops (EH) in both diseases. Transtympanic ECoG was performed in 91 patients with Ménière's disease and in 11 patients with confirmed Lyme disease. In both diseases the majority of patients had more than one complaint. There was one case with isolated hearing loss in the Lyme disease group. Typical clinical manifestations of Ménière's disease (vertigo, sensorineural hearing loss and tinnitus) were found in 6/11 patients (54.5%) in the Lyme disease group. The ECoG results indicated that there were 65/91 patients (71.4%) with Ménière's disease and 5 patients (45.5%) with Lyme disease who presented with EH. No statistically significant difference was found between the incidence of different symptoms of Ménière's and Lyme disease. On the basis of these results, patients with Lyme disease should undergo careful examination and investigation, especially in endemic regions. The presence of EH does not exclude the presence of infection with borreliosis as a cause of Ménière's disease-like symptoms.     

Long-term hearing outcome in patients receiving intratympanic gentamicin for Ménière's disease

OBJECTIVE: To determine the long-term hearing outcome in patients with intractable vertigo caused by unilateral Ménière's disease who were treated with intratympanic injection of gentamicin. STUDY DESIGN: The study was a longitudinal analysis of hearing and control of vertigo in patients with unilateral Ménière's disease who received intratympanic gentamicin. METHODS: Pure-tone thresholds and speech discrimination scores on audiometry were analyzed, along with the control of vertigo. Criteria described in 1995 by the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) were used. Patients treated with intratympanic gentamicin had "definite" Ménière's disease and had intractable vertigo despite optimal medical therapy, no symptoms suggestive of Ménière's disease in the contralateral ear, and serviceable hearing in the contralateral ear. The study analyzed the outcomes of 34 patients for whom follow-up data were available for periods greater than 24 months after intratympanic gentamicin. RESULTS: Complete control of vertigo (AAOHNS Class A) was obtained in 90% of the patients. Profound sensorineural hearing loss occurred as a result of gentamicin injection in 1 of the 34 patients (3%). When data from all patients were grouped together, hearing was improved in 5 (15%), unchanged in 23 (68%), and worse in 6 (17%) patients. This distribution of hearing outcome is similar to that in patients whose symptoms of Ménière's disease were managed with medical measures. Recurrent vertigo developed in 10 patients (29%) at an interval of 4 to 15 months after initially complete control. Treatment with additional intratympanic injection(s) of gentamicin did not result in a change in hearing. CONCLUSION: The risk of hearing loss in patients treated with infrequent intratympanic injection(s) of gentamicin is low.     

Rare cases of Ménière's disease in children

Classical Ménière's disease is rarely found in children and literature regarding it is scarce. In general, the frequency of Ménière's disease in children is only 0.4-7.0 per cent of that in adults. The progression pattern of Ménière's disease in children is not known well. Here, we report three cases of Ménière's disease in children less than 15 years old, treated over nine years. The three cases comprise 14- and 13-year-old boys and a nine-year-old girl. Two of the three patients initially complained only of recurrent bouts of vertigo, without any tinnitus, ear fullness or hearing impairment. In all three cases, the early pure tone audiograms showed only high tone frequency loss, regardless of subjective hearing loss, and the decrease in the hearing threshold was observed one to eight years after the dizziness attacks began. The hearing threshold was usually decreased to a level of mild or moderate hearing impairment. After diuretic treatment, vertigo was generally well controlled, and some cases showed improvement in hearing. Of the total number of patients with Ménière's disease who visited our department over nine years, 2.6 per cent (3/114) were children, and the overall incidence of Ménière's disease in children with vertigo was 2.0 per cent (3/147). In conclusion, Ménière's disease in children rarely develops and may have characteristics of high tone loss in initial audiograms.     

Intratympanic Steroid Injection

Intratympanic steroid therapy is an important treatment modality for managing certain inner ear pathology. Indications for intratympanic steroid injections include sudden sensorineural hearing loss, Ménière's disease, autoimmune inner ear disease and acoustic trauma. In this article, we discuss the evidence supporting various indications for intratympanic steroid therapy and describe the technical steps necessary to perform intratympanic injections.     

Histopathologic changes of contralateral human temporal bone in unilateral Ménière's disease.

HYPOTHESIS: To disclose the histopathologic findings in the contralateral temporal bone in unilateral Ménière's disease. BACKGROUND: Several functional studies reported abnormal findings in the contralateral ears in patients with unilateral Ménière's disease. METHODS: This study involved quantitative analysis, including the number of spiral ganglion cells, the loss of cochlear hair cells, the area of stria vascularis, and the density of fibrocytes in the spiral ligament. It included 14 temporal bones from 7 subjects with bilateral Ménière's disease, 30 temporal bones from 15 subjects with unilateral Ménière's disease, and 17 age-matched normal control temporal bones from 12 subjects. RESULTS: The mean number of spiral ganglion cells in the contralateral temporal bones in patients with unilateral Ménière's disease was 17,376.0 and was significantly lower than that in normal controls. The mean loss of inner and outer hair cells in the contralateral temporal bones in patients with unilateral Ménière's disease was significantly greater than that in normal controls in all turns. The stria vascularis was severely atrophic and degenerated in patients with Ménière's disease. The mean area of stria vascularis in contralateral temporal bones in patients with unilateral Ménière's disease was significantly smaller than normal controls. There was no significant difference in the density of fibrocytes in the spiral ligament between the diseased side and the contralateral side in patients with unilateral Ménière's disease and between normal control and contralateral side. CONCLUSION: The contralateral inner ear in patients with unilateral Ménière's disease has significantly more damage compared with inner ears of normal controls.     

A diagnostic test for Ménière's Disease and Cochlear Hydrops: impaired high-pass noise masking of auditory brainstem responses.

HYPOTHESIS: Endolymphatic hydrops in patients diagnosed with Ménière's disease causes changes in the response properties of the basilar membrane that lead to impaired high-pass noise masking of auditory brainstem responses to clicks. BACKGROUND: Ménière's disease is defined as the idiopathic syndrome of endolymphatic (cochlear) hydrops, which is an abnormal increase in the volume of cochlear fluid (endolymph) in the inner ear. Accurate detection and diagnosis are important but difficult because of the lack of sufficiently sensitive tests. METHODS: Two populations were compared: (1) 38 non-Ménière's normal-hearing subjects; and (2) 23 patients who, at the time of testing, continued to have at least three of the four hallmark symptoms (i.e., tinnitus, vertigo, fluctuating hearing loss, and fullness) used in the diagnosis of Ménière's disease. Auditory brainstem responses to clicks presented ipsilaterally with masking noise that was high-pass filtered at various frequencies were recorded. RESULTS: In the Ménière's patients, the masking noise is insufficient such that an undermasked Wave V is still present at a latency similar to that of Wave V in the response to the clicks alone. In the control non-Ménière's normal-hearing subjects, this undermasked component was either absent or significantly delayed because of the masking noise. The difference in the delays between these populations is such that the distributions do not overlap, resulting in 100% sensitivity and 100% specificity. CONCLUSION: This test is able to distinguish objectively active Ménière's disease in individuals and may show promise for tracking changes in the severity of the disease caused by progression or treatment.     

Experimental labyrinthine lesions through stylomastoid foramen.

It was confirmed that the substances injected into the facial nerve through the stylomastoid foramen, reached every part of the inner ear. In sensitized animals, by repeated provocative injections of antigen, acute reversible episodes, very similar to that of Ménière's disease, were observed. The injection of various solutions and the provocative injection into the sensitized animals, resulted in completely opposite reactions. In sensitized animals, the electrophysiological phenomena of the cochlea also showed fluctuations like Ménière's disease.     

Clinical significance of SP/AP ratio in inner ear diseases

Electrocochleogram was recorded from 28 ears affected by Ménière's disease , 27 ears affected by sensorineural hearing loss resulting from hair cell damage and 22 normal ears as controls in response to tone bursts in order to evaluate diagnostic significance of the summating potential/action potential (SP/AP) ratio and the SP amplitude. The fundamental aspects of the SP/AP ratio were studied in relation to frequency, intensity and rate of tone burst stimuli. It was concluded from present results that the SP/AP ratio can be a useful indicator to detect -SP dominance, while the SP amplitude can be an indicator to detect cell damage in inner ear diseases.     

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.     

Immunological responses in acute low-tone sensorineural hearing loss and Ménière's disease

OBJECTIVE: The autoimmune response appears to play an important role in some types of acute sensorineural hearing loss. Endolymphatic hydrops associated with fluctuating hearing loss has also been suggested to be caused by an immunological mechanism. Acute low-tone hearing loss (ALHL) associated with Ménière's disease (MD) is characterized by fluctuating hearing loss, and its etiology is thought to involve endolymphatic hydrops. The aim of this study was to attempt to determine the etiology of ALHL in MD. MATERIAL AND METHODS: A flow cytometer was used to analyze intracellular cytokine levels in peripheral blood from 19 patients with ALHL and 26 patients with MD and the data compared to those obtained from age- and gender-matched healthy volunteers. RESULTS: The patients with ALHL showed significantly increased levels of Th1 subsets (interferon-gamma-producing helper T cells) as compared to those in normal controls. The levels of Th2 (IL-4-producing helper T cells) subsets did not differ from those in the control group and thus Th1 predominated in ALHL patients. The patients with MD showed significantly increased natural killer cell activity but no Th1 dominance. These patients had no obvious systemic or local disease except in the inner ear. CONCLUSION: An abnormality of the Th1/Th2 balance in ALHL and increased natural killer cell activity in MD are thought to relate to inner ear disorder. These results are consistent with the possibility that the etiology of ALHL and MD involves an immune response.     

Electrocochleography and gentamicin therapy for Ménière's disease: a preliminary report

OBJECTIVE: It is widely held that an enlarged summating potential (SP) relative to the eighth nerve action potential (AP) is a reflection of endolymphatic hydrops. Aminoglycosides are an accepted treatment for incapacitating Ménière's disease and are known to affect both sensory and secretory cells of the inner ear. The intent of this study was to determine whether this effect on secretory cells could be objectively confirmed by virtue of changes in the electrocochleogram (ECoG) of patients receiving gentamicin therapy for Ménière's disease. STUDY DESIGN: This was a prospective longitudinal study of repeated ECoG measures in three groups of subjects. Ménière's patients undergoing gentamicin treatment were compared with two control groups: individuals with stable Ménière's disease and normal-hearing control subjects. SETTING: The study was conducted at a tertiary referral center. PATIENTS: The sample included 21 normal-hearing subjects, 15 patients with stable unilateral Ménière's disease, and 12 with disabling unilateral Ménière's disease. INTERVENTIONS: For patients with disabling Ménière's disease, gentamicin was administered transtympanically. Audiograms, impedance tests, and ECoG were performed twice for all subjects. MAIN OUTCOME MEASURES: The SP and AP amplitudes, AP latency, and SP/AP ratio of the EcoG were measured. RESULTS: A statistically significant reduction in the SP/AP ratio was observed after gentamicin administration (analysis of variance interaction effect: F2 = 5.64; p = 0.0065). CONCLUSIONS: The significant reduction in the SP/AP ratio in the gentamicin-treated Ménière's group supports the hypothesis that gentamicin improves the electrophysiologic function of the cochlea, possibly by reducing the severity of the associated endolymphatic hydrops.     

Postural control and glycerol test in Ménière's disease.

Ménière's disease is a clinical disorder, characterized by fluctuating hearing loss, recurrent spontaneous episodic vertigo, tinnitus and aural fullness, which may be defined as the idiopathic syndrome of endolymphatic hydrops. The most important test for diagnosis of Ménière's disease is the glycerol test. This is a simple and rapid method and several authors have confirmed its efficiency for identifying endolymphatic hydrops. This test provides information on the cochlear response to the osmotic changes produced by glycerol in the inner ear, whereas modifications in the vestibular labyrinth are usually not evaluated. The aim of this study was to evaluate the effects of glycerol on postural control during attacks of Ménière's disease, and to correlate this data with data on cochlear function. After the glycerol test, an improvement in postural control was recorded in 70% of patients, with all patients reporting a recovery of vertigo. The impairment of postural control during endolymphatic hydrops could be related to a pressure increase in the labyrinth, which interferes with the normal dynamics of the endolymph, and a rapid functional recovery could occur during an osmotic depletion. Dynamic posturography improves the sensitivity of the glycerol test and may therefore be useful in the diagnosis and staging of Ménière's disease.