Bilateral Ménière's disease is classified according to the time of appearance of symptoms in each ear into synchronous and metachronous types. A descriptive longitudinal study, involving 59 bilateral Ménière's disease patients, was carried out to assess the two forms of bilateral Ménière's disease.

Data on symptomatic chronology in each ear, auditory evolution and evolution of vertiginous crisis, among other aspects, were obtained, analysed and compared. Possible risk factors for Ménière's disease becoming bilateral were analysed after conducting nested case–control studies in a cohort.

The metachronous form was seen in 76.3 per cent of cases, and the time it took for the disease to become bilateral took a median time of seven years. The symptomatic triad was the most frequent symptomatic debut for the first ear in both forms. Synchronous debut presented a greater average hearing loss. Suffering from migraine and a symptomatic onset with a greater number of symptoms appear to be possible predictors of conversion to bilateral Ménière's disease.

Bilateral Ménière's disease temporal models presented differences. The study of them helps to better understand, prevent and predict the behaviour of these patients.

This study aimed to assess the clinical implications of delayed-acquisition post-gadolinium magnetic resonance imaging in identifying endolymphatic hydrops in Ménière's disease.

This study was a systematic review using Medline and Embase and following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines with predetermined criteria, namely Ménière's disease, post-gadolinium magnetic resonance imaging and endolymphatic hydrops. The Quality Assessment of Diagnostic Accuracy Studies-2 tool was used to assess bias.

Eleven studies were included; they all used 3T magnetic resonance imaging, with three-dimensional fluid-attenuated inversion recovery being the most common sequence. Intravenous gadolinium administration was more widely used compared with the intratympanic route. As for the timing of acquisition, 4 hours post-administration was universally used for the IV gadolinium and 24 hours was used for the intratympanic gadolinium. Despite patient-selection associated bias, all studies reported adequate visualisation of the endolymphatic spaces.

The use of delayed-acquisition magnetic resonance imaging is increasingly supported in visualising the endolymphatic spaces in Ménière's disease. Although the accessibility of 3T magnetic resonance imaging questions its wider applicability, it is a promising tool for the near future.

Recent developments in magnetic resonance imaging have enabled demonstration of endolymphatic hydrops, and the clinical application of these imaging studies in Ménière's disease is being explored.

To evaluate our centre's experience to date of hydrops magnetic resonance imaging in patients with episodic vertigo.

Magnetic resonance imaging was performed using a high-resolution three-dimensional fluid-attenuated inversion recovery sequence on a 3 Tesla scanner at 4 hours following double-dose gadolinium administration.

The study included 31 patients, 28 of whom had a clinical diagnosis of Ménière's disease. In unilateral Ménière's disease, magnetic resonance imaging was able to lateralise endolymphatic hydrops to the clinically symptomatic ear in all cases. Mild hydrops was often seen in clinically asymptomatic ears.

There is a good correlation between the clinical symptoms and lateralisation of hydropic changes on magnetic resonance imaging. Further refinements of imaging techniques and grading system will likely improve the diagnostic accuracy and clinical utilisation of hydrops magnetic resonance imaging.

Ménière's disease often presents with aural fullness, for reasons that are currently not well understood. Transtympanic ventilation tube insertion has been historically used for the management of this symptom, though the nature and mechanism of effectiveness is unclear.

To give an overview of the data available on the effects of ventilation tube insertion on aural fullness in Ménière's disease.

The databases PubMed, Embase, Medline, Scopus, Web of Science, Central and Google Scholar were searched to identify relevant records. Records were subsequently analysed and data extracted.

Only two studies directly measured the effect of ventilation tube insertion on aural fullness, while three others measured it as a placebo to assess another treatment. Considerable heterogeneity was found amongst the studies, including conflicting conclusions.

There is a paucity of evidence investigating the effect of grommet insertion on aural fullness in Ménière's disease. This work directs future research into this topic.

To evaluate the presence of endolymphatic hydrops in patients with immune-mediated inner-ear disease.

The presence of endolymphatic hydrops was prospectively evaluated in 17 patients clinically diagnosed with secondary (n = 5) or primary (n = 12) immune-mediated inner-ear disease, who attended the ENT department of a tertiary care centre for evaluation or treatment over the previous year. All patients underwent magnetic resonance imaging of the temporal bone.

Intratympanic gadolinium three-dimensional magnetic resonance imaging diagnosed hydrops in 11 of 12 patients with primary immune-mediated inner-ear disease (92 per cent). Of these, seven patients (64 per cent) presented only cochlear (n = 5) or predominantly cochlear (n = 2) hydrops. A positive magnetic resonance imaging result was observed in only one of five patients with secondary immune-mediated inner-ear disease (20 per cent).

This study confirms the presence of endolymphatic hydrops in immune-mediated inner-ear disease patients. The virtual absence of hydrops in patients with secondary immune-mediated inner-ear disease is remarkable, although firm conclusions cannot be drawn; this should be explored in a multicentre study with a larger sample of patients. A different immune reaction without development of endolymphatic hydrops should not be ruled out in secondary immune-mediated inner-ear disease patients.

To highlight a case from 1960 connecting endolymphatic sac tumour and von Hippel–Lindau disease.

In 1960, a 24-year-old woman presented with unilateral hearing loss, pulsatile tinnitus and a mass visible on otoscopy. The patient underwent surgical biopsy, which was complicated by haemorrhage, and ultimately resulted in death. At autopsy, a destructive temporal bone neoplasm with cystic and papillary architecture was observed that had eroded into the otic capsule. Intra-abdominal lesions consistent with von Hippel–Lindau disease were also observed, and the surgeon postulated a connection between endolymphatic sac tumour and von Hippel–Lindau disease.

A review of the literature was carried out using PubMed.

Endolymphatic sac tumours are rare neoplasms of the temporal bone that can occur sporadically or as part of von Hippel–Lindau disease. The connection between endolymphatic sac tumour and von Hippel–Lindau disease was first proposed in 1988 and formalised in 1997. We believe that this case represents the first documented connection between endolymphatic sac tumour and von Hippel–Lindau disease.

Although benign paroxysmal positional vertigo and endolymphatic hydrops are considered to be distinct diagnoses, a minority of vertiginous patients exhibit features of both conditions. This coincidence has been reported previously in the literature, and is reviewed here in terms of possible aetiology.

A new hypothesis to account for both conditions is offered, implicating free-floating degenerating debris from the otolithic apparatus. It is postulated that the gelatinous/proteinaceous component may account for an osmotically induced hydrops, while the calcified fragments may induce positional vertigo.

To describe the results obtained with endolymphatic sac drainage in patients with Ménière's disease.

A retrospective case review study was conducted of 95 Ménière's disease patients who underwent endolymphatic sac drainage in a tertiary care referral centre, after failing a long course of medical management. The main outcome measures were vertigo control and hearing preservation.

In patients with unilateral disease, vertigo control was obtained in 94.3 per cent of patients. A significant improvement in cochlear function was seen in 14 per cent of patients, and hearing was preserved or improved in 88 per cent. For the bilateral group, vertigo control was obtained in 85.7 per cent of patients and cochlear function improved in 28 per cent. Hearing preservation was attained in 71 per cent of these patients.

Endolymphatic sac drainage is a good surgical option for patients with incapacitating endolymphatic hydrops, providing a high percentage of vertigo control and hearing preservation.

To verify the hypothesis that intravenous frusemide reduces endolymphatic hydrops, as evaluated by three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging following intratympanic gadolinium administration.

The study comprised 12 patients (7 females and 5 males, aged 19–74 years) with Ménière's disease. Disease duration ranged from 0.5 to 8 years, with a frequency of 0.5 to 6 vertigo spells per month, as calculated in the last 6 months. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging was performed 24 hours after intratympanic injection of gadobutrol diluted eight-fold. Frusemide 20 mg was given intravenously immediately after imaging. Magnetic resonance imaging was repeated after 1 hour, using the same parameters and sequence.

All patients showed enhancement defects, indicating endolymphatic hydrops of variable degrees. No modifications occurred at the second magnetic resonance imaging performed 1 hour after frusemide administration.

There was no evidence of endolymphatic hydrops modification 1 hour after intravenously administered frusemide. Therefore, loop diuretics in Ménière's disease, which are today used on an empirical basis, must be reconsidered. Implications of these outcomes are discussed and related to the role of endolymphatic hydrops in the development of Ménière's disease.

To evaluate the effectiveness of tympanostomy tube placement in controlling symptoms of intractable Ménière's disease.

Fifteen patients with intractable Ménière's disease underwent tympanostomy tube placement in the affected ear. Post-operative changes in vertigo attacks and hearing level were recorded, and were evaluated according to American Academy of Otolaryngology–Head and Neck Surgery criteria.

At 12 months after treatment, 3 patients (20 per cent) showed complete control of vertigo, 7 (47 per cent) showed substantial control and 2 (13 per cent) showed limited control; 3 patients (20 per cent) required other treatment. At 24 months after treatment, 7 patients (47 per cent) showed complete control of vertigo, 3 (20 per cent) showed substantial control and 1 (7 per cent) showed limited control; 1 patient required other treatment 15 months after tympanostomy tube placement.

There is no definite pathophysiological explanation for the effect of tympanostomy tube placement in reducing vertigo attacks. This treatment is not effective for all patients with intractable Ménière's disease. However, tympanostomy tube placement might be an additional surgical therapeutic option to consider prior to contemplating other, more invasive treatments.

To determine whether the distribution of membrane lesions associated with Ménière's disease is random, as might be expected from a pervasive process such as hydrops, or orderly, as might be expected if membrane resistance is graded.

A meta-analysis of temporal bone reports on 184 specimens demonstrating endolymphatic hydrops was undertaken to determine membrane lesion evolution and distribution.

Lesion distribution was found to be orderly and cochleocentric. No random scattershot lesions were reported in any study. Disease always started in the cochlear apex, even in non-symptomatic cases, and then involved the saccule, utricle, ampullae and canal system in that precise sequence as the disease progressed.

The orderly lesion progression in the otopathology associated with Ménière's disease suggests that the hydropic process has a graded non-random effect on the labyrinth. These findings suggest a pathological staging system that may be useful in temporal bone evaluation.

Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging of the inner ear after intratympanic injection of gadolinium, together with magnetic resonance imaging scoring of the perilymphatic space, were used to investigate the positive identification rate of hydrops and determine the technique's diagnostic value for delayed endolymphatic hydrops.

Twenty-five patients with delayed endolymphatic hydrops underwent pure tone audiometry, bithermal caloric testing, vestibular-evoked myogenic potential testing and three-dimensional magnetic resonance imaging of the inner ear after bilateral intratympanic injection of gadolinium. The perilymphatic space of the scanned images was analysed to investigate the positive identification rate of endolymphatic hydrops.

According to the magnetic resonance imaging scoring of the perilymphatic space and the diagnostic standard, 84 per cent of the patients examined had endolymphatic hydrops. In comparison, the positive identification rates for vestibular-evoked myogenic potential and bithermal caloric testing were 52 per cent and 72 per cent respectively.

Three-dimensional magnetic resonance imaging after intratympanic injection of gadolinium is valuable in the diagnosis of delayed endolymphatic hydrops and its classification. The perilymphatic space scoring system improved the diagnostic accuracy of magnetic resonance imaging.

To establish a new magnetic resonance imaging scoring system for diagnosing endolymphatic hydrops.

A total of 214 ears of 107 patients were categorised into five groups: no symptoms, Ménière's disease, sudden deafness, delayed endolymphatic hydrops and other ear disorders. Gadolinium distribution within the labyrinth was scored separately and quantitatively by two radiologists. Multiple independent-sample non-parametric tests, Bayesian discriminant analysis, multivariate logistic regression and receiver operating characteristic curve analyses were performed.

The derived scoring model was highly accurate for diagnosing Ménière's disease and delayed endolymphatic hydrops. Two magnetic resonance imaging scoring methods for the perilymphatic space were proposed for the diagnosis of endolymphatic hydrops: a pre-1 value (a new variable that predicts individual probability) of more than 0.3982299, or a sum of all labyrinth component scores of less than 14.5.

A convenient method is proposed which offers reliable radiological diagnostic criteria for Ménière's disease and delayed endolymphatic hydrops.