Ménière's disease: a reappraisal supported by a variable latency of symptoms and the MRI visualisation of endolymphatic hydrops

Abstract

ObjectivesTo evaluate the onset of vertigo, hearing loss and tinnitus in Ménière's disease and the associated endolymphatic hydrops (EH) of the inner ear.DesignMulticentre evaluation of three patient groups.SettingsDisease-specific symptoms were reviewed among referred patients in a tertiary referral hospital in Finland and in members of a Finnish Ménière Association in Finland. The MRI of a separate group of patients was undertaken in a tertiary referral centre in Japan.Participants340 patients were reviewed in the referral hospital along with 740 members of the Ménière Association. MRI was undertaken in 224 patients in Japan.Primary and secondary outcome measuresLatency and symptom development in Ménière's disease, and the appearance of EH of the inner ear in monosymptomatic patients and in Ménière's disease.ResultsThe mean age of the first symptom was 43.8 years, with 10% of the patients being older than 65 years. The time delay between hearing loss and vertigo was more than 5 years in 20% of the members and of the patients. Gadolinium-contrasted MRI demonstrated EH in 90% of the patients with Ménière's disease, in which 75% was bilateral among patients with unilateral symptoms. In monosymptomatic patients with vertigo, tinnitus or hearing loss; EH was demonstrated in 55–90% of the patients either in the cochlea and/or the vestibulum of the symptomatic ear.ConclusionsMénière's disease often shows bilateral EH and comprises a continuum from a monosymptomatic disease to the typical symptom complex of the disease. We suggest that a 3T MRI measurement should be carried out in patients with sensory-neural hearing loss, vertigo and tinnitus, 4 h after the intravenous injection of a gadolinium-contrast agent to verify the inner ear pathology. This may lead to a better management of the condition.

Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI

“…The ability to visualize hydrops with this technique has been confirmed by multiple groups on different continents. [6][7][8]13 To date, however, no strong clinical application has been established for this technique, and existing research is mostly limited to showing a connection between a clinical diagnosis of MD and imaging evidence of hydrops.…”

“…4,5 Recent advances in MR imaging technology have allowed in vivo imaging of endolymphatic hydrops, which has the potential to advance our understanding of the natural history of the disease and assess changes in response to treatment. Several groups have confirmed the ability of delayed intravenous contrast-enhanced MR imaging to detect hydrops, [6][7][8] but no prior studies have assessed serial changes in hydrops during diuretic treatment by using delayed intravenous contrast-enhanced MR imaging, to our knowledge. One study that followed patients treated with betahistine by using intratympanic contrast-enhanced MR imaging did not show hydrops reversal despite symptomatic improvement.…”

Vestibular Schwannoma and Ipsilateral Endolymphatic Hydrops: An Unusual Association

“…In the recent years, several efforts have been made to develop reliable imaging able to identify ELH; some inner ear imaging techniques enable visualizing morphological changes in the cochlea and the vestibulum in vivo 24 . Current research demonstrates the validity of Locally Enhanced Inner Ear MRI (LEIM) after intratympanic contrast agent application and 3-Tesla MRI performed with intravenous gadolinium with 3D-FLAIR sequences for the diagnosis and grading of ELH; these techniques have shown similar sensitivity to histopathological series in identifying ELH [25][26][27] . The degree of perilymphatic enhancement in 3-Tesla MRI also depend on the interval between the intravenous contrast administration and imaging.…”

Ménière’s Disease and Tinnitus

“…Classical symptoms include luctuating hearing loss, aural fullness and periodic vertigo and tinnitus [2]. The diagnosis criteria from the American Academy of Otolaryngology and Head and Neck Surgery (AAO-HNS) and the Bárány Society do not recognize an eventual 'cochlear' Ménière disease [4], but recent imaging studies (gadolinium contrasted 3T MRI) are more sympathetic with this possibility [5].…”