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

Abstract

Objectives: To evaluate the onset of vertigo, hearing loss and tinnitus in Ménière's disease and the associated endolymphatic hydrops (EH) of the inner ear.

Design: Multicentre evaluation of three patient groups.

Settings: Disease-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.

Participants: 340 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 measures: Latency and symptom development in Ménière's disease, and the appearance of EH of the inner ear in monosymptomatic patients and in Ménière's disease.

Results: The 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.

Conclusions: Ménière'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.

Figure 1

The onset of symptoms of Ménière's disease and age of the individual—(A) in the tertiary referral centre (n=340), (B) in the Ménière Association (n=726).

Figure 2

Onset of symptom among patients with the Finnish Meniere Association (n=726). The patients could have one or several symptoms in the onset of the disease.

Figure 3

Distributions between the onset of hearing loss and the onset of vertigo—in the Finnish Ménière Association (n=726). (A) Indicates individuals who had vertigo as the initial symptoms and diagram (B) individuals who had hearing loss as the initial symptom.

Figure 4

MRI comparison of inpatients with Ménière's disease between three-dimensional (3D)-real IR and conventional 3D-FLAIR sequences following transtympanic injection of Gd-DOTA. The ears were imaged 24 h post-transtympanic injection of Gd-DOTA (0.1 M, 0.5 ml) in the left ear with 3T MRI. Using 3D-real IR sequence, the non-contrasted inner ear fluids showed black and can be distinguished from the surrounding bone which displayed grey in the right ear (A);Gd-DOTA-enhanced perilymph was singled out from the non-contrasted black endolymph and grey bone in the left ear (B). Using 3D-FLAIR sequence, the contrast between the Gd-DOTA-enhanced perilymph and the non-enhanced endolymph was higher than that imaged using 3D-real IR sequence, broader Gd-DOTA enhancement was detected, which was in the higher turns and modiolus (C). In the figure, the EH is seen in the cochlea and in the vestibulum(black areas), which showed enlargement of the endolymphatic spaces. CN, cochlear nerve; 8th N, cochleo-vestibular nerve; EH, endolymphatic hydrops; EV, endolymph in the vestibulum; FLAIR, Fluid Attenuation Inversion Recovery sequences; L, left ear; LS, lateral semicircular canal; Mod, modiolus; PS, posterior semicircular canal; PV, perilymph in the vestibulum; OSL, osseous spiral lamina; R, right ear; SM, scala media; ST, scala tympani; SV, scala vestibuli; VN, vestibular nerve.

Figure 5

MRI of a 72-year-old patient with Ménière's disease on the right side at 4 h post-intravenous injection of Gd-DTPA-BMA at a single dosage. Anatomy was demonstrated by T2-weighted MRI (A). Uptake of Gd-DTPA-BMA in the inner ear was shown using a heavy T2-weighted FLAIR sequence (B). Obvious enlargement of the scala media (SM) at the basal turn is a sign of endolymphatic hydrops and was observed in the cochlea. Coch, cochlea; CSF, cerebrospinal fluid; 8th N, cochleo-vestibular nerve; EV, endolymph in the vestibulum; FLAIR, Fluid Attenuation Inversion Recovery sequences; LS, lateral semicircular canal; PS, posterior semicircular canal; PV, perilymph in the vestibulum; Vest, vestibulum.