Symptom profile and onset of the disease
The symptoms of patients on their first visit to the tertiary hospital for evaluation and treatment were examined. Based on the initial symptoms, 38% of the 340 patients could be classified as definite Ménière's disease. In 62%, a definite diagnosis was not possible. Within this group, the mean age of onset of the first symptoms was 42.5 years among the 340 patients referred. shows the clustering of symptoms on referral for treatment.
Symptoms of Ménière's disease when first seen in a tertiary referral hospital (n=340)
Onset of symptoms
The age of onset of symptoms was evaluated among 726 patients belonging to a Finnish Ménière Association. This group comprised 35 individuals who had only Ménière's disease-like symptoms and ‘possible Ménière's disease’. The mean age of onset of the first symptoms was 43.8 years. In about 10% of the individuals, the symptoms started at an age of over 65 years ().
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).
We asked the individuals to recall the first symptoms associated with Ménière's disease. Most commonly, the symptoms started with vertigo, with or without tinnitus and pressure in the ear. Hearing loss, as an initial symptom, occurred significantly less frequently than vertigo (). Vertigo without hearing loss developed as an initial symptom among 300 individuals, and hearing loss without vertigo in 109 individuals. In Fisher's exact test, the onset of the disease with vertigo was significantly more common than onset with hearing loss (p=0.031).
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.
Latency of the disease entity
Among the members of the Finnish Ménière Association, the time delay between the onset of vertigo and hearing loss, with or without tinnitus, was long in many cases, irrespective of whether the disease started with vertigo (A) or with hearing loss (B). In 21% of the patients, the time delay in assigning a probable diagnosis was 1–4 years. In 11% of the individuals, the time difference between the occurrence of both hearing loss and vertigo was 5–10 years, and in 9%, the difference was longer than 10 years. Thus, in about 20% of the patients, the time delay between the development of the disease entity of probable Ménière's disease was more than 5 years.
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 (more ...)
MRI of Ménière's disease and different inner ear-related complaints: patients with a symptom entity referable to Ménière's disease
EH was present in 190 of 205 ears (93%) with symptoms attributable to Ménière's disease (as shown by MRI enhanced with both transtympanically and intravenously administered GdC). Of the 45 asymptomatic contralateral ears, 29 (65%) showed EH on MRI with intravenously administered GdC. shows the site of EH in patients investigated with GdC-enhanced MRI. The vestibule showed the presence of EH more frequently than did the cochlea (p<0.004).
Table 4 Endolymphatic hydrops (EH) in patients with symptoms associated with Ménière's disease classified with the American Academy of Otolaryngology—Head and Neck Surgery as possible, probable and definite Ménière's disease (more ...)
shows a representative inner ear MRI from a Finnish patient with EH, acquired with a 3D-FLAIR sequence at 24 h post-transtympanic injection of GdC. In this, the inner ear fluids of the unenhanced ear showed as a dark image, and the eighth nerve and bone were imaged as grey. The perilymph of the injected ear displayed a bright signal as a result of GdC contrast. Since GdC failed to pass the perilymph-endolymph barrier, the endolymphatic space in the vestibular and cochlear regions remained dark and obvious EH was visualised.
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 (more ...)
is a representative MRI of a Japanese patient with Ménière's disease, acquired by a heavy T2-weighted 3D-FLAIR sequence and 4 h postintravenous injection of GdC. The border between the endolymphatic and perilymphatic spaces was not as distinct as seen following transtympanic GdC administration.
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 (more ...)
Patients with monosymptomatic inner ear diseases
Patients with a single inner ear symptom such as vertigo, fluctuant hearing loss or tinnitus comprised a heterogeneous group on MRI findings. Fifty-three per cent of patients with sudden hearing loss showed a presence of EH. In total, 69–95% of the other patient groups showed EH (). EH was more frequently observed in the vestibulum than in the cochlea (p=0.025).
Endolymphatic hydrops (EH) inpatients with monosymptomatic diseases
The data were reclassified according to the AAO-HNS criteria of Ménière's disease (1972) with cochlear Ménière's disease and vestibular Ménière's disease. Thereafter, the extent of EH in the cochlea and vestibule among patients with cochlear and vestibular symptoms was classified (). In both groups, the vestibulum showed a more significant EH presence than the cochlea, and among patients with vestibular hydrops, usually no cochlear symptom was complained of (χ2, p=0.003).
Degree of endolymphatic hydrops in patients with cochlear and vestibular Ménière's disease based on the 1972 AAO-HNS classification on vestibular hydrops and cochlear hydrops