Abstract
Background: Menière's disease (MD) is an inner ear disorder characterized by recurrent episodes of spontaneous vertigo, unilateral low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Current diagnosis still often has to rely on subjective and audiometric criteria only, although endolymphatic hydrops is recognized as the pathophysiological substrate of the disease, having been demonstrated in anatomical pathological studies and by magnetic resonance (MRI). The modiolus has a close functional and anatomical relationship with the cochlear nerve and membranous labyrinth and can be evaluated with MRI but no data exist on the modiolar size in MD.Purpose: Our purpose is to examine the following hypothesis. Is cochlear modiolus smaller in symptomatic ears in MD?Methods: We used a retrospective 3 Tesla MR study (heavily T2-weighted 3D fast asymmetric spin-echo images and 0.5 mm slice thickness) comparing the mean modiolar area (MMA) in the index and best ears of eight patients with definite MD based on audiometric data. The obtained MMA values were compared against the audiometric data and the presence of vestibular endolymphatic hydrops.Results: No differences were seen in MMA between best and worst ears. Ears with a pure tone average (PTA) ≥25 dB and more pronounced endolymphatic hydrops showed lower MMA (not statistically significant). Two patients with extreme endolymphatic hydrops showed a noteworthy ipsilateral decrease in the cochlear modiolus area.Conclusion: No differences were seen in MMA between best and worst ears in definite MD. Worse hearing function (PTA ≥ 25dB) and more pronounced endolymphatic hydrops seem to be associated with lower MMA. This might be related to bone remodeling as a consequence of endolymphatic hydrops. Further research is needed to corroborate and explore these findings.
Highlights
Menière’s disease (MD) is a chronic disease with a prevalence of 200–500 per 100,000 individuals [1], characterized by a recurrent clinical syndrome of audiovestibular symptoms, namely spontaneous vertigo, unilateral hearing loss, aural fullness, and tinnitus [2].Prosper Ménière, in 1861, was the first to recognize the inner ear at the origin of the symptoms [3], with endolymphatic hydrops only later, in 1937, being described by British [4] and Japanese [5] researchers.Nowadays its cause remains undetermined, but the pathophysiological substrate seems to be the increase in the endolymphatic space of the membranous labyrinth, partially occupying the usual space of the perilymph
Pure tone average audiometry (PTA), and vestibular endolymphatic space were registered in Table 1 for “index” and “best” ears
When we analyzed PTA, modiolus area, and endolymphatic space for each individual patient, we found a consistently decreased modiolar area in the index ears in only three patients, but this was more evident in patients one and two, which interestingly had more extreme endolymphatic hydrops and a higher number of acute episodes and a relatively recent diagnosis of the disease (Figures 3, 4, Table 1)
Summary
MD is a chronic disease with a prevalence of 200–500 per 100,000 individuals [1], characterized by a recurrent clinical syndrome of audiovestibular symptoms, namely spontaneous vertigo, unilateral hearing loss, aural fullness, and tinnitus [2].Prosper Ménière, in 1861, was the first to recognize the inner ear at the origin of the symptoms [3], with endolymphatic hydrops only later, in 1937, being described by British [4] and Japanese [5] researchers.Nowadays its cause remains undetermined, but the pathophysiological substrate seems to be the increase in the endolymphatic space of the membranous labyrinth, partially occupying the usual space of the perilymph. MD is a chronic disease with a prevalence of 200–500 per 100,000 individuals [1], characterized by a recurrent clinical syndrome of audiovestibular symptoms, namely spontaneous vertigo, unilateral hearing loss, aural fullness, and tinnitus [2]. Menière’s disease (MD) is an inner ear disorder characterized by recurrent episodes of spontaneous vertigo, unilateral low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Current diagnosis still often has to rely on subjective and audiometric criteria only, endolymphatic hydrops is recognized as the pathophysiological substrate of the disease, having been demonstrated in anatomical pathological studies and by magnetic resonance (MRI). The modiolus has a close functional and anatomical relationship with the cochlear nerve and membranous labyrinth and can be evaluated with MRI but no data exist on the modiolar size in MD.
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