Abstract

Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD.

Highlights

  • Meniere’s disease (MD) is a long-lasting disorder of the inner ear characterized by episodes of vertigo lasting from 20 min to hours, low-to-middle frequencies sensorineural hearing loss (SNHL, Table 1), tinnitus, and aural fullness [1]

  • Apparently there were no clinical differences in the phenotype between sporadic and familial cases, Familial MD (FMD) had an earlier age of onset (p = 0.003) and a higher prevalence of autoimmune comorbidities (Table 2)

  • The diagnostic criteria for MD formulated by the Classification Committee of the Bárány Society state that bilateral involvement is determined by hearing loss defined in the audiogram

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Summary

Introduction

Meniere’s disease (MD) is a long-lasting disorder of the inner ear characterized by episodes of vertigo lasting from 20 min to hours, low-to-middle frequencies sensorineural hearing loss (SNHL, Table 1), tinnitus, and aural fullness [1]. The frequency of the spells of vertigo is typically greater during the earlier years [3,4,5], balance problems are observed during the course of the disease and might become severe if patients progress to a bilateral vestibular hypofunction [6, 7]. Several studies have reported contralateral ear involvement between 2 and 73% of cases, depending on the interval of follow-up and the diagnostic criteria used. There is a great disparity in the percentage of individuals with bilateral involvement, most of the studies highlighted that the number of patients with contralateral ear involvement increased with the duration of the disease [18, 21, 22]

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