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Review
. 2017 Aug;37(4):250-263.
doi: 10.14639/0392-100X-793.

The aetiopathologies of Ménière's disease: a contemporary review

B S Oberman  1 , V A Patel  1 , S Cureoglu  2 , H Isildak  1
Affiliations
Review

The aetiopathologies of Ménière's disease: a contemporary review

B S Oberman et al. Acta Otorhinolaryngol Ital. 2017 Aug.

Abstract
in English, Italian

Ménière's disease, a condition first described in the 1800's, has been an advancing area of clinical interest and scientific research in recent decades. Guidelines published by the American Academy of Otolaryngology - Head and Neck Surgery remained nearly static for almost 20 years, although we have certainly expanded our knowledge of the aetiology of the disease since that time. This review of the literature highlights the breadth and detail of the current theories in understanding the pathophysiology of this enigmatic disease. Histopathological specimens providing evidence of many of the aetiologies are presented as well. We aim to provide a centralised and updated resource regarding current and emerging theories for Ménière's disease.

La Sindrome di Ménière, una condizione descritta nel 1800, è stata un’area di grande interesse clinico e di ricerca scientifica negli ultimi decenni. Le linee guida pubblicate dall’ American Academy of Otolaryngology-Head and Neck Surgery sono rimaste pressoché invariate per quasi 20 anni, benché la ricerca scientifica sugli aspetti eziopatologici sia indubbiamente molto progredita nel frattempo. La presente revisione della letteratura evidenzia gli importanti progressi compiuti nella comprensione della fisiopatologia di questa malattia enigmatica. Le evidenze discusse sono inoltre accompagnate da una documentazione iconografica istopatologica. L’obiettivo della presente trattazione è fornire al lettore un quadro aggiornato ed accurato sulle teorie inerenti la Sindrome di Ménière.

Keywords: Aetiology; Ménière’s disease; Pathology; Update.

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Figures

Fig. 1.
Fig. 1.
Anatomy of the endolymph system. Used with permission from Rand S. Swenson. O'Rahilly R, Mueller F, Carpenter S, Swenson RS. Basic Human Anatomy: A Regional Study of Human Structure. 2009; http://www.dartmouth.edu/~humananatomy/figures/chapter_44/44-8.HTM. Accessed December, 24, 2014.
Fig. 2.
Fig. 2.
Aetiologies of endolymphatic hydrops.
Fig. 3.
Fig. 3.
a) HB 655 Lt 130 10x: A higher magnification of the cochlea showed outer hair cell loss (arrow) and stria vascularis atrophy (*), b) HB 655 Lt 100 1x: 72-year-old male patient diagnosed with Ménière's disease. He had a history of bilateral fluctuating hearing loss, tinnitus and vertigo episodes. Histopathologic exam of the left ear showed profound cochlear hydrops (arrow). Utricle (U), c) HB650Lt110. This 79-year-old patient had a history of bilateral profound mixed type hearing loss and left stapedectomy. She was extremely vertiginous right after the surgery for months. Histopathologic exam showed bilateral otosclerosis, which is located anterior to the oval window. This slide shows the surgery site, otosclerosis and hydropic saccular membrane that showed healed perforation. Interestingly, cochlea did not show any hydropic changes, d) HB593Lt 470. This was an 86-year-old patient who had a history of Ménière's disease and 8th nerve resection. Histopathologic exam showed profound hydropic changes in the cochlea, utricle and saccule. Note the outpouching, and hydropic saccular membrane.
Fig. 4.
Fig. 4.
a) HB333Lt 142. This was a 79-year-old patient who had a history of bilateral Ménière's disease. Histopathologic exam shows profound cochlea-saccular hydrops. Note that saccular membrane shows fistulae and touches to the footplate of stapes, b) HB839 Lt 260 1x: This 86-year-old male patient was diagnosed with left Ménière's disease. Histopathologic exam of his left ear showed profound hydrops in every turns of the cochlea as well saccule. Arrow shows that saccular membrane touches the footplate of stapes.
Fig. 5.
Fig. 5.
HB839 Lt 260 4x: A higher magnification of the cochlear hydrops is seen. Thick arrow shows profound hydrops; thin arrow indicates the rupture of Reissner's membrane.
Fig. 6.
Fig. 6.
a) HB 856 Rt 560 1x: This is an 80-year-old man who had bilateral mixed hearing loss and vertigo episodes. Histopathologic exam of his right ear showed otosclerosis that involves the otic capsule and endolymhatic hydrops in the basal turn of the cochlea (arrow), saccule (S) and utricle (U), b) HB 856 Rt 560 4x: A higher magnification of the basal turn of the cochlea showed clearly otosclerotic involvement of the otic capsule (arrow) and stria vascularis atrophy (*).
Fig. 7.
Fig. 7.
HB 693 Rt 570 1x: 78-year-old woman diagnosed with Ménière's disease. Histopathologic evaluation of her right ear indicated endolymphatic hydrops associated with extensive otosclerosis that involves the otic capsule and block the vestibular aqueduct (arrow). Note cochlear, saccular and utricular hydrops.
See this image and copyright information in PMC

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