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Ménière's Disease. Histopathological Changes European Review for Medical and Pharmacological Sciences 1999; 3: 189-193 Ménière’s disease. Histopathological changes: A post mortem study on temporal bones F. SALVINELLI, F. GRECO, M. TRIVELLI, F.H. LINTHICUM JR* Institute of Otolaryngology, “Campus Bio-Medico” University - Rome (Italy) *Department of Histopathology, “House Ear Institute” - Los Angeles, CA (USA) Abstract. – The histopathological Results and Discussion changes in the temporal bones of two deceased donors individuals with concomitant Ménierè’s disease have been studied. In one temporal Hydrops and its causes bone we have found a blockage of the endolym- Ménière’s disease is an affection of both phatic duct by abnormal bone. The histopatho- the hearing and balance organs of the inner logical modifications and the different therapeu- tic options are discussed. Clinical guidelines are ear, characterized by episodes of vertigo, proposed. hearing loss and tinnitus. Its pathological ba- sis is now firmly established as “hydrops” i.e. Key Words: distention of the endolymphatic spaces of the Ménière’s disease, Hydrops, Endolimphatic duct. labyrinth by fluid. The cause of the hydrops in Ménière’s disease is unknown. There are, however, other diseases of known pathogene- sis in which hydrops may be present as a complication. The common feature of these Introduction conditions is the presence of inflammatory or neoplastic involvement of the perilymphatic spaces. Thus otitis media complicated by per- The pathology of the vestibular system in ilymphatic labyrinthitis, syphilitic involve- man has been even less adequately investigat- ment of the labyrinth, or leukaemic deposits ed than the auditory one. Table I lists condi- in the perilymph spaces, may be associated tions the pathological bases of which have with hydrops4. been established by direct observation at au- topsy or surgery; affections such as “vestibu- Pathological appearances of hydrops lar neuronitis”, in which the possible structur- The hydrops of Ménière’s disease may af- al changes have been only guessed at from fect one or both inner ears. In most cases the the clinical symptoms, are not considered. cochlear duct and saccule are involved, but the utricular and semicircular ducts are usual- ly not. In some cases the cochlear duct alone is hydropic. A rare and debatable form of Materials and Methods Ménière’s is thought to affect the vestibule, but not the cochlea. Symptoms are those of We have studied the histopathological vertigo, but not hearing loss. changes in the temporal bones of two de- In the hydropic cochlear duct Reissner’s ceased individuals with Mènière’s disease. membrane, which is elastic, shows a variable These patients were donors and agreed degree of bulging. In the most severe cases, during their life to donate post mortem their the membrane reaches the top of the scala temporal bones to the House Ear Institute as vestibuli and may be in contact with a wide a contribution to a better knowledge of tem- area of cochlear wall. In the apical region it poral bone diseases. may bulge to such an extent that it fills the We have removed the temporal bones in helicotrema. In this way the distended scala our usual way1-3. media may even enter the scala tympani. The 189 F. Salvinelli, F. Greco, M. Trivelli, F.H. Linthicum Jr Table I. Pathology of the vestibular system. fibrous tissue in cases of Ménière’s hydrops external to the endolymphatic space has been Malformations described in the scala vestibuli by Hallpike Aging changes 7 Trauma and Cairns and in the vestibule deep to the 6 Ototoxicity footplate of the stapes by Schuknecht It is Infections: viral, bacterial, syphilis possible that the foci of connective tissue in Osseous lesions: Paget’s disease, otosclerosis these two situations are reactions to the irri- Hydrops: Ménière’s disease, syphilis, bacterial infec- tation produced by repeated distension and tion subsidence of the adjacent coclear duct and Positional vertigo: atrophy of superior division of saccule respectively. vestibular nerve; atrophy of utricle; “cupololithia- sis” Neoplasms: acoustic neuroma, metastatic carcinoma Changes in vestibular aqueduct and endolymphatic duct While hydrops involving the scala media and saccule is accepted by all as a basic fea- saccule swells up from its position on the me- ture of the pathology of Ménière’s disease, dial wall of the vestibule and frequently there is no such unanimity with regard to the touches the vestibular surface of the footplate alterations in the endolymphatic duct and its of the stapes. surrounding vestibular aqueduct. There have The utricle may be compressed in the been many descriptions of obstructive or po- process. In some cases the swollen saccule tentially obstructive lesions of these struc- may herniate from the vestibule into the tures associated with Ménières disease semicircular canals. Less frequently the utri- whereby restriction of the flow of endolymph cle may be distended, sometimes with small may have caused the hydrops. The following infoldings producing a scalloped appearance5. list gives a brief indication of the lesions dis- cussed at length in the literature: Changes in walls of a) Fibrosis8,9. membranous labyrinth b) Metastatic breast carcinoma10. Changes may be seen in the thin distended c) Decreased vascularity11. membranes of the hydropic endolymphatic d) Partial atresia of the intermediate por- spaces. Ruptures may be present particularly tion of the vestibular aqueduct with de- in Reissner’s membrane, and the terminal creased amounts of endoìymphatic duct tis- end of the ruptured membrane may be curled sue12. up. Such ruptures have been incriminated as e) Irregularity of the osseous wail of the possible pathological basis of the fluctuations vestibular aqueduct, sometimes with block- in pure tone thresholds which patients with age of the orifice of the vestibule13. Ménière’s disease may suffer. It has been sug- gested that the flooding of the perilymph with endolymph with its high potassium level may inhibit the bioelectric activity of the cochlea6. It is likely, however, that most of these ruptures are artefactual. They may be found in non-hydropic labyrinths. They are often multiple in the same membranous labyrinth. Outpouchings are often seen in which dilatation of part of the wall of the membranous labyrinth takes place and a lin- ing is present here that is thinner than else- where. These outpouchings have been ex- plained as healed ruptures, but, because of their regular features, it is more likely that they are simply areas of increased distension Figure 1. Meniere’s disease. Ruptures of the hydropic of parts of the labyrinthine wall, which are membrane are represented by redundancies in the normally thinner (Figure 1). The presence of membrane (arrows). ×35 190 Ménière’s Disease. Histopathological changes: A post mortem study on temporal bones f) Blockage of the vestibular aqueduct by Changes in the sensory epithelia syphilitic microgummata – “perivascular of the labyrinth round cell infiltrations”14. Alterations of the sensory cells of the or- g) Tumour-like papillary lesion resembling gan of Corti are mentioned by Hallpike and choroid plexus, which was removed surgically Cairns7, but they warn that such changes may from the endolymphatic sac; no Ménière’s be the result of post-mortem autolysis. The symptoms were present in this case15. effect of acid used in decalcification of the temporal bone is another possible source of Lesion similar to that mentioned under (g) damage to these cells after death. Such possi- were described in cases of Ménière’s disease bilitics of artefacts have been ignored in some by Schuknecht within the ductus reuniens re- reports. Changes, particularly in the apical re- gion of the hydropic cochlear duct6, by gion, have been described and associated Gussen9 in the hydropic crus commune and with low frequeney hearing loss6. In a recent by Hassard et al.16 at operation for possible study of 23 temporal bones from 17 patients insertion of an endolymphatic shunt. It is pos- with Ménière’s disease, however, no direct sible that such structures may secrete an ex- correlation was found between endolymphat- cess of endolymph and so produce lesions ic hydrops and hair cell loss14. Atrophy of the and symptoms of hydrops. In contrast to macula of the saccule may also be found, these observations it must be pointed out that which does not appear to be artefactual. in many careful studies of some Ménière’s hy- dropic temporal bones no changes whatever Relationsbip of symptoms to have been noted in the endoìymphatic duct pathological changes or vestibular aqueduct17. Image analysis of the areas in histological Ultrastructural alterations of a degenera- section of the cochìear duct (corresponding to tive nature have been observed in the epithe- volume in the whole structure) has been car- lial celìs of the endolymphatic sac in cases of ried out in two studies and related to the hear- Ménière’s disease18, but similar changes were ing loss. In the study of Antoli-Candela19 the also seen in cases of acoustic neuroma, sug- area of the cochlear duct was significantìy in- gesting that they are the result of the disease creased in relation to the degree of hearing process and not its cause. loss. Losses of over 70 dB showed a particular- We have found in a temporal bone en- ly high degree of hydropic expansion. In the dolymphatic hydrops due to a blockage of the study of Fraysse et al.14 a similar relationship endolymphatic duct by abnormal bone was found between cochlear duct size and the (Figure 2). total average hearing loss. There was also a correlation of those dimensions with the dura- tion of the disease: the longer the history of symptoms the more pronounced the cochlear duct dilatation. A relationship also seemed to be present between (a) the amount of dilata- tion of vestibular structures and (b) the re- sponse to caloric tests and the presence of posi- tional nystagmus, but this was less definite than the cochlear duct/hearing loss association.
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