Pathogenesis of Meniere's disease and Meniere's syndrome. 1984

M M Paparella

Meniere's disease can only be studied in patients, since it does not occur spontaneously in animals nor can it be induced in them. However, aspects of the disease such as endolymphatic hydrops can be usefully studied in animals. A study of the natural history (epidemiology) of Meniere's disease demonstrated the three major symptoms (triad) to be vestibular symptoms, auditory symptoms, and aural pressure. Bilaterality occurs in at least one out of 3 patients, and may approach 50% over full lifespans. Aural pressure (74.1%) was common, as was positional vertigo (85.9%) during and/or between attacks. Clinical variants such as vestibular Meniere's disease could persist for 25 years or more. Understanding the pathogenesis of Meniere's requires a study of known and unknown causes. In this study, Meniere's disease (cause unknown) was differentiated from Meniere's syndrome (cause known). Meniere's disease or syndrome can occur years after some inciting cause; thus all forms of Meniere's can be considered to have a delayed onset. Meniere's syndrome can occur as a sequel to syphilis, otosclerosis, infection (for example otitis media), or trauma. Endolymphatic hydrops explained on the basis of quantity and quality of endolymph is found in all forms of Meniere's disease and syndrome. Representative cases and pathological examples are discussed. Hydrops of the pars inferior (cochlear duct and saccule) is the most important finding in Meniere's disease. In some (but not most) cases, ruptures of the membranes are seen. The saccule can distend into the lateral semicircular canal. Symptomatic attacks are explained on the basis of both physical and biochemical phenomena. This study discusses concepts of pathogenesis of the disease, finding both theories of longitudinal (slow) and radial (fast) flow to be operational. Longitudinal flow, however, appears to be more important than radial flow, especially in advanced Meniere's where perilymph in the scala vestibuli and vestibule disappears and is replaced by membranous labyrinth. Meniere's disease (idiopathic) and Meniere's syndrome (cause known) probably occur as a result of endolymphatic absorptive dysfunction (the site being endolymphatic duct and sac). Hypocellularity of the mastoid and periaqueductal air cells, hypodevelopment of Trautmann's triangle, and anterior displacement of the lateral sinus are likely to be important findings associated with developmentally dysfunctional absorption of endolymph.(ABSTRACT TRUNCATED AT 400 WORDS)

UI MeSH Term Description Entries
D007758 Ear, Inner The essential part of the hearing organ consists of two labyrinthine compartments: the bony labyrinthine and the membranous labyrinth. The bony labyrinth is a complex of three interconnecting cavities or spaces (COCHLEA; VESTIBULAR LABYRINTH; and SEMICIRCULAR CANALS) in the TEMPORAL BONE. Within the bony labyrinth lies the membranous labyrinth which is a complex of sacs and tubules (COCHLEAR DUCT; SACCULE AND UTRICLE; and SEMICIRCULAR DUCTS) forming a continuous space enclosed by EPITHELIUM and connective tissue. These spaces are filled with LABYRINTHINE FLUIDS of various compositions. Labyrinth,Bony Labyrinth,Ear, Internal,Inner Ear,Membranous Labyrinth,Bony Labyrinths,Ears, Inner,Ears, Internal,Inner Ears,Internal Ear,Internal Ears,Labyrinth, Bony,Labyrinth, Membranous,Labyrinths,Labyrinths, Bony,Labyrinths, Membranous,Membranous Labyrinths
D007761 Labyrinthine Fluids Fluids found within the osseous labyrinth (PERILYMPH) and the membranous labyrinth (ENDOLYMPH) of the inner ear. (From Gray's Anatomy, 30th American ed, p1328, 1332) Fluid, Labyrinthine,Fluids, Labyrinthine,Labyrinthine Fluid
D008575 Meniere Disease A disease of the inner ear (LABYRINTH) that is characterized by fluctuating SENSORINEURAL HEARING LOSS; TINNITUS; episodic VERTIGO; and aural fullness. It is the most common form of endolymphatic hydrops. Meniere's Disease,Meniere's Syndrome,Vertigo, Aural,Auditory Vertigo,Aural Vertigo,Ménière Disease,Ménière's Disease,Ménière's Vertigo,Otogenic Vertigo,Auditory Vertigos,Disease, Meniere,Disease, Meniere's,Disease, Ménière,Disease, Ménière's,Diseases, Ménière,Diseases, Ménière's,Meniere Syndrome,Menieres Disease,Menieres Syndrome,Ménière Diseases,Ménière Vertigo,Ménière's Diseases,Ménière's Vertigos,Ménières Disease,Ménières Vertigo,Otogenic Vertigos,Syndrome, Meniere's,Vertigo, Auditory,Vertigo, Ménière's,Vertigo, Otogenic,Vertigos, Auditory,Vertigos, Ménière's,Vertigos, Otogenic
D009626 Terminology as Topic Works about the terms, expressions, designations, or symbols used in a particular science, discipline, or specialized subject area. Etymology,Nomenclature as Topic,Etymologies
D004712 Endolymphatic Sac The blind pouch at the end of the endolymphatic duct. It is a storage reservoir for excess ENDOLYMPH, formed by the blood vessels in the membranous labyrinth. Endolymphatic Sacs,Sac, Endolymphatic,Sacs, Endolymphatic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012444 Saccule and Utricle Two membranous sacs within the vestibular labyrinth of the INNER EAR. The saccule communicates with COCHLEAR DUCT through the ductus reuniens, and communicates with utricle through the utriculosaccular duct from which the ENDOLYMPHATIC DUCT arises. The utricle and saccule have sensory areas (acoustic maculae) which are innervated by the VESTIBULAR NERVE. Otolithic Organs,Utricle,Saccule,Organ, Otolithic,Otolithic Organ,Saccules,Utricle and Saccule,Utricles

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