The technique of transtympanic electrocochleography was initially developed as an objective hearing threshold test by Eggermont. Gibson et al. (1977) claimed that an enlarged direct current component of the action potential (AP) called the summating potential (SP) is an indication of endolymphatic hydrops, later confirmed by Coates who proposed an SP/AP ratio measure. This led to numerous publications using diagnostic ratios of 0.33-0.35. The insensitivity led to an eventual disenchantment with the test as a reliable objective test for Meniere's disease. It was further confused by audiologists employing remote canal or ear drum electrodes which give a response about one-fourth of the magnitude obtained by an electrode in contact with the cochlea. Subsequently Gibson stated that an SP/AP ratio of <0.5 is not diagnostic for hydrops. He then showed that a tone burst stimulus gave the test a significantly higher sensitivity and specificity, which has been supported by others. On MRI inner ear imaging with gadolinium hydrops can be seen, but the quality of images and what is seen may vary according to brand of scanner, settings, mode of gadolinium administration, and the possibility that gadolinium entry may favor the vestibule. Transtympanic tone burst electrocochleography is to date the simplest, cheapest and most sensitive technique for detecting cochlear endolymphatic hydrops to confirm a diagnosis of Meniere's disease.
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http://dx.doi.org/10.3389/fnins.2017.00301 | DOI Listing |
FEBS Open Bio
January 2025
Department of Medical Technology, Faculty of Health Sciences, Kumamoto Health Science University, Kumamoto, Japan.
FAM136A deficiency has been associated with Ménière's disease. However, the underlying mechanism of action of this protein remains unclear. We hypothesized that FAM136A functions in maintaining mitochondria, even in HepG2 cells.
View Article and Find Full Text PDFGenome Med
January 2025
Otology & Neurotology Group CTS495, Instituto de Investigación Biosanitario, Ibs.GRANADA, Universidad de Granada, 18071, Granada, Spain.
Background: Familial Meniere's disease (FMD) is a rare polygenic disorder of the inner ear. Mutations in the connexin gene family, which encodes gap junction proteins, can also cause hearing loss, but their role in FMD is largely unknown.
Methods: We retrieved exome sequencing data from 94 individuals in 70 Meniere's disease (MD) families.
Mol Neurobiol
January 2025
Otology & Neurotology Group CTS495, Division of Otolaryngology, Department of Surgery, Instituto de Investigación Biosanitaria, Ibs.GRANADA, Granada, Universidad de Granada, Granada, Spain.
Tinnitus is the perception of sound without an external source, often associated with changes in the auditory pathway and different brain regions. Recent research revealed an overload of missense variants in the ANK2 gene in individuals with severe tinnitus. ANK2, encoding ankyrin-B, regulates axon branching and inhibits microtubule invasion.
View Article and Find Full Text PDFActa Otolaryngol
January 2025
School of Audiology and Speech Language Pathology, Bharati Vidyapeeth (Deemed to be University), Pune, India.
Background: Meniere's disease (MD) affects 0.2% to 0.5% of the global population, with regional variations.
View Article and Find Full Text PDFActa Otolaryngol
January 2025
ENT Institute and Department of Otorhinolaryngology, Eye & ENT Hospital, State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Fudan University, Shanghai, PR China.
Background: Vestibular migraine (VM) and Menière's disease (MD) have numerous overlapping symptoms. Distinguishing the two common recurrent vestibulopathies was challenging.
Objectives: To assess the characteristics of hearing loss and the horizontal semicircular canal function in VM and MD.
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