Endolymphatic hydrops (EH) is the histopathological hallmark of Ménière's disease (MD) and has been found by magnetic resonance imaging (MRI) in patients with several inner ear syndromes without definite MD criteria. The incidence and relevance of this finding is under debate. The purpose of the study is to evaluate the prevalence and characteristics of EH and audiovestibular test results in groups of patients with fluctuating audiovestibular symptoms not fulfilling the actual criteria for definite MD and compare them with a similar group of patients with definite MD and a group of patients with recent idiopathic sudden neurosensory hearing loss (ISSNHL). 170 patients were included, 83 with definite MD, 38 with fluctuating sensorineural hearing loss, 34 with recurrent vertigo, and 15 with ISSNHL. The clinical variables, audiovestibular tests, and EH were evaluated and compared. Logistic proportional hazard models were used to obtain the odds ratio for hydrops development, including a multivariable adjusted model for potential confounders. No statistical differences between groups were found regarding disease duration, episodes, Tumarkin spells, migraine, vascular risk factors, or vestibular tests; only hearing loss showed differences. Regarding EH, we found significant differences between groups, with odds ratio (OR) for EH presence in definite MD group vs. all other patients of 11.43 (4.5-29.02; < 0.001). If the ISSNHL group was used as reference, OR was 55.2 (11.9-253.9; < 0.001) for the definite MD group, 9.9 (2.1-38.9; = 0.003) for the recurrent vertigo group, and 5.1 (1.2-21.7; = 0.03) for the group with fluctuating sensorineural hearing loss. The percentage of patients with EH varies between groups. It is minimal in the ISSNHL group and increases in groups with increasing fluctuating audiovestibular symptoms, with a rate of severe EH similar to the known rate of progression to definite MD in those groups, suggesting that presence of EH by MRI could be related to the risk of progression to definite MD. Thus, EH imaging in these patients is recommended.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8202684 | PMC |
http://dx.doi.org/10.3389/fsurg.2021.673847 | DOI Listing |
Worldwide, congenital deafness and pigmentation disorders impact millions with their diverse manifestations, and among these genetic conditions, mutations in the Microphthalmia-associated transcription factor (MITF: OMIM#156845) gene are notable for their profound effects on melanocyte development and auditory functions. This study reports a novel porcupine model exhibiting spontaneous deafness and pigmentation abnormalities reminiscent of human Waardenburg Syndrome Type 2 (WS2: OMIM#193510). Through phenotypic characterization, including coat color, skin, eye morphology, and auditory brainstem response (ABR) assessments, we identified hypopigmentation and complete deafness in mutant porcupines.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
December 2024
Hacettepe University, Faculty of Medicine, Department of Opthalmology, Ankara, Turkey.
Aims And Objectives: This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei.
Study Design: Cross-sectional observational study.
Setting: Tertiary referral centre.
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Bochkov Research Centre for Medical Genetics, Moscow, Russia.
A fifth world case of autosomal recessive Siddiqi syndrome (SIDDIS) related to ene is presented. In a consanguineous Lezgin (a Dagestan ethnicity) family, there were two affected brothers aged 28 yrs (proband, personally examined) and 32 yrs. Whole-exome sequencing followed by familial Sanger sequencing detected a novel missence variant c.
View Article and Find Full Text PDFZh Nevrol Psikhiatr Im S S Korsakova
December 2024
Federal Center of Brain Research and Neurotechnologies, Moscow, Russia.
According to the literature, a number of anti-epileptic drugs (AEDs) have an ototoxic effect. The mechanism of hearing dysfunction due to the use of AEDs is not well known. The main clinical manifestations of the cochleotoxic effect of the drugs are: tinnitus, sensorineural hearing loss, impaired pitch perception, hyperacusis.
View Article and Find Full Text PDFBMC Musculoskelet Disord
December 2024
Physical medicine & rehabilitation research center, School of medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Background: Pompe disease is a glycogen storage disease primarily affecting striated muscles. Despite its main manifestation in muscles, patients with Pompe disease may exhibit non-muscle symptoms, such as hearing loss, suggesting potential involvement of sensory organs or the nervous system due to glycogen accumulation.
Aims: This study aimed to evaluate the presence of concomitant small and large fiber neuropathy in patients with Pompe disease.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!