Fluctuating Sensorineural Hearing Loss

Abstract

Background: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière’s disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. Summary: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.

Atypical Presentation of Enlarged Vestibular Aqueducts Caused by SLC26A4 Variants

“…One proposed mechanism is that intracranial pressure changes results in inner ear pressure changes by a widened vestibular aqueduct [ 26 , 27 ]. Like Ménière’s disease, periodic microruptures in the already distended endolymphatic membranes leads to disturbances in ion concentration in the endolymphatic and perilymphatic spaces, loss of the membrane potential, and resultant alterations in hearing and vestibular function [ 26 , 28 ]. Due to the altered fluid flow of the inner ear system, worsening hearing thresholds have been associated with head trauma, strenuous exercise, and upper respiratory infections [ 28 ].…”

“…Like Ménière’s disease, periodic microruptures in the already distended endolymphatic membranes leads to disturbances in ion concentration in the endolymphatic and perilymphatic spaces, loss of the membrane potential, and resultant alterations in hearing and vestibular function [ 26 , 28 ]. Due to the altered fluid flow of the inner ear system, worsening hearing thresholds have been associated with head trauma, strenuous exercise, and upper respiratory infections [ 28 ]. A recent meta-analysis revealed that decreased hearing after minor head trauma was reported in one-third of patients with EVA [ 29 ].…”

Application of Human Stem Cells to Model Genetic Sensorineural Hearing Loss and Meniere Disease

“…The most common sensory disorder is hearing loss, affecting around 15% of the population [4]. Sensorineural hearing loss (SNHL) and vestibular disorders are caused by Disclaimer/Publisher's Note: The statements, opinions, and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s).…”

MiR-106a facilitates the sensorineural hearing loss induced by oxidative stress by targeting connexin-43

“…Sensorineural hearing loss (SNHL) is one of the most common clinical diseases of ophthalmology and otorhinolaryngology, which is the abnormality of the auditory system to feel and process the sound resulted from various factors [ 1 ]. With the acceleration of population aging, hearing loss is the third chronic disease affecting the healthy life of the elderly [ 2 ] and the incidence of SNHL is still on the rise [ 3 ]. The main effect of adult hearing loss is communication obstruction, even psychological disorders.…”