The neurologist and the hydrops

Abstract

The presence of endolymphatic hydrops has been studied in many neurological disorders. The pathophysiological mechanisms may involve CSF pressure variations, transmitted to the innear ear. This hydrops could play a role in vestibular or cochlear symptoms. For the ENT specialist, the etiological diagnosis of endolymphatic hydrops is a challenge, and neurological etiologies must be known. The treatment of these neurological causes could be effective on cochleo-vestibular symptoms. The knowledge of endolymphatic hydrops could also be a target for noninvasive tests, able to estimate CSF pressure variations. For the neurologist, this could represent a useful tool for the diagnosis and follow-up, in some of these neurological disorders, related to a CSF pressure imbalance. The purpose of this paper is to summarize literature data on endolymphatic hydrops in neurological disorders. We define some neurological conditions, for which there is a particular interest in noninvasive investigations of endolymphatic hydrops.

Predictive accuracy of wideband absorbance in children with large vestibular aqueduct syndrome: A single-center retrospective study

“…LVAS is diagnosed by radiological techniques (e.g., CT scan) according to VA size: a VA diameter of 1.5 mm or more at the operculum and 1/2 of the vestibular aperture indicates LVAS [ 2 ]. Enlarged VA may also alter cerebrospinal fluid (CSF) pressure dynamics in the inner ear [ 10 , 11 ]. CSF reaches the inner ear through the VA, the cochlear aqueduct, and the internal auditory canal [ 11 ].…”

“…Enlarged VA may also alter cerebrospinal fluid (CSF) pressure dynamics in the inner ear [ 10 , 11 ]. CSF reaches the inner ear through the VA, the cochlear aqueduct, and the internal auditory canal [ 11 ]. Changes in intracranial pressure in LVAS patients can propagate to the inner ear via the enlarged VA and cause excessive cochlear pressure.…”

Cranial nerve abnormalities in spontaneous intracranial hypotension and their clinical relevance

“…These symptoms are likely due to neuropathy affecting the abducens nerve (CN 6), the oculomotor nerve (CN 3), and the trochlear nerve (CN 4) with the former two being the most common 10,25–28 . CN 8 may also be affected, resulting in hearing and vestibular symptoms, including hearing loss, hyperacusis, tinnitus, positional nystagmus, and vertigo 10,29–31 . Although considered infrequent, there have been reports of facial numbness and paralysis as well as dysgeusia (altered taste) encompassing neuropathies in CNs 5, 7, 9, and 10 10,21,32,33 …”

“…10,[25][26][27][28] CN 8 may also be affected, resulting in hearing and vestibular symptoms, including hearing loss, hyperacusis, tinnitus, positional nystagmus, and vertigo. 10,[29][30][31] Although considered infrequent, there have been reports of facial numbness and paralysis as well as dysgeusia (altered taste) encompassing neuropathies in CNs 5, 7, 9, and 10. 10,21,32,33 As the incidence of associated CN symptoms and their corresponding abnormalities on MRI have not been widely described, this study is a retrospective review of CN symptoms and corresponding MRI findings in patients diagnosed and treated for SIH to better understand the association between SIH, cranial neuropathies, and imaging findings both before and after treatment.…”