Hearing abnormalities in multiple sclerosis: clinical semiology and pathophysiologic mechanisms

Roberto A. Cruz,Thomas Varkey, Ana Flavia, Ana Paula Amatuzzi Samways, Alejandro Garza, Gabrielle Greenlee, Margaret Friess, Jayne Sconzert, Afaf Aijaz, Walter Arruda, Juliana Khouri,Kent Ellington,Teresa C. Frohman,Elliot M. Frohman

Journal of Neurology(2022)

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摘要
Auditory manifestations from multiple sclerosis (MS) are not as common as the well-recognized sentinel exacerbations of optic neuritis, partial myelitis, motor weakness, vertiginous episodes, heat intolerance, and eye movement abnormalities. This paper discusses four cases of auditory changes, secondary to MS, and describes the first case, to our knowledge, of palinacousis, the perseveration of hearing, despite cessation of the sound stimulus. For each we characterize the initial complaint, the diagnostic work up, and ultimately, underscore the individualized treatment interventions, that allowed us to achieve a remission in all four cases. Individually codifying the treatment regimens served to mitigate, if not to abolish, the clinical derangements in hearing. Special attention is focused upon examination of the clinical manifestations and the pathophysiologic mechanisms which are responsible for them. We further emphasize the differential diagnostic considerations, and physical exam findings, along with the results of laboratory testing, neuro-imaging sequences, and lesion localization. Taken together, such information is germane to organizing cogently coherent strategic treatment plan(s). We believe that this small case series represents a clinically pragmatic example of ‘precision medicine’; a principal theme and goal throughout this paper, the achievement of such in MS, but also as an illustration for the assessment and management schema for neuroimmunologic disorders in general.
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关键词
Sensorineural hearing loss, Palinacousis, Multiple sclerosis, Cranial nerve VIII, Auditory nuclei, Trapezoid body, Lateral lemniscus, Inferior colliculus, Medial geniculate nucleus, Superior temporal gyrus, Heschl’s gyrus, Brodmann area 41
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