A 56-year-old woman with a history of uncontrolled hypertension, hyperlipidemia, and tobacco use developed sudden bilateral hearing loss preceded by one week of persistent vertigo. Physical examination demonstrated saccadic pursuit with spontaneous bilateral horizontal and vertical down-beating nystagmus, profound bilateral sensorineural hearing loss (bSNHL) confirmed by audiogram, and truncal ataxia. Neuroimaging showed occlusion of the right vertebral artery, basilar artery, and near-occlusion of bilateral anterior inferior cerebellar arteries (AICAs) (Figure 1) causing acute/subacute ischemic strokes (Figure 2). AICAs give rise to internal auditory arteries, almost a sole blood supply to inner ear, and supply anterior part of cerebellum, middle cerebellar peduncle, and flocculus. Sudden bSNHL is a rare phenomenon representing <5% of all acute SNHL cases dominated by unilateral presentation. In contrast to the latter, sudden bSNHL represents a medical emergency warranting immediate evaluation for life-threatening and/or reversible causes, such as stroke, autoimmune disorders (e.g., Susac, Cogan syndromes, and scleroderma), and drug toxicities (e.g., gentamicin).1 Occlusion/hypoperfusion of AICA in isolation or as part of vertebrobasilar ischemia can lead to sudden SNHL.2
The following structures are indicated: occlusion of right V4 vertebral artery (black arrowhead), patent left V4 vertebral artery (white arrowhead), occlusion of basilar artery (white arrow), and near-occlusion of bilateral AICAs (black arrows).
From right to left hyperintense signal on FLAIR, hyperintense areas of restricted diffusion on diffusion weighted imaging with correlating hypointense areas on apparent diffusion coefficient maps in bilateral anterior cerebellum (panel A), middle cerebellar peduncle, flocculus and pons (panels B and C).
Author Contributions
M. Buciuc, H. Ali, P. Gonzalez, C.L. Nankee, and C.A. Holmstedt: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data. M. Buciuc, C.L. Nankee, and C.A. Holmstedt: study concept or design. M. Buciuc, H. Ali, C.L. Nankee, and C.A. Holmstedt: analysis or interpretation of data.
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Resident and Fellow Section Editor Whitley Aamodt, MD, MPH.
- Received November 6, 2022.
- Accepted in final form April 7, 2023.
- © 2023 American Academy of Neurology
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