Vestibular Loss and Cerebellar Ataxia: A Practical Approach

Cerebellar ataxia has been the remit of neurology but increased awareness of genetic disorders affecting both cerebellar and vestibular function has led to a rise in their reported prevalence. It is not uncommon for such patients to be seen in otolaryngology clinics. This review explores the underlying etiologies of patients presenting with vestibular loss accompanied by signs of cerebellar ataxia and provides a practical approach to diagnosis. We provide a comprehensive overview of common conditions that can manifest with both vestibular dysfunction and cerebellar ataxia, acutely or chronically, including cerebellar strokes, thiamine deficiency, and neurodegenerative diseases such as spinocerebellar ataxias. The article explores key diagnostic approaches, including clinical examination, neuroimaging, and specialized vestibular testing, to aid in distinguishing these conditions. By focusing on practical aspects of diagnosis, we offer otolaryngology specialists an essential tool for more accurate identification and management of patients, aiming to improve patient care. We emphasize the importance of a systematic, multidisciplinary approach to managing complex vestibular cases.

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Publication Details

Journal
Ear and Hearing
Published
2025-08-12
DOI
https://doi.org/10.1097/aud.0000000000001708
Primary Topic
Genetic Neurodegenerative Diseases
Type
article
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article

Vestibular Loss and Cerebellar Ataxia: A Practical Approach

Lúcia Joffily, Diego Kaski, Paola Giunti, Nehzat Koohi et al.
Ear and Hearing
Genetic Neurodegenerative Diseases
article

Vestibular Loss and Cerebellar Ataxia: A Practical Approach

Lúcia Joffily, Diego Kaski, Paola Giunti, Nehzat Koohi, Alexander Andrea Tarnutzer, Syuzanna Simonyan
article en

Abstract

Cerebellar ataxia has been the remit of neurology but increased awareness of genetic disorders affecting both cerebellar and vestibular function has led to a rise in their reported prevalence. It is not uncommon for such patients to be seen in otolaryngology clinics. This review explores the underlying etiologies of patients presenting with vestibular loss accompanied by signs of cerebellar ataxia and provides a practical approach to diagnosis. We provide a comprehensive overview of common conditions that can manifest with both vestibular dysfunction and cerebellar ataxia, acutely or chronically, including cerebellar strokes, thiamine deficiency, and neurodegenerative diseases such as spinocerebellar ataxias. The article explores key diagnostic approaches, including clinical examination, neuroimaging, and specialized vestibular testing, to aid in distinguishing these conditions. By focusing on practical aspects of diagnosis, we offer otolaryngology specialists an essential tool for more accurate identification and management of patients, aiming to improve patient care. We emphasize the importance of a systematic, multidisciplinary approach to managing complex vestibular cases.

Ear and HearingVol. 47(1)
Universidade Federal do Rio de Janeiro (BR), University College London Hospitals NHS Foundation Trust (GB), University of Zurich (CH), Kantonsspital Baden (CH), National Hospital for Neurology and Neurosurgery (GB), University College London (GB), Yerevan State Medical University (AM), Universidade Federal do Estado do Rio de Janeiro (BR)
Good health and well-being
Openalex Percentile: Top 78%
Genetic Neurodegenerative Diseases
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Vestibular Loss and Cerebellar Ataxia: A Practical Approach — Lúcia Joffily, Diego Kaski, et al. · Ear and Hearing (2025) | TGRS Research Map | TGRS