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Double impairment: clinical identification of 33 cases of cerebellar ataxia with bilateral vestibulopathy.
Otolaryngol Head Neck Surg. 2012 May; 146(5):804-8.OH

Abstract

OBJECTIVE

Following recently described small series of patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV), the authors undertook a careful clinical and laboratory assessment of patients who presented to their unit with characteristics of this syndrome.

STUDY DESIGN

Case note review.

SETTING

Tertiary, university-based, multidisciplinary neurootology clinic.

SUBJECTS

Thirty-three patients whose characteristics fit this syndrome.

METHODS

Patients presenting to the Multidisciplinary Neurotology Clinic with characteristics of CABV were entered into a bespoke database. This was analyzed to identify the clinical findings and results of vestibular investigations for this group.

RESULTS

Patients presented at a mean age of 54 years (SD, 17.6) with symptoms having been present for a median of 3 years (interquartile ratio, 2.0-9.5). Caloric testing greatly underestimated the disorder, being subnormal in only 18% of patients; the head-thrust test was abnormal and dynamic visual acuity testing was abnormal 88% and 91% of the time, respectively. Of the patients, 76% demonstrated gaze-evoked nystagmus. Impaired smooth pursuit (97% of patients showed low gain with saccadic corrections) and impaired cancellation of the vestibulo-ocular reflex (in 97% of patients) were found. Impaired saccular otolithic function was abnormal in 33%, adding to patient imbalance.

CONCLUSION

The unique double-pathway balance impairment in CABV patients causes a high prevalence of subnormal function of both central and peripheral vestibular function. This is an easily missed clinical entity that is often associated with normal caloric investigations. As many patients with this syndrome are poor candidates for vestibular rehabilitation therapy, resources are better devoted to the early implementation of assistance with their safe ambulation and activities of daily living.

Authors+Show Affiliations

Department of Otolaryngology, Head and Neck Surgery, Toronto General Hospital, University Health Network, University of Toronto, Canada. mail@davepothier.comNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

22157390

Citation

Pothier, David D., et al. "Double Impairment: Clinical Identification of 33 Cases of Cerebellar Ataxia With Bilateral Vestibulopathy." Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery, vol. 146, no. 5, 2012, pp. 804-8.
Pothier DD, Rutka JA, Ranalli PJ. Double impairment: clinical identification of 33 cases of cerebellar ataxia with bilateral vestibulopathy. Otolaryngol Head Neck Surg. 2012;146(5):804-8.
Pothier, D. D., Rutka, J. A., & Ranalli, P. J. (2012). Double impairment: clinical identification of 33 cases of cerebellar ataxia with bilateral vestibulopathy. Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery, 146(5), 804-8. https://doi.org/10.1177/0194599811431788
Pothier DD, Rutka JA, Ranalli PJ. Double Impairment: Clinical Identification of 33 Cases of Cerebellar Ataxia With Bilateral Vestibulopathy. Otolaryngol Head Neck Surg. 2012;146(5):804-8. PubMed PMID: 22157390.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Double impairment: clinical identification of 33 cases of cerebellar ataxia with bilateral vestibulopathy. AU - Pothier,David D, AU - Rutka,John A, AU - Ranalli,Paul J, Y1 - 2011/12/09/ PY - 2011/12/14/entrez PY - 2011/12/14/pubmed PY - 2012/7/20/medline SP - 804 EP - 8 JF - Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery JO - Otolaryngol Head Neck Surg VL - 146 IS - 5 N2 - OBJECTIVE: Following recently described small series of patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV), the authors undertook a careful clinical and laboratory assessment of patients who presented to their unit with characteristics of this syndrome. STUDY DESIGN: Case note review. SETTING: Tertiary, university-based, multidisciplinary neurootology clinic. SUBJECTS: Thirty-three patients whose characteristics fit this syndrome. METHODS: Patients presenting to the Multidisciplinary Neurotology Clinic with characteristics of CABV were entered into a bespoke database. This was analyzed to identify the clinical findings and results of vestibular investigations for this group. RESULTS: Patients presented at a mean age of 54 years (SD, 17.6) with symptoms having been present for a median of 3 years (interquartile ratio, 2.0-9.5). Caloric testing greatly underestimated the disorder, being subnormal in only 18% of patients; the head-thrust test was abnormal and dynamic visual acuity testing was abnormal 88% and 91% of the time, respectively. Of the patients, 76% demonstrated gaze-evoked nystagmus. Impaired smooth pursuit (97% of patients showed low gain with saccadic corrections) and impaired cancellation of the vestibulo-ocular reflex (in 97% of patients) were found. Impaired saccular otolithic function was abnormal in 33%, adding to patient imbalance. CONCLUSION: The unique double-pathway balance impairment in CABV patients causes a high prevalence of subnormal function of both central and peripheral vestibular function. This is an easily missed clinical entity that is often associated with normal caloric investigations. As many patients with this syndrome are poor candidates for vestibular rehabilitation therapy, resources are better devoted to the early implementation of assistance with their safe ambulation and activities of daily living. SN - 1097-6817 UR - https://wwww.unboundmedicine.com/medline/citation/22157390/Double_impairment:_clinical_identification_of_33_cases_of_cerebellar_ataxia_with_bilateral_vestibulopathy_ L2 - https://journals.sagepub.com/doi/10.1177/0194599811431788?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -