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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1984 Jan;47(1):26–32. doi: 10.1136/jnnp.47.1.26

Neuro-otological abnormalities in Friedreich's ataxia.

J Ell, D Prasher, P Rudge
PMCID: PMC1027637  PMID: 6607322

Abstract

Ten patients with an accepted diagnosis of Friedreich's ataxia have been examined neuro-otologically, and oculomotor, vestibular and auditory function assessed. Brainstem auditory evoked potentials (BAEPs) were also recorded. A high incidence of various eye movement disorders was noted. Some of these were indicative of cerebellar dysfunction. Reduced vestibular function and impaired hearing were common to most of the patients. BAEPs were also abnormal in the majority; reasons underlying these abnormalities are discussed. Neuro-otologically, the patients did not constitute an homogeneous group. The findings cast doubt upon the accuracy and validity of the currently accepted criteria for the diagnosis and classification of the spinocerebellar degenerations.

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Selected References

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  1. Baloh R. W., Konrad H. R., Honrubia V. Vestibulo-ocular function in patients with cerebellar atrophy. Neurology. 1975 Feb;25(2):160–168. doi: 10.1212/wnl.25.2.160. [DOI] [PubMed] [Google Scholar]
  2. Barbeau A. Friedreich's ataxia 1976-an overview. Can J Neurol Sci. 1976 Nov;3(4):389–397. doi: 10.1017/s0317167100025646. [DOI] [PubMed] [Google Scholar]
  3. Dell'Osso L. F., Abel L. A., Daroff R. B. "Inverse latent" macro square-wave jerks and macro saccadic oscillations. Ann Neurol. 1977 Jul;2(1):57–60. doi: 10.1002/ana.410020109. [DOI] [PubMed] [Google Scholar]
  4. Geoffroy G., Barbeau A., Breton G., Lemieux B., Aube M., Leger C., Bouchard J. P. Clinical description and roentgenologic evaluation of patients with Friedreich's ataxia. Can J Neurol Sci. 1976 Nov;3(4):279–286. doi: 10.1017/s0317167100025464. [DOI] [PubMed] [Google Scholar]
  5. Harding A. E. Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features. Brain. 1981 Sep;104(3):589–620. doi: 10.1093/brain/104.3.589. [DOI] [PubMed] [Google Scholar]
  6. Hood J. D., Kayan A., Leech J. Rebound nystagmus. Brain. 1973 Sep;96(3):507–526. doi: 10.1093/brain/96.3.507. [DOI] [PubMed] [Google Scholar]
  7. Igarashi M., Miller R. H., O-Uchi T., King A. I. Temporal bone findings in Friedreich's ataxia. ORL J Otorhinolaryngol Relat Spec. 1982;44(3):146–155. doi: 10.1159/000275588. [DOI] [PubMed] [Google Scholar]
  8. Kirkham T. H., Guitton D., Katsarkas A., Kline L. B., Andermann E. Oculomotor abnormalities in Friedreich's ataxia. Can J Neurol Sci. 1979 May;6(2):167–172. doi: 10.1017/s0317167100119584. [DOI] [PubMed] [Google Scholar]
  9. Nuwer M. R., Perlman S. L., Packwood J. W., Kark R. A. Evoked potential abnormalities in the various inherited ataxias. Ann Neurol. 1983 Jan;13(1):20–27. doi: 10.1002/ana.410130106. [DOI] [PubMed] [Google Scholar]
  10. Sasaki S., Shimazu H. Reticulovestibular organization participating in generation of horizontal fast eye movement. Ann N Y Acad Sci. 1981;374:130–143. doi: 10.1111/j.1749-6632.1981.tb30866.x. [DOI] [PubMed] [Google Scholar]
  11. Satya-Murti S., Cacace A., Hanson P. Auditory dysfunction in Friedreich ataxia: result of spiral ganglion degeneration. Neurology. 1980 Oct;30(10):1047–1053. doi: 10.1212/wnl.30.10.1047. [DOI] [PubMed] [Google Scholar]
  12. Sharpe J. A., Herishanu Y. O., White O. B. Cerebral square wave jerks. Neurology. 1982 Jan;32(1):57–62. doi: 10.1212/wnl.32.1.57. [DOI] [PubMed] [Google Scholar]
  13. Spoendlin H. Optic cochleovestibular degenerations in hereditary ataxias. II. Temporal bone pathology in two cases of Friedreich's ataxia with vestibulo-cochlear disorders. Brain. 1974 Mar;97(1):41–48. doi: 10.1093/brain/97.1.41. [DOI] [PubMed] [Google Scholar]
  14. URICH H., NORMAN R. M., LLOYD O. C. Suprasegmental lesions in Friedreich's ataxia. Confin Neurol. 1957;17(6):360–371. doi: 10.1159/000105212. [DOI] [PubMed] [Google Scholar]
  15. Zee D. S., Robinson D. A. A hypothetical explanation of saccadic oscillations. Ann Neurol. 1979 May;5(5):405–414. doi: 10.1002/ana.410050502. [DOI] [PubMed] [Google Scholar]
  16. Zee D. S., Yee R. D., Cogan D. G., Robinson D. A., Engel W. K. Ocular motor abnormalities in hereditary cerebellar ataxia. Brain. 1976 Jun;99(2):207–234. doi: 10.1093/brain/99.2.207. [DOI] [PubMed] [Google Scholar]

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