Skip to main content Accessibility help

Audio-vestibular manifestations of Chiari malformation and outcome of surgical decompression: A case report

  • A. U. Ahmmed (a1), I. Mackenzie (a1), V. K. Das (a1), S. Chatterjee (a2) and R. H. Lye (a2)...


Sensorineural hearing loss, tinnitus, dizziness and ataxia are recognised symptoms associated with Chiari malformations but they are rarely the presenting complaints. Patients with such symptoms are frequently referred to otolaryngologists and audiological physicians. We report a case of a 13-year-old girl who presented complaining of tinnitus and impaired hearing, and was subsequently diagnosed as having a type I Chiari malformation. Pure tone audiogram showed a mild hearing impairment on the left side and the speech audiogram was normal. Auditory brain stem responses and the electronystagmography were abnormal. The patient underwent posterior fossa decompression following which her tinnitus disappeared, the hearing problem recovered and some of the abnormal electrophysiological parameters were corrected.


Corresponding author

A. U. Ahmmed, F.R.C.S., M.Sc., Senior registrar in Audiological Medicine, Manchester Royal Infirmary, Oxford Road, Manchester.


Hide All
Albers, F. W. J., Ingels, K. J. A. O. (1993) Clinical records Otoneurological manifestations in Chiari I malformation. Journal of Laryngology and Otology 107: 441443.
Arnold, A. C., Baloh, R. W., Yee, R. D., Hepler, R. S. (1990) Internuclear ophthalmoplegia in the Chiari type II malformation. Neurology 40(12): 18501854.
Barber, H. O., Stockwell, C. O. W. (1980) Manual of Electronystagmography. 2nd Edition. C.V. Mosby Co., p 94.
Bertrand, R. A., Martinez, S. N., Robert, F. (1973) Vestibular manifestations of cerebellar ectopia. Advances in Otorhinolaryngology 19: 355366.
Bronstein, A. M., Miller, D. H., Rudge, P., Kendal, B. E. (1987) Down beating nystagmus: magnetic resonance imaging and neuro-otological findings. Journal of Neurological Sciences (Netherlands) 81: 173184.
Camarda, R. M., Raimondo, D., Rossetti, M. (1991) Convergence nystagmus associated with Arnold Chiari malformation. Archives of Neurology 48(2): 131132.
Carmel, P. W., Markesbery, W. R. (1972) Early descriptions of Arnold Chiari malformation. The contribution of John Cleland. Journal of Neurosurgery 37: 543547.
Chait, G. E., Barber, H. O. (1979) Arnold Chiari malformation – some otoneurological features. Journal of Otolaryngology 8(1): 6570.
Chiari, H. (1891) Ueber Veranderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Deutsche Medizinische Wochenschr 17: 11721175.
Cleland, J. (1883) Contribution to the study of spina bifida encephalocele and anencephalus. Journal of Anatomy and Physiology 17: 257291.
Cogan, D. G. (1968) Down beat nystagmus. Archives of Ophthalmology 80: 757768.
Faria, M. A., Spector, R. H., Tindall, G. T. (1980) Downbeat nystagmus as the salient manifestation of Arnold Chiari malformation. Surgical Neurology 13: 333336.
Hendrix, R. A., Bacon, C. K., Sclafani, A. P. (1992) Chiari I malformation associated with asymmetrical sensorineura hearing loss. Journal of Otolaryngology 21(2): 102110.
Holliday, P. O. IIIrd, Pillsbury, D., Kelly, D. L. Jr, Dillard, R. (1985) Brainstem auditory evoked potentials in Arnold Chiari malformation: possible prognostic value and changes with surgical decompression. Neurosurgery 16(1): 4853.
Kumar, A., Pieri, A., Krol, G. (1993) Rebound caloric nystagmus. Laryngoscope 103: 12051213.
Longridge, N. S., Mallinson, A. L. (1985) Arnold Chiari malformation and the otolaryngologist: place of magnetic resonance imaging and electronystagmography. Laryngoscope 95(3): 335339.
Mohr, P. D., Strang, F. A., Sambrook, M. A., Boddie, H. G. (1977) The clinical and surgical features in 40 patients with primary cerebellar ectopia (adult Chiari malformation). Quarterly Journal of Medicine 46: 8596.
Mori, K., Uchida, Y., Nishimura, T., Eghwrudjakpor, P. (1988) Brainstem auditory evoked potentials in Chiari-II malformation. Child's Nervous System 4: 154157.
Mossman, S. S., Bronstein, A. M., Gresty, M. A., Kendall, B., Rudge, P. (1990) Convergence nystagmus associated with Arnold Chiari malformation. Archives of Neurology 47(3): 357359.
Paul, K. S., Lye, R. H., Strang, F. A., Dutton, J. (1983) Arnold Chiari malformation. Review of 71 cases. Journal of Neurosurgery 58: 183187.
Rydell, R. E., Pulec, J. L. (1971) Arnold Chiari malformation, neuro-otologic symptoms. Archives of Otolaryngology 94: 812.
Schwalbe, E., Gredig, M. (1907) Ueber Entwicklungs-storungendes Kleinhirns, Hirnstamms and Halsmarks bei Spina bifida (Arnold'sche und Chiari'sche Missbildung). Beitraege zur Pathologischen Anatomie 40: 132194.
Stone, J. L., Bouffard, A., Morris, R., Hovsepian, W., Mayers, H. L. (1983) Clinical and electrophysiological recovery in Arnold Chiari malformation. Surgical Neurology 20: 313319.
Waber, P. CCass, S. P. (1993) Neurotologic manifestations of Chiari I malformation. Otolaryngology – Head and Neck Surgery 109(5): 853860.
Yee, R. D., Baloh, R. W., Honrubia, V. (1984) Episodic vertical oscillopsia and downbeat nystagmus in a Chiari malformation. Achives of Ophthalmology 102(5): 723725.
Yeow, Y. K., Tjia, T. L. (1989) The localising value of downbeat nystagmus. Singapore Medical Journal 30(3): 273276.
Zimmerman, C. F., Roach, E. S., Troost, B. T. (1986) See-sawnystagmus associated with Chiari malformation. Archives of Neurology 43(3): 299300.



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed