Agenesis of the lateral semicircular canal: a case study


Authors: R. Brzezný 1;  R. Černý 2;  M. Kynčl 3;  P. Vrabec 4
Authors‘ workplace: Oddělení neurochirurgie FN v Motole, Praha 1;  Neurologická klinika dospělých 2. LF UK a FN v Motole, Praha 2;  Klinika zobrazovacích metod 2. LF UK a FN v Motole, Praha 3;  Klinika ORL a chirurgie hlavy a krku 1. LF UK a FN v Motole, Praha 4
Published in: Cesk Slov Neurol N 2008; 71/104(1): 81-84
Category: Case Report

Overview

The objective of the case study is to point out the importance of differential diagnosis of a rare otological disorder, in which congenital dysplasia of the inner ear is not to be omitted. A 63 year old patient diagnosed with an internal disease and with otological history of progressive hypoacusis and repeated surgeries due to exostoses and tympanosclerosis developed acute peripheral vestibular syndrome due to a fall and an injury. In view of the history of a rare otological disease, HRCT was performed showing agenesis of the lateral semicircular canal, with the failure in canal function confirmed by a head impulse test. Patients with lateral semicircular canal dysplasia present with evident findings in clinical examination as well as in ancillary and imaging methods, both before and during disease manifestation. Pyramid HRCT is a fast and accessible method to confirm the above congenital vestibular apparatus disorder with suspect anamnesis. The detection of patients with the above-mentioned congenital disorder is important in the prevention of injuries caused by falls in old age.

Key words:
congenital dysplasia, semicircular canals, HRCT, head impulse test, hearing disorder, falls


Sources

1. Brandt T, Dieterich M. Vestibular falls. J Vestib Res 1993; 3(1): 3-14.

2. Topinková E, Neuwirth J. Geriatrie pro praktického lékaře. 1. ed. Praha: Grada Publishing 1995.

3. Sando I, Takahara T, Ogawa A. Congenital anomalies of the inner ear. Ann Otol Rhinol Laryngol Suppl 1984; 112: 110-118.

4. Sennaroglu L, Saatci I. A New Classification for Cochleovestibular Malformations. Laryngoscope 2002; 112(12): 2230-2241.

5. Asiri S, Hasham A, al Anazy F, Zakzouk S, Banjar A. Tympanosclerosis: review of literature and incidence among patients with middle-ear infection. J Laryngol Otol 1999; 113(12): 1076-1080.

6. Timofeev I, Notkina N, Smith IM. Exostoses of the external auditory canal: a long-term follow-up study of surgical treatment. Clinical Otolaryngology & Allied Sciences 2004; 29(6): 588-594.

7. Brzezny R, Vyhnálek M, Jeřábek J. Centrální a periferní polohové závrativé stavy. Cesk Slov Neurol N 2005; 68/101(3): 148-153.

8. Halmagyi GM, Colebatch JG, Curthoys IS. New tests of vestibular function. Baillieres Clin Neurol 1994; 3(3):485-500.

9. Brzezny R, Glasauer S, Bayer O, Siebold Ch, Buttner U. Head Impulses in Three Orthogonal Planes of Space - The Influence of Age. Ann N Y Acad Sci. 2003; 1004: 473-477.

10. Brzezny R, Vyhnalek M, Cerny R, Jerabek J. Onemocnění otolitových struktur rovnovážného systému. I. Patofyziologie a symptomatologie. Cesk Slov Neurol N 2006; 69/102(4): 259-266.

11. Brzezny R, Vyhnalek M, Cerny R, Jerabek J. Onemocnění otolitových struktur rovnovážného systému. II. Diagnostika. Cesk Slov Neurol N 2006; 69/102(4): 267-271.

12. Sasaki O, Otsuka A, Asawa S, Sakaguchi M, Ishiyama T, Ezawa S et al. Neurotological evaluation of vertical semicircular canal function in inner ear malformation. A case report. ORL J Otorhinolaryngol Relat Spec 1999; 61(6):355-359.

13. McClay JE, Tandy R, Grundfast K, Choi S, Vezina G, Zalzal G et al. Major and minor temporal bone abnormalities in children with and without congenital sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2002; 128(6):664-671.

14. Okumura T, Takahashi H, Honjo I, Takagi A, Mitamura K. Sensorineural hearing loss in patients with large vestibular aqueduct. Laryngoscope 1995; 105(3 Pt 1):289-293.

15. Johnson JM, Lalwani K. Sensorineural and Conductive Hearing Loss Associated With Lateral Semicircular Canal Malformation. Laryngoscope 2000; 110(10): 1673-1679.

16. Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography. Arch Otolaryngol 1974; 100(1):11-18.

17. Murofushi T, Ouvrier RA, Parker GD, Graham RI, da Silva M, Halmagyi GM. Vestibular abnormalities in charge association. Ann Otol Rhinol Laryngol 1997; 106(2):129-134.

Labels
Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 1

2008 Issue 1

Most read in this issue

This topic is also in:


Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account