Hostname: page-component-6766d58669-88psn Total loading time: 0 Render date: 2026-05-17T19:50:52.581Z Has data issue: false hasContentIssue false
  • English
  • Français

Bilateral, isolated, lateral semicircular canal malformation without hearing loss

Published online by Cambridge University Press:  20 May 2008

I Dallan ,
S Berrettini ,
E Neri  and
A P Casani
I Dallan*
Affiliation:
Second ENT Unit, Azienda Ospedaliera Universitaria Pisana, Italy
S Berrettini
Affiliation:
First ENT Section, Neuroscience Department, University of Pisa, Italy
E Neri
Affiliation:
Division of Diagnostic and Interventional Radiology, Department of Oncology, University of Pisa, Italy
A P Casani
Affiliation:
First ENT Section, Neuroscience Department, University of Pisa, Italy
*
Address for correspondence: Dr Iacopo Dallan, 2nd ENT Unit, Azienda Ospedaliera Universitaria Pisana, Via Savi 10, 56126 Pisa, Italy. Fax: +39 050 993239 E-mail: iacopodallan@tiscali.it
Get access Rights & Permissions [Opens in a new window]

Abstract

Hypothesis:

Inner-ear malformations are frequently found in patients with sensorineural hearing loss. However, isolated anomalies of the vestibular part of the inner ear are seldom described, and for this reason their impact on balance is poorly understood.

Care report:

We present the case of a 38-year-old Caucasian man with recurrent vestibular complaints, with a sensation of linear tilting, but no hearing impairment. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was within normal limits. High-resolution magnetic resonance imaging of the inner ear, with three-dimensional reconstruction, demonstrated isolated vestibular anomalies involving both the lateral semicircular canal and the utricle.

Conclusions:

Bearing in mind this case, we speculate that isolated vestibular malformation may not be as rare as previously thought, and should be investigated with the aid of sophisticated imaging techniques. A review of the relative literature, focussing attention on the molecular aspects, is also reported.

Keywords

Semicircular CanalsInner EarSensorineural Hearing Loss

Information

Type
Clinical Record
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 8 , August 2008 , pp. 858 - 860
Copyright
Copyright © JLO (1984) Limited 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

References

1Johnson, J, Lawlani, AK. Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope 2000;110:1673–9CrossRefGoogle ScholarPubMed
2Antonelli, PJ, Varela, AE, Mancuso, AA. Diagnostic yield of high-resolution computed tomography for pediatric sensorineural hearing loss. Laryngoscope 1999;109:1642–7CrossRefGoogle ScholarPubMed
3Yu, KK, Mukherji, S, Carrasco, V, Pillsbury, HC, Shores, CG. Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases. Otolaryngol Head Neck Surg 2003;129:637–46CrossRefGoogle ScholarPubMed
4Rodriguez, K, Shah, RK, Kenna, M. Anomalies of the middle and inner ear. Otolaryngol Clin North Am 2007;40:81–97CrossRefGoogle ScholarPubMed
5Dallan, I, Bruschini, L, Neri, E, Nacci, A, Segnini, G, Rognini, F et al. The role of high-resolution magnetic resonance in atypical and intractable benign positional vertigo: our preliminary experience. ORL J Otorhinolaryngol Relat Spec 2007;69: 212–17CrossRefGoogle ScholarPubMed
6Neri, E, Caramella, D, Cosottini, M, Zampa, V, Jackson, A, Berrettini, S et al. High resolution magnetic resonance and volume rendering of the labyrinth. Eur Radiol 2000;10:114–18CrossRefGoogle ScholarPubMed
7Neri, E, Berrettini, S, Panconi, M. Middle and inner ear. In: Caramella, D, Bartolozzi, C, eds. 3D Image Processing (Techniques and Clinical Applications) Berlin: Springer Verlag, 2002;95–103Google Scholar
8Jackler, RK, Luxford, WM, House, WF. Congenital malformations of the inner ear: a classification based on organogenesis. Laryngoscope 1987;97(suppl 40):2–14CrossRefGoogle Scholar
9Parnes, LS, Chernoff, WG. Bilateral semicircular canal aplasia with near-normal cochlear development. Ann Otol Rhinol Laryngol 1990;99:957–9CrossRefGoogle ScholarPubMed
10Kenna, MA, Hirose, K. Embryology and developmental anatomy of the ear. In: Bluestone, CD, Stool, SE, Alpert, CM, eds. Pediatric Otolaryngology, 4th edn.Philadelphia: Saunders, 2003;129–45Google Scholar
11Morsli, H, Tuorto, F, Choo, D, Postiglione, MP, Simeone, A, Wu, DK. Otx1 and Otx2 activities are required for the normal development of the mouse inner ear. Development 1999;126:2335–43CrossRefGoogle ScholarPubMed
12Hadrys, T, Braun, T, Rinkwitz-Brandt, S, Arnold, HH, Bober, E. Nkx5-1 controls semicircular canal formation in the mouse inner ear. Development 1998;125:33–9CrossRefGoogle ScholarPubMed