Skip to main content Accessibility help
×
Home

Bilateral, isolated, lateral semicircular canal malformation without hearing loss

  • I Dallan (a1), S Berrettini (a2), E Neri (a3) and A P Casani (a2)

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.

Copyright

Corresponding author

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

References

Hide All
1Johnson, J, Lawlani, AK. Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope 2000;110:1673–9
2Antonelli, PJ, Varela, AE, Mancuso, AA. Diagnostic yield of high-resolution computed tomography for pediatric sensorineural hearing loss. Laryngoscope 1999;109:1642–7
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;129:637–46
4Rodriguez, K, Shah, RK, Kenna, M. Anomalies of the middle and inner ear. Otolaryngol Clin North Am 2007;40:8197
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: 212–17
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;10:114–18
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;95103
8Jackler, RK, Luxford, WM, House, WF. Congenital malformations of the inner ear: a classification based on organogenesis. Laryngoscope 1987;97(suppl 40):214
9Parnes, LS, Chernoff, WG. Bilateral semicircular canal aplasia with near-normal cochlear development. Ann Otol Rhinol Laryngol 1990;99:957–9
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;129–45
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;126:2335–43
12Hadrys, T, Braun, T, Rinkwitz-Brandt, S, Arnold, HH, Bober, E. Nkx5-1 controls semicircular canal formation in the mouse inner ear. Development 1998;125:33–9

Keywords

Metrics

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