Malformations of the lateral semicircular canal correlated with data from the audiogram

  • Aina VenkatasamyEmail author
  • Daphné Le Foll
  • Carine Eyermann
  • Hella Vuong
  • Dominique Rohmer
  • Anne Charpiot
  • Francis Veillon



Lateral semicircular canal (LSCC) malformations  are one of the most common inner ear malformations. The purpose of this study is to analyze the prevalence and type of hearing losses associated with LSCC malformations, compared to a control group.

Materials and methods

We retrospectively included 109 patients (166 ears) presenting with a CT-confirmed LSCC malformation, compared to a control group (24 patients). The bony island surface and the width of the inner portion of the LSCC were measured to confirm the malformation. There results were correlated to audiogram data: sensorineural (SHNL), mixed (MHL) or conductive hearing loss (CHL) by an otologist.


In the LSCC group, 60.9% of patients presented with an audiogram-confirmed hearing loss, especially SNHL (39.2%, n = 65) and MHL (12.7%, n = 21). Hearing was normal in 39.2% (n = 65) of the cases. Bilateral LSCC malformations (n = 57) were frequently associated with hearing loss (80.7%), SNHL in most of the cases (33.3%). Unilateral LSCC malformations were associated with hearing alterations (51.9%, n = 27), but we also observed a high rate (81%, n = 42) of contralateral abnormalities of the audiogram.


LSCC malformations are commonly associated with hearing loss (61%), especially SHNL (39%). The high rate (81%) of contralateral hearing disturbances in unilateral LSCC malformations should be taken into account in the patient’s daily life to avoid triggering or exacerbating any hearing loss. Otologists and radiologists must cooperate to ensure that all malformations are correctly described on CT, especially to improve the patient’s education regarding hearing preservation.


Lateral semicircular canal Congenital Malformation Hearing loss Audiogram 


Compliance with ethical standards

Conflict of interest

The authors have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all participants included in the study.


  1. 1.
    Johnson J, Lalwani KA (2000) Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope 110(10):1673–1679CrossRefPubMedGoogle Scholar
  2. 2.
    Purcell DD, Fischbein NJ, Patel A, Johnson J, Lalwani AK (2006) Two temporal bone computed tomography measurements increase recognition of malformations and predict sensorineural hearing loss. Laryngoscope 116(8):1439–1446CrossRefPubMedGoogle Scholar
  3. 3.
    Jackler RK, Luxfor WM, House WF (1987) Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 97(S40):2–14CrossRefPubMedGoogle Scholar
  4. 4.
    O’Rahilly R (1963) The early development of the otic vesicle in staged human embryos. Development 11(4):741–755Google Scholar
  5. 5.
    Phelps PD (1974) Congenital lesions of the inner ear, demonstrated by tomography: a retrospective study of 34 cases with special reference to the lateral semicircular canal. Arch Otolaryngol 100(1):11–18CrossRefPubMedGoogle Scholar
  6. 6.
    Matsunaga T, Hirota E (2003) Familial lateral semicircular canal malformation with external and middle ear abnormalities. Am J Med Genet Part A 116(4):360–367CrossRefGoogle Scholar
  7. 7.
    Dallan I, Berrettini S, Neri E, Casani AP (2008) Bilateral, isolated, lateral semicircular canal malformation without hearing loss. J Laryngol Otol 122(8):858–860CrossRefPubMedGoogle Scholar
  8. 8.
    Ozeki M, Kato Z, Sasai H, Kubota K, Funato M, Orii K et al (2009) Congenital inner ear malformations without sensorineural hearing loss in children. Int J Pediatr Otorhinolaryngol 73(10):1484–1487CrossRefPubMedGoogle Scholar
  9. 9.
    Verheij E, Elden L, Crowley TB, Pameijer FA, Zackai EH, McDonald-McGinn DM, Thomeer HG (2018) Anatomic malformations of the middle and inner ear in 22q11. 2 deletion syndrome: case series and literature review. Am J Neuroradiol 39(5):928–934CrossRefPubMedGoogle Scholar
  10. 10.
    Lan MY, Shiao JY, Ho CY, Hung HC (2009) Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss. Eur Arch Otorhinolaryngol 266(9):1361–1364CrossRefPubMedGoogle Scholar
  11. 11.
    Anson BJ, Donaldson JA (1973) Surgical anatomy of the temporal bone and ear, 3rd edn. WB Saunders Company, PhiladelphiaGoogle Scholar
  12. 12.
    Zalzal GH, Tomaski SM, Vezina LG, Bjornsti P, Grundfast KM (1995) Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg 121(1):23–28CrossRefPubMedGoogle Scholar
  13. 13.
    Piergallini L, Scola E, Tuscano B, Brambilla R, Campoleoni M, Raimondi G et al (2018) Flat-panel CT versus 128-slice CT in temporal bone imaging: assessment of image quality and radiation dose. Eur J Radiol 106:106–113CrossRefPubMedGoogle Scholar
  14. 14.
    Conte G, Scola E, Calloni S, Brambilla R, Campoleoni M, Lombardi L, Sina C (2017) Flat panel angiography in the cross-sectional imaging of the temporal bone: assessment of image quality and radiation dose compared with a 64-section multisection CT scanner. Am J Neuroradiol 38:1996–2002Google Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Service de Radiologie 1Hôpital de Hautepierre, Hôpitaux Universitaires de StrasbourgStrasbourg CEDEXFrance
  2. 2.Service de chirurgie ORLHôpitaux Universitaires de StrasbourgStrasbourg CEDEXFrance
  3. 3.Université de Strasbourg, INSERM IRFAC UMR_S1113, Laboratory Stress Response and Innovative Therapy “Streinth”StrasbourgFrance

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