Learning objectives
The objective of this review is to provide a practical anatomy overview of the three compartments of the ear and to summarize, classify and describe the most common congenital malformations (CM) and their main radiological findings on CT.
Background
INTRODUCTION
CM of the ear are usually the result of defects in the embryogenesis of the auditory system that compromises normal fetal development. The overall incidence of ear malformations is 1:3800 newborns [1], however, this figure varies greatly depending on the compartment affected and its etiology. CM may have a genetic or non-genetic background and they could appear in a syndromic context (Treacher-Collins, Pierre Robin, Goldenhar, or Kippel-Feil syndromes as the most common)[2].
Ear malformations may be silent but in most cases, they present as...
Findings and procedure details
EXTERNAL EAR
AURICLE
Auricular malformations (microtia) can affect the size, shape, position, and orientation of the pinna. Complete absence may also occur, and it is called anotia. Anterior to the auricle, ear appendages, ear sinus, and ear pits may also be found [4].Weerda's classification is the most accepted and commonly used for microtia at the present time [5].[Fig 6]
EAC
Failures in the canalization result in EAC malformations, stenotic or even absent canal. It can affect the fibrous canal, bony, or both. It must be...
Conclusion
CT imaging plays an important role in the clinical and surgical management of congenital ear malformations. There is a wide spectrum of malformations that could present isolated or associated with other systemic anomalies and radiologists should be aware of these scenarios.A detailed description is key to establishing an early diagnosis and treatment, in susceptible cases, and promoting the development of the child´s language and psychosocial skills.
Personal information and conflict of interest
M. Da Silva Torres:
Nothing to disclose
L. García Suárez:
Nothing to disclose
S. González Sánchez:
Nothing to disclose
Á. Meilán Martínez:
Nothing to disclose
M. Martínez-Cachero García:
Nothing to disclose
P. Corujo Murga:
Nothing to disclose
L. M. Guadalupe González:
Nothing to disclose
S. A. Jarreta:
Nothing to disclose
F. García Arias:
Nothing to disclose
References
Thorn L. Entwicklung des Ohres (einschließlich Entstehung von Missbildungen, experimentelle Embryologie und In-vitro-Studien) In: Helms J, editor.Oto-Rhino-Laryngologie in Klinik und Praxis. 1. Stuttgart: Thieme; 1994. pp. 1–22.
D’Arco F, Sanverdi E, O'Brien WT, et al. The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology 2020;62:539–44
Koral K. Embryology, anatomy, normal findings, and imaging techniques. In: Coley B, ed. Pediatric Diagnostic Imaging, 12th ed, Philadelphia: Elsevier Inc.; 2013:87–9.
Bartel-Friedrich S, Wulke C....