Clin Exp Otorhinolaryngol.  2019 May;12(2):176-180. 10.21053/ceo.2018.00857.

Analysis of Inner Ear Anomalies in Unilateral Congenital Aural Atresia Combined With Microtia

• Park E
• Lee G
• Jung HH
• Im GJ

• KMID: 2447426
• DOI: http://doi.org/10.21053/ceo.2018.00857

Abstract

OBJECTIVES
The aim of this study was to analyze the incidence of inner ear anomalies in patients with unilateral congenital aural atresia (CAA) combined with microtia.
METHODS
We retrospectively reviewed 61 patients with unilateral CAA combined with microtia who underwent high-resolution temporal bone computed tomography (TBCT) and hearing examination. Inner ear anomalies were analyzed using TBCT and evaluated according to the Jahrsdoerfer grading system, Marx classification, and extent of inferior displacement of the mastoid tegmen.
RESULTS
Inner ear anomalies were observed in 14 patients (23.0%). Lateral semicircular canal (LSCC) dysplasia was the most common inner ear anomaly, with an incidence of 16.4%. The incidence was significantly higher on the pathologic side than on the unaffected side (P=0.002). All vascular anomalies were observed in the high-riding jugular bulb, with an incidence of 24.6%. The incidence was significantly higher on the pathologic side than on the unaffected side (P<0.001). LSCC dysplasia was significantly more common in patients with a lower Jahrsdoerfer score (odds ratio, 0.66; P=0.004).
CONCLUSION
The incidence of inner ear anomalies was relatively high in patients with unilateral CAA combined with microtia; LSCC dysplasia was the most common anomaly and the probability of coexistence was higher in patients with a lower Jahrsdoerfer score.

Keyword

Inner Ear; Anomalies; Aural Atresia, Congenital; Congenital Microtia; Semicircular Canals

MeSH Terms

Classification
Congenital Microtia*
Ear, Inner*
Hearing
Humans
Incidence
Mastoid
Retrospective Studies
Semicircular Canals
Temporal Bone

Figure

• 

  Fig. 1. The extent of inferior displacement of the mastoid tegmen was calculated as the ratio of middle ear height to mastoid tegmen displacement (yellow arrows, middle ear height; red arrow, mastoid tegmen displacement).

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  Fig. 2. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing the dysmorphic features of the lateral semicircular canal (arrows).

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  Fig. 3. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing the dilated vestibular morphology (arrows).

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  Fig. 4. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing the medio-inferiorly displaced tympanic segment of the facial nerve (arrows).

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  Fig. 5. High-resolution temporal bone computed tomography images of unilateral congenital aural atresia combined with microtia. Axial (A) and coronal (B) views showing high-riding jugular bulb (arrows).

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