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Int J Pediatr Otorhinolaryngol. 2017 Apr;95:5-8. doi: 10.1016/j.ijporl.2017.01.028. Epub 2017 Jan 29.

Inner ear anomalies in children with isolated unilateral congenital aural atresia.

Author information

1
Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA.
2
Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 1800 Orleans Street, Baltimore, MD, 21287, USA.
3
Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA. Electronic address: ntodd@emory.edu.

Abstract

OBJECTIVES/HYPOTHESIS:

We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia.

METHODS AND MATERIALS:

Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve.

RESULTS:

Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P < 0.001 and P = 0.04, respectively).

CONCLUSION:

Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency.

KEYWORDS:

Aural atresia; Inner ear; Oval window; Round window

PMID:
28576532
DOI:
10.1016/j.ijporl.2017.01.028
[Indexed for MEDLINE]

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