Ann Rehabil Med.  2020 Jun;44(3):256-259. 10.5535/arm.19148.

Rare Occurrence of Internal Auditory Canal Stenosis Accompanied With Congenital Facial Palsy in a 3-Month-Old Infant: A Case Report

• Park SH
• Kim WJ
• Lim YJ
• On CW
• Park JH
• Min EH

• KMID: 2504446
• DOI: http://doi.org/10.5535/arm.19148

Abstract

Internal auditory canal (IAC) stenosis with hypoplasia of the facial and vestibulocochlear nerves is a rare cause of congenital facial palsy. In this case report, a 3-month-old female infant was referred for a neurological developmental assessment for developmental delay and congenital facial palsy. Upon evaluation of developmental delay, hearing loss was detected. Following a magnetic resonance imaging scan of the brain and a computed tomography scan of the temporal bone, IAC stenosis with hypoplasia of facial and vestibular nerves was diagnosed. This is a rare case of IAC stenosis in an infant with initial presentations of left facial palsy and developmental delay associated with hearing loss in the left ear. We strongly suggest that IAC stenosis be considered a cause of congenital facial palsy in infants, especially in patients with developmental delay. In infants with congenital facial palsy, a thorough physical examination and neurological developmental assessment should be performed.

Keyword

Developmental delay disorder; Facial paralysis; Internal auditory canal

Figure

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  Fig. 1. A 3-month-old infant with left facial palsy is shown. An incomplete closure of the left eye (A) and a deviation of the mouth angled to the right (B) are seen when she is crying.

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  Fig. 2. Audio brainstem response data are shown: the patient’s right ear (A) and left ear (B). The data show conduction thresholds of 50 dB nHL (right ear) and 100 dB nHL (left ear).

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  Fig. 3. An oblique sagittal magnetic resonance (MR) image of the both internal auditory canal (IAC), right (A) and left (B). The MR image shows poor delineation of the left facial and vestibulocochlear nerves and a relatively small diameter of nerves; this suggests hypoplasia of the left facial and vestibulocochlear nerves in the IAC. Each arrow indicates IAC with facial and vestibulocochlear nerves.

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  Fig. 4. A coronal computed tomography (CT) image of the patient’s right and left internal auditory canal (IAC). The CT image shows a narrowing of the left IAC (2.8 mm) compared with the right IAC (3.9 mm); this suggests congenital IAC stenosis. Arrows indicate bilateral IACs.

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