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previously visited 081230 CONGENITAL INTERNAL AUDITORY CANAL STENOSIS Random Case
Authored By: pedrad CSH, Radiologist, Chung Shan Medical University Hospital, Taiwan.
Patient: 3 month old female
History:

Chief complain:
Asymmetric face since birth

Infant of diabetic mellitus mother.
Premature Rupture of the Membranes(PROM): +
Cesarean section with gestational age: 34+ 5/7 weeks.
Birthbody weight: 2400gm. G2P2
Apgar score: 5 (at 1st min) to 7 (at 5th min).

Images:[small]mediumlargeas-submittedimages only

Fig. 1: Photograph when crying

Fig. 2: Photograph (3 m/o)

Fig. 3: Otoacoustic emissions (OAE)

Fig. 4: Auditory brainstem response (ABR)

Fig. 5: Low dose multidetector row CT (axial)

Fig. 6: Low dose multidetector row CT (coronal)
Images:[small]mediumlargeas-submittedimages only
Findings:

Photograph when crying (figure 1)
1. Asymmetric face
2. Difficult closing of left eye
3. Paralysis of left side of the mouth.
4. From above findings, left side peripheral facial palsy is considered.

Photograph (3 m/o) (figure 2)
1. Similar finding as figure 1

Otoacoustic emissions (OAE) (figure 3)
1. Downward displacement of left ear results (red arrows) below
   normal lower limit (normal range between blue arrows), suggesting
   abnormal function of outer hair cells in left cochlear.
2. Normal right ear results (green arrows) between normal range.

Auditory brainstem response (ABR) (figure 4)
1. Poor morphology of left side with loss normal V wave in left side,
   suggesting sensorineural hearing impairment.
2. Normal right side for comparison. Normal V waves (arrows).

Low dose multidetector row CT scan of temporal bone with
 multiplanar reconstruction, and without intravenous contrast
 (figure 5 and 6):

1. Increased opacity in left middle ear cavity and left mastoid air
   cells, may due to otitis media and mastoiditis.
   Less possibility: middle ear soft tissue mass.
2. Very small caliber of the orifice of left internal auditory
   canal in vertical diameter (arrow in figure 6), suggesting
   congenital hypoplastic internal auditory canal and possibly
   associated sensorineural hearing impairment, peripheral facial
   nerve palsy, or dizziness and tinnitus.

DDx: Asymmetric crying facies (ACF) is caused by congenital hypoplasia
 or agenesis of the depressor anguli oris muscle (DAOM) on one side
 of the mouth. Patients with this anomaly usually present with a droop
 of one corner of the mouth on the intact side while crying or
 grimacing. Forehead wrinkling, eye closure and nasolabial fold depth
 remain intact and equal on both sides.
Diagnosis: Congenital unilateral internal auditory canal stenosis, left side, with ipsilateral peripheral facial palsy and sensorineural hearing impairment.
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Additional Details:

Case Number: 22280482Last Updated: 12-30-2008
Rating:

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Anatomy: Face and Neck   Pathology: Congenital
Modality: CT, Photograph, OtherAccess Level: Readable by all users
Keywords: internal auditory canal, facial palsy, sensorineural hearing, oae, abrACR: 2134.1421
Case has been viewed 253 times.

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