This case was donated by Lindsay Baskin, AuD, of Broward County Schools.

We begin as the child, who has Crouzon Syndrome, is 1 years 8 months old, as we review the medical reports on file for Y.D.

But first, a question as always.

• Would you expect conductive or sensorineural loss with a craniofacial dysostosis such as Crouzon?

• After reading this first report, summarize it in more simple language.  What is wrong with Y.D.’s ears?

PATIENT:  Y.D.

AGE:  1 year, 8 months old

EXAM: CT of the internal auditory canals w/o contrast

INDICATION:  Hearing loss

DATE:  08/26/03

High resolution of the temporal bones was obtained in the axial and coronal planes without intravenous contrast using helical CT and 1mm intervals.

There is only partial visualization to the (right) external auditory canal.  There is marked diffuse narrowing to the external auditory canal.  There is diffuse sclerosis to the anterior and posterior aspects of the internal auditory canal.  The severe stenosis is visualized on axial images 27 and 28.  The tympanic membrane is not visualized.  There is a normal appearing malleus.  There is normal vestibule and cochlea.  The carotid canal is unremarkable.  The jugular bulb is unremarkable.  The facial nerve is normal.  The IAC is unremarkable.  There is no evidence for mastoiditis.  There is no evidence for cholesteatoma.  The tegmen is intact.  There is questionable mild enlargement to the vestibular aqueduct on the right side.

On the left side, there is marked stenosis to the external auditory canal.  Only the proximal aspect of the external canal is visualized; however, there is also associated complete bony atresia to the external auditory canal.  Only the proximal aspect of the external canal is visualized; however, there is also associated complete bony atresia to the external canal.  There is a complete osseous atresia.  The complete osseous atresia is best seen on images 22 to 28.  There is no evidence for fusion to the neck of the malleus to the atretic plate.

The vestibule and cochlea are seen.  The carotid canal is seen.  The jugular bulb is unremarkable.  The IAC is unremarkable.  The facial nerve is visualized and is unremarkable.  There is no marked enlargement to vestibular aqueduct on the left.

IMPRESSION:

1.  Left external auditory canal atresia.

2.  Right external stenosis.

3.  No marked abnormality to the middle ear.

4.  The bony labrum appears unremarkable.

5.  The internal auditory canals appear unremarkable bilaterally.

6.  There is questionable mild enlargement to the vestibular aqueduct on the right side.

7.  The findings are consistent with Crouzon disease (craniofacial dysostosis).