[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 54.211.207.116. Please contact the publisher to request reinstatement.
[Skip to Content Landing]
Citations 0
Clinical Problem Solving: Radiology
December 2004

Radiology Quiz Case 3—Diagnosis

Author Affiliations
 

NICK R.BRYANMDPATRICIA A.HUDGINSMD

Arch Otolaryngol Head Neck Surg. 2004;130(12):1449. doi:10.1001/archotol.130.12.1449

X-linked deafness is a mixed deafness syndrome with congenital fixation of the stapedial footplate. The DFN 3 gene was mapped to the Xq 21 region by linkage analysis and by identification of deletion in both syndromic and nonsyndromic patients.1

The hearing loss in the affected person has a conductive and sensorineural component. A large conductive component of more than 30 to 40 dB reflects stapes ankylosis, which becomes gradually smaller toward the higher frequencies. Smaller conductive losses of 20 dB are believed to be the result of a widened vestibule, leading to a loss of energy of fluid wave transmitted to the cochlea.2 Sensorineural loss is caused by cerebrospinal fluid changing pressure in the perilymphatic and endolymphatic component of the cochlea.

First Page Preview View Large
First page PDF preview
First page PDF preview
×