Isolated congenital internal auditory canal atresia with normal facial nerve function

The internal auditory canal forms as a result of mesoderm enveloping the eighth cranial nerve in the developing embryo. The mesoderm eventually transforms into cartilage and ultimately ossifies around the nerve, forming the internal auditory canal. It is theorized that atresia or stenosis of the int...

Full description

Saved in:
Bibliographic Details
Published in:International journal of pediatric otorhinolaryngology 1997-07, Vol.41 (1), p.1-8
Main Authors: Yates, Jay A., Patel, Pranay C., Millman, Brad, Gibson, William S.
Format: Article
Language:English
Subjects:
Child, Preschool
Deafness - congenital
Deafness - embryology
Deafness - physiopathology
Ear, Inner - abnormalities
Ear, Inner - embryology
Facial nerve
Facial Nerve - embryology
Facial Nerve - physiopathology
Female
Hearing Loss, Bilateral - congenital
Hearing Loss, Bilateral - embryology
Hearing Loss, Bilateral - physiopathology
Humans
Internal auditory canal atresia
Temporal bone
Tomography, X-Ray Computed
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:The internal auditory canal forms as a result of mesoderm enveloping the eighth cranial nerve in the developing embryo. The mesoderm eventually transforms into cartilage and ultimately ossifies around the nerve, forming the internal auditory canal. It is theorized that atresia or stenosis of the internal auditory canal results from altered cochleovestibular nerve development secondary to faulty chemotactic mechanisms or a lack of end organ targets. Unilateral internal auditory canal anomalies are frequently seen in conjunction with other inner ear anomalies and occasionally with middle or external ear anomalies. Infrequently, it will occur as either an isolated or bilateral finding, but rarely simultaneously. The few citations of isolated, unilateral or bilateral internal auditory canal anomalies that are reported in the literature are usually associated with other systemic developmental anomalies, such as, cardiac septal defects, polycystic kidney disease, skeletal deformities and duodenal atresia. We present a case report of a patient with bilateral, congenital, internal auditory canal atresia and cochleovestibular deficits but, normal facial nerve function. A review of the literature is discussed as well as diagnostic considerations and treatment options including audiologic and communication rehabilitation.
ISSN:0165-5876
1872-8464
DOI:10.1016/S0165-5876(97)00044-X