At about 8 weeks of age, we decided to have Sadie tested to see if we could determine what caused her hearing loss. We found out that it was due to the Connexin 26 gene disorder. Connexin 26 is a protein found on the (GJB2) gene and is the most common cause of congenital sensorineural hearing loss. In order for Sadie to have this disorder, Troy & I must both be carriers of a Connexin 26 mutated gene. The odds of this is only about .025%. Neither of us have hearing loss because we have one normal gene and one mutated gene. Unfortunately, we both passed our mutated gene along to Sadie (25% chance of this happening). With this genetic mutation, the potassium flow that is needed to stimulate hair cell growth in the cochlea is disrupted. You can read more about this disorder here:
http://www.californiaearinstitute.com/ear-disorders-connexin-26-bay-area.php
