Determine the Incidence of Long QT Amongst a Large Cohort of Subjects Diagnosed With Unilateral or Bilateral Sensorineural Hearing Loss.

The purpose of this study is to determine the true incidence of long QT (LQT) amongst a large cohort of subjects diagnosed with unilateral (right/left) or bilateral sensorineural hearing loss.

The goal of this study is to answer the following questions:

• What is the incidence of an abnormal ECG (QTc > 450 msec) in neonates greater than a week of age with either unilateral or bilateral Sensorineural hearing loss?
• What percentage of neonates greater than one week of age with either bilateral or unilateral sensorineural hearing loss and an abnormal ECG have an identifiable genetic mutation?
• What is the incidence of an abnormal genetic mutation consistent with long QT regardless of the ECG in neonates with bilateral sensorineural hearing loss?

Inclusion criteria

• All newborns who demonstrate a refer in one or both ears on a routine newborn hearing screen
• Documentation of informed consent
• Inborn
• Ability to perform an ABR (auditory brainstem response screen technology) screening test
• No major anomalies
• Subjects' parents willing to provide follow-up data on their child

Exclusion criteria

• Newborns with a syndromic cause of hearing loss
• Parents unwilling to provide follow-up data
• Major congenital anomalies
• Major medical problem or conditions. (i.e., hypoxic ischemic encephalopathy (HIE), persistent pulmonary hypertension neonate (PPHN), meconium aspiration, etc.)
• Congenital cytomegalovirus (CMV)