Long QT and Hearing Loss Registry

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:

1. 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?

2. 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?

3. 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)