Clinical trial NCT02082431: Determine the Incidence of Long QT Amongst a Large Cohort of Subjects Diagnosed With Unilateral or Bilateral Sensorineural Hearing Loss.
Official title: Long QT & Hearing Loss Prospective Study Registry
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Sensorineural Hearing Loss
Long QT Syndrome
Keywords: Sensorineural hearing loss
Long QT Syndrome
Lead sponsor: Pediatrix
Sponsor class: OTHER
First posted: 2014-03-10
Enrollment: 600
Sex: ALL
Minimum age (years): 1
Maximum age (years): 90
Standard ages: CHILD
Accepts healthy volunteers: yes
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Newborn infants

Brief summary: 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.

Detailed description: 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?

Eligibility criteria: 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)

Primary outcomes:
measure: The incidence of an abnormal ECG (QTc > 450) in neonates greater than a week of age with either unilateral or bilateral sensorineural hearing loss
timeFrame: 6 months
Secondary outcomes:
measure: In neonates > 1 week of age with either bilateral or unilateral sensorineural hearing loss and an abnormal ECG, percentage have an identifiable genetic mutation
timeFrame: One year
Other outcomes:
Locations:
city: Anchorage
country: United States
facility: Jack Jacob
state: Alaska
city: Phoenix
country: United States
facility: Banner Good Samaritan Hospital
state: Arizona
city: Phoenix
country: United States
facility: St. Joseph's Hospital & Medical Center
state: Arizona
city: Waterford
country: United States
facility: Lawrence and Memorial Hospital
state: Connecticut
city: Rockford
country: United States
facility: Swedish American Hospital
state: Illinois
city: Oklahoma City
country: United States
facility: Hearts for Hearing
state: Oklahoma
city: Austin
country: United States
facility: Dell Children's Medical Center
state: Texas
city: Austin
country: United States
facility: Child Cardiology Associates
state: Texas
city: San Antonio
country: United States
facility: Christus Santa Rosa Westover Hills
state: Texas
city: San Antonio
country: United States
facility: Children's Hospital of San Antonio
state: Texas
city: Alexandria
country: United States
facility: Alexandria Hospital- Inova Health System
state: Virginia
city: Fairfax
country: United States
facility: Pediatrix Audiology Services
state: Virginia
Study officials:
affiliation: Pediatrix
name: Mitchell Cohen, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Fenrich AL, Shmorhun DP, Martin GC, Young JA, Cohen MI, Kelleher AS, Anyebuno MA, Rider ED, Motta CL, Clark RH. Long QT and Hearing Loss in High-Risk Infants Prospective Study Registry. Pediatr Cardiol. 2022 Dec;43(8):1898-1902. doi: 10.1007/s00246-022-02939-4. Epub 2022 Jun 3.