Objective To analyze the presence of Joint Committee on Infant Hearing (JCIH) risk factors and co-occurring birth defects (CBDs) in children with unilateral hearing loss (UHL).
Design Retrospective review.
Setting Statewide registry of universal newborn hearing screen data for all children born in Virginia from 2002 through 2008.
Patients The study population comprised 371 children with confirmed UHL.
Main Outcome Measures Universal newborn hearing screen status, presence or absence of JCIH risk factors, and CBDs
Results Of the 371 children with confirmed unilateral hearing loss, 362 (97.5%) were identified through a failed universal newborn hearing screen. Of these 362 children, 252 (69.6%) had no JCIH risk factors and 110 (30.3%) had 1 or more risk factor reported. Nine children (2.5%) with 1 or more risk factors passed the universal newborn hearing screen but had later-onset UHL. Craniofacial anomaly was the most commonly reported JCIH risk factor in 48 children (43.6%). A family history of permanent childhood hearing loss was present in 24 children (21.8%). Twenty children (18.2%) had stigmata associated with a syndrome including hearing loss. Of the 110 children with UHL and a JCIH risk factor, additional CBDs were identified in 83 (75.5%). An ear-specific anomaly was most prevalent in 37 infants (44.6%), followed by cardiovascular anomalies in 34 infants (41.0%).
Conclusions Thirty percent of children with confirmed UHL had a JCIH risk factor, most commonly craniofacial anomalies, family history of hearing loss, and stigmata of syndromes associated with hearing loss. However, the absence of JCIH risk factors does not preclude the development of UHL. Further studies assessing the etiology of UHL and risk factor associations are warranted.