Author(s)
Emily Y. Huang, BS
Shi Liang, BS
Yang Fu, BS
Bridget Dorsey, BS
Ava Griffitt
Breanna Wong
Matthew A. Firpo, PhD, Albert H. Park, MD
Affiliation(s)
Johns Hopkins University School of Medicine; Division of Otolaryngology Head and Neck Surgery, University of Utah School of Medicine;
Abstract:
Approximately 5-15% of children with congenital cytomegalovirus (cCMV) present only with isolated sensorineural hearing loss (SNHL). Since most cCMV infected children do not undergo early CMV screening or testing, there is a need to determine other diagnosis methods. One approach would be to determine if hearing loss phenotype of cCMV infected children might be different from those with other causes of hearing loss. This study compared the audiograms of children with diagnosed asymptomatic cCMV, and other non-CMV causes of SNHL to identify characteristics unique to those with cCMV.
A retrospective chart review was performed at a Children’s hospital for infants, with cCMV, and non-CMV causes of SNHL, who received audiogram testing between 2005-2022. Congenital CMV was defined via a positive dry blood spot, or a positive urine CMV PCR test within 30 days of life. Non-CMV causes of SNHL were diagnosed via imaging (enlarged vestibular aqueduct), genetic testing (connexin mutation) or idiopathic (negative CMV IgG testing, nondiagnostic genetic testing and normal imaging). The first complete behavioral audiogram or auditory brainstem response testing was reviewed.
Eighty patients diagnosed with cCMV (n=23), LVA (n=22), Connexin 26 (n=12), or idiopathic non-CMV hearing loss (n=16) were included. One major audiologic characteristic notable in the cCMV cohort was the universal presence of threshold asymmetry. This difference was quantified by comparing the average pure tone average (PTA) for the better and worse hearing ear. The average PTA difference was significantly different among the four groups (ANOVA p< 0.05). Those with cCMV had the largest average difference in PTA compared to other groups; this difference was noted for all frequencies evaluated.
The cCMV cohort have a characteristic audiologic phenotype of threshold asymmetry when compared to those without cCMV-mediated SNHL. These findings may provide an approach to diagnose those with isolated SNHL not identified via early CMV screening.