The technology used for assessment, clinical protocol, and timing of assessment of infants can impact the timeline for diagnosis and treatment of congenital hearing impairment. Given the significant sequelae of delayed or missed diagnosis of hearing loss in infancy, implementing clinical practice changes should be considered at pediatric diagnostic centers.

BackgroundCongenital CMV infection is the leading cause of non-genetic sensorineural hearing loss in infancy. Antiviral therapy has been shown to improve hearing outcomes, and thus “targeted” CMV screening for newborns who do not pass the hearing screen has been recommended. Diagnosis of congenital CMV infection requires that the infant be tested for CMV in the first 3 weeks of age. Our objective was to determine when infants in the neonatal intensive care unit (NICU) have their first hearing screen performed and thus inform the practice of targeted screening for determination of CMV-related hearing loss.MethodsRetrospective review of the electronic health records of all infants admitted to the Level 4 outborn NICU at Nationwide Children’s Hospital, Columbus, OH from August 2016 to May 2017. Demographic and clinical data were obtained, and the age that the first hearing screen was performed was assessed.ResultsDuring the 10 month study period, 362 infants were admitted to the NICU and had a first hearing screen performed. The majority of neonates (204, 56%) had a first hearing screen performed in the first 3 weeks of age. However, 158 (44%; median birth weight [IQR], 1072 g [747–1766]; median gestational age [IQR], 28 weeks [25–32]) infants received the first hearing screen at >3 weeks of age when a positive CMV PCR or culture cannot distinguish congenital infection from intrapartum/postnatal acquisition of CMV. Of the 158 infants, 20 (13%) did not pass the first hearing screen (13, unilateral; 7, bilateral), and subsequently, 9 of them did pass a second hearing screen. However, 11 of the 20 infants did not pass a second hearing screen and had urine CMV PCR testing, and 1 (9%) was positive. This latter infant’s newborn dried blood spot CMV DNA PCR was negative so a diagnosis of congenital CMV infection was not possible.ConclusionTargeted screening in the NICU for CMV-related hearing loss is problematic as a substantial number of infants do not have a hearing screen performed until after 21 days of age, thus representing a missed opportunity for diagnosis of congenital CMV infection and institution of antiviral therapy if indicated. Our findings support universal CMV screening of neonates on admission to the NICU.Disclosures O. Adunka, MED-EL Corporation: Consultant, Consulting fee, Educational grant and Research support; Advanced Bionics: Consultant, Consulting fee and Licensing agreement or royalty; Advanced Cochlear Diagnostics: President, Ownership interest; AGTC Corporation: Consultant, Consulting fee

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BackgroundThe diagnosis of congenital cytomegalovirus (cCMV) infection requires that CMV be detected in a body fluid before 3 weeks of age. After 3 weeks, a diagnosis of cCMV infection is difficult since one cannot differentiate between prenatal, natal, and postnatal CMV acquisition. Infants who refer on the newborn hearing screen often are diagnosed with hearing loss after 3 weeks of age. Our objective was to describe the use of the newborn dried blood spot (DBS) for detection of CMV DNA in infants who are evaluated for sensorineural hearing loss (SNHL).MethodsRetrospective review of the electronic health records of infants who were referred to the Neonatal Infectious Disease (NEO-ID) Clinic at Nationwide Children’s Hospital, Columbus, OH since 2015 for evaluation of SNHL. Demographic, clinical, laboratory, and radiographic data were reviewed. With maternal informed consent, the newborn DBS was obtained from the Ohio Department of Health for detection of CMV DNA by polymerase chain reaction (PCR) testing as previously described (Boppana et al. JAMA, 2010).ResultsEighteen infants (gestational age [mean ± SD], 38 ± 4 weeks; birth weight, 3,094 ± 705 g) with SNHL were referred by Otolaryngology for evaluation of possible cCMV infection; 17 (94%) had referred on the newborn hearing screen. The 18 infants were first tested for CMV at 151 ± 124 days of age (mean ±SD; range, 21–521 days), and 3 (17%) had a positive CMV DBS. Fourteen (78%) of the 18 infants had a positive serum CMV IgG antibody while 5 (63%) of 8 infants had CMV DNA detected in urine by PCR. Of the 3 infants with a positive CMV DBS, 2 were tested for CMV DNA PCR in urine and both were positive. Of the 3 infants, 1 had a negative serum CMV IgG antibody test at 174 days of age but the urine CMV PCR test was positive. In comparison, of 54 infants with cCMV infection confirmed by a positive urine CMV PCR in the first 3 weeks of age, 37 (68%) had a positive CMV DBS.ConclusionDBS testing for CMV DNA by PCR testing identified a small minority of infants with SNHL and thus confirming congenital infection. However, the overall sensitivity of CMV DBS testing in our cohort was 68%, suggesting that some infants with SNHL due to congenital CMV infection are missed.Disclosures All authors: No reported disclosures.

BackgroundCongenital cytomegalovirus (cCMV) infection is the most common cause of non-genetic sensorineural hearing loss in infancy. Screening of newborns for cCMV infection has been performed utilizing saliva due to ease of collection and high sensitivity. Positive saliva screens for CMV DNA by polymerase chain reaction (PCR) testing has been reported to occur secondary to breast milk feeding without signifying congenital infection. The NICUs of Nationwide Children’s Hospital recently began universal saliva screening of all admissions. We report 3 neonates whose saliva CMV screen was positive yet the urine CMV PCR test was negative in order to inform CMV screening strategies.MethodsRetrospective review of the electronic health records of neonates admitted to the neonatal intensive unit (NICU) at Nationwide Children’s Hospital, Columbus, OH who had CMV detected by PCR from saliva specimens but not from urine. Pertinent demographic and clinical data were obtained.ResultsThree female neonates had a positive saliva CMV DNA PCR test but urine CMV PCR was negative. The first infant (gestational age [GA] 34 weeks, birth weight [BW]1790 Grams) was a monochorionic diamionic twin gestation and born vaginally with unknown duration of rupture of membranes (ROM). At 16 days of age, the infant had a positive saliva CMV PCR but a negative urine CMV PCR test. The infant received maternal milk. The twin’s CMV PCR tests of saliva and urine were negative. The second infant (GA 38 weeks, BW 2952 grams) was born vaginally after 9 hours of ROM. On the first day of age, the infant had a positive saliva CMV PCR test that was followed by a negative urine CMV PCR on the third day of age. The infant had not been breastfed. The third infant (GA 33 weeks, BW 1762 grams) was born by C-section delivery with ROM at delivery. Saliva CMV PCR screen was positive on the second day of age but urine PCR was negative twice (days 5 and 7). All 3 infants had no signs/symptoms of cCMV infection and passed the newborn hearing screen.ConclusionTesting of saliva for CMV DNA by PCR is not always confirmatory for cCMV infection as contamination of saliva specimens with CMV could result from exposure to maternal milk and possibly vaginal secretions. Definitive diagnosis of cCMV infection requires additional confirmatory testing preferably with urine.Disclosures All authors: No reported disclosures.