Sensorineural Hearing Loss In Newborns Research Articles

2124. Active surveillance for Congenital Cytomegalovirus infections as part of institutional protocol for newborns with failed hearing screening

Abstract Background Congenital cytomegalovirus (cCMV) is the leading infectious cause of sensorineural hearing loss in newborns. Universal screening for cCMV is not recommended in the United States. It is important to establish effective strategies for early detection of newborns with cCMV, as most of them are asymptomatic at birth. Methods As per institutional protocol at Louisiana State University Health Sciences Center in Shreveport, every newborn who fails auditory brainstem response (ABR) hearing screen is referred to Audiology and is evaluated with CMV polymerase chain reaction (PCR) from urine samples to rule out cCMV before they are discharged home. We intended to evaluate the proportion of newborns failing hearing screening during a two-year period that are positive for CMV by urine PCR and that are referred to Pediatric Infectious Diseases for cCMV treatment. Results From January 1st, 2020, to March 6th, 2022, 4,010 newborns had ABR based hearing screening at birth, with 125 (3%) failing on two attempts. Subsequently, 85 of them, corresponding to 68%, passed repeat testing at Audiology clinic, while 25 did not show up for their appointments. Out of the 67 infants that passed repeat hearing test and were tested for cCMV, none had positive urine CMV PCR results. Among the 15 newborns who failed repeat hearing test in Audiology clinic, 2 (13%) had positive urine CMV PCR results, 9 had negative results and 2 infants did not have tests done (Figure 1). Both infants diagnosed with cCMV showed severe to profound sensorineural hearing loss and are being followed by Otorhinolaryngology. They are also receiving oral valganciclovir for cCMV infection with a plan to receive treatment for 6 months, being followed periodically by Pediatric Infectious Diseases. Evaluations with urine CMV PCR in newborns with repeat hearing screening showed that all 67 that passed were negative; and out of the 15 infants that failed repeat testing, 2 had positive CMV PCR results. Conclusion Our institutional protocol recommends sending urine samples for CMV PCR in every baby that fails newborn ABR hearing screen, and this has helped identified neonates with cCMV infections in the setting of severe to profound sensorineural hearing loss. These patients diagnosed with cCMV are planned to receive treatment for 6 months. Our future goals involve evaluating if early treatment of cCMV for 6 months can prevent progression of hearing loss and help achieving improved audiological outcomes at 2 years of age. Disclosures All Authors: No reported disclosures.

Elimination of human cytomegalovirus DNA degradation in urine

Congenital cytomegalovirus infection (cCMVi) is an important cause of sensorineural hearing loss in newborns. Detection of human cytomegalovirus (HCMV) DNA in urine has been used to screen for cCMVi in newborns. However, the matrix effect of urine on HCMV DNA detection is unclear. To evaluate the matrix effect of urine on HCMV DNA detection and optimize the sample process strategy to eliminate or minimize the impact of urine on HCMV DNA detection, DNA in spiked samples was extracted using different DNA extraction methods, and urine samples that could inhibit HCMV DNA detection were mixed to evaluate the inhibitory substances, inhibitory mechanism, and elimination of the inhibitory effect. The optimal urine sample process strategy was evaluated using 42 adult female urine samples and 42 newborn urine samples spiked with HCMV. Some urine samples were found to inhibit HCMV DNA detection due to DNA degradation. The addition of ≥5 mM EDTA to the urine before extraction eliminated the inhibitory effect of urine and did not affect the detection results of urine exhibiting no inhibition. Of the 42 adult female and 42 newborn urine samples, four and two samples, respectively, could inhibit HCMV DNA detection. However, the inhibitory effects of these six urine samples were eliminated after the addition of EDTA. The collective results indicate that the addition of EDTA can completely eliminate the impact of inhibitors present in urine on HCMV DNA extraction and improve the detection of HCMV in urine.

Maternal hypertension, pre-eclampsia, eclampsia and newborn hearing: A retrospective analysis of 454 newborns

ObjectiveMaternal hypertension and preeclampsia have been related to sensorineural hearing loss in newborns. To investigate potential connections, we compared newborn hearing screening (NHS) results from newborns of mothers with chronic hypertension, preeclampsia, and eclampsia with results from newborns of healthy controls. The present study is unique with regard to its large sample size and the analysis of the possible effects of three different hypertensive disorders on newborn hearing. MethodsWe retrospectively searched the database of our hospital for pregnant women diagnosed with chronic hypertension, preeclampsia, and eclampsia according to the International Classification of Diseases Tenth Revision (ICD-10) diagnostic codes. The search covered the period from January 2010 to March 2020. NHS results were compared with those of newborns of healthy controls. ResultsThe auditory brainstem response (ABR) test results and categorical variables of infants of 146 women with preeclampsia, 71 women with chronic hypertension, and 10 women with eclampsia were compared with those of infants of 227 healthy women. Only the “bilateral pass” results were statistically significantly lower in the preeclampsia group in comparison to the control group (p = 0.036), but this was a temporary effect. Between the two groups, there was no significant difference in the second ABR (ABR refer) test. ConclusionThere was a statistically significant difference between the preeclampsia and control groups only in the first ABR test. But, the ABR refer test results of these groups did not differ significantly. Therefore, we conclude that these temporary effects may be related to newborns being born prematurely and being small for their gestational age.

Sensorineural Hearing Loss in Newborns Hospitalized in Neonatal Intensive Care Unit: An Observational Study.

Children hospitalized in Neonatal Intensive Care Units (NICU) present an increased risk for Sensorineural Hearing Loss (SNHL) due to prematurity, hypoxia-ischemia, hyperventilation, low birth weight and the use of ototoxic drugs. The aim of this study was to assess the prevalence of SNHL in newborns hospitalized in a NICU using Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Responses (A-ABR) and analyze the associated risk factors. A sample of 153 newborns hospitalized in NICU underwent TEOAE, A-ABR and clinical ABR to evaluate the presence of hearing deficits. Prevalence of SNHL was calculated and odds ratio for specific risk factors was measured. One-hundred fifteen babies (86.7%) presented normal hearing at TEOAE and A-ABR. Fifteen children had a REFER response at TEOAE and a PASS response at A-ABR. Twenty-five children (16.3%) had a REFER A-ABR and were addressed to clinical ABR. A diagnosis of SNHL was made in 12 (7.8%) newborns. An increased risk of SNHL was observed in preterm children <28 weeks (p=0.0135), in children with neurological disorders (p=0.02), that underwent surgery (p=0.0002), affected from premature retinopathy (p=0.0006), craniofacial malformation (p=0.007) and that had sepsis (p=0.04). Additional risk factors for SNHL in our sample were a maternal disease during pregnancy (p=0.0011), cesarean delivery (p<0.0001) and a twin pregnancy (p<0.0001). SNHL in newborns is correlated with hospitalization in NICU. An accurate hearing screening associated to a rigorous clinical medical collection of data is necessary to promptly identify cases of SNHL in children with a special attention to those hospitalized in NICU and plan proper intervention.

Prevalence of sensorineural hearing loss in newborns in a hospital from a developing country.

This study aimed to determine the prevalence of nonsyndromic congenital sensorineural hearing loss at the Hospital Universitario San Ignacio, Bogotá, Colombia, and to describe the risk factors associated with this condition. A prospective, observational cross-sectional study with bivariate analysis was conducted. A three-phase process using the Otoacoustic Emissions test screened all live newborns between June 2013 and June 2014. Negative cases were confirmed by Automated Auditory Brainstem Response test. A total of 962 newborns were screened with Otoacoustic Emissions test bilaterally: 401 males (46.36%), 464 females (53.64%). The mean weight was 2 798.10 g (95%CI: 2 766.51 - 2 839.76). The mean height was 48.60 cm (95%CI: 48.38 - 48.79). The mean age was 16.24 days (95%CI: 15.47 - 17.01). The mean maternal age was 27.37 years (95%CI: 26.76 - 27.98). There was a family history of hearing loss in 9.48% of the cases (n=90), and a family history of genetic diseases in 100 cases (10.56%). There were 14 cases of TORCH infections (1.45%), 375 admissions to the NICU (39.06%), 160 cases of neonatal jaundice (20.1%), and 79 cases of postpartum infections (8.21%). One live newborn presented with microtia. The prevalence of congenital sensorineural hearing loss was 0.31% in both ears, and 0.11% in one ear. Currently, Colombia lacks a public universal newborn hearing screening program, and its future implementation faces great challenges.

Aksaray Yöresinin Yenidoğan İşitme Taraması Sonuçlarının Değerlendirilmesi

Objective: To investigate the possible risk factors that may lead to hearing loss in newborns who failed the hearing screening tests. Material and Methods: In this retrospective study, 546 newborns who failed the evoked otoacoustic emission (TEOAE) test or who underwent screening auditory evoked brainstem response (ABR) due to detected risk factors were included between 2010 and 2018. The risk factors of 78 patients who failed the screening ABR test were documented. A total of 468 newborns who passed ABR screening test were evaluated as control group. The data were compared statistically. Results: In the comparison of groups who failed and passed the ABR screening test, the mean birth time was found to be significantly earlier (38.1±2.6 and 39.25±1.4 weeks, respectively) (p=0.049), and the mean birth weight was found to be significantly lower (3001 ±628.2 and 3334.82 ±418 g, respectively) (p=0.032) in the failed group. The incidence of neonatal jaundice and jaundice requiring phototherapy was higher in patients who failed the screening ABR test, and this difference was statistically significant (p=0.043 and p=0.028, respectively). The newborns receiving intensive care treatment were also found to be statistically higher (p=0.028). Conclusion: The highest risk group for sensorineural hearing loss in newborns may be considered as the ones with low birth weight, early birth time and receiving intensive care treatment.

Risk of Sensorineural Hearing Loss and Bilirubin Exchange Transfusion Thresholds.

High bilirubin levels are associated with sensorineural hearing loss (SNHL). However, few large studies of relative and excess risk exist. We sought to quantify the risk of SNHL in newborns who had bilirubin levels at or above American Academy of Pediatrics exchange transfusion thresholds (ETT). Infants born at ≥35 weeks gestation in 15 Kaiser Permanente Northern California hospitals from 1995-2011 were eligible (N = 525 409). We used a nested double cohort design. The exposed cohort included subjects with ≥1 bilirubin level at or above ETT. The unexposed cohort was a 3.6% random sample of subjects with all bilirubin levels below ETT (10 unexposed per exposed). An audiologist, blinded to bilirubin levels, reviewed the charts of children in whom SNHL had been diagnosed before age 8 years to confirm the diagnosis. We calculated Cox proportional hazard ratios for time to diagnosis of SNHL. SNHL was confirmed in 11 (0.60%) of the 1834 exposed subjects and in 43 (0.23%) of the 19 004 unexposed. Only bilirubin levels ≥10 mg/dL above ETT were associated with a statistically significant increased risk of SNHL (hazard ratio: 36 [95% confidence interval (CI): 13 to 101]). Likewise, only bilirubin levels ≥35 mg/dL were associated with a statistically significant increased risk of SNHL (hazard ratio: 91 [95% CI: 32 to 255]). For subjects with total serum bilirubin levels 0 to 4.9 mg/dL above ETT, the upper limit of the 95% CI for excess risk was 0.5%. Only bilirubin levels well above ETT were associated with SNHL. At lower bilirubin levels, the excess risk of SNHL was low.

Finite-element modeling of the newborn ear canal and middle ear

Available hearing-screening procedures cannot distinguish clearly between conductive and sensorineural hearing loss in newborns, and the results of available diagnostic tests in very young infants are difficult to interpret. Admittance measurements can help to detect conductive losses but do not provide reliable results for newborns, where the ear is anatomically different from the adult ear. Finite-element models of the newborn ear canal and middle ear were developed and their responses were studied for frequencies up to 2000 Hz. Material properties were taken from previous measurements and estimates, and the sensitivities of the models to these different parameters were examined. The simulation results were validated through comparison with previous experimental measurements. Preliminary simulations indicate that at frequencies up to 250 Hz the admittance of the canal wall is comparable to that of the middle ear in the newborn. Above 250 Hz the canal-wall admittance remains almost constant but for the middle ear there is a clearly defined resonance peak, which produces an admittance much larger than that of the canal wall. These results suggest that admittance measurements in the vicinity of the middle-ear resonance frequency can provide clinically useful information about the newborn middle ear.

Detection of Cytomegalovirus DNA in Dried Blood Spots of Minnesota Infants Who Do Not Pass Newborn Hearing Screening

Up to 15% of infants with asymptomatic congenital cytomegalovirus (CMV) infection will experience some degree of sensorineural hearing loss. Many infants who fail newborn hearing screening (NHS) are likely to have congenital CMV infection, but may escape definitive virologic identification because diagnostic evaluation may not commence until several weeks or months of age, making differentiation between congenital and postnatal CMV infection difficult. Early diagnosis linking virologic identification of congenital CMV infection to infants failing NHS may improve diagnostic precision and enhance opportunities for therapeutic intervention. The goal of this study was to compare newborn dried blood spots from Minnesota infants who had failed NHS, and were designated for referral, with control infants who passed NHS, for the presence of CMV DNA by real-time PCR, using hybridization probes for the CMV gene UL54. Of 479 infants with a failed NHS (bilateral failure), 13 had CMV DNA present in the blood spot (2.7%). This compared with only 2/479 positive results from a control group of infants who passed the NHS (0.4%; P = 0.007, Fisher exact test). Comparisons of the glycoprotein B (gB) genotype as well as direct DNA sequencing of selected positives revealed that PCR positive samples represented unique clinical isolates. The mean viral load among the 15 positive samples was 1.6 x 10(3) genomes/microgram of total DNA. Newborn bloodspot CMV screening by real-time PCR may be a useful and rapid adjunct to functional NHS and may enable more rapid etiologic diagnosis of sensorineural hearing loss in newborns.