Toxoplasma gondii IgG avidity for the diagnosis of primary infection in pregnant women: Comparison between chemiluminescent microparticle immunoassay and enzyme-linked immunosorbent assay.

INTRODUCTION: This study evaluated whether IgG avidity measured by chemiluminescent microparticle immunoassay (CMIA) compared with enzyme-linked immunosorbent assay (ELISA) was useful to detect primary T. gondii infection during pregnancy and to estimate the risk for congenital T. gondii infection. METHODS: One hundred six women with positive tests for T. gondii IgG and T. gondii IgM, comprising 21 women (19.8%) with low (<30%), 6 (5.7%) with borderline (30%-35%), and 79 (74.5%) with high (>35%) IgG avidity measured by ELISA were selected. Their stored sera were used for T. gondii IgG avidity measurements by CMIA. RESULTS: In CMIA, 72 (67.9%) women had low (<50%), 12 (11.3%) had borderline (50%-59.9%), and 22 (20.8%) had high (≥60%) IgG avidity. The ratio of low T. gondii IgG avidity index in CMIA was more than three-fold than that in ELISA. Eighteen (85.7%) of 21 women with ELISA low avidity also had CMIA low avidity, and 26 (96.3%) of 27 women with ELISA low or borderline avidity corresponded to CMIA low or borderline avidity, whereas 21 (26.6%) of 79 women with ELISA high avidity were diagnosed with CMIA low avidity. All three cases with congenital T. gondii infection showed coincidentally low IgG avidity in both methods. A positive correlation in IgG avidity indices was found between of ELISA and CMIA. CONCLUSIONS: CMIA for T. gondii avidity measurements compared with ELISA was clinically useful to detect pregnant women at a high risk of developing congenital T. gondii infection.

Two pregnant women with immune-mediated thrombotic thrombocytopenic purpura: A case report.

Thrombotic thrombocytopenic purpura (TTP) during pregnancy is life-threatening. We encountered two pregnant women with immune-mediated TTP (iTTP). A 40-year-old primigravida woman was referred at 19 gestational weeks (GWs) owing to iTTP. She received plasma exchange (PE) and steroid therapies and delivered a live infant at 27 GWs by cesarean delivery. A 29-year-old primigravida woman was referred owing to intrauterine fetal death and thrombocytopenia at 20 GWs. She was diagnosed with iTTP and received PE therapy. She required additional PE and steroid therapies owing to relapse. Before her second pregnancy, she received prednisolone and hydroxychloroquine according to the therapy for systemic lupus erythematosus (SLE). She had induced labor at 37 GWs owing to decrease plasma level of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) activity. Close monitoring of plasma ADAMTS13 activity level and treatments for underlying SLE may prevent iTTP relapse and lead to a good prognosis.

Anti-ß2-glycoprotein I/HLA-DR Antibody and Adverse Obstetric Outcomes.

Anti-ß2-glycoprotein I/HLA-DR (anti-ß2GPI/HLA-DR) antibody has been reported to be associated with antiphospholipid syndrome and recurrent pregnancy loss (RPL). We conducted a prospective multicenter cross-sectional study aimed at evaluating whether the anti-ß2GPI/HLA-DR antibody is associated with adverse obstetric outcomes and RPL. From 2019 to 2021, serum anti-ß2GPI/HLA-DR antibody levels (normal, <73.3 U) were measured in 462 women with RPL, 124 with fetal growth restriction (FGR), 138 with hypertensive disorders of pregnancy (HDP), 71 with preterm delivery before 34 gestational weeks (preterm delivery (PD) ≤ 34 GWs), and 488 control women who experienced normal delivery, by flow cytometry analysis. The adjusted odds ratios (aORs) of anti-ß2GPI/HLA-DR antibody positivity for adverse obstetric outcomes and RPL were evaluated on the basis of comparisons between the control and each patient group, using multivariable logistic regression analysis. The following were the positivity rates for the anti-ß2GPI/HLA-DR antibody in the patient and control groups: RPL, 16.9%; FGR, 15.3%; HDP, 17.4%; PD ≤ 34 GWs, 11.3%; and the control, 5.5%. It was demonstrated that anti-ß2GPI/HLA-DR antibody positivity was a significant risk factor for RPL (aOR, 3.3 [95% confidence interval {CI} 1.9-5.6], p < 0.001), FGR (2.7 [1.3-5.3], p < 0.01), and HDP (2.7 [1.4-5.3], p < 0.01) although not for PD ≤ 34 GWs. For the first time, our study demonstrated that the anti-ß2GPI/HLA-DR antibody is involved in the pathophysiology underlying FGR and HDP, as well as RPL.

A prospective cohort study of newborns born to mothers with serum Toxoplasma gondii immunoglobulin M positivity during pregnancy.

INTRODUCTION: The aims were to investigate the clinical characteristics of Toxoplasma gondii (T. gondii) immunoglobulin (Ig) M-positive mothers and to clarify the incidences of serum T. gondii IgM or blood T. gondii DNA positivity in newborns born to the mothers and the actual congenital T. gondii infection. METHODS: Mothers with T. gondii IgM positivity and newborns born to the mothers from 2013 to 2020 were prospectively investigated. Serum T. gondii IgG and IgM were measured by enzyme-linked immunosorbent assay. Blood T. gondii DNA was detected by semi-nested polymerase chain reaction. Congenital T. gondii infection was diagnosed based on clinical characteristic manifestations with serum T. gondii IgG positivity at any age or T. gondii IgG positivity after 12 months of age. RESULTS: Among 71 T. gondii IgM-positive mothers, including one with triplets, 41% had low T. gondii IgG avidity index and 73% received maternal therapy. Among 73 newborns who were examined for serum T. gondii IgG and IgM at birth, none had clinical manifestations, and one (1.4%) had T. gondii IgM positivity. Among 32 newborns who were examined for blood T. gondii DNA at birth, two (6.3%) were positive. All patients with serum T. gondii IgM or blood T. gondii DNA positivity showed T. gondii IgG negativity within 12 months of age. CONCLUSIONS: A few newborns born to T. gondii IgM-positive mothers were suspected of having congenital T. gondii infection based on serum T. gondii IgM or blood T. gondii DNA testing at birth. However, none developed congenital T. gondii infection.

Predictive factors for postpartum glucose intolerance in women with gestational diabetes mellitus.

AIM: The aim of this prospective cohort study was to evaluate the risk factors for postpartum glucose intolerance (GI) in women with gestational diabetes mellitus (GDM). METHOD: A total of 140 women with GDM were enrolled. Of these, 115 underwent a 75-g oral glucose tolerance test (OGTT) at 12 weeks after delivery. Clinical factors and parameters in the antepartum 75-g OGTT associated with postpartum GI were evaluated by logistic regression analyses. RESULTS: Twenty-two (19.1%) of the 115 women with GDM developed postpartum GI. The univariate and multivariable logistic regression analyses revealed that low oral disposition index (DI) was a risk factor for postpartum GI (OR, 0.2; 95% CI, 0.04-0.7; p < 0.05), and that no clinical factors were associated with postpartum GI. CONCLUSIONS: Lower oral DI on the antepartum 75-g OGTT may be a useful marker for identifying GDM women who are at high risk for postpartum GI.

Administration of intravenous benzylpenicillin in 13 infants born to mothers with syphilis infection: A case series.

The incidence of syphilis infection among pregnant women is persistently high in Japan and in several developed countries. Here, we report the utility of intravenous benzylpenicillin in 13 infants born to mothers with syphilis infection. Because the recommended treatment (intramuscular benzathine benzylpenicillin) is not available in Japan, we intravenously administered benzylpenicillin for 10 days, which is used for treatment in high-risk cases. The administration of benzylpenicillin in low-risk infants resulted in an extended duration of parent-to-infant separation and increased the infants' exposure to invasive procedures. Thus, establishing evidence of the adequacy of no-treatment follow-up in low-risk groups and introducing intramuscular injections of benzathine benzylpenicillin may improve the management of infants suspected with congenital syphilis in Japan.

Abdominal compartment syndrome in a monochorionic-triamniotic triplet pregnancy complicated by feto-fetal transfusion syndrome.

A 40-year-old primigravida woman with a monochorionic-triamniotic (MT) triplet pregnancy was hospitalized due to threatened abortion at 16 gestational weeks. Polyhydramnios in two fetuses and oligohydramnios in the third supported a diagnosis of feto-fetal transfusion syndrome (FFTS) at 23 weeks and 3 days of gestation. Severe dyspnea and liver dysfunction required intensive care unit admission and mechanical ventilation support, and abdominal compartment syndrome (ACS) caused by polyhydramnios was clinically diagnosed. When her general condition was not improved regardless of intensive care, the patient delivered the three fetuses by cesarean section at 23 weeks and 5 days gestation. Abdominal decompression was achieved with delivery, and the patient was discharged 13 days after operation without morbidity. This is the first case report of ACS caused by FFTS in a MT triplet pregnancy resulting in extremely preterm birth.

Sudden fetal death with placental mesenchymal dysplasia complicated by placenta previa.

Placental mesenchymal dysplasia (PMD) is a rare placental abnormality that is closely related to severe pregnancy complications. A 27-year-old woman with fetal growth restriction and placenta previa was referred to a university hospital at 22 gestational weeks (GW). She was suspected of having a twin pregnancy with a complete or partial hydatidiform mole and coexisting normal live fetus, because two separate placentas, an enlarged one with multiple cystic lesions and a normal one, were shown on ultrasound examinations. At 27 GW, she experienced a sudden intrauterine fetal death (IUFD) after bleeding due to placenta previa, despite confirmation of fetal well-being at 2 h before bleeding. After delivery, histopathological examination confirmed the diagnosis of PMD. This is the first documented case of a woman with PMD and placenta previa who had a sudden IUFD after bleeding. Patients with both PMD and placenta previa should be considered at extremely high risk for IUFD.

Efficacy of superselective transcatheter arterial embolization for intractable postpartum hemorrhage due to genital tract trauma after vaginal delivery.

PURPOSE: To evaluate the efficacy of superselective transcatheter arterial embolization (TAE) for intractable postpartum hemorrhage (PPH) due to genital tract trauma (GTT) after vaginal delivery. METHODS: We evaluated 27 patients who underwent TAE for intractable PPH due to GTT after vaginal delivery at our institution between January 2008 and December 2020. Patients were divided into two groups according to TAE procedure; TAE performed as close as possible to the bleeding point, at least more peripherally than the second branch of the anterior division of the internal iliac artery, was defined as superselective TAE (S-TAE). TAE performed from the proximal segment of the internal iliac artery was defined as proximal TAE (P-TAE). Patient characteristics, pre-procedural contrast-enhanced computed tomography (CE-CT), procedure details, technical/clinical success, and complications were evaluated separately for the S-TAE and P-TAE groups. RESULTS: The combined technical/clinical success rate was 92%. No major procedure-related complications were seen (mean follow-up: 6.12 ± 3.93 days). The combined technical/clinical success rate of S-TAE was 100% and of P-TAE was 67% (p = 0.04). S-TAE was performed more frequently in patients with pre-procedural CE-CT (p = 0.01) and use of permanent embolic materials (p = 0.003). CONCLUSION: S-TAE is safe and effective for intractable PPH due to GTT. Pre-procedural CE-CT may be useful for detecting the culprit artery and be helpful in performing S-TAE.

The Current Challenges in Developing Biological and Clinical Predictors of Congenital Cytomegalovirus Infection.

Congenital cytomegalovirus (CMV) infection may cause severe long-term sequelae. Recent studies have demonstrated that early antiviral therapy for infants with symptomatic congenital CMV (cCMV) infection may improve neurological outcomes; thus, accurate identification of newborns at high risk of cCMV infection may contribute to improved outcomes in affected children. However, maternal serological screening for cCMV infection by diagnosing primary infection during pregnancy, which is a popular screening strategy, is inefficient, because the number of cCMV infections with nonprimary causes, including reactivation of or reinfection with CMV, is larger than that of cCMV infections with primary causes. Low levels of neutralizing antibodies against pentameric complex and potent CMV-specific T cell-mediated immune responses are associated with an increased risk of cCMV infection. Conversely, our prospective cohort studies revealed that the presence of maternal fever/flu-like symptoms, threatened miscarriage/premature delivery, or actual premature delivery are risk factors for cCMV infection among both women with normal pregnancies and those with high-risk ones, regardless of whether the infection is primary or nonprimary. This review focused on host immune responses to human CMV and current knowledge of potential biological and clinical factors that are predictive of cCMV infection.

Clinical Factors Associated With Congenital Cytomegalovirus Infection: A Cohort Study of Pregnant Women and Newborns.

BACKGROUND: The aim of this prospective cohort study was to determine clinical factors associated with the occurrence of congenital cytomegalovirus infection (cCMV) in pregnant women. METHODS: Between March 2009 and November 2017, newborns born at a primary maternity hospital received polymerase chain reaction (PCR) analyses for CMV DNA in their urine with informed consent of the mothers at a low risk. Clinical data, including age, gravidity, parity, body mass index, occupation, maternal fever/flulike symptoms, pregnancy complications, gestational weeks at delivery, birth weight, and automated auditory brainstem response, were collected. Logistic regression analyses were performed to determine clinical factors associated with cCMV. RESULTS: cCMV was diagnosed by positive PCR results of neonatal urine in 9 of 4125 pregnancies. Univariate and multivariable analyses revealed that the presence of fever/flulike symptoms (odds ratio [OR], 17.9; 95% confidence interval [CI], 3.7-86.7; P < .001) and threatened miscarriage/premature labor in the second trimester (OR, 6.0; 95% CI, 1.6-22.8; P < .01) were independent clinical factors associated with cCMV. Maternal fever/flulike symptoms or threatened miscarriage/premature labor in the second trimester had 100% sensitivity, 53.2% specificity, and a maximum Youden index of .85. CONCLUSIONS: This cohort study for the first time demonstrated that these clinical factors of pregnant women and newborns were associated with the occurrence of cCMV. This is useful information for targeted screening to assess risks of cCMV in low-risk mothers, irrespective of primary or nonprimary CMV infection.

Vaginal microbiota associated with preterm delivery.

OBJECTIVES: The aim of this study was to evaluate whether vaginal microbiota is associated with threatened premature labor and preterm delivery. METHODS: This prospective study enrolled 64 pregnant women who underwent vaginal microbiome analyses using 16S ribosomal RNA sequence method with informed consent. The 64 pregnant women consisted of 47 women with threatened premature labor and 17 women with other diseases (non-threatened premature labor) in a case-control study. In a cohort study of threatened premature labor group, 23 pregnancies ended in preterm delivery, and the remaining 24 ended in full-term deliveries. The differences in vaginal microbiota between threatened and non-threatened premature labor groups, and between preterm and full-term delivery groups were evaluated. RESULTS: There were no differences in vaginal microbiota between threatened and non-threatened premature labor groups. There were significant differences between preterm and full-term delivery groups in Nugent score [median 3 (range 0-7) vs. 0 (0-4), p < 0.05], percentage of Lactobacillus species [88% (0-100) vs. 99.8% (55.4-100), p < 0.01], the number of bacterial species [3 (1-13) vs. 2 (1-5), p < 0.05], and positivity of Ureaplasma species (61% vs. 17%, p < 0.01). Univariate and multivariable analyses revealed that positivity of Ureaplasma species was a predictive factor of preterm delivery in women with threatened premature labor (OR, 6.5; 95% CI, 1.3-33.0; p < 0.05). CONCLUSION: Increased positivity of Ureaplasma species in vaginal microbiota was a risk factor for preterm delivery among women with threatened premature labor. Vaginal microbiome analysis may identify high risk pregnancies for preterm delivery.

Case report of eight pregnant women with syphilis.

Congenital syphilis may lead to severe sequelae in affected infants. The prevalence of syphilis in women of childbearing age has increased worldwide. From 2015 to 2018, we encountered eight pregnant women with syphilis including six with late latent and two with early latent syphilis. Seven pregnant women with syphilis received antibiotic therapies of oral amoxicillin, intravenous penicillin G, or the both. The syphilotherapies in four cases were considered effective, because rapid plasma reagin titers decreased. None of the seven pregnant women who received syphilotherapies had congenital syphilis. The remaining one woman who did not undergo a maternity checkup or syphilotherapy delivered a stillbirth with congenital syphilis at 29 gestational weeks.

A cohort study of the universal neonatal urine screening for congenital cytomegalovirus infection.

OBJECTIVES: This prospective cohort study aimed to evaluate the efficacy of the universal neonatal urine screening, followed by diagnosis, workup and antiviral therapy for symptomatic congenital cytomegalovirus (CMV) infection to reduce neurological impairments and sequelae. METHODS: Neonates born in three facilities underwent the universal urine screening of PCR analyses for CMV-DNA. Neonates with symptomatic congenital CMV infection (cCMV) received oral valganciclovir (VGCV) of 32 mg/kg/day for six weeks or six months, and were evaluated for neurological outcomes including developmental quotient (DQ) and hearing function at around 18 months of corrected age. RESULTS: cCMV was diagnosed in 56 (0.48%) of 11,736 neonates, consisting of 23 neonates with symptomatic and 33 with asymptomatic cCMV. The incidence of cCMV in the general perinatal medical center (0.69%) was higher than that in the primary maternity hospital (0.23%, p<0.01%). Twenty of the 23 infants with symptomatic cCMV received VGCV therapy, and 19 underwent neurological assessment. Eight neonates (42%) had severe sequelae of DQ < 70, bilateral hearing dysfunction, and/or epilepsy. Four neonates (21%) had mild sequelae of DQ 70-79 or unilateral hearing dysfunction only, and seven (37%) showed normal development without any impairment. CONCLUSIONS: This study on a large scale demonstrated that a series of universal neonatal urine screening, diagnosis, workup, and VGCV therapy for neonates with symptomatic cCMV may decrease neurological impairments, because 58% of the treated infants had normal development or mild sequelae. The universal urine screening likely identifies subclinical symptomatic cCMV. Mothers with fetuses of cCMV seem to be selectively transferred to perinatal medical centers before deliveries.

Recurrence of post-partum hemorrhage in women with a history of uterine artery embolization.

AIM: To evaluate pregnancy outcome and complications in subsequent pregnancies after severe post-partum hemorrhage (PPH) between women with and without a history of uterine artery embolization (UAE). METHODS: Women who had a history of severe PPH, and delivered newborns at ≥22 gestational weeks in subsequent pregnancies were enrolled. Severe PPH was defined as blood loss volume of more than 2000 mL. RESULTS: The blood loss volume (median 1581 mL) in women with UAE (n = 14) was significantly more than that in women without UAE (median 1021 mL, n = 32, P < 0.01), and the recurrence rate of severe PPH in women with UAE (n = 5, 35.7%) was significantly higher than that in women without UAE (n = 3, 9.4%, P < 0.05). There were no significant differences in frequencies of premature delivery, hypertensive disorders of pregnancy, fetal growth restriction, or placenta previa/low lying placenta. Of 14 women with UAE, 7 (50.0%) had abnormally invasive placenta, whereas of 32 women without UAE, none had abnormally invasive placenta. CONCLUSION: Subsequent pregnancies after UAE for severe PPH had high risks for recurrence of severe PPH.

Hepatitis B virus infection: Prevention of mother-to-child transmission and exacerbation during pregnancy.

The aims of this study were to assess the effect of maternal screening for hepatitis B (HB) virus and a perinatal prevention program of mother-to-child transmission, and to identify clinical characteristics and findings associated with HB exacerbation during pregnancy. This prospective cohort study enrolled 3796 pregnant women and their neonates with informed consent. Pregnant women underwent maternal universal screening for HBs antigen (Ag) in the first trimester. If HBs Ag was positive, serum levels of HBe Ag, alanine transaminase (AST), aspartate aminotransferase (ALT), and HB virus (HBV) DNA were measured. All neonates delivered from HBs Ag-positive women were given HB immune globulin and HB vaccine based on the guidelines of the perinatal prevention program. Of the 3796 pregnant women, 40 (1.05%) tested positive for HBs Ag. Three (7.5%) of the 40 HBs Ag-positive women experienced exacerbation of HBV infection during pregnancy. Serum levels of AST (median 776 vs. 22 mIU/ml, p < 0.01), ALT (median 325 vs. 15 mIU/ml, p < 0.01), and HBV-DNA (median 9.1 vs. 5.4 log copies/ml, p < 0.05), and frequencies of HBe Ag-positive (100% vs. 29.7%, p < 0.05) and symptoms of itching or general fatigue (66.7% vs. 0%, p < 0.01) in three women with exacerbation of HBV infection were significantly higher than those in 37 women without exacerbation. There was no case of mother-to-child transmission, suggesting the perinatal HBV prevention program was effective. Levels of HBe Ag, liver enzymes, and HBV-DNA as well as symptoms of itching and general fatigue should be carefully monitored for HBs Ag-positive women during pregnancy and the postpartum period.

A cohort study of maternal screening for congenital Toxoplasma gondii infection: 12 years' experience.

Primary infection with Toxoplasma gondii (T. gondii) during pregnancy may cause congenital infection of the infant. This study evaluated whether screening using IgG avidity and multiplex-nested polymerase chain reaction (PCR) methods was effective for detecting a high-risk pregnancy for congenital T. gondii infection. In a prospective cohort study serum T. gondii IgG avidity was measured in 469 pregnant women who had a positive test for T. gondii antibody plus a positive or equivocal test for IgM. Multiplex-nested PCR for T. gondii DNA on amniotic fluid, maternal blood, and neonatal blood was performed with informed consent. Low (<30%), borderline (30-35%), and high (>35%) IgG avidity indices were found in 104 (22.2%), 30 (6.4%), and 305 (71.4%), respectively. A total of 12 cases had a positive PCR test for amniotic fluids of the prenatal amniocentesis or at birth, or neonatal blood. Seven of the 12 cases were diagnosed as having congenital T. gondii infection, and they had low IgG avidity indices. Congenital T. gondii infection screening using of IgG avidity and multiplex-nested PCR methods for pregnant women with a positive test for T. gondii antibody plus a positive or equivocal test for T. gondii IgM was useful for detecting a high-risk pregnancy and diagnosing congenital T. gondii infection.

Clinical factors associated with pregnancy outcome in women with recurrent pregnancy loss.

The aim of this prospective cohort study was to evaluate clinical factors associated with pregnancy outcomes in women with recurrent pregnancy loss (RPL). Women with a history of two or more pregnancy losses underwent workups for clinical factors of RPL and their pregnancies were followed-up with informed consent. Two hundred eleven (81.5%) of 259 women with RPL became pregnant. The multivariable analyses demonstrated that age (p < .01, OR 0.9, 95%CI 0.97-0.83), uterine abnormality (p < .05, OR 0.3, 95%CI 0.11-0.8), and protein C (PC) deficiency (p < .01, OR 0.14, 95%CI 0.03-0.6) were independent factors for becoming pregnancy in women with RPL. The number of previous pregnancy loss (p < .01, OR 0.57, 95%CI 0.43-0.75) and natural killer (NK) cell activity ≥33% (p < .01, OR 0.31, 95%CI 0.13-0.73) were independent factors for live birth in the subsequent pregnancy. Advanced age, the presence of uterine abnormality, and PC deficiency were risk factors for reduced pregnancy rate in women with RPL. Increased number of previous pregnancy loss and high NK cell activity were risk factors for miscarriage in the subsequent pregnancy. These results involve important information and are helpful for clinical practitioners.

Maternal and neonatal screening methods for congenital cytomegalovirus infection.

Human cytomegalovirus (CMV) is a common cause of congenital infection that may lead to severe long-term sequelae. Because there are no established vaccines, fetal interventions or neonatal treatments, neither maternal nor neonatal screening is recommended. However, recent studies have indicated that early antiviral treatment may improve neurological outcomes in symptomatic infants with congenital infection. Therefore, prenatal detection may be important in newborns at high risk of such infection. Polymerase chain reaction for CMV DNA in the amniotic fluid is considered the gold standard for diagnosis of intrauterine infection, but its use is limited because amniocentesis is an invasive procedure. In a prospective cohort study, we have reported that the presence of CMV DNA in secretions of the maternal uterine cervix were predictive of congenital infection in groups at high risk. However, we also recently demonstrated that maternal serological screening for primary CMV infection using specific immunoglobulin G, the immunoglobulin G avidity index or specific immunoglobulin M can overlook many cases. Previous research has indicated that the combination of early detection by universal neonatal screening of urinary CMV DNA combined with early antiviral therapy can improve outcomes in infants with symptomatic congenital infection. In this article, we review the current state of maternal and neonatal screening for congenital CMV infection.

Efficacy of Valganciclovir Treatment Depends on the Severity of Hearing Dysfunction in Symptomatic Infants with Congenital Cytomegalovirus Infection.

Although earlier studies have shown that antiviral treatment regimens using valganciclovir (VGCV) improved hearing function in some infants with congenital cytomegalovirus (CMV) infection; its efficacy on the severity of hearing dysfunction is unclear. We conducted a prospective study among 26 infants with congenital CMV infections from 2009 to 2018. Oral VGCV (32 mg/kg/day) was administered for 6 weeks (November 2009 to June 2015; n = 20) or 6 months (July 2015 to March 2018, n = 6). Hearing function was evaluated by measuring the auditory brainstem response before VGCV treatment and at 6 months. Hearing dysfunction, defined as a V-wave threshold >40 dB, was categorized into: most severe, ≥91 dB; severe, 61⁻90 dB; and moderate, 41⁻60 dB. Hearing improvement was defined as a decrease of ≥20 dB from the pretreatment V-wave threshold. Of 52 ears in 26 infants with congenital CMV infection, 29 (56%) had hearing dysfunction, and of 29 ears, 16 (55%) improved after VGCV treatment. Although, 16 (84%) of 19 ears with moderate or severe hearing dysfunction improved after treatment (p < 0.001), 10 ears with the most severe form did not. In conclusion, VGCV treatment is effective in improving moderate and severe hearing dysfunction in infants with congenital CMV infection.