Consensus recommendation for prenatal, neonatal and postnatal management of congenital cytomegalovirus infection from the European congenital infection initiative (ECCI).

Congenital cytomegalovirus (cCMV) infection carries a significant burden with a 0.64% global prevalence and a 17-20% chance of serious long-term effects in children. Since the last guidelines, our understanding, particularly regarding primary maternal infections, has improved. A cCMV guidelines group was convened under the patronage of the European Society of Clinical Virology in April 2023 to refine these insights. The quality and validity of selected studies were assessed for potential biases and the GRADE framework was employed to evaluate quality of evidence across key domains. The resulting recommendations address managing cCMV, spanning prevention to postnatal care. Emphasizing early and accurate maternal diagnosis through serological tests enhances risk management and prevention strategies, including using valaciclovir to prevent vertical transmission. The guidelines also strive to refine personalized postnatal care based on risk assessments, ensuring targeted interventions for affected families.

Analysis of congenital hearing loss after neonatal hearing screening.

Introduction: Neonates undergo neonatal hearing screening to detect congenital hearing loss at an early stage. Once confirmed, it is necessary to perform an etiological workup to start appropriate treatment. The study objective was to assess the different etiologies, risk factors, and hearing results of infants with permanent hearing loss and to evaluate the efficacy and consequences of the different screening devices over the last 21 years. Methods: We conducted a single-center retrospective cohort analysis for all neonatal hearing screening program referrals and performed an etiological workup in case of confirmed hearing loss. We analyzed the evolution of the etiological protocols based on these results. Results: The governmental neonatal hearing screening program referred 545 infants to our center. Hearing loss was confirmed in 362 (66.4%) infants and an audiological workup was performed in 458 (84%) cases. 133 (24.4%) infants were diagnosed with permanent hearing loss. Ninety infants (56 bilateral and 34 unilateral) had sensorineural hearing loss, and the degree was predominantly moderate or profound. The most common etiology in bilateral sensorineural hearing loss was a genetic etiology (32.1%), and in unilateral sensorineural hearing loss, an anatomical abnormality (26.5%). Familial history of hearing loss was the most frequently encountered risk factor. Conclusion: There is a significant number of false positives after the neonatal hearing screening. Permanent hearing loss is found only in a limited number of infants. During the 21 years of this study, we noticed an increase in etiological diagnoses, especially genetic causes, due to more advanced techniques. Genetic causes and anatomical abnormalities are the most common etiology of bilateral and unilateral sensorineural hearing loss, respectively, but a portion remains unknown after extensive examinations.

Congenital facial nerve palsy: Single center study.

Objectives: This study will list the most common comorbidities of congenital facial nerve palsy and how to detect and treat them, with special attention for ENT-problems such as hearing loss. Congenital facial nerve palsy is a very rare entity but in UZ Brussels hospital there was a follow-up of 16 children in the last 30 years. Methods: Literature review has been done, combined with thorough research of our own series of 16 children with congenital facial nerve palsy. Results: Congenital facial nerve palsy can be part of a known syndrome, most commonly Moebius syndrome, but can also appear solely. It appears often bilateral and with a severe gradation. In our series, hearing loss is frequently seen in association with congenital facial nerve palsy. Other abnormalities are dysfunction of the abducens nerve, ophthalmological problems, retro- or micrognathism and abnormalities of limbs or heart. The majority of the children in our series underwent radiological imaging (CT and/or MRI): the facial nerve but also the vestibulocochlear nerve and middle and inner ear can be evaluated. Conclusion: A multidisciplinary approach of congenital facial nerve palsy is recommended as it can affect various bodily functions. Radiological imaging needs to be done to acquire additional information that can be useful for diagnostic and therapeutic purposes. Although congenital facial nerve palsy may not be treatable itself, its comorbidities can be treated and improve the quality of life of the affected child.

Hearing aid use and gender differences in the auditory-cognitive cascade in the oldest old.

OBJECTIVES: This study analyzed cognitive differences between hearing-aid (HA) and non-HA users. We hypothesized that HA-use attenuates the auditory-cognitive cascade, thereby, the latter is more conspicuous in non-HA users. Since hearing impairment (HI) shows male predominance, we hypothesized gender differences within the auditory-cognitive relationship. METHODS: Non-frail community-dwellers ≥ 80 years were assessed for HI (pure tone audiogram-PTA; speech reception threshold-SRT) and global and domain-specific cognitive impairments (Mini-Mental State Examination-MMSE; Montreal Cognitive Assessment-MOCA; Reaction Time Test-RT1-4). Pearson and partial correlations (correcting for age and PTA) assessed auditory-cognitive associations within gender and HA subgroups. Fisher's z test compared correlations between HA and non-HA users. RESULTS: 126 participants (age range 80-91 years) were included. HA-use prevalence was 21%. HA-users were older with worse HI (mean PTA 49.5dBHL). HA-users exhibited no significant auditory (PTA, SRT) and cognitive (MMSE, MOCA, RT1- RT4) correlations. Male non-HA users, displayed a significant association between HI and global cognition, processing speed, selective and alternating attention. Significant differences were noted between MMSE and PTA and SRT (z-score 2.28, 3.33, p = 0.02, <0.01, respectively) between HA and non-HA users. CONCLUSION: Male non-HA users displayed an association between HI and global and domain-specific (processing speed; selective and alternating attention) cognitive decline. Associations between global cognition and HI were significantly different between HA and non-HA users. This may be partially attributable to underlying subgroups sample sizes and statistical power disparity. If larger scale longitudinal or interventional studies confirm these findings, timely HI assessment and management may be the cornerstone for delaying cognitive decline.

Audiometric Findings in Senior Adults of 80 Years and Older.

Objective: To examine hearing thresholds in senior adults of 80 years and older and compare this data to the current ISO 7029 reference values. Design: A descriptive, prospective study testing pure-tone and speech audiometry in senior adults participating in the BUTTERFLY study or the BrUssels sTudy on The Early pRedictors of FraiLtY. A Gerontological study to identify determinants for active aging and for early stages of frailty in the oldest population. Using the formula given by ISO 7028:2017 the median value of hearing was calculated based on the sex and age of the participant and compared to the measured hearing thresholds. Results: 151 senior adults were included. The prevalence of hearing loss was 90.7% (PTA > 20 dB HL). The results were compared to the mean ISO values, calculated for every participant. Both males and females in our study population had worse hearing thresholds than could be expected based on the ISO reference values. In our study population with moderate hearing loss (PTA > 40 dB HL), 38% is underserved in term of hearing restoration healthcare and yet another 38% is unsatisfied with the result of the hearing aids. Given the vast impact on the individual and society, this is a problem in need of our attention. Conclusion: The ISO 7029 reference values may be an underestimation of hearing loss in senior adults of 80 years and older. Therefore we present a statistical distribution of hearing thresholds on different frequencies related to age and sex that can be used as a baseline for further development of the reference values.

Three Years of Vestibular Infant Screening in Infants With Sensorineural Hearing Loss.

OBJECTIVES: Although vestibular deficits are more prevalent in hearing-impaired children and can affect their development on many levels, a pediatric vestibular assessment is still uncommon in clinical practice. Since early detection may allow for timely intervention, this pioneer project has implemented a basic vestibular screening test for each six-month-old hearing-impaired infant in Flanders, Belgium. This study aims to report the vestibular screening results over a period of three years and to define the most important risk factors for abnormal vestibular screening results. METHODS: Cervical Vestibular Evoked Myogenic Potentials with bone-conduction were used as a vestibular screening tool in all reference centers affiliated to the Universal Newborn Hearing Screening Program in Flanders. From June 2018 until June 2021, 254 infants (mean age: 7.4 months, standard deviation: 2.4 months) with sensorineural hearing loss were included. RESULTS: Overall, abnormal vestibular screening results were found in 13.8% (35 of 254) of the infants. The most important group at risk for abnormal vestibular screening results were infants with unilateral or bilateral severe to profound sensorineural hearing loss (20.8%, 32 of 154) (P < .001, odds ratio = 9.16). Moreover, abnormal vestibular screening results were more prevalent in infants with hearing loss caused by meningitis (66.7%, 2 of 3), syndromes (28.6%, 8 of 28), congenital cytomegalovirus infection (20.0%, 8 of 40), and cochleovestibular anomalies (19.2%, 5 of 26). CONCLUSIONS: The vestibular screening results in infants with sensorineural hearing loss indicate the highest risk for vestibular deficits in severe to profound hearing loss, and certain underlying etiologies of hearing loss, such as meningitis, syndromes, congenital cytomegalovirus, and cochleovestibular anomalies.

Pediatric myringoplasty: A study of effectiveness and influencing factors.

OBJECTIVES: Until today, there is no consensus about the ideal age for a myringoplasty in children. In this retrospective study, we study our own series to characterize different prognostic factors to answer questions/dilemmas such as when to carry out surgery in a child with an ear drum perforation, when to postpone surgery or when to use a different technique to improve the outcome after tympanoplasty. METHODS: We performed a retrospective study on charts of 97 children who underwent a myringoplasty. The same surgeon (IF) treated all included children and with the same classical surgical technique: retro-auricular approach and microscopic underlay placement of fascia of the musculus temporalis. Children with associated disease (cholesteatoma, revision surgery and ossicular chain defects) were excluded. All children had a minimum follow up of 12 months. A successful procedure was defined as a closed eardrum after 12 months and an air bone gap <20 dB. Prognostic factors were inventoried and studied. RESULTS: Success rate after myringoplasty is 80.2% in this pediatric case series. Age was not a statistical significant prognostic factor. Only the history of an adenoidectomy had a positive effect on tympanic closure (p = 0.047). A negative prognostic factor was the size of the perforation: large perforations showed only 42.9% eardrum closure (p = 0.040). There was a complication rate of 28.9%, in which formation of granulation tissue and ear discharge were most common but easily treated. CONCLUSION: Tympanoplasty type 1 with musculus temporalis fascia in underlay is a safe and successful technique in children of all ages with eardrum perforations. Our data suggests using a different technique (cartilage tympanoplasty) in cases with large perforation. Postponing surgery is not advocated, unless perhaps in children with poor Eustachian function or adenoidhyperplasia.

Vestibular Infant Screening (VIS)-Flanders: results after 1.5 years of vestibular screening in hearing-impaired children.

Due to the close anatomical relationship between the auditory and vestibular end organs, hearing-impaired children have a higher risk for vestibular dysfunction, which can affect their (motor) development. Unfortunately, vestibular dysfunction often goes unnoticed, as vestibular assessment in these children is not standard of care nowadays. To timely detect vestibular dysfunction, the Vestibular Infant Screening-Flanders (VIS-Flanders) project has implemented a basic vestibular screening test for hearing-impaired infants in Flanders (Belgium) with a participation rate of 86.7% during the first year and a half. The cervical Vestibular Evoked Myogenic Potentials (cVEMP) test was applied as vestibular screening tool to map the occurrence of vestibular (mainly saccular) dysfunction in this population. At the age of 6 months, 184 infants were screened. No refers on vestibular screening were observed in infants with permanent conductive hearing loss. In infants with permanent sensorineural hearing loss, a cVEMP refer rate of 9.5% was observed. Failure was significantly more common in infants with severe-profound compared to those with mild-moderate sensorineural hearing loss (risk ratio = 9.8). Since this is the first regional study with a large sample size and successful participation rate, the VIS-Flanders project aims to set an example for other regions worldwide.

Percutaneous tracheostomy for long-term ventilated COVID-19-patients: rationale and first clinical-safe for all-experience.

INTRODUCTION: COVID-19 infection has resulted in thousands of critically ill patients admitted to ICUs and treated with mechanical ventilation. Percutaneous tracheostomy is a well-known technique utilised as a strategy to wean critically ill patients from mechanical ventilation. Worldwide differences exist in terms of methods, operators, and settings, and questions remain regarding timing and indications. If tracheostomy is to be performed in COVID-19 patients, a safe environment is needed for optimal care. MATERIAL AND METHODS: We present a guidewire dilating forceps tracheostomy procedure in COVID-19 patients that was optimised including apnoea-moments, protective clothing, checklists, and clear protocols. We performed a retrospective analysis of the outcome after tracheostomy in COVID-19 patients between March 2020 and May 2020. RESULTS: The follow-up of the first 16 patients, median age 62 years, revealed a median intubation time until tracheostomy of 18 days and median cannulation time of 20 days. The overall perioperative complication rate and complication rate while cannulated was 19%, mainly superficial bleeding. None of the healthcare providers involved in performing the procedure developed any symptoms of the disease. CONCLUSIONS: This COVID-19-centred strategy based on flexibility, preparation, and cooperation between healthcare providers with different backgrounds facilitated percutaneous tracheostomy in COVID-19 patients without an increase in the overall complication rate or evidence of risk to healthcare providers. Our findings provide initial evidence that tracheostomy can be performed safely as a standard of care for COVID-19 patients requiring prolonged mechanical ventilation as was standard practice in ICU patients prior to the COVID-19 pandemic to promote ventilator weaning and patient recovery.

Congenital Cytomegalovirus Infection: A Narrative Review of the Issues in Screening and Management From a Panel of European Experts.

Maternal primary and non-primary cytomegalovirus (CMV) infection during pregnancy can result in in utero transmission to the developing fetus. Congenital CMV (cCMV) can result in significant morbidity, mortality or long-term sequelae, including sensorineural hearing loss, the most common sequela. As a leading cause of congenital infections worldwide, cCMV infection meets many of the criteria for screening. However, currently there are no universal programs that offer maternal or neonatal screening to identify infected mothers and infants, no vaccines to prevent infection, and no efficacious and safe therapies available for the treatment of maternal or fetal CMV infection. Data has shown that there are several maternal and neonatal screening strategies, and diagnostic methodologies, that allow the identification of those at risk of developing sequelae and adequately detect cCMV. Nevertheless, many questions remain unanswered in this field. Well-designed clinical trials to address several facets of CMV treatment (in pregnant women, CMV-infected fetuses and both symptomatic and asymptomatic neonates and children) are required. Prevention (vaccines), biology and transmission factors associated with non-primary CMV, and the cost-effectiveness of universal screening, all demand further exploration to fully realize the ultimate goal of preventing cCMV. In the meantime, prevention of primary infection during pregnancy should be championed to all by means of hygiene education.

Cytomegalovirus infection during pregnancy: state of the science.

Cytomegalovirus is the most common congenital infection, affecting 0.5-2% of all live births and the main nongenetic cause of congenital sensorineural hearing loss and neurological damage. Congenital cytomegalovirus can follow maternal primary infection or nonprimary infection. Sensorineurological morbidity is confined to the first trimester with up to 40-50% of infected neonates developing sequelae after first-trimester primary infection. Serological testing before 14 weeks is critical to identify primary infection within 3 months around conception but is not informative in women already immune before pregnancy. In Europe and the United States, primary infection in the first trimester are mainly seen in young parous women with a previous child younger than 3 years. Congenital cytomegalovirus should be evoked on prenatal ultrasound when the fetus is small for gestation and shows echogenic bowel, effusions, or any cerebral anomaly. Although the sensitivity of routine ultrasound in predicting neonatal symptoms is around 25%, serial targeted ultrasound and magnetic resonance imaging of known infected fetuses show greater than 95% sensitivity for brain anomalies. Fetal diagnosis is done by amniocentesis from 17 weeks. Prevention consists of both parents avoiding contact with body fluids from infected individuals, especially toddlers, from before conception until 14 weeks. Candidate vaccines failed to provide more than 75% protection for >2 years in preventing cytomegalovirus infection. Medical therapies such as cytomegalovirus hyperimmune globulins aim to reduce the risk of vertical transmission but 2 randomized controlled trials have not found any benefit. Valaciclovir given from the diagnosis of primary infection up to amniocentesis decreased vertical transmission rates from 29.8% to 11.1% in the treatment group in a randomized controlled trial of 90 pregnant women. In a phase II open-label trial, oral valaciclovir (8 g/d) given to pregnant women with a mildly symptomatic fetus was associated with a higher chance of delivering an asymptomatic neonate (82%), compared with an untreated historical cohort (43%). Valganciclovir given to symptomatic neonates is likely to improve hearing and neurological symptoms, the extent of which and the duration of treatment are still debated. In conclusion, congenital cytomegalovirus infection is a public health challenge. In view of recent knowledge on diagnosis and pre- and postnatal management, health care providers should reevaluate screening programs in early pregnancy and at birth.

A focused 35-minute whole body MRI screening protocol for patients with von Hippel-Lindau disease.

BACKGROUND: Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited tumor syndrome. Affected patients develop central nervous system hemangioblastomas and abdominal tumors, among other lesions. Patients undergo an annual clinical screening program including separate magnetic resonance imaging (MRI) of the brain, whole spine and abdomen. Consequently, patients are repeatedly subjected to time-consuming and expensive MRI scans, performed with cumulative Gadolinium injections. We report our experience with a 35-min whole body MRI screening protocol, specifically designed for detection of VHL-associated lesions. METHODS: We designed an MRI protocol dedicated to the typical characteristics of VHL-associated lesions in different imaging sequences, within the time frame of 35 min. Blank imaging of the abdomen is carried out first, followed by abdominal sequences with Gadolinium contrast. Next, the full spine is examined, followed by imaging of the brain. A single dose of contrast used for abdominal imaging is sufficient for further highlighting of spine- and brain lesions, thus limiting the Gadolinium dosage. We used 1.5 Tesla equipment, dealing with fewer artifacts compared to a 3 Tesla system for spine- and abdominal imaging, while preserving acceptable quality for central nervous system images. In addition, imaging on a 1.5 Tesla scanner is slightly faster. RESULTS: From January 2016 to November 2018, we performed 38 whole body screening MRIs in 18 VHL patients; looking for the most common types of VHL lesions in the abdomen, spine, and brain, both for new lesions and follow-up. The one-step approach MRI examinations lead to 6 surgical interventions for clinically significant or symptomatic hemangioblastomas in the brain and spine. One renal cell carcinoma was treated with radiofrequency ablation. In comparison with previous conventional MRI scans of the same patients, all lesions were visible with the focused protocol. CONCLUSIONS: Annual screening in VHL disease can be done in a rapid, safe and sensitive way by using a dedicated whole body MRI protocol; saving MRI examination time and limiting Gadolinium dose.

Hearing Loss With Congenital Cytomegalovirus Infection.

OBJECTIVE: In this study, we determined the prevalence of hearing loss in 157 children with proven congenital cytomegalovirus (cCMV) infection. We looked at possible risk determinants for developing hearing loss and proposed recommendations for screening and follow-up in the newborn. METHODS: In a prospective 22-year study, 157 children with proven cCMV infection were evaluated for sensorineural hearing loss (SNHL). The development of SNHL was correlated with the type of maternal infection (primary versus nonprimary), the gestational age of maternal primary infection, imaging findings at birth, and the presence of symptomatic or asymptomatic infection in the newborn. RESULTS: Of all children, 12.7% had SNHL, and 5.7% needed hearing amplification because of SNHL. Improvement, progression, and fluctuations of hearing thresholds were seen in 45%, 53.8%, and 5.7% of the children, respectively. Hearing loss was more common in the case of a symptomatic infection at birth (P = .017), after a maternal primary infection in the first trimester of pregnancy (P = .029), and in the presence of abnormalities on a neonatal brain ultrasound and/or MRI (P < .001). CONCLUSION: SNHL is a common sequela in children with cCMV infection. Risk factors for SNHL were primary maternal infections before the 14th week of pregnancy, the presence of a disseminated infection at birth, and imaging abnormalities in the newborn. These children may benefit from a more thorough investigation for SNHL than children who do not present with those risk factors.

Detection of CMV DNA in the perilymph of a 6-year-old boy with congenital cytomegalovirus infection.

We present the case of a 6-year-old boy who received a cochlear implant for profound sensorineural hearing loss after being born with cytomegalovirus (CMV) infection. Even after 6 years, CMV DNA was still found in the perilymph of the cochlea. Our case shows that CMV DNA can be present in the cochlea years after congenital CMV infection, and it can explain why progressive and/or late-onset hearing loss occurs in these children.

Primary maternal cytomegalovirus infections: accuracy of fetal ultrasound for predicting sequelae in offspring.

BACKGROUND: Cytomegalovirus infection is the most common perinatal viral infection that can lead to severe long-term medical conditions. Antenatal identification of maternal cytomegalovirus infections with proven fetal transmission and potential postnatal clinical sequelae remains a major challenge in perinatology. There is a need to improve the prenatal counseling offered to patients and guide future clinical management decisions in cases of proven primary cytomegalovirus infection. OBJECTIVE: We sought to evaluate the accuracy of fetal ultrasound for predicting sequelae in fetuses infected with congenital cytomegalovirus after maternal primary infection. STUDY DESIGN: We conducted a prospective observational study from 1996 through 2012 in pregnant women with serological evidence of primary cytomegalovirus infection and proven vertical transmission to the fetus, based on viral load in the amniotic fluid. Fetal ultrasound was performed in all patients. Pregnancy termination was presented as an option for infected fetuses. Hearing and neurological clinical assessments were performed for all neonates with cytomegalovirus-positive urine samples. RESULTS: A total of 67 patients (69 fetuses) with proven vertical transmission were included in this study, including 64 singleton and 3 twin pregnancies. Eight fetuses were lost to follow-up. Of the remaining 61 fetuses, termination of the pregnancy was performed for 26, including 11 with fetal ultrasound anomalies. Autopsy provided histological evidence of fetal cytomegalovirus infection in all cases. In the 15 terminated fetuses without ultrasound anomalies, histological evidence of damage caused by fetal infection was detected in 13 cases. Among the 35 live-born infants, 12 had fetal ultrasound anomalies suggestive of congenital infection. Of these 12 infants, 6 had normal clinical evaluations, whereas 6 presented with either hearing and/or neurological anomalies, classified as severe in 4 cases. Among the 23 live-born infants with normal prenatal ultrasound, 5 developed hearing impairments and 1 showed mild neurological developmental delay. CONCLUSION: Fetal ultrasound anomalies were detected in 37.7% of pregnant women with primary cytomegalovirus infection acquired in early pregnancy and proven fetal infection, and were confirmed by autopsy or postnatal clinical evaluation in 73.9%. Autopsy or postnatal clinical evaluation also detected cytomegalovirus-related anomalies in 55% of infants with normal fetal ultrasound evaluations.

Congenital cytomegalovirus infection: contribution and best timing of prenatal MR imaging.

OBJECTIVE: To predict sensorineural hearing loss (SNHL) and neurological impairment in congenital cytomegalovirus (cCMV) infection using MR imaging and define the best timing in pregnancy for prenatal assessment. METHODS: In 121 patients with confirmed cCMV infection, brain features at MR imaging were respectively graded from 1 to 5: normal; isolated frontal/parieto-occipital hyperintensity; temporal periventricular hyperintensity; temporal/occipital cysts and/or intraventricular septa; migration disorders. Grading was correlated with postnatal SNHL and neurological impairment using regression analysis. In 51 fetuses with MR examinations at 26.9 and 33.0 weeks, the predictive value of SNHL and neurological impairment was compared using ROC curves. RESULTS: Postnatal follow-up showed SNHL in 18 infants and neurological impairment in 10. MR grading was predictive of SNHL and of neurological impairment (P < 0.001). In grade 1 or 2, none had SNHL and 1/74 had neurological impairment. The areas under ROC curves for prediction of postnatal SNHL and of neurological impairment from first and second MR examination were comparable. CONCLUSION: Our data suggest that in cCMV infection, prediction of SNHL and neurological impairment is feasible by fetal MR imaging with a high negative predictive value and can equally be done at 27 or 33 weeks of gestation. KEY POINTS: • In cCMV, isolated periventricular T2-weighted signal hyperintensity has a good postnatal prognosis. • In cCMV, SNHL and neurological impairment can be predicted at 27 or 33 weeks. • In cCMV, fetal MR has a high NPV in predicting SNHL. • In cCMV, fetal MR has a high NPV in predicting neurological impairment.

Hearing configuration in children with cCMV infection and proposal of a flow chart for hearing evaluation.

OBJECTIVE: This study had three main goals: (1) to determine the hearing configuration in hearing-impaired children born with a congenital CMV (cCMV) infection, (2) to see whether auditory neuropathy spectrum disorder (ANSD) was present, and (3) to propose a flow chart for the follow-up of hearing in children with cCMV. DESIGN: Hearing configuration and the presence of ANSD in cCMV infected children was analysed. Selection criteria were: hearing-impaired children with a regular audiometric follow-up for at least 36 months, no other major risk factors for hearing loss, a normal middle-ear status, and an appropriate behavioral response to the given pure-tone stimuli. STUDY SAMPLE: Out of a cohort of 206 cCMV infected children, 18 hearing-impaired children were selected. RESULTS: Audiograms of all children showed a flat configuration of SNHL: the slope between octave bands was never greater than 10 decibels. None of the 18 children were found to have ANSD. CONCLUSIONS: Hearing impairment in cCMV infants affected all frequencies equally and ANSD does not appear to be a feature of cCMV infection. A flow chart for hearing follow-up in children with cCMV infection was suggested in order to provide guidance, improve uniformity in follow-up, and to make results easier to compare.

Hearing thresholds in children with a congenital CMV infection: a prospective study.

OBJECTIVE: Hearing thresholds in children with a congenital cytomegalovirus (cCMV) infection are not always stable. Children can develop late onset hearing loss, fluctuations, progression (worsening) and improvement of hearing loss. Knowledge about these characteristics is important to understand why long term follow up in these children is mandatory. METHODS: We prospectively follow a cohort of 154 children with cCMV infection, 68 of which met the inclusion criteria of at least 3 hearing evaluations over a period of at least 18 months in the absence of other risk factors for hearing loss. In those 68 children we evaluated the occurrence of unstable hearing thresholds: late onset hearing loss, fluctuations, progression and improvement of hearing loss. RESULTS: Unstable hearing thresholds were observed in 29.4% of children with cCMV infection of which 19.2% were found in the group of children with ultimately normal hearing and in 62.5% of children with sensorineural hearing loss (SNHL) (p=0.0027). Fluctuations occurred in 16.2%. Late onset hearing loss occurred in 4.3% of children with a normal hearing at birth. In children with SNHL, progression or worsening of hearing thresholds occurred in 27.3% and improvement of thresholds in 40.9%. Important changes in thresholds only occurred in 13.2% of all children and predominantly in children who finally develop SNHL. CONCLUSIONS: Unstable hearing thresholds are frequently found in children with cCMV infection and occur not only in children who develop hearing losses but also in children who have a normal hearing at the last visit. Important changes in hearing thresholds of > 30 dB are more frequently seen in children who ultimately will develop SNHL.

Hearing loss in children with congenital cytomegalovirus infection in relation to the maternal trimester in which the maternal primary infection occurred.

OBJECTIVES: The purpose of this work was to study the relation between maternal trimester of primary infection with cytomegalovirus and the occurrence of sensorineural hearing loss in the congenitally infected offspring. PATIENTS AND METHODS: Thirty-four consecutive live-born children diagnosed with a congenital cytomegalovirus infection after maternal primary cytomegalovirus infections were included in the study. Five were lost for follow-up, and 1 died. Of the remaining 28 congenitally infected children, an estimation of the maternal trimester in which cytomegalovirus primary infection occurred was performed. All of the children were investigated for potential sensorineural hearing loss. RESULTS: Five of the maternal infections occurred in the first trimester, 12 in the second trimester, and 11 in the third trimester of pregnancy. Sensorineural hearing loss was detected in 4 (80%) of the 5 congenitally infected children who were infected after a primary maternal infection in the first trimester of pregnancy and in 1 (8%) of the 12 children when the maternal infection occurred in the second trimester of pregnancy. No sensorineural hearing loss was detected after primary maternal infection occurring in the third trimester. Fluctuation and improvement of sensorineural hearing loss were seen regardless the trimester of pregnancy during which maternal primary infection occurred. Progression of sensorineural hearing loss occurred in 2 children born after a maternal primary infection of the first trimester. CONCLUSIONS: Hearing loss seemed more common in infants with congenital cytomegalovirus infection who were born to women who experienced a primary cytomegalovirus infection in the first trimester of pregnancy than when infection took place later in pregnancy.