Thyroglossal duct cysts in children: a 30-year survey with emphasis on clinical presentation, surgical treatment, and outcome.

Background: Thyroglossal duct cysts (TGDC) are one of the most common congenital anomalies found in the anterior neck region of children. Sistrunk's procedure, described in 1920 already, is still considered as the gold standard. However, clinical reality shows that in a minority of patients, marsupialization and simple cyst excision are still performed as well. Cyst recurrence is the most feared complication. The main goal of this retrospective study is to determine risk factors of recurrence. Furthermore, data on presentation characteristics, management and outcome were collected as well. Methods: The data of 104 patients aged between 0 and 16 years who underwent surgery for TGDC at the University Hospital of Brussels between 1986 and 2016 were retrospectively analyzed. We focused on aspects of clinical presentation, intra- and postoperative treatment and long-term follow-up. Results: Overall recurrence of TGDC was seen in twelve of the 104 cases (11.5%). Eight out of these 12 showed a preoperative infection, 4 out of 12 had intra-operative cyst rupture. Five out of the 12 patients had not been treated by the Sistrunk procedure, but by cyst excision or marsupialization only. Non-adherence to the Sistrunk procedure appeared to be the only significant risk factor of TGDC recurrence. Conclusion: Our study shows that Sistrunk's operation for thyroglossal duct cyst in pediatric patients is significantly superior in reducing the risk of cyst recurrence compared to other surgical treatments. Preoperative infection and cyst rupture did not influence the recurrence rates.

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.

Fusobacterium necrophorum and other Fusobacterium spp. isolated from head and neck infections: A 10-year epidemiology study in an academic hospital.

BACKGROUND: Fusobacterium spp. from clinical specimens are increasingly reported. We sought to describe the epidemiology, the microbiological, and the clinical characteristics of head and neck infections caused by Fusobacterium necrophorum and other Fusobacterium spp. MATERIALS AND METHODS: Retrospective cohort study between October 1st, 2004 and September 30(th), 2014 performed in an academic hospital. Electronic patient charts and the laboratory information system were reviewed for demographic and microbiological data. The number and percentages of specific diagnosis and treatment among patients with positive Fusobacterium spp. culture were calculated. The incidence was calculated based on the number of specimens investigated each year. RESULTS: Included were 230 cultures of 230 patients (median age of 28 years, 61.7% men). F. necrophorum was often found in young patients with high C-reactive protein (CRP) and high number of leukocytes in blood. Other Fusobacterium spp. were often found in middle aged patients with relatively high CRP and slightly increased leukocytes. Three major causes of the isolation of F. necrophorum and other Fusobacterium spp. were acute tonsillitis (n = 18, incidence of 0.2%), peritonsillar abscess (n = 39, 0.5%) and acute otitis (n = 45, 0.1%). While F. necrophorum was found in majority (37/57) of patients with acute tonsillitis or peritonsillar abscess, Fusobacterium spp. other than F. necrophorum were found in the majority (35/45) of patients with acute otitis. Isolated fusobacteria were susceptible to beta-lactam antibiotics, clindamycin and metronidazole. The outcomes of patients with Fusobacterium spp. were good. CONCLUSION: F. necrophorum and other Fusobacterium spp. are rare cause of head and neck infections. The infections are well treated by combination of antibiotics and surgery.

Characteristics of a Dysphonic Population Referred for Voice Assessment and/or Voice Therapy.

OBJECTIVE: The aim of this study was to define patient characteristics of a dysphonic population in terms of voice disorder, gender, age, and subjective and objective vocal parameters and to explore the relevant characteristics of the most frequent groups of voice disorders. PATIENTS AND METHODS: Patient records from 4,447 subjects referred for voice assessment and/or voice therapy were analyzed. RESULTS: Significantly more cases of dysphonia were structural as compared to nonstructural. This significant difference was found in almost all age groups. Significantly more women were diagnosed with dysphonia than men. The most common symptom was light-to-moderate hoarseness. The average voice handicap index was 31 and the average dysphonia severity index was -0.6. Vocal fold nodules (VFN), functional voice disorders (FVD) and vocal fold paralysis (VFP) were the three most frequently diagnosed vocal pathologies and were analyzed in detail. Women were found to be significantly more vulnerable to FVD, VFN and cysts, whereas men were significantly more often diagnosed with carcinoma, hyperkeratosis, laryngitis, papillomatosis, presbyphonia, puberphonia and VFP. CONCLUSIONS: The results of this study allow a better estimation of the clinical needs and costs for a specific dysphonic population looking for help and highlight the gender-related risks of specific voice disorders.

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.

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.

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.

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.

Floating-Harbor syndrome associated with middle ear abnormalities.

Floating-Harbor syndrome is a rare syndrome of unknown etiology, which was first described in 1973. A triad of main features characterizes Floating-Harbor syndrome: short stature, characteristic face, and an expressive speech delay. We present a patient in whom the hearing thresholds improved insufficiently after placement of grommets. High-resolution CT scan of the temporal bone showed a prominent soft-tissue thickening suspected of causing fixation of the malleus, and fusion of the malleus head with the body of the incus. To our knowledge this is the first reported abnormal middle ear anatomy in a patient with Floating-Harbor syndrome. A conservative treatment with hearing aids was preferred as an initial treatment in favor of a surgical exploration.

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.

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.

A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection.

OBJECTIVE: To determine the incidence, characteristics, and evolution of sensorineural hearing loss (SNHL) in infants with a congenital cytomegalovirus infection (cCMV). STUDY DESIGN: In a prospective 10-year study, 14 021 unselected live-born infants were screened for cCMV by virus isolation in urine. Congenitally infected newborns were evaluated for SNHL during the first 5 years of life. RESULTS: A total of 74 of the 14 021 infants (0.53%) were congenitally infected; of these, 4 (5.4%) were symptomatic at birth. Hearing testing could be performed in 60 of the infants. SNHL was found in 21% of the asymptomatic and in 33% of symptomatic congenitally infected infants. Late-onset hearing loss was detected in 5%, progression in 11%, fluctuation in 16%, and improved hearing threshold in 18% of the infants with cCMV. SNHL was observed in 15% of infected infants born after a maternal primary infection, in 7% born after a maternal recurrent infection, and in 40% after a maternal infection of indeterminate timing. CONCLUSIONS: In our study population, 0.53% of the infants had cCMV infection, 22% of whom developed SNHL. Long-term follow up and repeated audiologic testing is needed, because progression, fluctuation, improvement, and late-onset hearing loss are important features of cCMV infection. The search for a neonatal screening program to detect all cCMV is worthwhile.

Audiometric analyses confirm a cochlear component, disproportional to age, in stapedial otosclerosis.

OBJECTIVE: To report the preoperative audiometric profile of surgically confirmed otosclerosis. STUDY DESIGN: Retrospective, multicenter study. SETTING: Four tertiary referral centers. PATIENTS: One thousand sixty-four surgically confirmed patients with otosclerosis. INTERVENTIONS: Therapeutic ear surgery for hearing improvement. MAIN OUTCOME MEASURES: Preoperative audiometric air conduction (AC) and bone conduction (BC) hearing thresholds were obtained retrospectively for 1064 patients with otosclerosis. A cross-sectional multiple linear regression analysis was performed on audiometric data of affected ears. Influences of age and sex were analyzed and age-related typical audiograms were created. Bone conduction thresholds were corrected for Carhart effect and presbyacusis; in addition, we tested to see if separate cochlear otosclerosis component existed. Corrected thresholds were than analyzed separately for progression of cochlear otosclerosis. RESULTS: The study population consisted of 35% men and 65% women (mean age, 44 yr). The mean pure-tone average at 0.5, 1, and 2 kHz was 57 dB hearing level. Multiple linear regression analysis showed significant progression for all measured AC and BC thresholds. The average annual threshold deterioration for AC was 0.45 dB/yr and the annual threshold deterioration for BC was 0.37 dB/yr. The average annual gap expansion was 0.08 dB/year. The corrected BC thresholds for Carhart effect and presbyacusis remained significantly different from zero, but only showed progression at 2 kHz. CONCLUSION: The preoperative audiological profile of otosclerosis is described. There is a significant sensorineural component in patients with otosclerosis planned for stapedotomy, which is worse than age-related hearing loss by itself. Deterioration rates of AC and BC thresholds have been reported, which can be helpful in clinical practice and might also guide the characterization of allegedly different phenotypes for familial and sporadic otosclerosis.

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.

Acute sore throat in children at the emergency department: best medical practice?

BACKGROUND: Belgian antibiotic (AB) policy guidelines discourage the use of ABs in sore throat in otherwise healthy individuals; yet, 35% of all children with sore throat in our pediatric emergency department receive a prescription for ABs. OBJECTIVE: To identify factors influencing our physician's prescription behavior. PATIENTS AND METHODS: Using a retrospective study, we analyzed all medical records of children younger than 16 years of age diagnosed with sore throat (in 2009 and 2010). We included 1345 files. RESULTS: Children younger than 5 years of age received an AB prescription more easily (37.8 vs. 27.7%; P=0.0007). Children of White origin received less frequent ABs compared with non-Whites (31.6 vs. 37.8%; P=0.02). More ABs were prescribed during night shifts (38.7 vs. 31.8%; P=0.008). Physicians with a Belgian degree prescribed less frequently compared with those with a Dutch degree (23.4 vs. 46.4%; P<0.0001). CONCLUSION: We find several 'practical' factors to have an influence on the AB prescription rate.

Centor criteria in children in a paediatric emergency department: for what it is worth.

INTRODUCTION: Centor criteria (fever >38.5°C, swollen, tender anterior cervical lymph nodes, tonsillar exudate and absence of cough) are an algorithm to assess the probability of group A ß haemolytic Streptococcus (GABHS) as the origin of sore throat, developed for adults. We wanted to evaluate the correlation between Centor criteria and presence of GABHS in children with sore throat admitted to our paediatric emergency department (PED). DESIGN: Retrospective cohort study. SETTING: The emergency department of a large tertiary university hospital in Brussels, with over 20 000 yearly visits for children below age 16. PARTICIPANTS: All medical records (from 2008 to 2010) of children between ages 2 and 16, who were diagnosed with pharyngitis, tonsillitis or sore throat and having a throat swab culture for GABHS. Children with underlying chronic respiratory, cardiac, haematological or immunological diseases and children who had already received antibiotics (AB) prior to the PED consult were excluded. Only records with a full disease history were selected. Out of a total 2118 visits for sore throats, 441 met our criteria. The children were divided into two age groups, 2-5 and 5-16 years. RESULTS: The prevalence of GABHS was higher in the older children compared to the preschoolers (38.7 vs 27.6; p=0.01), and the overall prevalence was 32%. There was no significant difference in the prevalence of GABHS for all different Centor scores within an age group. Likelihood ratios (LR) demonstrate that none of the individual symptoms or a Centor score of ≥3 seems to be effective in ruling in or ruling out GABHS. Pooled LR (CI) for Centor ≥3 was 0.67 (CI 0.50 to 0.90) for the preschoolers and 1.37 (CI 1.04 to 1.79) for the older children. CONCLUSIONS: Our results confirm the ineffectiveness of Centor criteria as a predicting factor for finding GABHS in a throat swab culture in children.

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.

A pilot with an intravestibular schwannoma: to fly or not to fly?

OBJECTIVE: To report on the professional repercussions of an intravestibular schwannoma in a commercial airline pilot. PATIENTS: The case of a single patient with a unilateral intravestibular schwannoma. INTERVENTION: Return to a flying status with specific restrictions and under tight conditions depending on the outcomes of a mandatory periodic watch-and-rescan policy. MAIN OUTCOME MEASURES: Consecutive magnetic resonance imaging results coupled to the clinical otovestibular status of the patient. RESULTS: After 16 uneventful months of flying, the pilot was grounded after a first episode of vertigo. CONCLUSION: The combination of an intravestibular schwannoma-as one of the rarest intralabyrinthine schwannomas-and a pilot-for whom proper otovestibular functioning is of the uppermost importance-challenges the responsibilities of all involved. Depending on the clinical circumstances and under tight conditions with specific restrictions, a return to a flying status can very exceptionally be considered. An open discussion and the establishment of a relation of mutual trust are felt to be an absolute prerequisite.

Mastoid pneumocele with secondary pneumatocele causing external auditory canal obstruction and the influence of forced nose blowing.

We present a case involving a 36-year-old man who had a feeling that his right ear was obstructed and who had associated conductive hearing loss that was exacerbated by nose blowing. On otoscopy, a soft-tissue swelling was seen in the posterosuperior aspect of the right external auditory canal. Computed tomography revealed the presence of hyperaerated mastoid air cells and an air-containing cavity connected with the enlarged mastoid air cells, narrowing the external auditory canal. A radiographic diagnosis of a mastoid pneumocele with secondary pneumatocele was made. A novel hypothesis is presented, that high nasal pressures play an important role in the progressive formation of a pneumocele/pneumatocele as a result of air invasion via the eustachian tube. Symptoms disappeared after a myringotomy tube was placed. Computed tomography performed 6 months later showed an impressive remodeling of the temporal bone.

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.