Disorder-Not CHARGE Syndrome-Presenting as Isolated Cochleovestibular Dysfunction.

CHARGE syndrome, characterized by a distinct set of clinical features, has been linked primarily to mutations in the gene. Initially defined by specific clinical criteria, including coloboma, heart defects, choanal atresia, delayed growth, and ear anomalies, CHARGE syndrome's diagnostic spectrum has broadened since the identification of . Variants in this gene exhibit considerable phenotypic variability, leading to the adoption of the term " disorder" to encompass a wider range of associated symptoms.

Cognitive outcomes in Susac syndrome: A 2-year neuropsychological follow-up study.

Background And Purpose: Susac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long-term neuropsychological outcomes of patients with SuS.

Risk Factors for Natural Hearing Evolution in Newborns With Congenital Cytomegalovirus Infection.

Importance: Congenital cytomegalovirus (cCMV) is the major cause of congenital nonhereditary sensorineural hearing loss in children. Currently, criteria to identify infants at increased risk for unfavorable hearing outcome are lacking.

Objective: To identify risk factors associated with cCMV-related hearing improvement, hearing deterioration, and late-onset hearing loss.

Vestibular Follow-up Program for Congenital Cytomegalovirus Based on 6 Years of Longitudinal Data Collection.

Objectives: Congenital cytomegalovirus (cCMV), the leading nongenetic cause of pediatric sensorineural hearing loss, can also affect vestibular function. Literature findings suggest clinical presentation of vestibular loss in cCMV to be as variable as the hearing loss. Still, probably due to the considerable additional burden it entails for both patients and diagnostic centers, longitudinal vestibular follow-up in cCMV is not well-established in clinical practice.

Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort.

Susac syndrome (SuS) is a rare immune-mediated endotheliopathy that affects the brain, retina and inner ear and is characterised by the variable clinical triad of encephalopathy, visual and vestibulocochlear dysfunction. Here, we present clinical and paraclinical data of 19 SuS patients followed at Ghent University Hospital and highlight some atypical clinical and novel radiological findings. Our findings suggest that spinal involvement expands the clinical phenotype of SuS.

Risk Factors for Hearing Loss at Birth in Newborns With Congenital Cytomegalovirus Infection.

Importance: With a prevalence between 0.2% and 6.1% of all live births, congenital cytomegalovirus (cCMV) infection is a major cause of congenital nonhereditary sensorineural hearing loss.

Vestibular Infant Screening-Flanders: What is the Most Appropriate Vestibular Screening Tool in Hearing-Impaired Children?

Objectives: As children with sensorineural hearing loss have an increased risk for vestibular impairment, the Vestibular Infant Screening-Flanders project implemented a vestibular screening by means of cervical vestibular evoked myogenic potentials (cVEMP) at the age of 6 months for each child with hearing loss in Flanders (Belgium). Given that vestibular deficits can affect the child's development, this vestibular screening should allow early detection and intervention. However, less is currently known about which screening tool would be the most ideal and how vestibular impairment can evolve.

Long-term anatomic and functional outcome of pediatric myringoplasty in primary and revision cases.

Objectives: To evaluate the long-term anatomical and functional results of myringoplasty in a large cohort of children and analyse factors determining outcome of surgery.

Methods: A retrospective analysis of 469 cases of primary and revision pediatric myringoplasties conducted between 2003 and 2018 at the Ghent University Hospital was performed. Anatomical success was defined as an intact tympanic membrane postoperatively.

Predicting Early Vestibular and Motor Function in Congenital Cytomegalovirus Infection.

Objectives: Congenital cytomegalovirus (cCMV) can affect vestibular function, which is an important cornerstone for early motor development. This study aims to identify risk factors for early vestibular dysfunction with severe repercussions on the motor outcome.

Methods: This prospective cohort study included 169 cCMV-patients with complete vestibular assessment (lateral video Head Impulse Test and cervical Vestibular Evoked Myogenic Potentials) before the age of 18 months (mean 8.

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.

Hearing Loss in Malignant Infantile Osteopetrosis: A Case-Based Review.

Osteopetrosis, or marble bone disease, is a rare genetic disease of bone resorption. It includes a clinically heterogeneous group of conditions that are characterized by increased bone density on radiographs due to a defect in osteoclasts. A most common feature of osteopetrosis of the temporal bone is hearing impairment.

Twelve years of neonatal hearing screening: audiological and etiological results.

Purpose: Most developed countries have implemented some form of universal newborn hearing screening program. Early identification and rehabilitation of congenital hearing loss is important in functional outcome, and the need to identify the cause of hearing impairment has become clear. We aimed to evaluate audiological and etiological outcomes in a large group of patients with failed neonatal hearing screening.

Congenital Unilateral Hearing Loss: Characteristics and Etiological Analysis in 121 Patients.

Objective: To describe the characteristics and etiological analysis in patients with congenital unilateral hearing loss.

Study Design: Retrospective cohort analysis.

Setting: Tertiary referral center.

Investigation of the urinary excretion of prednisolone and metabolites after nasal administration: Relevance to doping control.

Glucocorticosteroid use in sport is restricted to non-systemic (nasal/ophtamological/dermatological/intra-articular) use. Systemic use is prohibited because of strong inflammatory suppressing effects. Prednisolone is a GC proven to be very effective in the treatment of nasal congestions and allergic rhinitis and its therapeutic use is allowed.

Congenital CMV-Associated Hearing Loss: Can Brain Imaging Predict Hearing Outcome?

Objectives: Congenital cytomegalovirus (cCMV) infection is the leading cause of nonhereditary sensorineural hearing loss in childhood and is also associated with CNS abnormalities. The main objective is to investigate the prognostic value of neonatal cranial ultrasound (cUS) and cranial magnetic resonance imaging (cMRI) in predicting long-term hearing outcome in a large cohort of cCMV-infected symptomatic and asymptomatic patients.

Design: Data were prospectively collected from a multicentre Flemish registry of children with cCMV infection born between 2007 and 2016.

Vestibular Function in Children With a Congenital Cytomegalovirus Infection: 3 Years of Follow-Up.

Objectives: Congenital cytomegalovirus (cCMV) infection is the most common nongenetic cause of sensorineural hearing loss in children. Due to the close anatomical relationship between the auditory and the vestibular sensory organs, cCMV can also be an important cause of vestibular loss. However, the prevalence and nature of cCMV-induced vestibular impairment is still underexplored.

The impact of cleft lip and/or palate on parental quality of life: A pilot study.

Background: Cleft lip and/or palate (CL/CP/CLP) is one of the most common congenital anomalies. Children may suffer from a variety of health problems including difficulties with feeding and speech, middle ear problems, hearing loss and associated psychosocial concerns. The extent of impact of this disorder on the parents, however, has not yet been thoroughly evaluated.

Ear- and hearing-related impact on quality of life in children with cleft palate: Development and pretest of a health-related quality of life (HRQOL) instrument.

Objectives: To investigate to what extent middle ear problems and associated hearing loss affect quality of life (QoL) of children born with a cleft palate.

Methods: Fifty-five children aged between 6 and 18 years, born with non-syndromic cleft palate ± cleft lip (CP/L) were included. A new health-related quality of life (HRQOL) questionnaire was generated with consideration of the following domains of QoL: communication, hearing loss, physical symptoms, limitation of activities and socio-emotional impact.

Correction to: Long-term hearing results of stapedotomy: analysis of factors affecting outcome.

The given and family names of all the co-authors are incorrect in the published article. The correct names should read as follows.

Long-term hearing results of stapedotomy: analysis of factors affecting outcome.

Objective: To evaluate long-term hearing results of stapedotomy and analyze the influence of patient-, disease-, and procedure-related variables.

Study Design: Retrospective case series.

Setting: Tertiary referral center.

Comparison of the Motor Performance and Vestibular Function in Infants with a Congenital Cytomegalovirus Infection or a Connexin 26 Mutation: A Preliminary Study.

Objectives: Hearing-impaired children are at risk for vestibular damage and delayed motor development. Two major causes of congenital hearing loss are cytomegalovirus (CMV) infection and connexin (Cx) 26 mutations. Comparison of the motor performance and vestibular function between these specific groups is still underexplored.

Haemophilus influenzae biofilm formation in chronic otitis media with effusion.

Otitis media with effusion (OME) is a highly prevalent disease in children, but the exact pathogenesis and role of bacteria are still not well understood. This study aimed to investigate the presence of otopathogenic bacteria in the middle ear effusion (MEE) and adenoid of children with chronic OME (COME), and to investigate in vivo whether these bacteria, especially Haemophilus influenzae, are organized as a biofilm in the middle ear fluid. MEE and adenoid samples were collected from 21 patients with COME.

Hearing in Children with Congenital Cytomegalovirus Infection: Results of a Longitudinal Study.

Objectives: To evaluate hearing outcome, to characterize the nature of symptomatic and asymptomatic congenital cytomegalovirus (cCMV) infection and associated hearing loss, and to compare results with data from previous studies.

Study Design: A prospective multicenter registry was set up in 2007. Six centers participated in the development of a standardized protocol for diagnosis, treatment, and follow-up.

Early motor development of children with a congenital cytomegalovirus infection.

Background: Congenital cytomegalovirus (cCMV) infection is the most important etiology of non-hereditary childhood hearing loss and an important cause of neurodevelopmental delay. The current study aimed to investigate the early motor development of symptomatic and asymptomatic cCMV infected children with and without sensorineural hearing loss (SNHL).

Methods: Sixty-four children with a cCMV infection, without cerebral palsy, were compared to a control group of 107 normal hearing children.