Neurologic morbidity associated with congenital cytomegalovirus (CMV) infection is a major public health concern. The pathogenesis of cerebral lesions

Congenital choanal atresia (CA) is a rare cause of upper airway obstruction. Its incidence varies from between 1 in 5000 to 1 in 8000 live births. Anatomically, it consists of an enlarged vomer and medialized lateral pterygoid plate causing a complete nasal obstruction.¹ It is more frequently unilateral (usually the right side) and seems to affect girls more than boys. Although the unilateral condition may be left undiagnosed, bilateral CA may be life-threatening, newborns being solely nasal breathers. Some CA may be associated with other congenital anomalies; however, in 50% of cases, no genetic relationship may be found.²

BACKGROUND: Congenital cytomegalovirus (CMV) infection is the leading infectious cause of neurologic disabilities and sensorineural hearing loss in children. Sensorineural hearing loss prevalence in CMV suggests a viral tropism for the inner ear. Vestibular disorders induced by CMV infection are underestimated. This is the largest and most thorough study to assess the incidence of vestibular disorders and their correlation with hearing thresholds in children with CMV. METHODS: This retrospective study assessed a cohort of 52 children with congenital CMV infection and sensorineural impairment who received a complete hearing and vestibular assessment. Vestibular evaluation included clinical examination, caloric bithermal test, earth vertical axis rotation, off-vertical axis rotation, and vestibular evoked myogenic potential. The prevalence, progression, and clinical impact of vestibular disorders were studied and correlated with hearing thresholds and the severity of congenital CMV infection. RESULTS: Forty-eight children (92.3%) had hearing loss and vestibular disorders. Of those, vestibular disorders were complete and bilateral in 33.3%, partial and bilateral in 43.7%, and partial and unilateral in 22.9%. Serial testing in 14 children showed stable vestibular function in 50% and deterioration in 50%. Congenital CMV infection has a negative impact on postural development that is correlated with neurologic and vestibular impairment. Vestibular disorders were significantly associated with hearing disorders, but their respective severities showed no concordance. CONCLUSIONS: Vestibular disorders are frequent and severe in CMV-infected children. Routine screening and appropriate management of vestibular lesions is essential to initiate adapted care.

The objective of this study was to review the characteristics of congenital nasal pyriform aperture stenosis (CNPAS) in a series of 20 children seen between 1993 and 1996. The diagnosis was made by physical examination and computed tomography scan. A single central maxillary incisor was detected in 12 cases (60%). Three children had morphological abnormalities of the pituitary gland shown on magnetic resonance imaging. One child had an antidiuretic hormone deficiency, and another child had a growth hormone deficiency. Two children had craniosynostoses, 1 of which was Apert's syndrome. All patients underwent operation by a sublabial approach, and 1 was referred for a columellar necrosis after nasal stenting. After surgery, all patients showed improvement, and the nasal stenting was usually removed 1 week after surgery. Follow-up revealed normal breathing. In conclusion, CNPAS was previously considered to be an unusual cause of nasal obstruction in neonates and infants. The number of cases treated recently in our department suggests that this newly recognized entity is more common than expected.