Congenital nasal pyriform aperture stenosis is an unusual and previously undescribed cause of nasal airway obstruction in the newborn. The nasal pyriform aperture is narrowed due to bony overgrowth of the nasal process of the maxilla. This anomaly may produce signs and symptoms of nasal airway obstruction in newborns and infants similar to those seen in bilateral posterior choanal atresia. Computed tomography confirms the diagnosis and delineates the anomaly. A series of six patients with nasal pyriform aperture stenosis is presented. Four patients were treated with surgical enlargement of the nasal pyriform aperture via a sublabial approach. One patient was repaired via a transnasal approach, and one patient did not undergo surgical intervention. Follow-up reveals normal nasal airway and facial growth in all patients. Mildly symptomatic patients with congenital nasal pyriform aperture stenosis may be treated expectantly, while severely symptomatic patients benefit from repair via the sublabial approach.

To evaluate the effectiveness of endoscopic cauterization as definitive treatment for fourth branchial cleft sinuses. Design: Retrospective chart review with follow-up questionnaire. Setting: Tertiary care children's hospital. Patients: Ten children (age range, 10 months to 10 years) with fourth branchial cleft sinuses treated with endoscopic cauterization between 1995 and 2002. Main Outcome Measure: Recurrence of neck infections after endoscopic cauterization of fourth brachial cleft sinus tracts. Results: Seven of the 10 patients treated with endoscopic cauterization of the fourth branchial cleft sinuses showed no recurrence with an average follow-up of 3 years. Three of the patients were unavailable for followup, but medical records of the hospital showed no additional admissions for those patients for neck masses. No morbidity of the procedure was identified. All patients were discharged the day of surgery. Conclusions: Endoscopic cauterization of fourth branchial cleft sinuses appears to be an effective alternative to open excision.

CHARGE association is a recently described cluster of congenital defects including ocular coloboma, heart disease, choanal atresia, retarded development and/or CNS abnormalities, genital hypoplasia, and ear anomalies. Although congenital hearing loss has been reported in CHARGE association, no information regarding the underlying temporal bone disease is available in the literature to date. The authors evaluated four patients with multiple anomalies consistent with CHARGE syndrome. Two surviving patients have bilateral severe hearing loss on auditory brain stem response testing. Two patients did not survive, and their temporal bones were obtained at autopsy for histologic examination. All four temporal bones showed severe middle ear defects including ossicular deformities, absence of the stapedius muscle, absence of the oval window, aberrant course of the facial nerve, and dehiscence of the facial nerve canal. In the more severely affected case, a Mondini-type malformation of the cochlea was present, together with multiple anomalies of the vestibular apparatus. Vestibular defects also occurred in the other case; however, the cochleae were found to be normally developed.

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