Nobuko Kawashiro

Delayed post-neonatal intensive care unit hearing disturbance.

Ten children, who developed hearing disturbance after discharge from the neonatal intensive care unit (NICU), are reported. All were born at full term, between November 1988 and November 1992, weighed at least 2500 g and had normal auditory brainstem response (ABR) at discharge from the NICU. All ten underwent thorough evaluation to determine the cause of the subsequent hearing loss. All had severe cardiovascular and/or pulmonary disorders at birth, and persistent pulmonary hypertension of the newborn (PPHN) was diagnosed in eight. A total of 25 PPHN cases were treated at our facility during the 1988-1992 period, the rate of hearing disturbance in PPHN patients being 32%. The 10 infants in this study were mechanically ventilated for, on average, 30 days. High frequency oxygenation (HFO) was required in five cases, extra corporeal membrane oxygenation (ECMO) in six. During this period, only eight ECMO-treated infants survived. The rate of hearing loss in surviving cases was therefore very high, 75% (6 8). All 10 of our cases were treated with an aminoglycoside, furosemide and a muscle relaxant. We recommend follow-up at 6 and 12 months of age, as these children are at very high risk of developing hearing disturbance.

Idiopathic deafness or hearing loss of unknown etiology following discharge from the NICU.

The clinical course, treatment and respiratory management of 6 infants who developed profound hearing loss (n = 5) or high tone hearing loss (n = 1) some months after discharge from the NICU are presented. All 6 infants had congenital and/or perinatal diseases causing hypoxia, and all were treated with gentamycin. All were nearly normal at discharge but later rose to 80-90 dB, or 60 dB in the case with high tone hearing loss. The hearing impairment was thought to be due to hypoxia, in combination with medications and invasive procedures. Follow-up audiologic testing is recommended after NICU discharge.

LINGUAL CYST IN INFANCY : IMPORTANCE OF PALPATION FOR DIAGNOSIS

Two infants, 5 and 12 weeks of age, with lingual cysts were presented. Histological findings were compatible with a salivary retention cyst in one and a thyroglossal duct cyst in the other. Both infants were admitted to our hospital because of severe stridor that had developed from one to two weeks of age. Their lingual cysts were easily recognizable by simple palpation and were confirmed by non‐invasive imaging techniques, such as ultrasound sonography, computed tomography and magnetic resonance imaging. Lingual cyst in infancy may be large enough to cause stridor and dyspnea, and occasionally result in sudden infant death, although once diagnosis is made a radical operation can be easily performed. Therefore, it is important that palpation in the oral cavity should be performed with all infants with persistent stridor as a part of a physical examination.

Choanal Atresia and Congenital Pharyngeal Stenosis

From 1980 to 1992, 8 patients with choanal atresia were treated in our facility. The female to male ratio was 5:3. One patient, a girl, had unilateral osseous obstruction with membranous closure of the opposite naris while the other 7 had bilateral osseous obstruction. Seven of the 8 had other congenital anomalies, CHARGE association (2 cases), Aperts syndrome (1 case), meningocele and banded fingers (1 case), omphalocele (1 case), subdural hematoma (1 case), and nasal fistula (1 case). Of the 7 patients who underwent endonasal surgical repair, 3 (40%) experienced reclosure. The remaining one patient, whose obstruction was of the bilateral osseous type, was successfully operated on via a transpalatal approach. Surgical correction is particularly difficult in those with craniofacial abnormalities, e.g. Aperts syndrome and maxillary hypoplasia. We treated 5 congenital pharyneal stenosis cases (CPS). Our CPS cases were divided into Types 1, 2 and 3, the latter being a new type not previously reported on. We consider Type 3 to be the result of fetal tissue remnants. We performed uvula splitting surgery in 4 cases and obtained good results in 3.

Radiographic diagnosis of congenital bony nasal stenosis.

Congenital bony nasal stenosis (CBNS) is a rare condition that causes respiratory distress in neonates. Between 1986 and 1996, we encountered 13 cases of CBNS. Recently, CT measurements have been used to evaluate the features of this type of stenosis, but no satisfactory investigation of the severity of bony nasal stenosis has been reported. We compared the nasal width (NW), facial width (FW), and interorbital distance (IOD) measured from occipitofrontal (Caldwells) projections of plain radiographs in nine CBNS patients and nine normal infants. NW was significantly narrower in the CBNS patients than in the controls, and never exceeded the IOD in any of the CBNS patients. This measuring method can be used to diagnose CBNS, because NW in normal subjects of all age groups is always greater than IOD on Caldwells view. We also investigated one patient radiographically, to determine how NW increased until the age of 4 years. However, there was no significant change in nasal width, although the FW increased significantly. This method seems useful for determining the severity of stenosis, and its simplicity makes it useful for routine investigation of breathing difficulty in neonates and infants. We review and discuss the CT images of CBNS reported in the literature.