Primary ciliary dyskinesia is a hereditary defect in the ultrastructure of cilia, leading to poor ciliary motility. The sinonasal and the bronchial manifestations of the disease are well documented; whereas its otological aspects have received less attention. In this report, we describe the clinical profile of 16 patients with primary ciliary dyskinesia laying particular emphasis on the otological manifestations. All children (11 patients) had bilateral otitis media with effusion. Of the five adults, three had tympanosclerosis; one had bilateral cholesteatoma; and one patient had bilateral keratosis obturans in combination with tympanosclerosis. Hearing improvement and a dry ear was achieved in all the children treated by tympanostomy tube insertion. The study suggests that otitis media is a prominent feature of this disorder. Most subjects suffer from protracted bilateral otitis media with effusion throughout childhood.
This is a report of three cases of mixed hearing loss that resulted from inner ear disorders. Two cases were unilateral and the third was bilateral. The diagnosis was based on the findings of normal middle and external ears in association with the absence of round window reflexes. The contralateral stapedial reflex was present in the two unilateral cases. This is the first documentation of conductive deafness due to inner ear abnormality. This diagnosis should be considered in cases of conductive hearing loss if the middle and external ears are normal. More studies are needed to establish the pathophysiology of this entity.
Objective: Ultrasound-guided core renal biopsies were performed in children to describe the accuracy of this technique and compare it with the other techniques. Methods: Forty-six children, aged 9 months to 13 years, were biopsied with 14 G and 18 G Tru-cut needles, using a real-time ultrasound sector scanner. Results: Adequate biopsy samples were obtained from 88.2 and 86.9% of the biopsy procedures with 14 G and 18 G Tru-cut needles, respectively. No major complications were encountered. Minor complications were also minimal and seen in only 3 of our patients who were managed conservatively. Conclusion: Ultrasound-guided Tru-cut renal biopsy is a safe and efficient method for performing renal biopsies in pediatric patients. The use of small needles (18 G) would reduce the complication rate while allowing retrieval of sufficient tissue for histological diagnosis.
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