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Background and Objectives@#The speech tests used to evaluate language performance in patients with bilateral deafness (BiD) and cochlear implant (CI) are problematic if applied to patients with single-sided deafness (SSD) because normal ear hearing should be excluded. Thus, we investigated the feasibility of using wireless connection to evaluate speech intelligibility of the CI ear in patients with SSD. @*Subjects and Methods@#Patients with BiD and SSD were administered the word recognition scores (WRS) and speech intelligibility tests using an iPadbased wireless connection and conventional methods. To exclude normal side hearing in patients with SSD, masking noise and “plugged and muffed” method were used in the WRS and speech intelligibility tests, respectively. @*Results@#In patients with BiD, the WRS and speech intelligibility tests results using wireless connection and conventional methods were similar. In patients with SSD, the WRS using masking noise in the normal hearing ear was similar to that of using wireless connection. However, 3 of 11 patients with SSD showed under-masked results if using the “plugged and muffed” method. @*Conclusions@#Speech intelligibility testing using wireless connection is a convenient and reliable method for evaluating CI performance in patients with SSD. The “plugged and muffed” method is not recommended for evaluating CI performance in patients with SSD.
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"Rickets" is the term applied to impaired mineralization at epiphyseal growth plate, resulting in deformity and impaired linear growth of long bones. Rickets may arise as a result of vitamin D deficiency or abnormality in metabolism. Vitamin D-dependent rickets (VDDR) is rare autosomal recessive disorder in which affected individuals have clinical features of vitamin D deficiency. In 1961, Prader first described this disorder including severe clinical features of rickets, such as hypophosphatemia, hypocalcemia, muscle weakness and seizure. Two distinctive hereditary defects, type I VDDR and type II VDDR have been recognized in vitamin D metabolism. Type I VDDR may be due to congenital defects of renal 1 alpha-hydroxylase, the enzyme responsible for conversion of 25 (OH) D3. These patients have low to detectable 1,25(OH)2D3 in presence of normal to raised 25 (OH) D3. In type II VDDR, renal production of 1,25(OH)2D3 is intact but 1,25(OH)2D3 is not used effectively and target organ resistant to 1,25(OH)2D3 is respectively derived from the abnormality in the vitamin D receptor. We report a case of a 25 month-old girl with typical clinical features of VDDR type I rickets, hypocalcemia, increased alkaline phosphatase and secondary hyperparathyroidism.