Influence of Semicircular Canal Dehiscence on Cochlear Implant Outcome.

INTRODUCTION: Semicircular canal dehiscence (SCD) is defined as a defect of the bone overlying the semicircular canal. It has a relatively high prevalence of 3% in the general population, which makes it likely that a certain number of patients receiving a cochlear implant (CI) would have it. However, little is known about the influence of SCD on the CI outcome. Therefore, the aim of this study was to determine the influence of SCD on CI outcome with regard to short- and long-term word perception and hearing preservation. METHODS: This study was a retrospective analysis of postoperative word perception ability in the electric-only condition after 6, 12, and ≥18 months and of hearing preservation 4 weeks after surgery in CI recipients with and without SCD. All patients received a preoperative 1.5- or 3-tesla magnetic resonance imaging. RESULTS: Fifty-five patients were included. Forty-eight patients (87%) had no SCD, and 7 patients (13%) had SCD. Mean postoperative word perception scores were 66% in the non-SCD group versus 50% in the SCD group (p = 0.17) after 6 months, 74 versus 64% (p = 0.28) after 12 months, and 77 versus 73% (p = 0.62) after 18 or more months. The mean postoperative hearing loss in patients with functional residual hearing before surgery (n = 34) was 22 dB in the non-SCD group versus 31 dB in the SCD group (p = 0.15). CONCLUSIONS: CI outcome is comparable between recipients without and with SCD. Specifically, hearing preservation rate and word perception ability in the electric-only condition seem not affected by SCD. The rate of progress of word perception ability in the first 12 months after cochlear implantation is not influenced by SCD.

Normal Growth in PKU Patients Under Low-Protein Diet in a Single-Center Cross-Sectional Study.

Dietary phenylalanine restriction in phenylketonuria (PKU) patients is usually mandatory in order to prevent cognitive impairment. The influence of a low-protein diet on growth has raised concerns in families and caregivers. This paper aims to investigate the growth in PKU patients treated with a low-protein diet including supplementation of amino acids and other nutrients according to standard protocols.We performed a single-center, cross-sectional study on growth in pediatric PKU patients (n = 51) treated with low-protein diet over a 20-month period. Height of healthy siblings (n = 44) and target height, calculated based on parents' height, served as controls.No statistically significant differences were found comparing mean height z-scores between patients and siblings (p = 0.261). Patients <12 years showed a reduction in mean height z-scores compared to the target height (p = 0.020), whereas postpubertal patients ≥12 years did not differ significantly in height z-scores compared to the target height (p = 0.071). Healthy siblings' height did not differ from target height in neither age group (p = 0.100/p = 0.301).Our results suggest that PKU patients treated with low-protein diet can achieve normal growth with patients making up the leeway after puberty. While prepubertal patients were shorter than expected based on their target height, older patients were within their expected target height. This study indicates that current practice of low-protein diet in PKU patients allows normal growth.