An open-label study evaluating the safety and efficacy of AMO-01 for the treatment of seizures in Phelan-McDermid syndrome.

Phelan-McDermid syndrome (PMS) is a neurodevelopmental disorder caused by haploinsufficiency of the SHANK3 gene. Approximately 25% of individuals with PMS have epilepsy. Treatment of epilepsy in PMS may require multiple anticonvulsants, and in a minority of cases, seizures remain poorly controlled.

Frequency-to-Place Mismatch and Cochlear Implant Outcomes by Electrode Type.

Importance: Speech recognition outcomes with a cochlear implant (CI) are highly variable. One factor suggested to correlate with CI-aided speech recognition is frequency-to-place mismatch, or the discrepancy between the natural tonotopic organization of the cochlea and the electric frequency allocation of the CI electrodes within the patient's cochlea.

Objective: To evaluate the association between frequency-to-place mismatch and speech recognition outcomes in a large cohort of postlingually deafened adult CI users, while controlling for various clinical factors known to be associated with those outcomes.

Patient Preferences in Cochlear Implant Manufacturer Selection.

Objective: To assess the factors that drive a patient's selection of cochlear implant (CI) manufacturer.

Study Design: Prospective survey study.

Setting: Tertiary referral center.

Accuracy of adult height predictions in patients with axial leg deviations using the Modified and the Abbreviated Modified Fels Knee System.

Background: The accurate estimation of residual growth is crucial for the appropriate timing of growth-guiding surgery in patients with axial leg deviations. Skeletal age methods such as the Modified and the Abbreviated Modified Fels Knee System were developed on historical patient cohorts and the applicability to the modern pediatric population with axial leg deviation has not yet been evaluated.

Questions/purposes: Are both final adult height prediction methods (the Modified Fels Knee System (FKS) and the Abbreviated Modified Fels Knee System (aFKS)) accurate to determine SA and the final adult height on long leg radiographs in patients with axial leg deviations?Which multiplier table shows highest association between predicted and true final adult body height?Do FKS- and aFKS- skeletal age determination methods improve final adult body height prediction accuracy compared to the simple use of chronological age?

Methods: A single center, retrospective study of 31 patients who underwent temporary hemiepiphysiodesis due to axial leg deviations in the frontal plane between 2018 and 2020 was conducted.