Childhood Hearing Loss in Patients With Sickle Cell Disease in the United States.

This study sought to examine if modern medical evaluations including newborn screening and early diagnosis along with better methods of disease control have improved rates of hearing loss in children with sickle cell disease (SCD). Audiometric and medical data for patients with SCD was obtained from the AudGen Database and analyzed for the presence of hearing loss, type of hearing loss, severity of hearing loss, and correlation with comorbid conditions. Children with sickle cell trait (SCT) were used as a comparison group. A total of 189 patients with SCD and 244 patients with SCT had sufficient audiologic data available. Hearing loss was present in 62% of children with SCD and 50% of children with SCT in the study population. Patients with SCD were significantly more likely than those with SCT to have a sensorineural component to their hearing loss (P<0.001, odds ratio: 2.41 [1.53 to 3.79]) and to have severe or profound hearing loss (P=0.02, odds ratio: 4.00 [1.14 to 14.04]). The true prevalence of hearing loss in children with SCD has not been established as routine screening is not being performed. Routine auditory testing should be done for these children to detect this loss before it impacts development.

A Randomized Controlled Trial of Balloon Dilation as a Treatment for Persistent Eustachian Tube Dysfunction With 1-Year Follow-Up.

OBJECTIVE: Compare Eustachian tube balloon dilation versus continued medical therapy (control) for treating persistent Eustachian tube dysfunction (ETD). STUDY DESIGN: Prospective, multicenter, randomized controlled trial. SETTING: Tertiary care academic center and private practice. PATIENTS: Diagnosed with medically refractory persistent ETD. INTERVENTIONS: 1:1 Randomization to balloon dilation or control. After 6 weeks, control participants had the option to undergo balloon dilation if symptoms persisted. MAIN OUTCOME MEASURES: Primary efficacy endpoint was the comparison between treatment arms in the mean change from baseline in the 7-item Eustachian Tube Dysfunction Questionnaire (ETDQ-7) score. Primary safety endpoint was complication rate. RESULTS: Sixty participants were randomized (31 balloon dilation, 29 control). Mean (SD) change in overall ETDQ-7 score at 6 weeks was -2.9 (1.4) for balloon dilation compared with -0.6 (1.0) for control: balloon dilation was superior to control (p < 0.0001). No complications were reported in either study arm. Among participants with abnormal baseline assessments, improvements in tympanogram type (p < 0.006) and tympanic membrane position (p < 0.001) were significantly better for balloon dilation than control. Technical success was 100% (91 successful dilations/91 attempts) and most procedures (72%) were completed in the office under local anesthesia. Improvements in the ETDQ-7 scores were maintained through 12 months after balloon dilation. CONCLUSIONS: Balloon dilation is a safe and effective treatment for persistent ETD. Based on improved ETDQ-7 scores, balloon dilation is superior to continued medical management for persistent ETD. Symptom improvement is durable through a minimum of 12 months. Procedures are well tolerated in the office setting under local anesthesia.

Assessment of weight gain following adenotonsillectomy in children with Down syndrome.

INTRODUCTION: Adenotonsillectomy (T&A) has been associated with postoperative weight gain in children. The purpose of this study is to determine whether a similar association exists in children with Down syndrome (DS). METHODS: The medical records of 311 DS patients were reviewed. Subjects were classified into either a control group or surgical group based on whether they had undergone adenotonsillectomy (T&A). Subjects were excluded if they only had one recorded BMI. Cases were analyzed in a pairwise fashion to maximize available data. 113 total patients with DS were identified: 84 (74.3%) in the control group and 29 (25.7%) in the T&A group. Height, weight, BMI, and Z-score data were compared between the control and T&A groups at 6-month intervals over a 24-month period. RESULTS: Children with DS who underwent T&A were comparable by demographics to children with DS who did not undergo T&A. Mean weight gain at 24 months for the T&A group was 8.07 ± 5.66 kg compared with 5.76 ± 13.20 kg in controls. The median Z-score at 24 months for the T&A group was 1.11 (0.10-1.88) compared with 1.17 (0.80-1.75) in controls. Children undergoing T&A had a stable median Z-score change of 0.09 at 24 months (p = 0.861, compared to baseline) while children who did not undergo T&A had a significantly increased median Z-score of 0.52 (p = 0.035, compared to baseline). Despite this, there were no significant intergroup differences between weight change, BMI, nor Z-score at any interval (p > 0.05). CONCLUSIONS AND RELEVANCE: Children with DS did not have an increased rate of weight gain or increased BMI after T&A. BMI Z-scores were shown to stabilize over 24 months in the T&A group and increase in the control group. While this suggests that T&A provides an added benefit of weight control in patients with DS, the results should be interpreted with caution due to the small sample size and the fact that not all patients had complete follow up across a 24-month period.