Sensorineural Hearing Loss in themdx Mouse: A Model of Duchenne Muscular Dystrophy

Sensorineural hearing loss has been identified in several types of muscular dystrophy, but few studies have investigated any relationship between Duchenne muscular dystrophy and hearing. An animal model of Duchenne muscular dystrophy, themdx mouse, exhibits the same genetic defect as humans. We performed brainstem auditory evoked responses on mdx and control mice in order to assess sensorineural hearing loss. The amplitude and latency of wave I for each animal were measured at increasing sound pressure levels. A significant increase in threshold and a decrease in wave I amplitude were found in the mdx mice. These results indicate that significant sensorineural hearing loss is associated with muscular dystrophy in the mdx mouse. Possible cellular mechanisms contributing to the hearing deficit are presented.     

Patterns and predictors of inpatient falls and fall-related injuries in a large academic hospital.

OBJECTIVES: Most research on hospital falls has focused on predictors of falling, whereas less is known about predictors of serious fall-related injury. Our objectives were to characterize inpatients who fall and to determine predictors of serious fall-related injury. METHODS: We performed a retrospective observational study of 1,082 patients who fell (1,235 falls) during January 2001 to June 2002 at an urban academic hospital. Multivariate analysis of potential risk factors for serious fall-related injury (vs no or minor injury) included in the hospital's adverse event reporting database was conducted with logistic regression to calculate adjusted odds ratios (aORs) with 95% confidence intervals (CI95) RESULTS: The median age of patients who fell was 62 years (interquartile range, 49-77 years), 50% were women, and 20% were confused. The hospital fall rate was 3.1 falls per 1,000 patient-days, which varied by service from 0.86 (women and infants) to 6.36 (oncology). Some (6.1%) of the falls resulted in serious injury, ranging by service from 3.1% (women and infants) to 10.9% (psychiatry). The most common serious fall-related injuries were bleeding or laceration (53.6%), fracture or dislocation (15.9%), and hematoma or contusion (13%). Patients 75 years or older (aOR, 3.2; CI95, 1.3-8.1) and those on the geriatric psychiatry floor (aOR, 2.8; CI95, 1.3-6.0) were more likely to sustain serious fall-related injuries. CONCLUSIONS: There is considerable variation in fall rates and fall-related injury percentages by service. More detailed studies should be conducted by floor or service to identify predictors of serious fall-related injury so that targeted interventions can be developed to reduce them.     

Stereopsis and long-term stability of alignment in esotropia.

PURPOSE: Recent studies of infantile and accommodative esotropia (ET) have focused on stereoacuity as a final outcome measurement for judging the success or failure of treatment. The purpose of the present study was to extend this approach by evaluating whether the presence of stereopsis developing immediately after surgical alignment or optical correction plays a role in maintenance of long-term alignment. METHODS: Random-dot stereoacuity was assessed within 3 months of initial surgical alignment in 70 children with infantile ET and within 3 months of initial optical correction in 66 children with accommodative ET. At > or = 5 years of age, adverse outcomes were assessed including loss of alignment, amblyopia, and nil stereopsis. Risk-factor analysis was used to evaluate whether early nil stereopsis increased the risk for subsequent adverse outcomes. RESULTS: In the infantile ET cohort, early nil stereopsis was associated with a 3.6 times (95% confidence interval [CI] 2.4 to 4.1) greater risk of surgery for recurrent ET or consecutive exotropia and a 4.2 times (95% CI 3.3 to 4.4) greater risk for nil stereopsis at > or = 5 years of age. In the accommodative ET cohort, early nil stereopsis was associated with a 17.4 times (95% CI 3.3 to 32.2) greater risk of surgery for ET and a 32.2 times (95% CI 15.8 to 35.6) greater risk for nil stereopsis at > or = 5 years of age. CONCLUSION: Treatment protocols designed to optimize stereoacuity outcomes promote long-term stability of alignment.     

Characterization of long‐term outcomes for pediatric patients with epithelioid hemangioma

Epithelioid hemangioma (EH) is a rare benign vascular tumor that occurs in soft tissues and bone and presents between the third and sixth decades of life. Little is known about the clinical course and outcomes of pediatric EH. We report 11 patients diagnosed with EH at a median age of 14.4 years. One patient treated with interferon and one with sirolimus exhibited partial response for >2 years. Although a benign neoplasm, EH is difficult to manage without standard protocols and portends considerable morbidity. Our findings suggest medical management, particularly sirolimus, may benefit these patients; however, long‐term follow‐up is needed.     

Hearing impairment and hearing aid usage in Singapore

Objective: To examine the epidemiological and audiological profile, pattern of hearing aid (HA) fitting and usage among HA users in Singapore. Design: Retrospective cross-sectional study. Study sample: Cohort of 1068 subjects issued with HAs at a tertiary hospital between 2001 and 2013. Results: Mean age was 70?years with 50.8% male. Mean hearing loss (HL) was 63.1?dB and 69.5% had at least moderate-severe HL. Sensorineural HL was present in 66.4% and 69.8% had symmetrical HL. “Behind-the-Ear” HAs were cheaper than “In-Ear” HAs. Standard “Behind-The-Ear” HAs were also cheaper than “Receiver-in-Canal” HAs. Among In-Ear HAs, “In-the-Canal” and “Completely-in-Canal” were more popular than “In-the-Ear” HAs despite costing more. HA was used ≥4?days/week by 85.6% but >7?h per day by only 35.7%. Only 18% received bilateral first HA fitting. In multivariate analysis, younger age and symmetrical HL were predictors of bilateral HA uptake while better Pure-Tone-Audiometry of aided ear and >7?h of daily HA usage were predictors of successive HA fitting. Conclusions: HA users in Singapore were elderly and presented with advanced HL. Bilateral HA adoption and average daily use were low compared to other developed countries. Future research on understanding the suboptimal HA usage should explore patients' motivation, hearing disability, and HA effectiveness.