Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.

OBJECTIVES: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. STUDY DESIGN: Meta-analysis. METHODS: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. RESULTS: We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. CONCLUSION: This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021.

High-fidelity, inexpensive surgical middle ear simulator.

HYPOTHESIS: A high-fidelity, inexpensive middle ear simulator could be created to enhance surgical training that would be rated as having high face validity by experts. BACKGROUND: With rapid prototyping using additive manufacturing technology (AMT), one can create high-resolution 3-dimensional replicas of the middle ear at low cost and high fidelity. Such a simulator could be of great benefit for surgical training, particularly in light of new resident training guidelines. METHODS: AMT was used to create surgical middle ear simulator (SMS) with 2 different materials simulating bone and soft tissue. The simulator is composed of an outer box with dimensions of an average adult external auditory canal without scutum and an inner cartridge based on an otosclerosis model. The simulator was then rated by otology experts in terms of face validity and fidelity as well as their opinion on the usefulness of such a device. RESULTS: Eighteen otologists from 6 tertiary academic centers rated the simulator; 83.3% agreed or highly agreed that SMS has accurate dimensions and 66.6% that it has accurate tactile feedback. When asked if performance of stapedotomy with the SMS improves with practice, 46% agreed. As to whether practicing stapedotomy with the SMS translates to improvement with live surgery, 78% agreed with this statement. Experts' average rating of the components of SMS (of possible 5) was as follows: middle ear dimensions, 3.9; malleus, 3.7; incus, 3.6; stapes, 3.6; chorda tympani, 3.7; tensor tympani, 4.1; stapedius, 3.8; facial nerve, 3.7; and promontory, 3.5. Overall, 83% found SMS to be at least "very useful" in training of novices, particularly for junior and senior residents. CONCLUSION: Most experts found the SMS to be accurate, but there was a large discrepancy in rating of individual components. Most found it to be very useful for training of novice surgeons. With these results, we are encouraged to proceed with further refinements that will strengthen the SMS as a training tool for otologic surgery.