Congenital Absence of Stapes Suprastructure and Footplate: A Case Report.

INTRODUCTION: Congenital ossicular chain anomalies are rare conductive hearing loss conditions that remain difficult to diagnose even with high-resolution computed tomography (CT). The preoperative diagnosis is helpful for surgical planning and counseling patients regarding treatment outcomes. CASE PRESENTATION: We report a case involving a 14-year-old boy presenting with left conductive hearing loss without history of trauma for 5 years, physical examination showed normal otoscopic examination bilaterally and high-resolution CT showed absent of stapes suprastructure and footplate. Subsequent diagnosis was done via endoscopic middle ear exploration which revealed an absent long process of the incus, stapes suprastructure and footplate, but with intact oval window membrane. The residual incus was removed, and a tragal perichondrium graft was used over the oval window. A total ossicular replacement prosthesis was placed between the malleus and oval window to repair the chain. Postoperatively, the patient had no complications. Preoperative pure tone average revealed an air/bone result of 52/8 dB. Follow-up after surgery at 6 months showed a pure tone average air/bone result of 15/3 dB. The air-bone gap was reduced from 44 to 12 dB. CONCLUSION: Congenital absence of the stapes suprastructure and footplate remains a rare condition compared to the myriad of middle ear anomalies in the literature.

App-Based Hearing Screenings in Preschool Children With Different Types of Headphones: Diagnostic Study.

Background: Hearing disability in preschool children can delay or impact oral communication and social skills. Provision of hearing screening tests by standard audiometry in low- to middle-income countries is problematic due to a lack of pediatric audiologists, standard hearing equipment, and standard soundproof rooms. Therefore, an innovative hearing screening tool that is easily accessible and inexpensive such as a mobile app should be considered. Headphones have been a crucial part of hearing screenings. Audiometric headphones, which serve as the reference standard, have been used in most studies. However, since audiometric headphones are not accessible in rural areas, we hypothesized that generic headphones can also be used in hearing screenings. Objective: This study aimed to determine the sensitivity, specificity, κ coefficiency, and time consumption of the PASS-Pro (Preschool Audiometry Screening System-Pro) app when using TDH39 headphones, Beyerdynamic DT 770 PRO headphones, and generic earmuff headphones compared to standard conditioned play audiometry. Methods: We recruited preschool children aged 4 to 5 years to participate in this study. The children received 3 PASS-Pro screening tests using different types of headphones in a quiet room and 1 standard conditioned play audiometry in a soundproof room. All tests were administered in random order. The agreement coefficient, sensitivity, specificity, and mean test duration were determined. Results: A total of 44 children participated in this study. For mild hearing loss screening, the κ coefficients between standard conditioned play audiometry and the PASS-Pro app using TDH39 headphones, Beyerdynamic DT 770 PRO headphones, and generic earmuff headphones were 0.195, 0.290, and 0.261 (P=.02, P=.002, and P=.004), respectively. The sensitivity for all headphones was 50% and the specificity was more than 88%. For moderate hearing loss screening, the κ coefficients were 0.206, 0.272, and 0.235 (all Ps=.001), respectively. The sensitivity for all headphones was 100% and the specificity was more than 92%. There were no statistical differences in sensitivity and specificity between the reference headphone (TDH39), Beyerdynamic DT 770 PRO headphone, and generic earmuff headphones (all Ps >.05). The PASS-Pro app used significantly less time to carry out hearing tests than conditioned play audiometry (P<.001). Conclusions: The PASS-Pro app, used with generic headphones, is effective for conducting hearing screening tests in preschool children with high sensitivity and specificity.