Study of hearing features of Congenital Malformation of the Middle and Outer Ears (CMMOE)

Abstract

Background: There is no study on the hearing features of congenital malformation of middle and outer ears (CMMOE), including classification, grades, and frequency characteristics, which play a decisive role in the selection of precise hearing solutions for patients. Aims/Objectives: To analyze the hearing features of CMMOE and provide guidance for clinical practice.Material and Methods: 298 cases (351 ears) with CMMOE were retrospectively analyzed for the features of 0.5 ∼ 4KHz pure tone hearing, including the classification, grades and frequency characteristics. Results: We observed conductive deafness in 84.3% (296/351), mixed deafness in 15.7% (55/351), and 0% (0/351) sensorineural deafness. Grades measured by average Air Conduction Thresholds (ACT) of pure tone: Mild deafness (26–40dB HL) 0.6% (2/351), moderate deafness (41–55dB HL) 10.3% (36/351), moderate to severe deafness (56–70dB HL) 46.1%(162/351), severe deafness (71–90dB HL) 39.9%(140/351), extremely severe deafness (> 90 dB HL) 3.1%(11/351). The average ACT of 296 ears conductive deafness was 67 ± 10 dB HL, of which 56-80dB HL accounted for 78.1% (274/351). In 55 ears with mixed deafness, 32 ears (32/55 = 58.2%) increased Bone Conduction Threshold (BCT) at a single frequency, and out of 32 ears, 31ears (31/55 = 56.4%) ≤40dB HL, 25(25/32 = 78.1%) ears at 2KHz. In 55 ears with mixed deafness, 87.3% (48/55) increased BCT at 2KHz, and the average BCT was 35 ± 10dB HL. Conclusions and Significance: CMMOE result mainly in conductive deafness, moderate to severe and severe deafness. In mixed deafness, the BCT increased mainly at a single frequency, 2KHz and ≤40dB HL. These data suggest that bone-conductive hearing devices are a good solution for CMMOE hearing impairment.