Surgical outcome in class 4 congenital anomalies of the ossicular chain: a systematic review of the literature

OBJECTIVE: To review hearing and surgical outcomes after reconstructive middle ear surgery in class 4 congenital middle ear anomalies (CMEA), e.g., patients with oval- or round window atresia of dysplasia. DATA SOURCES: Pubmed/Medline, Embase and Cochrane library. REVIEW METHODS: Articles containing data on hearing outcomes and complications after reconstructive ear surgery in class 4 anomalies were analyzed and critically appraised. The following data were included and reviewed: patient demographics, audiometric testing, surgical techniques, complications, revision surgeries and their outcomes. Risk of bias was determined, and GRADE certainty of evidence was assessed. Primary outcomes were postoperative air conduction thresholds (AC), change in AC, and success rates (closure of the ABG to within 20 dB), the occurrence of complications (most importantly sensorineural hearing loss) and the long-term stability of hearing results (> 6-month follow-up) and occurrence of recurrence of preoperative hearing loss. RESULTS: Success rates varied from 12.5 to 75% at long-term follow-up with larger cohorts reporting success rates around 50%, mean postoperative gain in AC varied from 4.7 to 30 dB and − 8.6 to 23.6 dB at, respectively, short- and long-term follow-up. No postoperative change in hearing occurred in 0–33.3% of ears, and recurrence of hearing loss occurred in 0–66.7% of ears. SNHL occurred in a total of seven ears across all studies of which three experienced complete hearing loss. CONCLUSION: Reconstructive surgery can be an effective treatment option which should be considered in patients with very favorable baseline parameters, while also considering the substantial risk of recurrence of hearing loss, the possibility of unchanged hearing despite surgery and the rare occurrence of SNHL. LEVEL OF EVIDENCE: 2c. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00405-023-08091-w.