Abstract
We describe the youngest case of bilateral stapes agenesis and systematically reviewed the literature. Among those cases, 8/12 (66.7%) showed a bilateral agenesis. There is a male predominance 8/12 (66.7%). The most common associated anomaly reported being either a dehiscent or displaced facial nerve (7/12–58%). Furthermore, 5/12 patients (42%) had a narrow or an absent oval window. In our case, a 5-year-old male patient was referred for bilateral conductive hearing loss (air-bone gap 65 dB, bilateral). Operating findings on the left side included an inferiorly displaced facial nerve over the promontory, a narrow ossified oval window and an absent stapes. A stapedotomy with insertion of a modified stapes prosthesis allowed us to close the conductive gap. Even with possible good outcomes, surgical management should not be taken lightly, considering amplification is almost always a safe and effective option.
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