The impact of a syndromal diagnosis on surgery for congenital minor ear anomalies.

Between 1964 and 1986, 104 ears of 86 patients with a minor congenital ear anomaly underwent an exploratory tympanotomy at the Institute of Otorhinolaryngology of the University Hospital Nijmegen. A classification of these anomalies is proposed based on the surgical findings and results. The 4 groups in this classification are: isolated stapes ankylosis, stapes ankylosis associated with an anomaly of the malleus and incus, an isolated anomaly of the malleus and incus with a mobile stapes footplate and finally, aplasia of the oval and/or round window. In a total of 29 ears (22 patients) out of these 104 ears, the anomaly formed part of a syndrome. The various syndromes and the anomalies encountered are discussed. The impact of a syndromal diagnosis on the outcome of reconstructive ear surgery is discussed per syndrome.