The surgical findings in 144 successive ears operated on for congenital conductive hearing loss were analyzed, and the results were evaluated in terms of hearing gain. All the patients underwent middle ear surgery at the University Hospital Nijmegen between 1964 and 1990. A classification system was developed to analyze the findings. Class 1 comprises ears with congenital isolated stapes ankylosis. Class 2 comprises ears with congenital stapes ankylosis in combination with a congenital anomaly of the ossicular chain. Class 3 comprises ears with congenital anomalies of the ossicular chain and at least a mobile stapes footplate. Class 4 comprises ears with aplasia or severe dysplasia of the oval window or round window.
Because of the important function of the embryologic stapedial artery, it is taken for granted by many surgeons that the finding of such a persistent artery in postnatal humans during middle ear surgery should urge maximal caution in order not to damage the artery. Often, discontinuation of the surgery is recommended. Yet this attitude is based on theoretic considerations rather than on any clinical evidence of complications following injury to this vessel. The present paper describes the embryology in relation to this specific aspect and reviews the literature on the persistent stapedial artery, emphasizing the papers dealing with injury to this vessel. In addition, we report 4 cases of persistent stapedial artery from the files of almost 20,000 patients in whom tympanotomy was performed. From all these data we conclude that injury to this artery or even complete section probably does not cause major, if any, postoperative sequelae, and that consequently, middle ear surgery is not necessarily hindered by the presence of this vessel.
Preoperative and postoperative hearing results and long-term results of stapedectomy have been investigated in 58 ears (47 patients) with osteogenesis imperfecta. After 3 months, hearing gain had been achieved in 49 (85%) of 58 ears. Twenty-seven (68%) of 40 ears followed up for an average of 9.6 years (range, 2 to 24 years) had no deterioration of their immediate postoperative hearing gain. In the other ears, the decrease in hearing gain in the long term was due to progression of the sensorineural component of the hearing loss. Complete closure of the air-bone gap remained unchanged in 26 (70%) of 37 ears. In 5 (9%) of 58 ears the sensorineural component of the hearing loss increased as an immediate result of the operation. In 6 other ears (10%) progressive sensorineural hearing loss was seen only after more than 1 year. A natural course of the disease is assumed as the cause because progressive sensorineural hearing loss has also been seen in the nonoperated on contralateral ears of these patients.
A newly recognized autosomal dominant inherited syndrome associated with congenital conductive deafness, hyperopia, broad thumbs, broad first toes, short distal phalanges, and syndactyly is reported. The conductive loss was the result of congenital stapes ankylosis and, in two cases, was associated with ankylosis of the short process of the incus in the fossa incudis. Stapedectomy improved hearing in these patients. Fused cervical vertebrate are also an associated feature.
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