Surgery for congenital conductive deafness in Klippel-Feil syndrome

A wide variety of anomalies of the middle and inner ear has been reported in association with congenital deafness in Klippel-Feil syndrome. Findings of six new exploratory tympanotomies are added to a review of 14 previously reported; for this entire group, better hearing was achieved in eight ears. When the only anomaly is a disconnection of the incudostapedial joint, good results can be expected from reconstruction of the ossicular chain. With the addition of the six newly reported stapedectomies, the success rate for this procedure now equals 50%. None of the fenestrations of the promontory performed for absence of the stapes and oval window have been successful. Since these results are encouraging, it is concluded that an exploratory tympanotomy should be considered during the course of hearing rehabilitation in cases of Klippel-Feil syndrome and a significant conductive hearing loss. Anomalies of inner ear structures established preoperatively may be a contraindication for stapedectomy.