Congenital aural cholesteatoma: results of surgery in 60 cases

From 1978 to 1993, 59 patients (60 ears) with congenital middle ear cholesteatoma were treated at the House Ear Clinic. The median patient age at presentation was 5 years, and the period of postoperative follow-up was 4.8 years. An intact canal wall was maintained in 58 of 60 cases and a closed middle ear space in all cases. In 12 operations, lateral graft tympanoplasty eradicated the cholesteatoma in one stage; 32 patients required a second-stage surgery to rule out recurrence, and the remaining 16 cases required three or more operations to eradicate disease and reconstruct the hearing mechanism. Thirty-five (63%) of 56 patients had a postoperative air-conduction threshold pure-tone average (PTA) within 10 dB of the best bone-conduction PTA; 91% were within 20 dB. Average speech reception threshold improved from 32 dB hearing level (HL) preoperatively to 20 dB HL postoperatively.