Evolution of Acquired Middle Ear Cholesteatoma in Patients With Ectrodactyly, Ectodermal Dysplasia, Cleft Lip/Palate (EEC) Syndrome

OBJECTIVE: To review an institutional experience with the surgical and clinical management of acquired middle ear cholesteatoma in patients with ectrodactyly, ectodermal dysplasia, cleft lip/palate (EEC) syndrome. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Eight patients with medical history significant for EEC syndrome who underwent surgery for acquired middle ear cholesteatoma between 1996 and 2016. INTERVENTION(S): Appropriate surgical interventions at the time of admission. MAIN OUTCOME MEASURE(S): History of ventilation tube insertion, status of the contralateral ear, surgical technique, cholesteatoma recidivism, presence of postoperative external auditory canal stenosis, pre and postoperative audiograms. RESULTS: Cholesteatoma was diagnosed in all patients, 3 (37.5%) unilateral and 5 (62.5%) bilateral, totalizing 13 ears. Six ears (46.2%) underwent a canal wall up mastoidectomy but required conversion to a canal wall down technique in a second procedure due to recurrent cholesteatoma. In the remaining seven ears (53.8%) a canal wall down mastoidectomy was performed. Of all meatoplasty performed, seven (53.8%) evolved with stenosis of the external auditory canal. CONCLUSIONS: Our results suggest that most patients with EEC syndrome and middle ear cholesteatoma should be considered for a canal wall down mastoidectomy due to extensive disease and a high rate of recidivism. In addition, a high percentage of postoperative stenosis of the external auditory canal was found in this group.