Clinical Analysis Of Congenital Aural Deformity With Cholesteatoma

Objective:To investigate the clinical features and prognosis of congenital external auditory canal malformation with cholesteatoma.Methods:This study presents a retrospective review and follow-up of 19 patients(20 ears)with congenital aural stenosis(CAS)and congenital aural atresia(CAA) accompanied by ear canal cholesteatoma which underwent surgery at First Affiliated Hospital of Fujian Medical University from August 2013 to October 2019.Clinical features were summarized and review of relevant literature was made.Results:(1)Of the 19 patients(20 ears)evaluated,there were 10 males and 9 females.Eight ears were affected on the left side and twelve ears were affected on the right side.The mean age at surgery was 9.4 years(range: 2–30 years).(2)Hearing loss was noted in 20(100%)patients,otalgia in 17(85%),otorrhea in 12(60%)and facial nerve paralysis in 2(19%).(3)Eighteen ears undertook pure tone audiometry and Auditory Brainstem Response.Bone conduction was tested on fourteen ears,of which 12 ears revealed conductive hearing loss,1 profound sensorineural hearing loss and 1 moderate sensorineural hearing loss.(4)Sixteen cases of congenital aural stenosis and 4 cases of congenital aural atresia were identified in the study.Cholesteatoma arose from bony auditory canal in 16 ears,cartilaginous auditory canal in 1 ear and external ear canal and middle ear in 16 ears.Among 16 patients with congenital aural stenosis accompanied by cholesteatoma,tympanic membrane was found to be present intraoperatively in 14 ears,and absent in 2 ears due to cholesteatoma erosion from external auditory canal to middle ear.While tympanic membrane was absent in all four ears with aural atresia,among which three tympanic membranes were replaced by bone and one other bone-replaced eardrum was partly damaged by cholesteatoma.(5)All 20 ears underwent surgery for cholesteatoma and meatoplasty.Auditory canal reconstruction by skin grafting was performed on fifteen ears,mastoidectomy on three,post-auricular fistulectomy for two,type I tympanoplasty for two and type II tympanoplasty for two.(6)All patients were followed up for 2-75 months.7 ears(35%)had recurrent aural stenosis,of which one ear had cholesteatoma recurrence(5%).Conclusion: Congenital aural stenosis and atresia is usually accompanied by cholesteatoma.Conductive hearing loss is common.Clinicians should have a high index of suspicion of cholesteatoma when otalgia,otorrhea and redness of auricle occurred in these patients.High resolution temporal computed tomography provides important information to diagnosis and treatment.Cholesteatoma usually arises in bony auditory canal.Surgery is effective and should be performed at the earliest possible.A large reconstructed canal and EAC stent prevents postoperative ear canal stenosis.