Abstract
Background: Conductive hearing loss in children is usually acquired. However congenital causes: ranging from deformities of external and middle ear to isolated malformations of the ossicular chain are noted. The incidence of congenital middle ear anomalies is 0.28 per 1,00,000 persons. The diagnosis and management are both not only challenging but demanding with respect to surgical expertise, to ensure optimum outcome in terms of hearing and thereby improved quality of life.
Case report: Our patient a 7-year-old boy was referred with complaints of decreased hearing noticed for the past 2years. The clinical examination was normal except for left preauricular sinus and skin tag. Audiological evaluation showed bilateral moderately severe conductive hearing loss with ‘Ad’ type tympanogram on left side. HRCT temporal bone showed agenesis of right stapes and dysplastic left stapes. Exploratory tympanotomy on left side under general anaesthesia revealed a deformed incus with only an arch of bone in the area of stapes and the facial nerve coursing through the arch. The intra-operative identification of the congenital anomaly was followed by appropriate ossicular reconstruction with aim to restore hearing and avoid facial palsy. A fenestra was created anterior to the arch and Teflon piston placed from malleus (Malleovestibulopexy). The post operative hearing improvement was beneficial to the patient with an air-bone gap closure to 15dB on pure tone audiometry.
Conclusion: Congenital anomalies of middle ear are relatively rare. Early identification of anomalies and anatomical variants, through proper preoperative evaluation along with imaging techniques is necessary to initiate appropriate treatment ensuring improved quality of life.