Conductive hearing loss – “Need to think out of the box”
Main Article Content
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.
Article Details
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
2. Garg S, Kohli C, Mangla V, Chadha S, Singh MM, Dahiya N. An Epidemiological Study on Burden of Hearing Loss and Its Associated Factors in Delhi, India. Ann Otol Rhinol Laryngol. 2018 Sep;127(9): 614-619
3. Miller, S.M., Mikulec, A.A. (2013). Congenital Conductive Hearing Loss. In: Kountakis, S.E. (eds) Encyclopedia of Otolaryngology, Head and Neck Surgery. Springer, Berlin, Heidelberg. 2013;539-543
4. Thomeer HG, Kunst HP, Cremers CW. Congenital stapes ankylosis associated with another ossicular chain anomaly: surgical results in 30 ears. Arch Otolaryngol Head Neck Surg. 2011 Sep;137 (9):935-41.
5. Briggs RJ, Luxford WM. Correction of conductive hearing loss in children. OtolaryngolClin North Am. 1994;27(3):607
6. Stewart JM, Downs MP. Congenital conductive hearing loss: the need for early identification and intervention. Pediatrics. 1993;91(2):355-359.
7. Esteves SDS, Silva AP, Coutinho MB, Abrunhosa JM, Sousa CA. Congenital defects of the middle ear - uncommon cause of pediatric hearing loss. Braz J Otorhinolaryngol. 2014;80:251-6.
8. Charachon R, Barthez M, Lavieille JP. Minor malformations of the ear ossicles: new classification and therapeutic results. Ann OtolaryngolChirCervicofac. 1994;111(2):69-74.
9. Teunissen EB, Cremers WR. Classification of congenital middle ear anomalies: report on 144 ears. Ann OtolRhinolLaryngol. 1993;102(8, pt 1): 606-612.
10. Zeifer B,Sabini P, Sanne J.Congenital absence of oval window:radiologic diagnosis and associated anomalies.AJNR Am J Neuroradiol. 2000;21(1):171-4
11. Sara E Henkemans , Adriana L Smit,Robert J Stokroos ,Hans GXM Thomeer. Congenital Anomalies of the Ossicular Chain: Surgical and Audiological Outcomes Ann Otol Rhinol Laryngol. 2021 Jun 11;131(4):388–396.
12. Kisilevsky V E, Bailie N A, Dutt S N, Halik J J. Hearing results of stapedotomy and malleovestibulopexy in congenital hearing loss. Int J Ped Otorhinolaryngol; 2009; Sep 30; p135-151.
13. Gargule S,Daval M,Arej N,Veyrat M,Corre A, Ayache D. Malleostapedotomy for otosclerosis, our experience of nitinol piston on twelve patients. J Otol.2020(5):129-132.