Congenital Anomalies of the Ossicular Chain: Surgical and Audiological Outcomes.

Sara E Henkemans,Adriana L Smit,Hans G.X.M Thomeer,Robert J Stokroos

doi:10.1177/00034894211025405

Sara E Henkemans, Adriana L Smit + Show 2 more

Open Access

https://doi.org/10.1177/00034894211025405

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Abstract

Objectives:In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies.Methods:In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes.Results:Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered.Conclusion:middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss.

Highlights

A rare cause of congenital conductive hearing loss (HL) are congenital middle ear anomalies (CMEAs)
Other possible causes of hearing loss for example, ossicular fixation due to multiple episodes of otitis media or otosclerosis were excluded by combining anamnestic data with referrals and findings during exploratory tympanotomy
A syndromal diagnosis was encountered in 2 patients: branchio-oto-renal syndrome (BORs) in patient 10 and 22.q.11. deletion syndrome (22.q.11.ds) in patient 14

Summary

Introduction

A rare cause of congenital conductive hearing loss (HL) are congenital middle ear anomalies (CMEAs). They are defined as malformations of the auditory ossicles of any type.[1] During the embryological development the auditory ossicles are formed from branchiogenic origin and malformations can occur at any stage of this development.[2] Typically, conductive hearing losses of 30 to 50 dB are encountered in CMEA patients. CMEAs manifesting as isolated anomalies of the auditory ossicles are considered minor ear anomalies. When additional tympanic membrane or other external auditory canal anomalies (ie, atresia) are present, this deficit is described as a major anomaly.[1] The incidence of CMEAs is around 0.28 per 100,000 persons,[3] and CMEAs occur both uni- and bilaterally. CMEAs are predominantly sporadic, yet are described to be part of a syndromal diagnosis in more than 25% of cases.[4]

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