Abstract
We describe the novel solution adopted in positioning middle ear implant in a child with bilateral congenital aural atresia and craniofacial dysmorphism that have posed a significant challenge for the safe and correct management of deafness. A five-year-old child, affected by a rare congenital disease (Van Maldergem Syndrome), suffered from conductive hearing loss. Conventional skin-drive bone-conduction device, attached with a steel spring headband, has been applied but auditory restoration was not optimal. The decision made was to position Vibrant Soundbridge, a middle ear implant, with an original surgical application due to hypoplasia of the tympanic cavity. Intubation procedure was complicated due to child craniofacial deformities. Postoperative hearing rehabilitation involved a multidisciplinary team, showing improved social skills and language development.
Highlights
We describe the novel solution adopted in positioning middle ear implant in a child with bilateral congenital aural atresia and craniofacial dysmorphism that have posed a significant challenge for the safe and correct management of deafness
Congenital aural atresia is a general term to describe a spectrum of ear deformities characterized by aplasia or hypoplasia of the external auditory canal
Care has been taken that the axis of the floating mass transducer (FMT) was put parallel to the orientation of the stapes, so that the device vibrated simulating the natural movement of the ossicular chain
Summary
Congenital aural atresia is a general term to describe a spectrum of ear deformities characterized by aplasia or hypoplasia of the external auditory canal. It is associated with microtia and occasionally with anomalies of the inner ear [1]. Conventional air-conduction hearing aids are common and easy means to improve patient’s deafness. These devices cannot give an acceptable benefit, if air-bone gaps are as great as up to 60 dB or the external auditory canal is absent or hypoplastic [5]. The best solution should be provided after careful multidisciplinary assessment about risks and benefits of all possible treatments [8]
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