Unilateral Aural Atresia: Current Management Issues and Results

Abstract

Unilateral aural atresia (UAA) presents both diagnostic and therapeutic challenges to the clinician. The absence or stenosis of the ear canal is usually associated with aberrant middle ear morphology and a variable conductive hearing loss (CHL). While the scholastic deficits associated with unilateral sensorineural hearing loss and otitis media with effusion have been well-studied, the disabilities associated with unilateral (or bilateral) aural atresia with its attendant moderate or moderate-severe CHL are less well understood. With the advent of new and more widely and easily accessible implantable hearing devices, more options are available for hearing rehabilitation in patients with unilateral or bilateral aural atresia. Preoperative tools such as high resolution computed tomography and pure tone and speech audiometry are typically used to provide some insight on the middle ear anatomy and thus predict hearing outcomes following surgical atresia repair. In this review, we explore treatment options for patients with UAA, including the use of implantable devices such as bone-anchored hearing devices and implantable middle ear devices. Predictors of success for atresia surgery will be highlighted as these may be the primary measures by which patients are counseled and ultimately choose treatment.