Children with aural atresia (AA) present with a hearing loss due to differing degrees of malformations of the ear. The effects of this hearing loss on audition, speech/language development, and academic achievement are not well understood. The literature is quite sparse on this topic, including studies with only small numbers of children or retrospective chart reviews. The few studies that have been completed demonstrate that the hearing loss caused by AA affects auditory, as well as speech and language, development and, therefore, results in delays. Children with bilateral atresia are more adversely affected than children with unilateral atresia. However, the delays are not well understood with respect to whether or not amplification mitigates them. This paper presents the current evidence in the literature on the audiological management, the auditory and speech/language development, and the academic achievement of children with aural atresia.