Aplasia of the Cochlea

OBJECTIVE: Hearing rehabilitation of patients with severe inner ear malformations remains controversial. Our objective was to describe the radiologic findings of aplasia of the cochlea (AC) and evaluate the existing therapeutic options in such patients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Pediatric and adult patients with AC evaluated at our institution from 1995 to 2010. INTERVENTIONS: The precise radiologic findings were identified using high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) scans of the inner ear. In cases of auditory implantation on the AC side, the achieved outcome was recorded using categories of auditory performance (CAP). Related surgical aspects were analyzed. RESULTS: Twenty-three patients (28 ears) with AC were found. In 5 patients AC was bilateral. The remaining unilateral cases had contralateral normal ears (2 patients), cochlea hypoplasia (5 patients), common cavity (6 patients), incomplete partition Type I (4 patients), and atresia of the internal auditory canal (1 patient). Four patients (3 bilateral, 1 unilateral ACs) were treated with cochlear implants in ears with AC, and 1 patient underwent auditory brainstem implantation. All implanted patients achieved speech perception with limited vocabulary (CAP scores between 4 and 5). CONCLUSION: AC is defined as the total absence of the cochlea, with a present, although malformed, vestibule. Although a distinct auditory nerve was not seen in these cases of AC, results following cochlear implantation suggest functional cochlear nerve fibers in the remaining dysplastic inner ear structures. In selected cases, cochlear implantation may be a reasonable option for the habilitation of deafness associated with AC.