Cochlear implantation in patients with enlarged vestibular aqueduct. A case series with literature review

Objectives: To report our institutional experience of the management of patients with enlarged vestibular aqueduct (EVA) and compare it to the literature.

Methods: We carried out a retrospective review of patients’ records from 1993 to 2015. The age, sex, associated malformations, relevant past medical history, genetic screening results, possible surgical incident, implant model and duration of follow- up, outcome in terms of Categories of Auditory Performance (CAP scores), and integration or resuming mainstream school or work were recorded.

Results: We had 11 patients (six boys and five girls) with EVA who underwent cochlear implant surgery in our center during the 22-year study period, out of a total of 827 implanted (1.3%). The mean age at surgery was 8.9 years ranging from 0.6 to 35 years. EVA was bilateral in 10 cases, isolated anatomical finding in seven cases, and associated with other malformations in four. Cochlear implantation was bilateral in five cases and unilateral in six. The mean follow- up duration was 48.3 months (range: 3–120). No postoperative complication was observed and all the patients could regain a serviceable hearing, attending normal school and working normally.

Conclusion: EVA is frequently observed in the deaf population without an identifiable cause. The hearing loss is usually progressive and may result in cochlear implantation which has proved its efficiency in rehabilitating EVA patients.