The large vestibular aqueduct - case report and review of the literature

Patients with a large vestibular aqueduct (LVA) suffer from a loss of hearing in childhood at an early onset. An acute loss of hearing can be precipitated by minor head trauma. Until now there seems to be no sufficient therapy for stopping the progression of a loss of hearing. It has been shown that a cochlear implantation is a worthwhile procedure if the patient is almost deaf. We report the case of a patient with a bilateral LVA. A loss of hearing was confirmed at the age of 16 months. Exposure to loud noise triggered an acute progression of the hearing loss. At the age of 18 years, LVA was confirmed radiologically, revealing an enlarged endolymphatic duct and sac in MRI scans and an enlarged vestibular aqueduct in the CT scan. We successfully performed a cochlear implant (MED-EL, Combi 40+ flex). Proceeding from this case report, the paper reviews the literature on LVA.