Cochlea Implantation in Patients with EVA-Syndrome

Authors: R. Parllangaj, V. Onnebrink, J.J.-H.Park Cochlear implantation in patients with EVA syndrome

Introduction An enlarged vestibular aqueduct (EVA) is a deformity in the inner ear associated with hearing loss in childhood. The vestibular aqueduct acts as a bony canal between the inner ear and the cranial cavity and contains the endolymphatic duct, which transports endolymph from the inner ear to the endolymphatic sac into the cranial cavity. The EVA syndrome consists of a dilation of the endolymphatic duct > 1.5 mm. This results in backflow of endolymph into the inner ear.

Case Report Patient A (34 years) presented with hearing loss close-by deafness since childhood. Otorrhea, vertigo were denied. Sufficiency of communication was not possible despite maximum hearing aid fitting. During preoperative preparation a CT – temporal bone/ MRT-cerebellopontine angle was performed, which showed the dilation of the endolymphatic aqueduct on both sides > 4 mm. Patient B (45 years) presented for CI-implantation at known deafness on the left side. She reported tinnitus bilaterally. No vertigo. Hearing impairment has been present since childhood. During the operation in both cases cerebrospinal fluid (CSF) emerged at the opening of the round window membrane. Covering was performed without complications.

Results In case of hearing loss unilaterally or bilaterally in childhood EVA should always be considered. CI implantation is possible but associated with complications of CSF. Postoperatively, attention should be paid to otorrhea and CSF in the nasopharynx. In addition, other syndromes such as Pendred syndrome should be considered.

Publikationsverlauf

Artikel online veröffentlicht:
12. Mai 2023