Cochlear implantation in isolated Large Vestibular Aqueduct Syndrome (LVAS)

Introduction:

LVAS is characterized by an enlargement of the endolymphatic duct and sac. It is associated with sensorineural hearing loss. LVAS can be detected by imaging (MRI or CT). An increased intracranial pressure is considered to be transferred to the endolymphatic space, causing LVAS [1].

Case-Report:

A 34-year-old patient presented in our clinic with bilateral, intermittent progressive sensorineural severe to profound hearing loss. LVAS was diagnosed by MRI and CT.

First, unilateral cochlear implantation was performed without any complications. In particular, Gusher phenomenon did not occur. Two days postoperatively, the patient developed vertigo and spontaneous nystagmus. The symptoms disappeared completely under corticosteroid therapy. Two weeks after activating the implant, the patient achieved a speech recognition of 80% at 65 dB in the Freiburg speech intelligibility test.

One year later, cochlear implantation of the other side was performed without any complications. Postoperatively the patient again developed severe vestibular symptoms, which again disappeared under corticosteroid therapy.

Conclusion:

In case of progressive sensorineural hearing loss, isolated LVAS should be considered and searched for by imaging. Audiological prognosis for cochlear implantation is good in patients with LVAS and inner ear deafness [2]. Intraoperatively, the risk of Gusher phenomenon has to be considered. Postoperatively, vestibular symptoms must be expected.

Publication History

Publication Date:
18 April 2018 (online)