Cochlear implantation in Pendred syndrome

Abstract

Objective

The objective of this study was to examine the anatomical, audiological, and surgical factors that can affect the hearing outcome in Pendred syndrome cochlear implant recipients. Patients with Pendred syndrome represent challenging cochlear implant candidates, combining goiter, severe-to-profound hearing loss, and inner-ear dysplasias. The hearing outcome and further relevant parameters in such patients have not been adequately evaluated yet.

Material and methods

Retrospective study in a tertiary referral cochlear implant centre. Data of patients with Pendred syndrome, who had undergone cochlear implantation in the 10-year period 1999–2008, were evaluated.

Results

Five cochlear implant recipients with Pendred syndrome were identified, four unilateral and one bilateral. The average postoperative follow-up duration for these patients was 4.8 years. Enlarged vestibular aqueduct and Mondini dysplasia were the most common anatomical findings. Although these anatomical variances did not affect the electrode insertion, they prolonged the duration of the surgery. Cerebrospinal fluid gusher was the most common intraoperative complication. All patients enjoyed satisfactory hearing rehabilitation.

Discussion

Cochlear implantation is the proper method for optimal hearing rehabilitation in patients with Pendred syndrome. Although the precise pathogenetic mechanism and the genetic background of deafness in Pendred syndrome have not been completely understood, the preexisting hearing experience represents a positive factor for satisfying hearing outcome. The inner-ear malformations can cause mild surgical difficulties and extended surgery duration. The cochlear implant surgeon should be aware of these difficulties to avoid complications.

Keywords:

• Cochlear implant
• Pendred syndrome
• Enlarged vestibular aqueduct
• Mondini dysplasia
• Cerebrospinal fluid gusher
• Hearing outcome