COCHLEAR IMPLANT IN A CASE OF PENDRED SYNDROME- BILATER AL SENSORINEURAL HEARING LOSS WITH HYPOTHYROIDISM AND GOITRE

Abstract

INTRODUCTION The clinical symptom of severe SNHL or deafness in children has a genetic background in about 65%of the cases, with 85% of them in a nonsyndromic appearance. Concerning the pathogenesis of hearing loss in patients with proven EVA, EVA seems not to be the causative factor for the hearing loss, but more of an accompanying morphological result of an altered fluid balance in the inner ear during embryo - logical development, due to malfunction of Pcndrin AIMS & OBJECTIVES We present a child of Indian origin with Pendred syndrome who underwent cochlear implant at Mehrotra ENT hospital, Kanpur, India. Patients with Pendred syndrome represent challenging cochlear implant candidate, combining goiter, severe-to-profound hearing loss, and inner-ear dysplasias. METHODOLOGY Cochlear implantation is the proper method for optimal hearingrehabilitation in patients with Pendred syndrome. The genetic background is a mutation of theSLC26A4gene, coding for a transmembrane protein with anion transport function, called Pendrin [3,4,5]. Child was implantedand rehabilitated at our centre. CONCLUSION Outcomes in terms of hearing, speech and quality of life is comparable to cases within on syndromic hearing loss. KEYWORDS: - Pendred syndrome, Pendrin, Hypothoidism, Goiter