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

Neha Gupta*, Neeraj Sainy, Sumit Maheshwari, Rohit Mehrotra and Ujjwal Mehrotra

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. Cochlear implantation is the proper method for optimal hearing rehabilitation in patients with Pendred syndrome. The genetic background is a mutation of the SLC26A4 gene, coding for a transmembrane protein with anion transport function, called Pendrin. Child was implanted and rehabilitated at our centre. Outcomes in terms of hearing, speech and quality of life are comparable to cases with non-syndromic hearing loss.