Cochlear Implantation in Biotinidase Enzyme Deficiency.

Metabolic syndromes associated with hearing loss are rare and are characterized by specific enzyme pathway deficiencies involving lysosomal storage, peroxisomes, fatty acid enzymes, organic acids and amino acids. The deficiency of biotinidase, an enzyme involved in the metabolism of biotin, is one such rare cause of congenital hearing loss estimated at 1:60,000 newborns. The parents of a 5-year-old girl presented to the clinic with complaints that she was hard of hearing with no speech development.

A clinical profile of revision cochlear implant surgery: MERF experience.

To study the prevalence of revision cochlear implant study in a tertiary care referral center. To assess the various indications and surgical outcomes of revision cochlear implant surgery. A retrospective chart review of revision cochlear implant surgery done from June 1997 to December 2019.

Posterior semi-circular canal electrode misplacement in Goldenhar's syndrome.

Literature documents the incidence of electrode misplacement within the range of 0.2% to 5.8% with the superior SCC as the most common site, followed by the vestibule.

Subtotal Petrosectomy in Cochlear Implant Surgery: Our Experience.

Aims: Subtotal petrosectomy (SP) with cochlear implant (CI) is required in certain specific situations in the management of patients who are candidates for cochlear implants. To study and review the indications, surgical issues, and complications of this procedure.

Materials And Methods: Retrospective review of all patients who underwent subtotal petrosectomy with cochlear implant during the period January 2010-December 2016 at a tertiary care and referral centre.