Cochlear Implantation in Goldenhar Syndrome.

Goldenhar syndrome is a rare genetic condition characterized by hemifacial microsomia, mandibular hypoplasia, auricular malformations, and epibulbar dermoids. The syndrome has both sporadic and familial occurrence. Incidence of congenital hearing loss in these patients is 1:1000 in children with a male to female ratio of 3:2.

New SMS Classification of Cochleovestibular Anomalies: Our Experience with 25 Cases of Type I Anomaly.

Purpose: To study and analyse the radiological and surgical findings of 25 cochlear implantees with SMS type-I cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and post-operatively.

Methods: Retrospective analysis of cochlear implanted candidates over a period of 8 year from 3 institutions was undertaken and 25 patients suffering from pre-lingual profound congenital sensori-neural hearing loss along with presence of SMS type I cochleovestibular malformation were studied. Pre-operative radiology, surgical difficulties and complication, and post-operative hearing and speech outcomes upto a period of 2 years, using IT-MAIS scores were noted.