Audiometric Findings in Waardenburg's Syndrome Amongst the Institutionalised Deaf / Blind in Kaduna-Nigeria

Abstract

Introduction: Waardenburg's syndrome is a rare inherited disorder of congenital hearing loss and Pigmentary disturbances of the eyes, hair, skin and neural crest derivatives. Methodology: 620 students in a deaf/blind school were examined and four had Waardenburg's syndrome with a frequency of 0.65%. 2 males and 2 females with Waardenburg's syndrome and age ranges between 10-19years (mean 15.75years) All 4 subjects had complete blue irides, white forelock and sensorineural hearing loss, and thus met the diagnostic criteria. They were then subjected to Audiometric assessment. Results: Otoscopy was essentially normal but Audiometry revealed sensorineural hearing loss [SNHL] in all the subjects ranging from severe to profound with one subject being stone deaf. Conclusion: Waardenburg's syndrome is a rare disorder in our environment although it may be under reported. Two of the subjects benefited from amplification and were given hearing Aids. Provision of early amplification cannot be over emphasized.

Keywords: Waardenburg Syndrome, SNHL, blue irides, white forelock, Amplification