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