Audiometric findings in Waardenburg's syndrome...

Audiometric findings in Waardenburg's syndrome amongst the institutionalised deaf/blind in Kaduna-Nigeria.

Abstract

INTRODUCTION: Waardenburg's syndrome (WS) is a rare inherited disorder of congenital hearing loss and Pigmentary disturbances of the eyes, hair, skin and neural crest derivatives. MATERIALS & METHODS: 620 students in a deaf/blind school were examined and four had WS with a frequency of 0.65%. 2 males and 2 females with WS and age ranges between 10-19yrs {mean 15.75yrs.} 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: WS is a rare disorder in our environment although it may be under reported. Two ofthe subjects benefited from amplification and were given hearing Aids. Provision of early amplification cannot be over emphasized.