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

Ahmed AO, Samaila E, Abah ER, Oladigbolu KK, Merali H, Abubakar A

Available online Jul 14, 2018.

[ Original ] Volume 19, Issue 4, 2010, Pages 382-385


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.,

October - December 2010

Volume 19 | Issue 4

Page Nos. 382-385

Online since Jul 13, 2018

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