title: Audiometric findings in Waardenburg's syndrome amongst the institutionalised deaf/ blind in Kaduna-Nigeria.
abstract: BACKGROUND
  Waardenburg's syndrome is a rare inherited disorder of congenital hearing loss and Pigmentary disturbances of the eyes, hair, skin and neural crest derivatives.
  
  
  METHODS
  620 students in a deaf/blind school were examined end four had Waardenburg's syndrome with a frequency of 0.65%. 2 males and 2 females with Waardenburg's syndrome and age ranges between 1019 years (mean 15.75 years) 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Otorhinolaryngology, Bayero University/Aminu Kano Teaching Hospital, Kano-Nigeria, PO. E 14529, Postcode: 70008, Kano. drahmedzizo@yahoo.com
authors.forename: A O
authors.initials: AO
authors.lastname: Ahmed
authors.forename: E
authors.initials: E
authors.lastname: Samaila
authors.forename: E R
authors.initials: ER
authors.lastname: Abah
authors.forename: K K
authors.initials: KK
authors.lastname: Oladigbolu
authors.forename: H
authors.initials: H
authors.lastname: Merali
authors.forename: A
authors.initials: A
authors.lastname: Abubakar
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D001299:Audiometry / Q000379:methods*; D002648:Child; D003638:Deafness / Q000150:complications / Q000151:congenital; D005260:Female; D006310:Hearing Aids; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000209:etiology / Q000534:rehabilitation; D006801:Humans; D017496:Hypopigmentation / Q000150:complications; D008297:Male; D009549:Nigeria; D019986:Persons with Hearing Disabilities / Q000534:rehabilitation; D017678:Sex Distribution; D014849:Waardenburg Syndrome / Q000150:complications / Q000175:diagnosis*; D055815:Young Adult