title: Audiological findings in children with ataxia-telangiectasia (A-T) syndrome.
abstract: OBJECTIVE
  To assess peripheral and central hearing in children with A-T.
  
  
  METHODS
  3 children diagnosed with A-T according to the diagnostic criteria for A-T of the European Society for Immunodeficiencies. Involuntary movements were seen in the form of chorea-athetosis together with tremors. They were examined to assess both peripheral and central hearing was assessed (hearing thresholds). Sound-field testing, tympanometry, acoustic reflexes, Otoacoustic Emissions (OAEs) and Auditory Brainstem Responses (ABR) were done for all of them.
  
  
  RESULTS
  Basic Audiological evaluation is of a limited value as the children are not co-operative. Sound field testing could not be done. Bilateral normal middle ear functions as reflected by Tympanometry and Acoustic Reflexes. Advanced Audiological evaluation including OAEs and ABR are more valuable in assessing hearing in children with A-T. Bilateral pass response at all test frequencies in DPOAEs. Abnormal ABR findings were obtained in the form of a delay in wave V latency more than 2 SD with subsequent increased in I-V interpeak latency with no significant interaural latency difference.
  
  
  CONCLUSIONS
  Consistent with bilateral normal peripheral hearing sensitivity with central hearing affection.
  
  
  CONCLUSIONS
  The rarity of the disease, make it difficult to be applied on many cases.
authors.affiliation: Lecturer of Audiology, Otorhinolaryngology Department, Ain Shams University, Cairo, Egypt. Electronic address: pretty_afifi@hotmail.com.
authors.forename: Pretty O
authors.initials: PO
authors.lastname: Afifi
authors.affiliation: Assistant Professor of Audiology, Otorhinolaryngology Department, Faculty of Medicine, Tanta University, Egypt.
authors.forename: Hanaa Hussein
authors.initials: HH
authors.lastname: Elsanadiky
mesh_terms: D000158:Acoustic Impedance Tests; D001260:Ataxia Telangiectasia / Q000150:complications / Q000503:physiopathology*; D001309:Auditory Threshold; D002648:Child; D002675:Child, Preschool; D016057:Evoked Potentials, Auditory, Brain Stem; D034381:Hearing Loss / Q000209:etiology / Q000503:physiopathology; D006313:Hearing Loss, Central / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous
keywords: ABR findings; Ataxia-telangiectasia; Audiological assessment