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Updated: Mar 9, 2026
Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities
Published on: January 29, 2014
Audiological findings in children with ataxia-telangiectasia (A-T) syndrome
Pretty O Afifi1, Hanaa Hussein Elsanadiky2
1Lecturer of Audiology, Otorhinolaryngology Department, Ain Shams University, Cairo, Egypt.
Insights
Children with Ataxia-Telangiectasia (A-T) show normal peripheral hearing but central auditory processing is affected. Advanced audiological tests like Auditory Brainstem Responses (ABR) are crucial for diagnosis.
Area of Science:
- Audiology
- Pediatrics
- Neurology
Background:
- Ataxia-Telangiectasia (A-T) is a rare genetic disorder affecting multiple systems.
- Hearing impairment can be a feature of A-T, but its characteristics are not fully understood.
Purpose of the Study:
- To evaluate peripheral and central auditory function in children diagnosed with A-T.
- To determine the utility of various audiological tests in this population.
Main Methods:
- Three children with A-T underwent comprehensive audiological assessments.
- Tests included tympanometry, acoustic reflexes, Otoacoustic Emissions (OAEs), and Auditory Brainstem Responses (ABR).
Main Results:
- While middle ear function was normal (tympanometry, acoustic reflexes) and OAEs showed normal responses, ABR revealed abnormalities.
- Specifically, delayed wave V latency and increased I-V interpeak latency were observed, indicating central auditory pathway dysfunction.
Conclusions:
- Children with A-T have normal peripheral hearing sensitivity.
- Central auditory pathways are affected in A-T, necessitating advanced audiological evaluations like ABR for accurate assessment.
Aim:
To assess peripheral and central hearing in children with A-T.
Method:
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.
Conclusion:
Consistent with bilateral normal peripheral hearing sensitivity with central hearing affection.
Limitations:
The rarity of the disease, make it difficult to be applied on many cases.
