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Auditory brainstem responses in Rett syndrome: effects of hyperventilation, seizures, and tympanometric variables
J P Pillion1, V W Rawool, S Naidu
1Department of Audiology, Kennedy Krieger Institute, Baltimore, Maryland 21205, USA.
Insights
Auditory brainstem response (ABR) abnormalities in Rett syndrome (RS) are linked to middle ear issues and seizures. Wave I latency prolongation suggests sensorineural hearing loss in some children with RS.
Area of Science:
- Neuroscience
- Audiology
- Genetics
Background:
- Rett syndrome (RS) is a complex neurodevelopmental disorder affecting females.
- Auditory brainstem response (ABR) and tympanometry are crucial for assessing auditory pathway function.
- Understanding factors influencing ABR in RS is vital for early diagnosis and intervention.
Purpose of the Study:
- To investigate the impact of tympanometric variables, disease stage, hyperventilation, and seizures on ABR in Rett syndrome.
- To identify specific ABR abnormalities and their correlation with clinical factors in children with RS.
- To explore the potential presence of sensorineural hearing impairment in Rett syndrome.
Main Methods:
- Thirty-four female children with Rett syndrome underwent ABR and tympanometry.
- Interpeak latency intervals (IPLIs) were calculated for identifiable ABR peaks (I, III, V).
- ABR abnormalities were defined by absent peaks or latencies exceeding 2 standard deviations from normative data.
Main Results:
- Significant prolongation of ABR wave I latencies was observed in children with abnormal tympanograms.
- ABR wave III latencies were significantly affected by seizures and hyperventilation.
- Wave III and V abnormalities showed significant differences based on hyperventilation and seizure presence.
Conclusions:
- Abnormal middle ear function and seizures are associated with specific ABR alterations in Rett syndrome.
- The presence of wave I abnormalities with normal middle ear function suggests sensorineural hearing impairment in some RS patients.
- These findings highlight the importance of comprehensive audiological assessment in managing Rett syndrome.
Abstract:
This study examined the effects of tympanometric variables, stage of disease, hyperventilation, and seizures on the auditory brainstem response (ABR) in Rett syndrome (RS). Thirty-four female children with RS ranging in age from 2 years, 3 months to 15 years, 7 months participated in the study. ABRs and tympanograms were recorded from all of the subjects. When the ABR peaks were identifiable, interpeak latency intervals (IPLIs) for I-III, III-V, and I-V were computed for each waveform. The peaks (I, III, and V) and IPLIs were characterized as abnormal if either the peaks were absent or the latencies were greater than 2 SD from the normative data (obtained on female children). Analyses revealed significant prolongation of wave I latencies in Rett children with abnormal tympanograms. ABR wave III latencies were significantly affected by the presence of seizures and hyperventilation. The Pearson chi-square statistic revealed significant differences in the rate of wave III and V abnormalities due to the presence and degree of hyperventilation and the presence of seizures. Wave I abnormalities were also observed in the presence of normal middle ear function, suggesting the presence of sensorineural hearing impairment in some RS children. Clinical implications of these findings are discussed.
