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Original Article

Auditory-evoked response analysis in Brazilian patients with sickle cell disease

, , , &
Pages 272-276 | Received 13 Mar 2009, Accepted 09 Sep 2009, Published online: 24 Feb 2010
 

Abstract

Abstract

The objective of the present study was to evaluate the integrity of the peripheral and central auditory systems of sickle cell disease (SCD) patients, through electrophysiological evaluation utilizing auditory evoked potentials, and comparing the results obtained in SCD patients with individuals without SCD. A total of 80 individuals were evaluated: 40 SCD patients; and 40 healthy age- and sex-matched controls. Brainstem auditory evoked response (BAER) was used to check neural integrity and electrophysiological thresholds, and cognitive potential (P300) to analyse the auditory selective attention. Despite the exclusion of individuals with comorbidities typical of SCD, the predominance of hearing loss among the patients was detected in 16 ears (20%). The absolute latencies of the BAER were within the expected range but the SCD group showed a small but statistically significant reduction of the interpeaks I–V, indicative of coch-lear alteration. P300 latency and amplitude were adequate for both groups suggesting the absence of central auditory system abnormalities. The present findings suggest that SCD causes variable degree of cochlear abnormalities without evidence of neural problems.

Sumario

El objetivo de este estudio fue evaluar la integridad del sistema auditivo central y periférico en pacientes con anemia de células falciformes (SCD) por medio de una evaluación electrofisiológica utilizando potenciales evocados auditivos y comparando los resultados obtenidos en pacientes con SCD ante los de pacientes sin la enfermedad. Se evaluó un total de 80 individuos: 40 con SCD y 40 individuos sanos pare-ados por edad y sexo como grupo control. Se utilizaron los potenciales evocados auditivos de tallo cerebral (BAER) para verificar la integridad neural y los umbrales y el potencial cognitivo (P300) para analizar la atención selectiva. A pesar de la exclusión de individuos con enfermedades típicamente asociadas a la SCD, la predominancia de hipoacusia entre los pacientes fue detectada en 16 oídos (20%). Las latencias absolutas de los BAER estuvieron dentro del rango esperado, pero el grupo con SCD mostró una menor, pero sustancialmente significativa reducción del intervalo I-V, indicativa de la alteración coclear. La latencia y la amplitud de la P300 fueron adecuadas en ambos grupos, lo que sugiere una ausencia de anormalidades en el sistema auditivo central. Estos hallazgos sugieren que la SCD ocasiona grados variables de anormalidades cocleares sin evidencia de problemas neurales.

Acknowledgements

We thank the hematology staff and patients at the Pediatric Oncology and Hematology Center (SES, DF), and the parents and children of the control group for their cooperation.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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