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Updated: Jun 26, 2026

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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Hearing in Sickle Cell Disease: Cochlear and Subcortical Dysfunction without Cortical Impairment.
Mara Renata Rissatto-Lago1, Geovana da Silva Conceição1, Caique Dos Santos Reis1
1Department of Life Sciences, Speech-Language Pathology Program, State University of Bahia (UNEB), Bahia, Brazil.
Acta Otorrinolaringologica Espanola
|June 24, 2026
Summary
Sickle cell disease (SCD) patients show high rates of sensorineural hearing loss (SNHL) and early auditory pathway dysfunction. Regular hearing tests are crucial for early detection and monitoring of auditory changes in SCD.
Area of Science:
- Audiology
- Hematology
- Neuroscience
Background:
- Sickle cell disease (SCD) can affect various organ systems, including the auditory system.
- Auditory pathway dysfunction may occur in SCD patients, but comprehensive evaluations are limited.
Purpose of the Study:
- To assess peripheral and central auditory pathways in individuals with SCD.
- To identify patterns of auditory impairment and potential lesion sites in SCD.
Main Methods:
- Cross-sectional study of 107 participants (54 with SCD, 53 controls).
- Evaluations included pure-tone audiometry, tympanometry, contralateral acoustic reflex testing, brainstem auditory evoked potentials (BAEP), and cortical auditory evoked potentials (P300).
Main Results:
- Sensorineural hearing loss (SNHL) was significantly more prevalent in the SCD group (29.6%) compared to controls.
- Elevated contralateral acoustic reflex thresholds were more frequent in SCD patients, suggesting subclinical dysfunction.
- No significant differences in BAEP or P300 latencies were found between groups.
Conclusions:
- Individuals with SCD have a high prevalence of SNHL and subclinical auditory dysfunction.
- Alterations in acoustic reflexes indicate early brainstem auditory circuit dysfunction, even with normal hearing.
- Regular audiological monitoring is recommended for early detection and follow-up of auditory changes in SCD.
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