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Sickle cell anemia: audiological findings.

R A Berry

    Journal of the American Audiology Society
    |September 1, 1975
    PubMed
    Summary

    This study suggests sickle cell anemia (SCA) may cause hearing loss, particularly sensorineural deficits in high frequencies. More frequent hemolytic crises correlate with poorer hearing acuity, possibly due to cochlear microcirculatory issues.

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    Area of Science:

    • Otorhinolaryngology
    • Hematology
    • Audiology

    Background:

    • Sickle cell anemia (SCA) is a genetic blood disorder with potential systemic complications.
    • Auditory system involvement in SCA is not well-characterized, yet hearing loss can impact communication.
    • Hearing acuity offers an objective measure to explore SCA-related complications.

    Purpose of the Study:

    • To investigate characteristic auditory system manifestations associated with sickle cell anemia.
    • To determine if hearing loss is a potential complication in individuals with SCA.
    • To explore the relationship between SCA severity (hemolytic crises) and hearing function.

    Main Methods:

    • Standardized audiological procedures were used to examine hearing acuity.
    • Ten adult male subjects diagnosed with sickle cell anemia participated.
    • Subjects were categorized based on the frequency of reported hemolytic crises (short vs. long recurrence intervals).

    Main Results:

    • Subjects experiencing more frequent hemolytic crises (1-6 month recurrence) showed consistently poorer hearing threshold responses compared to those with less frequent crises (12-36 month recurrence).
    • A correlation between frequent crises and poorer hearing suggests potential audiometric changes.
    • Sensorineural deficits in high frequencies and abnormal high Short Increment Sensitivity Index (SISI) scores were observed, possibly linked to cochlear microcirculatory issues.

    Conclusions:

    • Hearing impairment, specifically sensorineural deficits in high frequencies, may be a characteristic manifestation of sickle cell anemia.
    • The frequency of hemolytic crises appears to influence the severity of hearing loss.
    • Cochlear microvascular thrombosis is a potential mechanism underlying SCA-related hearing impairment, warranting further investigation.

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