Absence of hearing impairment in adult onset facioscapulohumeral muscular dystrophy.

摘要

Hearing impairment has long been associated with the rarer forms of severe childhood and infantile facioscapulohumeral muscular dystrophy. Recent studies have suggested a high prevalence in classic, adult or adolescent, onset cases as well. We undertook detailed pure tone audiometric examination of 21 adult onset facioscapulohumeral muscular dystrophy cases. Patient results were compared with normative data obtained from the (United Kingdom) National Study of Hearing (The prevalence and distribution of hearing impairment and reported hearing disability in the MRC Institute of Hearing Research's National Study of Hearing, Whurr, 1995). There was no significant difference in the prevalence of hearing impairment in facioscapulohumeral muscular dystrophy patients at any single or averaged frequency tested. In this group of patients, pure tone audiometric thresholds were consistently better in the facioscapulohumeral muscular dystrophy patients, although these reached statistical significance only at 4 and 6 kHz. Age of onset, disease duration, clinical severity, degree of facial weakness and double-digest fragment size made no significant difference to mean hearing thresholds. We conclude that hearing impairment is not more common in adult onset facioscapulohumeral muscular dystrophy, and according to this data, may even, be less prevalent than in the normal population.