神経筋疾患にみられた感音難聴
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概要
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A description is given of 6 cases of impaired hearing function coexisting with generalized neuromuscular involvement, encounted out of a total of 288 hospital patients in the neurological clinic during 1981. These included acute mixed polyradiculitis (38 year-old female), familial polyneuropthy probably of Rosenberg-Chutorian type (siblings of 20 year-old brother and 24 year-old sister), chronic progressive polyneuropathy of predominantly sensory type (57 year-old male), acrocephalosyndactyly (16 year-old female), and myopathy akin to Kearns-Shy syndrome with hypo-TBG-emia (58 year-old female).<BR>In addition to the preceding report, the following results were obtained:<BR>(1) Impaired hearing loss of sensori-neural type may occur in association with a variety of generalized neuro-muscular diseases.<BR>(2) Hearing loss as an initial symptom may precede and be followed by the remaining neurological deficits.<BR>(3) Hearing loss is variable, ranging from mild high-frequency loss to profound deafness. Occasionally audition is almost normal in one ear.<BR>(4) The mode of onset of hearing loss may be relatively acute or subacute in some cases, though the underlying illness was of insidious onset or of slowly progressive course.<BR>(5) Hearing loss is detected even in some cases without complaint.<BR>(6) There exists a significant relation between sensorineural hearing loss and decreased vibratory sensation in the extremities.<BR>(7) There were some clinical evidences suggesting the brainstem lesion along with that of the peripheral cohlear nerve.
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