Sensori-Neural Hearing Loss Appearing in Generalized or Systemic Diseases

Abstract

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).
In addition to the preceding report, the following results were obtained:
(1) Impaired hearing loss of sensori-neural type may occur in association with a variety of generalized neuro-muscular diseases.
(2) Hearing loss as an initial symptom may precede and be followed by the remaining neurological deficits.
(3) Hearing loss is variable, ranging from mild high-frequency loss to profound deafness. Occasionally audition is almost normal in one ear.
(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.
(5) Hearing loss is detected even in some cases without complaint.
(6) There exists a significant relation between sensorineural hearing loss and decreased vibratory sensation in the extremities.
(7) There were some clinical evidences suggesting the brainstem lesion along with that of the peripheral cohlear nerve.