晩発性先天性迷路梅毒の長期観察の1例 : Hennebert症候,聴力変動所見,Herxheimer反応,難聴効所見の検討

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A case of late congenital syphilitic labyrinthitis (male, first examined at 35 years of age) which has been observed over a long period (for a total of ten years) is described along with a discussion on the clinical findings.1.A positive fistula test with an intact tympanic membrane, or Henneberts sign, was evident in both ears over a period of about one year.The form of induced mystagmus, which lasted for 1 to 2 seconds with I to 3 beats, was chiefly vertical with strong rotatory elements. From these findings the underlying pathogenetic mechanism of the sign in the present case was inferred to be due to pressure transmission via the oval window.The direction of the nystagmus was reversed on positive and negative pressure.2.Marked fluctuations in auditory thresholds were noted in both ears over a period of years. The patient further showed a tendency for the auditory disturbance to slowly progress into gradual high tone loss with repeated fluctuations bilaterally.In the right or better hearing ear, the fluctuations in hearing acuity presented different features at the high, middle and low tone levels:They were most marked for the low frequencies below 500 cps, less for the high fre- quencies above 4000 cps, and least for the middle frequencies of 1000 to 2000 cps.This finding seemed to be due to possible difference in vulnerability or in ability to recover in the respective regions of the cochlea.3.Conspicuous exacerbation of hearing impairment occurred bilaterally during and in a few days after antiluetic therapy with penicillin or erythromycin, being not accompanied with fever or any other general symptoms.The manifestation was interpreted as representing Jarisch-Herxheimer reaction that developed in an atypical fashion.It has been suggested that this reaction will rather be manifested in such atypical form in the antiluetic treatment for late congenital syphilis of the inner ear.4.Audiometric examinations performed in this case on frequent occasions have demonst- rated that amelioration in hypacusis with consequent stabilization of the hearing acuity may be achieved successfully by prolonged, high-dose corticosteroid therapy for.:this, ;disease.It:has been emphasized that, in antiluetic therapy for this condition, concomitant administration of the drug is advisable to prevent aggravation of labyrinthine disorders which may develop in the form of Herxheimer-effect due to the use of antisyphilitics.
社団法人日本耳鼻咽喉科学会の論文

晩発性先天性迷路梅毒の長期観察の1例 : Hennebert症候,聴力変動所見,Herxheimer反応,難聴効所見の検討

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