Lermoyez syndrome revisited: 100-year mystery 沈耿仲 楊怡和 臺大醫院耳鼻喉部

(Marcel Lermoyez, 1858-1929) Increasing illness Progressive deafness Sudden vertigo Hearing recovery (Lermoyez , La Presse Médicale, 1919)

Patients (1992-2017) Case no.: 7 (8 ears) Gender: M / F = 5 / 2 Age: 27 - 85 (54) years Laterality: R / L / B = 4 / 2 / 1 Prevalence of LS / MD = 7 / 4,096 (0.2%)

Methods Otoscopy Audiometry Caloric test cVEMP test after 2000 oVEMP test after 2008

Clinical manifestation (n=7) (%) 100 86 86 71 71 43

Types of audiogram (n=8 ears) Abnormality rate: 100% 4 3 比較兩組臨床症狀： A組在20名在突發性耳聾後在1~5年間發生眩暈，90%耳鳴、75%耳悶，沒有人頭痛 B組在20名在突發性耳聾後不發生眩暈，75%耳鳴、25%耳悶，10%頭痛 兩組在臨床症狀上無顯著差異 1

Chen, M85 Plasma expander Vertigo 2-4 2-6 2-7 Hearing level (dB) .125 .25 .5 1 2 4 8 Frequency (kHz) 30 40 50 60 70 80 90 100 110 20 10 -10 2010-2-3 2-5 2010-2-5 2010-2-7 2010-2-3 2010-2-5

Liang, F40 right ear Vertigo Hearing level (dB) 1992-8-24 1992-8-26 1992-9-4 .125 .25 .5 1 2 4 30 40 50 60 70 80 90 100 110 20 10 -10 30 40 50 60 70 80 90 100 110 20 10 -10 30 40 50 60 70 80 90 100 110 20 10 -10 30 40 50 60 70 80 90 100 110 20 10 -10 Hearing level (dB) 1992-8-24 8 Frequency (kHz) 1992-8-24 1992-8-26 1992-9-4 2018-2-22 Vertigo

Liang, F40 left ear Vertigo Hearing level (dB) 1992-8-24 1992-8-26 .125 .25 .5 1 2 4 Frequency (kHz) 30 40 50 60 70 80 90 100 110 20 10 -10 30 40 50 60 70 80 90 100 110 20 10 -10 1992-9-4 30 40 50 60 70 80 90 100 110 20 10 -10 30 40 50 60 70 80 90 100 110 20 10 -10 Hearing level (dB) 1992-8-24 8 1992-8-24 1992-8-26 1992-9-4 2018-2-22 Vertigo

MHL: pre- vs. post-Lermoyez attacks -20 Post-attack ＊ ＊ ＊ Hearing level (dB) 20 40 60 ＊ : p<0.05 80 Pre-attack 100 250 500 1000 2000 4000 8000 Frequency (Hz)

Clue 1: From demographic viewpoint Lermoyez syndrome Meniere’s disease Middle aged Elderly

Clue 2: From sequence of inner ear deficits oVEMP Caloric cVEMP PTA 38 43 (%) 100 Lermoyez syndrome Abnormality 100 (%) Meniere’s disease Abnormality 65 45 20 25

Mechanism of Lermoyez attack

Conclusion The mechanism of Lermoyez syndrome is blockage in the ductus reuniens probably caused by dislodged saccular otoconia. Aging and trauma are two precipitating factors for the dislodged saccular otoconia. (Acta Otolaryngol, 2018)

World heritage: Chateau de Chenonceau