▁synd rome ▁in ▁the ▁title , ▁forever ▁linking ▁this ▁disease ▁with ▁him . ▁ ▁Path ogen esis ▁In ▁the ▁March ▁ 1 9 7 9 ▁report ▁in ▁Ne uro log y , ▁D rs . ▁Sus ac , ▁Hard man ▁and ▁Sel hor st ▁reported ▁two ▁patients ▁with ▁the ▁tri ad ▁of ▁en ce phal op ath y , ▁hearing ▁loss ▁and ▁micro ang iop ath y ▁of ▁the ▁ret ina . ▁The ▁first ▁patient ▁under w ent ▁brain ▁bi ops y , ▁which ▁revealed ▁s cl eros is ▁of ▁the ▁media ▁and ▁advent it ia ▁of ▁small ▁p ial ▁and ▁cort ical ▁vessels , ▁suggest ive ▁of ▁a ▁he aled ▁ang i itis . ▁Both ▁patients ▁under w ent ▁flu ores ce in ▁ret inal ▁ang i ography ▁that ▁demonstrated ▁mult if ocal ▁ret inal ▁ar tery ▁occ lus ions ▁without ▁evidence ▁of ▁em bol ic ▁disease . ▁Though ▁the ▁exact ▁path ogen esis ▁of ▁this ▁dis order ▁is ▁unknown , ▁the ▁ret inal ▁and ▁brain ▁bi ops y ▁find ings ▁suggest ▁a ▁small ▁vessel ▁vas cul op ath y ▁leading ▁to ▁ar ter i olar ▁occ l usion ▁and ▁micro inf ar ction ▁of ▁c ere br al , ▁ret inal ▁and ▁co ch lear ▁t issue . ▁Dem y el ination ▁is ▁not ▁a ▁typical ▁feature ▁of ▁Sus ac ' s ▁synd rome . ▁Mus cle ▁bi ops ies ▁from ▁such ▁patients ▁are ▁usually ▁normal , ▁but ▁some ▁have ▁also ▁shown ▁n ons pec ific ▁signs ▁of ▁infl amm ation