Plm. Huygen et Rjc. Admiraal, AUDIOVESTIBULAR SEQUELAE OF CONGENITAL CYTOMEGALOVIRUS-INFECTION IN 3CHILDREN PRESUMABLY REPRESENTING 3 SYMPTOMATICALLY DIFFERENT TYPES OFDELAYED ENDOLYMPHATIC HYDROPS, International journal of pediatric otorhinolaryngology, 35(2), 1996, pp. 143-154
Three cases of congenital cytomegalovirus (CMV) infection with long-te
rm audiovestibular sequelae are presented. Case 1 had no hearing in on
e ear and severe progressive hearing loss in the other ear; he showed
vestibular symptoms at the age of 4.5 years. Case 2 had severe but sta
tionary hearing loss in one ear and showed hearing impairment symptoms
in the other ear at 9-13 years of age. Case 3 did not have hearing im
pairment Symptoms, or vestibular symptoms, but was found to have sever
e progressive hearing loss from the age of 15 months onwards, which le
d to profound deafness at the age of 2 years and vestibular areflexia
at or before the age of 4 years. These cases may represent 3 symptomat
ically different types of delayed endolabyrinthine hydrops. Type 1 (ip
silateral hydrops) incorporates vestibular symptoms only because of a
lack of hearing in the offending labyrinth. Type 2 (contralateral hydr
ops) incorporates hearing impairment symptoms only because of a lack o
f vestibular function on both sides and type 3 does not incorporate he
aring impairment symptoms or vestibular symptoms (other than those rel
ating to a complete lack of function). Given the present findings, tho
se described by Weiss and Ronis (Trans. Pa. Acad. Ophthalmol. Otolaryn
gol., 30 (1977) 52-54) in one case and other reported findings relatin
g to histopathological or imaging methods in somewhat similar cases,it
seems appropriate to include congenital CMV infection in the differen
tial diagnosis of delayed endolymphatic hydrops.