Delayed Endolymphatic Hydrops

Diagnosis and Treatment

Abstract

Delayed endolymphatic hydrops (DEH), a clinical entity which can be differentiated from Meniere's disease, was first reported simultaneously by Nadol et al. and Wolfson et al. in 1975, and was specified by Schuknecht in 1978. In the past 6 years and 8 months, we encountered 30 cases of DEH,28 ipsilateral and 2 contralateral, in our clinic (Univ. of Tokyo Hospital). In 14 cases, the genesis of the hearing loss was juvenile unilateral deafness of unknown etiology. Other causes were mumps in 8 cases, sudden deafness in 5, head trauma, measles and progressive sensorineural hearing loss in 1 respectively. The latency between hearing loss and vertigo ranged from 3 to 49years.
In the ipsilateral cases the result of the Furosemide Test was 86% positive in the deafened ear. This test seems to be very useful for the diagnosis of DEH. It is very difficult to determine which ear is responsible for the vertigo. Added to the cochlear symptoms and other clinical signs, the furosemide test is often of great advantage in the determination of the responsible ear.
On the basis of the furosemide test we have been able to choose the method of treatment, conservatively or surgically. In many patients conservative treatment with diuretics and steroids yielded good results. Eleven cases were treated by epidural shunt operation on the endolymphatic sac. Ten of these are free from violent vertigo and doing very well after an average postoperative period of 18 months, and 3of them have no complaint 3 years after the operation.