A longitudinal electrocochleographic study of a case of long-standing bilateral Lermoyez's syndrome

An 18-year follow-up of a case of bilateral Lermoyez's syndrome is presented. The left ear having reached a stabilized hearing loss about 9 years after the onset of the disease, the right ear, apart from some isolated early periods of hearing loss, started to show the full extent of clinical symptoms after about 16 years. Electrocochleographic observations are presented. Studies were performed twice in the right ear during a period of strongly fluctuating hearing thresholds, once in an impaired and once in a relatively good condition. Electrocochleography of the stabilized left ear was performed as well. The data are compared with electrocochleographical observations in the left ear in its early fluctuating stage. Variation of cochlear physiological data during the fluctuating stage of the disease shows remarkable correspondence between the two ears. The stabilized Lermoyez ear is shown to have developed considerable hair cell loss, but may still have preserved its endolymphatic hydrops. These findings in Lermoyez's syndrome fit well into the observations reported in patients with Menière's disease.