Recovery of bilateral vestibular loss in Cogan's syndrome--a case report

To show that both hearing and vestibular loss can be reversible and quantified in Cogan's syndrome.

Immunosuppressive medication was continued for more than 6 years in a young woman with Cogan's syndrome. Standard pure tone audiometry (PTA) and speech discrimination score (SDS) for hearing, motorized head impulse test (MHIT) for horizontal, angular vestibulo-ocular reflex, and erythrocyte sedimentation rate were followed serially.

The PTA before medication was 59/74 dB, and it stabilized to 13/46 dB in 2 years. The initial SDS of 92/72% improved later to 100/100%, respectively. The gain of the vestibulo-ocular reflex was asymmetric (32%) and decreased to 0.48/0.25 at the beginning. Two years after the treatment, both the asymmetry (7%) and the gain (0.95/0.82) were recovered within normal limits. Erythrocyte sedimentation rate improved from 40 to 5 mm/h.

Our case report of Cogan's syndrome demonstrates objective, simultaneous deterioration of hearing and vestibular function, which improved and stabilized after the introduction of immunosuppressive medication. The efficacy of immunosuppressive medication on vestibular function may be followed repetitively using MHIT in patients with Cogan's syndrome.