Acute Bilateral Vestibulopathy with Concomitant Progressive Deterioration of Binaural Hearing

Bilateral vestibulopathy is a condition with vestibular hypofunction of both inner ears. Patients with this diagnosis will often complain of dizziness and/or imbalance in darkness and when walking in uneven terrain and will often also experience oscillopsia. Predominant etiology is idiopathic. A 73-year old man with complaints of dizziness for 2 days. Objective findings included spontaneous nystagmus, a positive Romberg test with eyes closed, and a pathological video head impulse test. Initial audiometry only revealed bilateral presbycusis. Following gradual non-complete remission of vertiginous symptoms, the patient was discharged and scheduled for follow-up. The patient was later readmitted due to gradual progressive bilateral hearing deterioration alongside persisting vertiginous symptoms. Various additional tests all came out negative, and the condition was classified as idiopathic acute bilateral vestibulopathy with concomitant progressive deterioration of binaural hearing. The patient was later referred to bilateral cochlear implantation. Acute monosymptomatic bilateral vestibulopathy is difficult to diagnose, as it requires very specific tests that are not routinely done by neurologists. Acute bilateral vestibulopathy with concomitant progressive deterioration of binaural hearing leading to bilateral anacusis is indeed so rare that it has not been possible to find any literature describing a similar case.