Audio-vestibular symptoms can arise from vertebrobasilar dolichoectasia (VBD) and basilar dolichoectasia (BD). Given the dearth of available information, herein we reported our experience with different audio-vestibular disorders (AVDs) observed in a case series of VBD patients. Furthermore, a literature review analyzed the possible relationships between epidemiological, clinical, and neuroradiological findings and audiological prognosis. The electronic archive of our audiological tertiary referral center was screened. All identified patients had a diagnosis of VBD/BD according to Smoker’s criteria and a comprehensive audiological evaluation. PubMed and Scopus databases were searched for inherent papers published from 1 January 2000 to 1 March 2023. Three subjects were found; all of them had high blood pressure, and only the patient with high-grade VBD showed progressive sensorineural hearing loss (SNHL). Seven original studies were retrieved from the literature, overall including 90 cases. AVDs were more common in males and present in late adulthood (mean age 65 years, range 37–71), with symptoms including progressive and sudden SNHL, tinnitus, and vertigo. Diagnosis was made using different audiological and vestibular tests and cerebral MRI. Management was hearing aid fitting and long-term follow-up, with only one case of microvascular decompression surgery. The mechanism by which VBD and BD can cause AVD is debated, with the main hypothesis being VIII cranial nerve compression and vascular impairment. Our reported cases suggested the possibility of central auditory dysfunction of retro-cochlear origin due to VBD, followed by rapidly progressing SNHL and/or unnoticed sudden SNHL. More research is needed to better understand this audiological entity and achieve an evidence-based effective treatment.