Paraneoplastic cochleovestibulopathy associated with breast cancer: a case report of two patients and literature review

Abstract

Background: Paraneoplastic cochleovestibulopathy (PCVP) is a rare, atypical neurological paraneoplastic syndrome of the nervous system that is often misdiagnosed and mistreated. Case presentation: We report two cases of middle-aged women who presented with bilateral sensorineural hearing loss (SNHL) as the initial symptom. Pure-tone audiometry (PTA) demonstrated bilateral hearing impairment, while brainstem auditory evoked potentials (BAEP) and cochlear electrograms were within normal limits. Otoacoustic emissions indicated bilateral cochlear dysfunction. Cranial imaging excluded intracranial organic and vascular lesions. After corticosteroid therapy proved ineffective, positron emission tomography/computed tomography (PET/CT) combined with histopathological examination confirmed the presence of breast cancer, leading to a diagnosis of PCVP. Despite receiving endocrine therapy (Case 1) or surgical excision (Case 2), neither patient exhibited significant improvement in PTA during one year of follow-up. Conclusion: PCVP should be considered in middle-aged patients who present with progressive bilateral hearing loss, often accompanied by vestibular dysfunction and clinical signs of cerebellar or brainstem involvement. Regular monitoring with PET/CT is recommended, and female patients in particular should undergo screening for breast cancer.