Abstract
Background
Paraneoplastic cochleovestibulopathy (PCVP) is an atypical neurological paraneoplastic syndrome characterized by rapidly progressive hearing loss with or without vestibular dysfunction, usually detectable malignancy, and paraneoplastic antibodies. PCVP is rare and easy to be misdiagnosed and mistreated. Previous studies have suggested that KLHL11-IgG and seminomas are associated with male patients. However, the tumors associated with female patients are not known.
Case presentation:
We report two cases of middle-aged women with binaural sensorineural hearing loss (SNHL) as the first symptom. Pure tone audiograms showed binaural hearing impairment, brainstem auditory evoked potentials (BAEP) and cochlear electrograms were normal, and otoacoustic emissions showed binaural cochlear dysfunction. Intracranial organic lesions and vascular lesions were excluded by cranial imaging. After steroid treatment is invalid, the positron emission tomography/computedtomography (PET/CT) and pathologic examination, and breast cancer was screened. After treatment with endocrine therapy (patient #1) and surgical excision (patient #2) for breast cancer, no significant improvement in pure tone average (PTA) was seen at the 1 year of follow-up.
Conclusion
PCVP should be considered in middle-aged patients presenting with progressive hearing loss in both ears without organic pathology, mostly with vestibular dysfunction and signs of cerebellar and brainstem pathology. Regular review of PET/CT is warranted. Female patients need to be concerned about breast cancer.