A case of a brain stem hemangioblastoma with recurrent episodes of hypacusis due to progression of hydrocephalus is reported. The patient was a 25-year-old female, admitted to the department of otorhinolaryngology with complaints of hearing difficulty, headache and blurred vision. Neuroradiological studies showed a tumor from the medulla oblongata, obliterating the IVth ventricle, and a secondary hydrocephalus. Hearing loss fluctuated as hydrocephalus progressed. Multiple V-P shunting procedures relieved episodic hypacusis. The patient remains asymptomatic at present and has resumed normal activity. The mechanism of episodic hearing loss due to hydrocephalus is though to be due to the fact that through the ductus perilymphaticus and the ductus endolymphaticus, especially the former, increased intracranial pressure is transmitted to the inner ear. Through the ductus perilymphaticus there is communication between the perilymphatic space and the intracranial subarachnoid space. Through the ductus endolymphaticus there is communication with the subdural space. Increased ICP effects the inner ear. It is suspected that, in this particular case, the progression of hydrocephalus effected the patient's hearing.