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  • Title: A case of cortical deafness and loss of vestibular and somatosensory sensations caused by cerebrovascular lesions in bilateral primary auditory cortices, auditory radiations, and postcentral gyruses - complete loss of hearing despite normal DPOAE and ABR.
    Author: Kaga K, Shinjo Y, Enomoto C, Shindo M.
    Journal: Acta Otolaryngol; 2015 Apr; 135(4):389-94. PubMed ID: 25631671.
    Abstract:
    CONCLUSION: A right-handed 38-year-old man's complete loss of hearing could be diagnosed as cortical deafness caused by cerebral vascular lesions in bilateral auditory cortices. OBJECTIVE: The aim of this case report was to study the pathophysiology of a particular patient who manifested deafness without residual hearing and lost somatosensory sensation, vestibular sensation, and articulation ability after a right internal carotid-posterior communicating artery (IC-PC) aneurysm and subarachnoidal hemorrhage. METHODS: MRI, aphasia and neurological tests, subjective and objective audiometry, and vestibular function tests were performed. RESULTS: The neurological test revealed system loss of somatosensory sensation with normal motor function and articulation ability. Brain imaging revealed extensive infarction in the bilateral primary auditory cortices, postcentral gyruses, and the bilateral partial third frontal gyruses. Pure-tone audiometry of both ears revealed off-the-scale results and speech audiometry demonstrated 0% maximum speech discrimination. However, objective audiometry showed normal distortion product otoacoustic emissions (DPOAE) and normal auditory brainstem response (ABR). The patient showed 0% perception of environmental, speech, and music sounds in both ears. He was unable to feel vestibular sensation despite normal caloric nystagmus. He showed no damage to his larynx or articulation organs.
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