Otoscopic examination revealed normal-appearing external auditory canals, intact tympanic membranes bilaterally, and normal middle ear anatomy. Audiogram at that time showed bilateral profound hearing loss with no responses to pure-tone or speech testing. Treatment
Auditory perception can improve with time. There seems to be a level of neuroplasticity that allows patients to recover the ability to perceive environmental and certain musical sounds. Patients presenting with cortical hearing loss and no other associated symptoms recover to a variable degree, depending on the size and type of the cerebral lesion. Patients whose symptoms include both motor deficits and aphasias often have larger lesions with an associated poorer prognosis in regard to functional status and recovery.Cochlear or auditory brainstem implantation could also be treatment options. Electrical stimulation of the peripheral auditory system may result in improved sound perception or cortical remapping in patients with cortical deafness. However, hearing aids are an inappropriate answer for cases like these. Any auditory signal, regardless if has been amplified to normal or high intensities, is useless to a system unable to complete its processing. Ideally, patients should be directed toward resources to aid them in lip-reading, learning American Sign Language, as well as speech and occupational therapy. Patients should follow-up regularly to evaluate for any long-term recovery. History
Early reports, published in the late 19th century, describe patients with acute onset of deafness after experiencing symptoms described as apoplexy. The only means of definitive diagnosis in these reports were postmortem dissections.
Although physiologic measures demonstrate normal peripheral hearing sensitivity, this patients speech has the inflection and prosodic characteristics associated with profound peripheral hearing loss, and she is unable to understand spoken communication. Behaviorally obtained pure-tone thresholds were variable, ranging from normal to moderate hearing loss with normal middle ear muscle reflexes and normal ABRs to high- and low-intensity stimuli. Auditory middle latency and cortical evoked potentials were grossly abnormal, consistent with the central nature of cortical deafness. Because of her inability to communicate auditorily, this patient was ultimately taught American Sign Language and educated at the Louisiana School for the Deaf. At the completion of the case study, the patient was married and expecting a child. A more recent study, published in 2013 the patient described is a 56-year-old woman a history of hypertension, hypercholesterolemia, and multiple cerebrovascular accident (CVA) who presented in March 2009 with a complaint of complete bilateral hearing loss. In March 2009, she experienced an acute right-sided insulotemporal intracerebral hemorrhage. Immediately after this event, the patient complained of hearing loss with the inability to hear all sounds except for severe bilateral tinnitus. Imaging revealed sequelae in the left cerebral cortex from her previous CVA. The new right-sided hemorrhage was centered on the posterior putamen with surrounding edema involving the posterior portion of the posterior limbs of the internal, external, and extreme capsules. Signal abnormalities extended into the right temporal lobe. The patient had no other neurologic deficits and spoke fluently, although with poor internal volume control of her voice.