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Images in Neurology
November 2013

Reversible Pure Word Deafness

Author Affiliations
  • 1Departments of Neurology and Radiology (Neuroradiology), University of Miami Miller School of Medicine, Miami, Florida

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JAMA Neurol. 2013;70(11):1454-1455. doi:10.1001/2013.jamaneurol.509

A 29-year-old right-handed human immunodeficiency virus (HIV)–seropositive (CD4 lymphocyte nadir, 7/mm3) woman commenced antiretroviral therapy (emtricitabine, 200 mg; tenofovir, 300 mg; and efavirenz, 600 mg, once a day) in December 2011. In February 2012, she developed headache and hearing impairment, which progressed to complete deafness over 3 weeks. She reported “muffled” and “gibberish” conversational and musical sounds in March 2012. Her communication was confined to lip reading and reading from written language. Her spontaneous speech was normal. She was able to read aloud and comprehend the written speech. She could write spontaneously and copy transcription. Auditory verbal repetition and writing (to auditory dictation) were impossible. Auditory word recognition score using NuChip picture pointing cards showed severe impairment. She could hear and recognize nonverbal sounds such as telephone ringing and her parents’ voices. Her nonverbal gestural, emotional, and prosodic elements in communication appeared intact. Pure tone audiometry and otoacoustic reflexes were intact bilaterally. Brain magnetic resonance imaging showed multiple cerebral lesions including bilateral superior temporal lesions (Figure). A biopsy from the right frontal lesion showed findings suggestive of HIV encephalitis and immune reconstitution inflammatory syndrome1 (data not shown).

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