Cortical deafness: a case report and review of the literature.
OBJECTIVE
To review the literature regarding cortical hearing loss and document a case of cortical hearing loss including its presentation, diagnosis, and evolution over 32 months of follow-up.


METHODS
A 56-year-old woman with profound bilateral sensorineural hearing loss secondary to sequential hemorrhagic, temporal lobe infarctions separated in time by 8 months.


METHODS
Diagnostic.


RESULTS
Sequential infarctions affecting the patient's auditory radiations and primary auditory cortices bilaterally combined to cause cortical hearing loss. At presentation, audiogram revealed a bilateral profound sensorineural hearing loss with no reliable responses to pure-tone or speech audiometry. She has subsequently recovered the ability to distinguish environmental sounds. At her 32-month follow-up, she had a pure-tone average (PTA) of 62 dB on the right and 70 dB on the left but continued to display a poor word recognition score (0%). A literature review was performed from the year 1891 until the present.


CONCLUSIONS
Cortical deafness is an exceedingly rare entity. Presentation and recovery of hearing are dependent on the extent of the initial lesions. The majority of patients can expect improvements in pure-tone auditory thresholds over time; however patients should be counseled that recovery of the ability to understand speech is unlikely.
Department of Otolaryngology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania 19107, USA.
Robert M
RM
Brody
Brian D
BD
Nicholas
Michael J
MJ
Wolf
Paula B
PB
Marcinkevich
Gregory J
GJ
Artz
D001301:Audiometry, Pure-Tone; D001302:Audiometry, Speech; D001921:Brain / Q000473:pathology; D003054:Cochlear Implants; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006313:Hearing Loss, Central / Q000175:diagnosis / Q000473:pathology*; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D006801:Humans; D020300:Intracranial Hemorrhages / Q000150:complications / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D017084:Otoacoustic Emissions, Spontaneous; D020127:Recovery of Function; D013067:Speech Perception