176:
deafness. Although physiologic measures demonstrate normal peripheral hearing sensitivity, this patient's 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.
134:, also called brainstem auditory evoked responses, show the neuronal activity in the auditory nerve, cochlear nucleus, superior olive, and inferior colliculus of the brainstem. They typically have a response latency of no more than six milliseconds with an amplitude of approximately one microvolt. The latency of the responses gives critical information: if cortical deafness is applicable, long latency responses are completely abolished and middle latency responses are either abolished or significantly impaired. In auditory agnosia, long and middle latency responses are preserved.
57:
167:. He was comatose for several weeks and awoke quadriparetic, cognitively impaired and completely deaf. He exhibited a response towards the occasional sudden, loud sound, however, by turning his head. Reading and writing capabilities were maintained, and he was able to communicate by lip-reading. His own speech was dysarthric, but comprehensible. Normal tympanograms and stapedial reflexes imply that the middle and
218:
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.
172:
pathways were functioning through the brainstem, but there was a lesion present in the mid-brain. With these findings, it was determined the patient had cortical deafness due to bilateral interruption of the ascending auditory pathway associated with hemorrhagic lesions of both internal capsules. Therefore, cochlear implantation was not performed.
187:, and multiple strokes who presented 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
217:
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
213:
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,
203:
In a case study by
Sasidharan et al. (2020), a patient developed cortical deafness following bacterial meningitis at 5 months old. The case was evaluated when the patient was 7 years old. Objective tests showed normal peripheral hearing, but the patient did not respond to sounds during pure-tone
175:
Published in 1994, this patient was monitored over the course of almost 20 years after exhibiting signs of hearing impairment as an infant. Audiologic and related test results in concurrence with MRI confirmed bilateral absence of considerable portions of her temporal lobes resulting in cortical
199:
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. Otoscopic
171:
remained functioning and the auditory nerve was intact. His auditory nerve was tested by evoking responses with normal auditory nerve potentials at 10 dB bilaterally. The results of the brainstem auditory evoked responses waves were normal, but an abnormal complex IV-V suggested that the
145:
can be unaware of their deficit, and insist that they are not deaf. Verbal deafness and auditory agnosia are disorders of a selective, perceptive and associative nature whereas cortical deafness relies on the anatomic and functional disconnection of the auditory cortex from acoustic impulses.
112:
It is thought that cortical deafness could be a part of a spectrum of an overall cortical hearing disorder. In some cases, patients with cortical deafness have had recovery of some hearing function, resulting in partial auditory deficits such as auditory verbal agnosia. This syndrome might be
89:. Patients with cortical deafness cannot hear any sounds, that is, they are not aware of sounds including non-speech, voices, and speech sounds. Although patients appear and feel completely deaf, they can still exhibit some reflex responses such as turning their head towards a loud sound.
29:
200:
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.
230:. The only means of definitive diagnosis in these reports were postmortem dissections. Subsequent cases throughout the 20th century reflect advancements in diagnoses of both hearing loss and stroke. With the advent of audiometric and
214:
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.
238:, greatly improved the diagnosis and localization of cerebral infarcts that coincide with primary or secondary auditory centers. Neurological and cognitive testing help to distinguish between total cortical deafness and
632:
Brody, Robert M., Brian D. Nicholas, Michael J. Wolf, Paula B. Marcinkevich, and
Gregory J. Artz. "Cortical Deafness: A Case Report and Review of the Literature." Otology and Neurology 34.7 (2013): 1226–229. Ovid.
125:
have many similarities, diagnosing the disorder proves to be difficult. Bilateral lesions near the primary auditory cortex in the temporal lobe are important criteria. Cortical deafness requires demonstration that
204:
audiometry. Late latency response tests showed absent bilateral responses, confirming cortical deafness. This case highlights that meningitis can lead to cortical deafness in addition to peripheral hearing loss.
154:
Although cortical deafness has very specific parameters of diagnosis, its causes on the other hand can vary tremendously. The following are three case studies with different reasons for cortical deafness.
1076:
105:
of the brain. The ascending auditory pathways are damaged, causing a loss of perception of sound. Inner ear functions, however, remains intact. Cortical deafness is most often caused by
159:
A case published in 2001 describes the patient as 20-year-old man referred for cochlear implants because of bilateral deafness following a motorcycle accident two years earlier. His
1069:
565:
Denes, G.; Pizzamiglio, P. "Comprehension
Disorders." Handbook of Clinical and Experimental Neuropsychology. Hove, East Sussex, UK: Psychology, 1999. 210. Print.
1062:
109:, but can also result from brain injury or birth defects. More specifically, a common cause is bilateral embolic stroke to the area of Heschl's gyri.
77:. Cortical deafness is an auditory disorder where the patient is unable to hear sounds but has no apparent damage to the structures of the ear (see
729:
195:
in the left cerebral cortex from her previous strokes. The new right-sided hemorrhage was centered on the posterior putamen with surrounding
226:
Early reports, published in the late 19th century, describe patients with acute onset of deafness after experiencing symptoms described as
1054:
276:
1315:
996:
234:
studies, investigators could diagnose cortical deafness with increasing precision. Advances in imaging techniques, such as
1354:
131:
1121:
819:
1111:
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1507:
141:
deaf. They are aware of their inability to hear environmental sounds, non-speech and speech sounds. Patients with
1116:
931:
337:
Graham J, Greenwood R, Lecky B (October 1980). "Cortical deafness—a case report and review of the literature".
1345:
1383:
876:
715:
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1310:
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235:
70:
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Sasidharan, Megha; Elsa Itty, Jinsi; Hinduja, Ghanta; Hasna, Shabnam; Priya, Deena (31 October 2020).
1481:
1423:
1410:
1322:
1295:
991:
456:
Narayanan, Santhosh; Majeed, K. Abdul; Subramaniam, Gomathy; Narayanan, Arathi; Navaf, K. M. (2017).
56:
1350:
690:
1388:
1360:
809:
410:
179:
A more recent study, published in 2013 the patient described is a 56-year-old woman a history of
82:
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1327:
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268:
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308:
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1440:
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926:
881:
520:
184:
8:
1476:
1404:
1154:
242:, resulting in the inability to perceive words, music, or specific environmental sounds.
50:
524:
409:
Silva, JĂşlia; Sousa, Marisa; Mestre, Susana; Nzwalo, Isa; Nzwalo, HipĂłlito (July 2020).
137:
Another important aspect of cortical deafness that is often overlooked is that patients
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difficult to distinguish from a bilateral temporal lesion such as described above.
86:
911:
695:
533:
263:
Neurolinguistics: an introduction to spoken language processing and its disorders
98:
78:
1372:
1184:
869:
1496:
1204:
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1001:
859:
647:
Hood L, Berlin C, Allen P (1994). "Cortical deafness: a longitudinal study".
483:
286:
102:
592:
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auditory responses are normal, but cortical evoked potentials are impaired.
901:
814:
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552:
474:
434:
180:
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682:
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916:
906:
1263:
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967:
168:
127:
46:
509:"Preservation of auditory P300-like potentials in cortical deafness"
1230:
1089:
774:
577:"Cortical deafness as a sequela to meningitis: a single case study"
458:"A Case of Cortical Deafness due to Bilateral Heschl Gyrus Infarct"
455:
227:
188:
1126:
962:
751:
739:
192:
160:
122:
97:
Cortical deafness is caused by bilateral cortical lesions in the
28:
1225:
1139:
574:
411:"Cortical deafness of following bilateral temporal lobe stroke"
106:
507:
Cavinato M, Rigon J, Volpato C, Semenza C, Piccione F (2012).
1093:
196:
506:
408:
267:. Cambridge, UK: Cambridge University Press. pp.
1346:
Upper dorsal pontine syndrome/Raymond–Céstan syndrome
737:
672:
336:
36:
Location of the primary auditory cortex in the brain
258:
81:). It has been argued to be as the combination of
1494:
646:
415:Journal of Stroke and Cerebrovascular Diseases
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330:
328:
326:
1070:
723:
306:
649:Journal of the American Academy of Audiology
323:
252:
1077:
1063:
730:
716:
427:10.1016/j.jstrokecerebrovasdis.2020.104827
55:
27:
600:
542:
532:
491:
473:
389:"Cortical Deafness: A longitudinal study"
163:shows hemorrhagic lesions involving both
581:Intractable & Rare Diseases Research
1495:
1311:Lateral medullary syndrome/Wallenberg
1058:
711:
628:
626:
624:
622:
620:
121:Since cortical deafness and auditory
997:Benign paroxysmal positional vertigo
386:
382:
380:
378:
376:
315:. Cambridge: Cambridge UP. pp.
302:
300:
298:
296:
132:Brainstem auditory evoked potentials
802:
449:
13:
1323:Medial medullary syndrome/Dejerine
640:
617:
14:
1524:
668:
373:
293:
149:
1092:associated with lesions of the
396:The American Journal of Nursing
568:
559:
500:
402:
307:Bogousslavsky, Julien (2001).
1:
245:
1384:Internuclear ophthalmoplegia
877:Auditory processing disorder
534:10.1371/journal.pone.0029909
351:10.1016/0022-510X(80)90148-3
208:
116:
7:
259:Ingram, John Henry (2007).
10:
1529:
827:Sensorineural hearing loss
221:
71:sensorineural hearing loss
1482:Upper motor neuron lesion
1469:
1449:
1397:
1338:
1303:
1294:
1251:
1215:
1104:
1017:
976:
955:
946:
894:
852:
820:Superior canal dehiscence
795:
759:
750:
676:
40:
35:
26:
21:
1351:Lateral pontine syndrome
468:. Hindawi Limited: 1–3.
462:Case Reports in Medicine
92:
73:caused by damage to the
1508:Complications of stroke
1389:One and a half syndrome
1365:Millard–Gubler syndrome
1361:Medial pontine syndrome
1040:Vestibulo–ocular reflex
810:Conductive hearing loss
593:10.5582/irdr.2020.03072
99:primary auditory cortex
83:auditory verbal agnosia
75:primary auditory cortex
1457:Alternating hemiplegia
844:Nonsyndromic deafness
1007:Labyrinthine fistula
932:visual reinforcement
882:Spatial hearing loss
475:10.1155/2017/6816748
387:Hood, Linda (1999).
185:hypercholesterolemia
1477:Pseudobulbar affect
1431:Parinaud's syndrome
1422:ventral tegmentum,
1236:Parkinson's disease
1155:Hemispatial neglect
771:Excessive response
525:2012PLoSO...729909C
191:. Imaging revealed
51:otorhinolaryngology
1409:ventral peduncle,
1379:Locked-in syndrome
1369:Foville's syndrome
1269:Dysdiadochokinesia
1180:Cortical blindness
1160:Gerstmann syndrome
1135:Expressive aphasia
1086:Signs and symptoms
232:electrophysiologic
69:is a rare form of
1490:
1489:
1465:
1464:
1441:Claude's syndrome
1418:Benedikt syndrome
1284:Cerebellar ataxia
1243:Thalamic syndrome
1200:Cortical deafness
1175:Bálint's syndrome
1150:Receptive aphasia
1052:
1051:
1048:
1047:
1025:Dix–Hallpike test
992:Ménière's disease
942:
941:
890:
889:
837:Cortical deafness
705:
704:
278:978-0-521-79190-8
165:internal capsules
67:Cortical deafness
64:
63:
22:Cortical deafness
16:Medical condition
1520:
1405:Weber's syndrome
1301:
1300:
1274:Intention tremor
1079:
1072:
1065:
1056:
1055:
1030:Unterberger test
984:Balance disorder
953:
952:
865:Wolfram syndrome
800:
799:
757:
756:
732:
725:
718:
709:
708:
674:
673:
664:
634:
630:
615:
614:
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572:
566:
563:
557:
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504:
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406:
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371:
370:
334:
321:
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311:Stroke Syndromes
304:
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240:auditory agnosia
143:auditory agnosia
87:auditory agnosia
60:
59:
31:
19:
18:
1528:
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1523:
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1518:
1517:
1493:
1492:
1491:
1486:
1461:
1445:
1393:
1334:
1290:
1247:
1211:
1171:Occipital lobe
1105:Cerebral cortex
1100:
1083:
1053:
1044:
1013:
972:
938:
912:Tone decay test
886:
848:
791:
746:
736:
706:
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641:Further reading
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101:located in the
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79:auditory system
54:
17:
12:
11:
5:
1526:
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1222:Basal ganglia
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1210:
1209:
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1196:Temporal lobe
1194:
1193:
1192:
1187:
1185:Anton syndrome
1182:
1177:
1169:
1168:
1167:
1162:
1157:
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1146:Parietal lobe
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1035:Romberg's test
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909:
904:
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870:Usher syndrome
867:
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849:
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677:Classification
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669:External links
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587:(4): 247–250.
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339:J. Neurol. Sci
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103:temporal lobes
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62:
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44:
38:
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33:
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24:
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15:
9:
6:
4:
3:
2:
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1234:
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1205:Prosopagnosia
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1165:Astereognosis
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1131:Frontal lobe
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1002:Labyrinthitis
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860:Deafblindness
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738:Disorders of
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719:
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710:
697:
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684:
679:
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662:
658:
655:(5): 330–42.
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519:(1): e29909.
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481:
476:
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421:(7): 104827.
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150:Case examples
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1199:
1122:PCA syndrome
1117:MCA syndrome
1112:ACA syndrome
902:Hearing test
836:
815:Otosclerosis
767:Hearing loss
689:
652:
648:
584:
580:
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345:(1): 35–49.
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181:hypertension
153:
138:
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120:
111:
96:
66:
65:
1357:) (lateral)
1190:Pure alexia
988:Peripheral
832:Presbycusis
785:Phonophobia
780:Hyperacusis
1497:Categories
1253:Cerebellum
922:Audiometry
917:Weber test
907:Rinne test
246:References
1513:Audiology
1296:Brainstem
1264:Dysmetria
1216:Subcortex
1098:brainstem
1090:syndromes
968:nystagmus
927:pure tone
484:1687-9627
443:218585923
287:297335127
209:Treatment
169:inner ear
128:brainstem
117:Diagnosis
47:Neurology
42:Specialty
1503:Deafness
1398:Midbrain
1260:Lateral
1231:Dystonia
956:Symptoms
775:Tinnitus
760:Symptoms
611:33139984
553:22272260
513:PLOS One
435:32386850
367:36026035
228:apoplexy
193:sequelae
189:tinnitus
1373:basilar
1304:Medulla
1280:Medial
1127:Aphasia
977:Disease
963:Vertigo
948:Balance
796:Disease
752:Hearing
744:balance
740:hearing
696:D006313
661:7987023
602:7586874
544:3260175
521:Bibcode
493:5368354
359:7420124
222:History
123:agnosia
1226:Chorea
1140:Abulia
1088:, and
659:
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551:
541:
490:
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441:
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365:
357:
285:
275:
271:–171.
107:stroke
53:
1470:Other
1450:Other
1094:brain
1018:Tests
895:Tests
853:Other
439:S2CID
392:(PDF)
363:S2CID
197:edema
93:Cause
1355:AICA
1339:Pons
1316:PICA
1096:and
803:Loss
742:and
691:MeSH
657:PMID
633:Web.
607:PMID
549:PMID
480:ISSN
466:2017
431:PMID
355:PMID
283:OCLC
273:ISBN
139:feel
85:and
1424:PCA
1411:PCA
1328:ASA
597:PMC
589:doi
539:PMC
529:doi
488:PMC
470:doi
423:doi
347:doi
317:153
269:160
236:MRI
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419:29
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375:^
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343:48
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325:^
295:^
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183:,
161:CT
49:,
1375:)
1371:(
1367:/
1363:/
1353:(
1078:e
1071:t
1064:v
731:e
724:t
717:v
683:D
663:.
653:5
613:.
591::
585:9
555:.
531::
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517:7
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349::
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