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

Kaga, Kimitaka; Shinjo, Yukiko; Enomoto, Chieko; Shindo, Mitsuko

doi:10.3109/00016489.2014.980914

Cited by 12 publications

(7 citation statements)

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“…The primary finding from the present experiment is that RT was significantly (~100 ms) shorter in the presence of a concurrent auditory stimulus (Fig. 2), despite the participant being unable to consciously process sound 21 . This novel result indicates that secondary auditory pathways can contribute to the processes involved in response initiation, even when the auditory stimulus is not perceived.…”

Section: Discussionmentioning

confidence: 47%

“…One male participant (Age = 40 years) with complete bilateral cortical deafness participated in this case study. His condition has been thoroughly described previously 21 , but in short, as the result of a 2007 rupture of the right internal carotid-posterior communicating artery and a subarachnoidal hemorrhage, the patient suffered extensive infarction of both left and right primary auditory cortices and auditory radiations (Fig. 1a).…”

Section: Patientmentioning

confidence: 90%

“…While other localized damage occurred resulting in the loss of some motor function, this was recovered completely; however, complete loss of hearing and some somatosensory and vestibular function did not recover at all. Pure tone audiometry showed thresholds that were "off the scale" (i.e., no perception even at the highest tested intensity) and speech audiometry showed 0% speech discrimination 21 . The patient reports no sensation or perception of sound whatsoever; however, audiometry showed a normal ABR with thresholds of 20 dB in both ears (Fig.…”

Section: Patientmentioning

confidence: 98%

“…In order to investigate the involvement of subcortical circuits in response initiation, we carried out a simple RT experiment in a patient with bilateral cortical deafness, in which an auditory stimulus of varying intensities was presented in conjunction with a visual go-signal. Complete bilateral cortical deafness is an exceedingly rare condition, and the patient reports no conscious perception of sound, a result of cortical auditory damage following a bilateral stroke 21 (Fig. 1a).…”

mentioning

confidence: 99%

“…Additional details are provided in the Methods section. See Kaga et al21 for original images and a more detailed patient description and testing protocols. Reproduced with permission.…”

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confidence: 99%

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An unperceived acoustic stimulus decreases reaction time to visual information in a patient with cortical deafness

Carlsen

Maslovat

Kaga

2020

Sci Rep

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Responding to multiple stimuli of different modalities has been shown to reduce reaction time (RT), yet many different processes can potentially contribute to multisensory response enhancement. To investigate the neural circuits involved in voluntary response initiation, an acoustic stimulus of varying intensities (80, 105, or 120 dB) was presented during a visual RT task to a patient with profound bilateral cortical deafness and an intact auditory brainstem response. Despite being unable to consciously perceive sound, RT was reliably shortened (~100 ms) on trials where the unperceived acoustic stimulus was presented, confirming the presence of multisensory response enhancement. Although the exact locus of this enhancement is unclear, these results cannot be attributed to involvement of the auditory cortex. Thus, these data provide new and compelling evidence that activation from subcortical auditory processing circuits can contribute to other cortical or subcortical areas responsible for the initiation of a response, without the need for conscious perception. Responding to a stimulus with a voluntary action is typically thought to involve several processes that are mediated by cortical structures. For example, a sprinter will "get set" to launch their body out of the starting blocks, and once the starting pistol fires the sprinter must recognize that the signal has occurred and initiate that planned response. The processes involved in planning a response (preparation) and holding it ready until the go-signal occurs (storage) have been ascribed principally to cortical motor and premotor areas 1-3. Similarly, processes involved in stimulus recognition (detection/discrimination) and response initiation are thought to occur in cortical sensory and association areas as well as frontal cortical regions 4. However, data from a paradigm involving the presentation of an unpredictable high-intensity auditory stimulus that is capable of eliciting a startle reflex has suggested that lower subcortical structures may play a role in response initiation processes 5. Typical mean reaction time (RT) to the onset of an auditory stimulus is approximately 140-150 ms, with reaction to a visual stimulus being slightly longer at 180-200 ms 6. Yet, when a loud startling acoustic stimulus (SAS) is presented, RT is dramatically shortened to approximately 70-80 ms on average, while the characteristic features of the response remain largely unchanged 7,8. The startle reflex is primarily mediated by brainstem nuclei 9 and elicits a pattern of generalized flexion in various muscles with latencies of 50-100 ms. Given the short latency of both the startle reflex and prepared response, early explanations of RT speeding by startle were that the "voluntary" reaction was too short to have involved the normal cortically mediated stimulus-response processing pathways and was somehow driven at the speed of the startle response. In order for this to occur, it was argued that storage and/or initiation of the response must have involved subcortical struc...

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Section: Discussionmentioning

confidence: 47%

Section: Patientmentioning

confidence: 90%

Section: Patientmentioning

confidence: 98%

mentioning

confidence: 99%

“…Additional details are provided in the Methods section. See Kaga et al21 for original images and a more detailed patient description and testing protocols. Reproduced with permission.…”

mentioning

confidence: 99%

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