First report of prevalence of non‐syndromic hereditary prosopagnosia (HPA)

Kennerknecht, Ingo; Grueter, Thomas; Welling, Brigitte; Wentzek, Sebastian; Horst, Jürgen; Edwards, Steve; Grueter, Martina

doi:10.1002/ajmg.a.31343

Cited by 242 publications

(230 citation statements)

References 27 publications

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“…The diagnosis of hereditary prosopagnosia was based on a standardized semistructured interview (Tables S3 and S4) (41,42), which has been validated with objective face recognition tests in previous studies (41,43).…”

Section: Methodsmentioning

confidence: 99%

Simulation of talking faces in the human brain improves auditory speech recognition

Kriegstein

Doğan

Grüter

et al. 2008

Proc. Natl. Acad. Sci. U.S.A.

140

199

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Human face-to-face communication is essentially audiovisual. Typically, people talk to us face-to-face, providing concurrent auditory and visual input. Understanding someone is easier when there is visual input, because visual cues like mouth and tongue movements provide complementary information about speech content. Here, we hypothesized that, even in the absence of visual input, the brain optimizes both auditory-only speech and speaker recognition by harvesting speaker-specific predictions and constraints from distinct visual face-processing areas. To test this hypothesis, we performed behavioral and neuroimaging experiments in two groups: subjects with a face recognition deficit (prosopagnosia) and matched controls. The results show that observing a specific person talking for 2 min improves subsequent auditory-only speech and speaker recognition for this person. In both prosopagnosics and controls, behavioral improvement in auditory-only speech recognition was based on an area typically involved in face-movement processing. Improvement in speaker recognition was only present in controls and was based on an area involved in face-identity processing. These findings challenge current unisensory models of speech processing, because they show that, in auditory-only speech, the brain exploits previously encoded audiovisual correlations to optimize communication. We suggest that this optimization is based on speaker-specific audiovisual internal models, which are used to simulate a talking face.fMRI ͉ multisensory ͉ predictive coding ͉ prosopagnosia H uman face-to-face communication works best when one can watch the speaker's face (1). This becomes obvious when someone speaks to us in a noisy environment, in which the auditory speech signal is degraded. Visual cues place constraints on what our brain expects to perceive in the auditory channel. These visual constraints improve the recognition rate for audiovisual speech, compared with auditory speech alone (2). Similarly, speaker identity recognition by voice can be improved by concurrent visual information (3). Accordingly, audiovisual models of human voice and face perception posit that there are interactions between auditory and visual processing streams (Fig. 1A) (4, 5).Based on prior experimental (6-8) and theoretical work (9-12) we hypothesized that, even in the absence of visual input, the brain optimizes auditory-only speech and speaker recognition by harvesting predictions and constraints from distinct visual face areas (Fig. 1B).Experimental studies (6,8) demonstrated that the identification of a speaker by voice is improved after a brief audiovisual experience with that speaker (in contrast to a matched control condition). The improvement effect was paralleled by an interaction of voice and face-identity sensitive areas (8). This finding suggested that the associative representation of a particular face facilitates the recognition of that person by voice. However, it is unclear whether this effect also extends to other audiovisual dependencies in human ...

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Section: Methodsmentioning

confidence: 99%

Simulation of talking faces in the human brain improves auditory speech recognition

Kriegstein

Doğan

Grüter

et al. 2008

Proc. Natl. Acad. Sci. U.S.A.

140

199

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“…DP affects approximately 2% of the population (Kennerknecht et al, 2006;Kennerknecht, Pluempe, & Welling, 2008), and evidence from family and twin studies suggests that there may be a genetic component to this disorder (Duchaine, Germine, & Nakayama, 2007;Lee, Duchaine, Wilson, & Nakayama, 2010). The exact nature of the face processing deficits in DP is still largely unknown.…”

Section: Introductionmentioning

confidence: 99%

Face identity matching is selectively impaired in developmental prosopagnosia

Fisher

Towler

Eimer

2017

Cortex

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Individuals with developmental prosopagnosia (DP) have severe face recognition deficits, but the mechanisms that are responsible for these deficits have not yet been fully identified. We assessed whether the activation of visual working memory for individual faces is selectively impaired in DP. Twelve DPs and twelve age-matched control participants were tested in a task where they reported whether successively presented faces showed the same or two different individuals, and another task where they judged whether the faces showed the same or different facial expressions. Repetitions versus changes of the other currently irrelevant attribute were varied independently. DPs showed impaired performance in the identity task, but performed at the same level as controls in the expression task. An electrophysiological marker for the activation of visual face memory by identity matches (N250r component) was strongly attenuated in the DP group, and the size of this attenuation was correlated with poor performance in a standardized face recognition test. Results demonstrate an identity-specific deficit of visual face memory in DPs. Their reduced sensitivity to identity matches in the presence of other image changes could result from earlier deficits in the perceptual extraction of image-invariant visual identity cues from face images.

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“…Prosopagnosia was first studied as an acquired impairment arising as a consequence of brain injury, but it is now clear that severe face recognition problems can be present from childhood in the absence of any history of serious injury or disease. The reasons for failing to develop normal adult face recognition skills are not currently known, but appear to often include a genetic element [3,4]. DP may also result from prenatal or early minor brain damage, or inadequate visual input during key developmental periods (for example, due to severe myopia, or suppression of input from the left eye in amblyopia) [5].…”

Section: Introductionmentioning

confidence: 99%

“…It is now believed that the prevalence of DP may be as high as 2% of the general population [4]. However, the condition is seldom diagnosed since people with prosopagnosia can identify people in many situations by using general appearance and manner (including hair and clothes), semantic features (e.g., bushy eyebrows), voice, and contextual cues.…”

Section: Introductionmentioning

confidence: 99%

Psychosocial consequences of developmental prosopagnosia: A problem of recognition

Yardley

McDermott

Pisarski

et al. 2008

Journal of Psychosomatic Research

175

151

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Objective: To provide the first systematic in-depth description of the consequences of developmental prosopagnosia (DP; 'face blindness') for psychosocial functioning and occupational disability, in order to determine what kind of professional intervention may be needed. Methods: Semi-structured telephone interviews were carried out with 25 people whose selfreports of face recognition problems were confirmed by impaired scores on the Cambridge Face Recognition Test. Thematic analysis was used to inductively identify and understand common psychosocial consequences of DP. Results: All participants described recurrent and sometimes traumatic social interaction difficulties caused by recognition problems, such as failing to recognize close friends, work colleagues, and family members. These problems often led to chronic anxiety about offending others and feelings of embarrassment, guilt, and failure. Most participants described some degree of fear and avoidance of social situations in which face recognition was important, including family and social gatherings, and meetings at work. Long-term consequences could include dependence on others, a restricted social circle, more limited employment opportunities, and loss of self-confidence. Conclusion: The potential for negative psychosocial consequences and occupational disability posed by DP is as great as that posed by conditions which are currently afforded professional recognition and support, such as stuttering and dyslexia. Wider recognition of the problems prosopagnosia can cause could reduce anxiety about social interaction difficulties by making it easier to explain and justify recognition problems to other people, including employers. Greater professional awareness could facilitate detection and referral of those requiring support with coping with social interactions.

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First report of prevalence of non‐syndromic hereditary prosopagnosia (HPA)

Cited by 242 publications

References 27 publications

Simulation of talking faces in the human brain improves auditory speech recognition

Simulation of talking faces in the human brain improves auditory speech recognition

Face identity matching is selectively impaired in developmental prosopagnosia

Psychosocial consequences of developmental prosopagnosia: A problem of recognition

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