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  • Updated 05.14.2024
  • Released 07.02.2002
  • Expires For CME 05.14.2027

Cognitive aspects of central auditory disorders



Central auditory disorder is a continuum of dysfunction involving the processing and interpretation of sounds, ranging from detecting the presence of simple sounds to comprehending complex auditory stimuli such as environmental sounds and music. This article reviews the range of clinical syndromes occurring in both acquired and developmental cases and their associated pathophysiology.

Key points

• Central auditory disorders are rare disorders that affect specific forms of auditory processing.

• Various distinct subtypes affect the processing of different auditory stimuli, including words (word deafness), environmental sounds (nonverbal auditory agnosia), and music (amusia).

• These subtypes usually co-occur but can occasionally be affected in isolation.

• Central auditory disorders usually result from damage in the temporal regions near the auditory and auditory association cortex.

• Diagnosis must be comprehensive due to co-occurrence of other deficits and should include audiological as well as neuropsychological tests.

Historical note and terminology

Central auditory disorder refers to a dysfunction in the ability to recognize sounds, despite adequate hearing. There are several types of central auditory disorders, including the inability to recognize environmental sounds (nonverbal auditory agnosia), understand spoken language (word deafness, also called verbal auditory agnosia), process certain aspects of music (amusia), and recognize the identity of a speaker from the sound of their voice (phonagnosia). Whereas “pure” cases of these subtypes have been reported, patients usually exhibit mixed forms of two central auditory disorders. Central auditory disorders usually occur following temporal lobe lesions to the left or right hemisphere or both. Although central auditory disorders usually occur in conjunction with aphasias, they can occur in the absence of any deficits in language processing.

Central auditory disorders were first described in the 19th century, including reports by German physician Ludwig Lichtheim (1845-1923), French psychiatrist (1864-1947), and French neurologist Jules Dejerine (1849-1917) (51; 79; 14; 15).

Auditory agnosia can be divided into perceptual-discriminative and associative-semantic subtypes. Early research by Finkelnburg (1870) and Hughlings Jackson (1878) found a high instance of nonverbal auditory agnosia in patients with aphasia, suggesting an intimate relationship between processing verbal and nonverbal stimuli. Word deafness can also be divided into two subtypes: a temporal processing disorder and a phonemic processing disorder (04).

Historically, there has been considerable debate about the existence of truly “pure” cases of word deafness, with many studies failing to adequately assess performance in the processing in other domains (08). For instance, Yaqub and colleagues reported that their patient could “recognize and appreciate music,” but did not carry out formal testing (100). In that vein, using more controlled laboratory tests, Pinard and colleagues found that a patient who was previously classified as a “pure” case of word deafness (59) also had nonverbal auditory agnosia and amusia (65). However, as several cases that can be classified as “pure” do exist in the literature (60; 84; 31; 81), pure word deafness may be a real, but very rare, disorder.

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