Cognitive aspects of central auditory disorders

Luke Miller MS (Mr. Miller of the University of California, San Diego, has no relevant financial relationships to disclose.)
Victor W Mark MD, editor. (Dr. Mark of the University of Alabama at Birmingham has no relevant financial relationships to disclose.)
Originally released July 2, 2002; last updated March 11, 2017; expires March 11, 2020

This article includes discussion of cognitive aspects of central auditory disorders, auditory agnosia, cortical auditory processing disorders, and word deafness. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

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. In this article, the authors discuss the range of clinical syndromes occurring in both acquired and developmental cases, some of the proposed anatomical mechanisms behind these disorders, and their associated pathophysiology.

Key points

 

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

 

• There are many distinct subtypes affecting the processing of different types of 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 there are “pure” cases of these disorders in the literature, patients usually exhibit the symptoms of at least 2 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 (Lichtheim 1885) and have since received considerable research interest from neuropsychologists. Research on auditory agnosia has suggested that the disorder can be divided into 2 subtypes: perceptual-discriminative and associative-semantic. 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. It has also been proposed that word deafness should also be divided into 2 subtypes: a temporal processing disorder and a phonemic processing disorder (Auerbach et al 1982).

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 (Buchman et al 1986). For instance, Yaqub and colleagues reported that their patient could “recognize and appreciate music,” but did not carry out formal testing (Yaqub et al 1988). 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 (Mesulam 1982) also had nonverbal auditory agnosia and amusia (Pinard et al 2002). However, as several cases that can be classified as “pure” do exist in the literature (Metz-Lutz and Dahl 1984; Stefanatos et al 2005; Hayashi and Hayashi 2007; Slevc et al 2011), pure word deafness may be a real, albeit very rare, disorder.

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