Symptomatology, neuroanatomical correlates and pathomechanisms of central hearing disorders (pure word deafness, verbal/nonverbal auditory agnosia, cortical deafness) - A review

Pure word deafness (auditory verbal agnosia) is characterized by an impairment of auditory comprehension, repetition of verbal material and writing to dictation whereas spontaneous speech production and reading largely remain unaffected. Sometimes, this syndrome is preceded by complete deafness (cortical deafness) of varying duration. Perception of vowels and suprasegmental features of verbal utterances (e.g., intonation contours) seems to be less disrupted than the processing of consonants and, therefore, might mediate residual auditory functions. Often, lip reading and/or slowing of speaking rate allow within some limits to compensate for speech comprehension deficits. Apart from a few exceptions, the available reports of pure word deafness documented a bilateral temporal lesion. In these instances, as a rule, identification of nonverbal (environmental) sounds, perception of music, temporal resolution of sequential auditory cues and/or spatial localization of acoustic events were compromised as well. The observed variable constellation of auditory signs and symptoms in central hearing disorders following bilateral temporal disorders, most probably, reflects the multitude of functional maps at the level of the auditory cortices subserving, as documented in a variety of non-human species, the encoding of specific stimulus parameters each. Thus, verbal/nonverbal auditory agnosia may be considered a paradigm of distorted "auditory scene analysis" (Bregman 1990) affecting both primitive and schema-based perceptual processes. It cannot be excluded, however, that disconnection of the Wernicke-area from auditory input (Geschwind 1965) and/or an impairment of suggested "phonetic module" (Liberman 1996) contribute to the observed deficits as well. Conceivably, these latter mechanisms underly the rare cases of pure word deafness following a lesion restricted to the dominant hemisphere. Only few instances of a rather isolated disruption of the discrimination/identification of nonverbal sound sources, in the presence of uncompromised speech comprehension, have been reported so far (nonverbal auditory agnosia). As a rule, unilateral right-sided damage has been found to be the relevant lesion.