Word deafness: one hundred years later.

Abstract

Since its original description the diagnosis of word deafness has been greatly expanded. Confusion has arisen with regard to the usage of the related terms pure word deafness, auditory agnosia, and cortical deafness. Three new cases of word deafness are presented including one case with CT and necropsy correlation. These cases are compared with 34 previously reported cases of various cortical auditory disorders. Our review establishes that patients with word deafness who have had formal testing of linguistic and non-linguistic sound comprehension and musical abilities always demonstrated a more pervasive auditory agnosia. Despite the spectrum of auditory deficits and associated language abnormalities, patients with word deafness share common features including aetiology, pathology, clinical presentation and course. These common features justify inclusion of heterogeneous cortical auditory disorders under the rubric of word deafness. Despite some limitations the term "word deafness" should be retained for this syndrome, since inability to comprehend spoken words is the most distinctive clinical deficit. Word deafness is most frequently caused by cerebrovascular accidents of presumed cardiac embolisation, with bitemporal cortico-subcortical lesions. The sequence of cerebral injury is not predictive of resulting auditory deficits. Impairment of musical abilities parallels the severity of the auditory disorder.