Stroke-Associated Cortical Deafness: A Systematic Review of Clinical and Radiological Characteristics

Abstract

Background: Stroke is the leading cause of cortical deafness (CD), the most severe form of central hearing impairment. CD remains poorly characterized and perhaps underdiagnosed. We perform a systematic review to describe the clinical and radiological features of stroke-associated CD. Methods: PubMed and the Web of Science databases were used to identify relevant publications up to 30 June 2021 using the MeSH terms: “deafness” and “stroke”, or “hearing loss” and “stroke” or “auditory agnosia” and “stroke”. Results: We found 46 cases, caused by bilateral lesions within the central auditory pathway, mostly located within or surrounding the superior temporal lobe gyri and/or the Heschl’s gyri (30/81%). In five (13.51%) patients, CD was caused by the subcortical hemispheric and in two (0.05%) in brainstem lesions. Sensorineural hearing loss was universal. Occasionally, a misdiagnosis by peripheral or psychiatric disorders occurred. A few (20%) had clinical improvement, with a regained oral conversation or evolution to pure word deafness (36.6%). A persistent inability of oral communication occurred in 43.3%. A full recovery of conversation was restricted to patients with subcortical lesions. Conclusions: Stroke-associated CD is rare, severe and results from combinations of cortical and subcortical lesions within the central auditory pathway. The recovery of functional hearing occurs, essentially, when caused by subcortical lesions.

Auditory Agnosia in Autoimmune Encephalitis

“…Optimal therapy for patients with auditory agnosia remains unclear but speech therapy targeted at using alternate forms of communication including lip reading is recommended. 4 This case highlights that while many patients recover very well long term with AIE, even with early aggressive therapy, persistent focal deficits do occur and can result in significant long-term disability.…”

Auditory Agnosia in Autoimmune Encephalitis

“…Optimal therapy for patients with auditory agnosia remains unclear but speech therapy targeted at using alternate forms of communication including lip reading is recommended. 4 This case highlights that while many patients recover very well long term with AIE, even with early aggressive therapy, persistent focal deficits do occur and can result in significant long-term disability.…”

Cortical deafness following embolic strokes

“…Central deafness is a complex syndrome characterised by the failure of central auditory signal processing, despite intact peripheral auditory function 1. A systematic review in 2021 identified 44 cases of strokeinduced central deafness, reported in the literature 2. Several syndromes fall within the classification of central deafness, including cortical deafness, verbal or pure word auditory agnosia, non-verbal agnosia and amusia.…”

Cerebral infarction presenting with bilateral sudden deafness as the primary symptom: a case report

“…Patients with cortical deafness retain brainstem auditory-evoked potentials, lose the perception of nonverbal and verbal sounds, show no response to all auditory stimuli, and have abnormal pure tone hearing test results; cortical deafness is thus considered the most serious kind of cortical hearing impairment. 20 Early diagnosis and prompt treatment are essential for reducing the mortality and disability caused by cerebral infarction with sudden deafness as the first manifestation. 21 A neurological examination of our patient did not reveal any definitive abnormalities at the time of admission.…”

Auditory Agnosia in Autoimmune Encephalitis

“…Optimal therapy for patients with auditory agnosia remains unclear but speech therapy targeted at using alternate forms of communication including lip reading is recommended. 4 This case highlights that while many patients recover very well long term with AIE, even with early aggressive therapy, persistent focal deficits do occur and can result in significant long-term disability.…”

Cortical deafness following embolic strokes

“…Central deafness is a complex syndrome characterised by the failure of central auditory signal processing, despite intact peripheral auditory function 1. A systematic review in 2021 identified 44 cases of strokeinduced central deafness, reported in the literature 2. Several syndromes fall within the classification of central deafness, including cortical deafness, verbal or pure word auditory agnosia, non-verbal agnosia and amusia.…”

Cerebral infarction presenting with bilateral sudden deafness as the primary symptom: a case report

“…Patients with cortical deafness retain brainstem auditory-evoked potentials, lose the perception of nonverbal and verbal sounds, show no response to all auditory stimuli, and have abnormal pure tone hearing test results; cortical deafness is thus considered the most serious kind of cortical hearing impairment. 20 Early diagnosis and prompt treatment are essential for reducing the mortality and disability caused by cerebral infarction with sudden deafness as the first manifestation. 21 A neurological examination of our patient did not reveal any definitive abnormalities at the time of admission.…”

Auditory Agnosia in Autoimmune Encephalitis

“…Optimal therapy for patients with auditory agnosia remains unclear but speech therapy targeted at using alternate forms of communication including lip reading is recommended. 4 This case highlights that while many patients recover very well long term with AIE, even with early aggressive therapy, persistent focal deficits do occur and can result in significant long-term disability.…”

Cortical deafness following embolic strokes

“…Central deafness is a complex syndrome characterised by the failure of central auditory signal processing, despite intact peripheral auditory function 1. A systematic review in 2021 identified 44 cases of strokeinduced central deafness, reported in the literature 2. Several syndromes fall within the classification of central deafness, including cortical deafness, verbal or pure word auditory agnosia, non-verbal agnosia and amusia.…”

Cerebral infarction presenting with bilateral sudden deafness as the primary symptom: a case report

“…Patients with cortical deafness retain brainstem auditory-evoked potentials, lose the perception of nonverbal and verbal sounds, show no response to all auditory stimuli, and have abnormal pure tone hearing test results; cortical deafness is thus considered the most serious kind of cortical hearing impairment. 20 Early diagnosis and prompt treatment are essential for reducing the mortality and disability caused by cerebral infarction with sudden deafness as the first manifestation. 21 A neurological examination of our patient did not reveal any definitive abnormalities at the time of admission.…”