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.

NIHSS is deficient in acute stroke presenting with cortical deafness; clinical skills remain the backbone: a case report

“…In a systematic review by Silva and colleagues in 2021 a total of only 44 cases were found dating from 1977 to 2021. Along this review a conclusion was made that CD is mostly underdiagnosed as well as being rare and that it develops from cortical and subcortical vascular insults as well as other lesions interfering with the central auditory pathway [ 9 ].…”

“…Yet, CD occurs only from bilateral lesions [ 10 ]. This was the condition in the presented case; the patient suffered earlier large right subcortical hemispheric infarction with mRS zero and actually there was no recall of initial deficit and on suffering a new left infarction that was presenting initially in the form of TIA a profound CD developed, although patient was still able to pantomime, read orders and write and this by itself might differentiate CD patients from aphasic patients [ 9 ].…”

“…Only after her second return rtPA was considered but again on a zero NIHSS score and after analyzing the patient’s entire available data including imaging. Acute CD and as a result of missed identification by NIHSS sometimes may be considered psychiatric [ 9 ].…”

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.…”

“…[1][2][3] Auditory agnosia may result from bilateral lesions to any structure along the auditory pathway, including the temporal lobes or the medial geniculate bodies of the thalamus. 4 Unilateral lesions, typically affecting the auditory cortex, have also been reported as well as bilateral lesions of the pons and midbrain. 2,4 Vascular etiologies remain the most common cause of auditory agnosia, often located in the bilateral temporal lobes.…”