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

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.

Multidisciplinary Evaluation and Management of Cortical Deafness and Other Related Central Hearing Impairments

Purpose A summary of literature surrounding cortical hearing impairment (cortical deafness, auditory agnosia, and pure word deafness) was studied to identify causes and prognoses of the disorders, in addition to assessment methods and rehabilitation strategies for the multidisciplinary care team setting. Method A comprehensive review of the literature surrounding cortical hearing impairment with a focus on clinical evaluation and management of patients with these conditions was performed. Results An extensive literature search resulted in 109 relevant articles discussing cases of cortical hearing impairment. Conclusions Cortical deafness continues to be the rarest of the cortical hearing impairment spectrum of disorders, with reports of auditory agnosia and pure word deafness appearing more commonly. In order to assess and manage patients with these conditions, audiologists must utilize a comprehensive set of clinical tools. This information must be combined with results of thorough multidisciplinary evaluations in order to better characterize diagnoses and outcomes for these patients. Continued reporting of case studies and summaries of the literature is encouraged, with a specific call for more consistent assessment and reporting formats.