Hearing loss may be found in patients with systemic autoimmune disorders such as connective tissue diseases (CTD). This work aimed to review the literature on ear involvement in CTD. A systematic search of articles published in PubMed/MEDLINE, EMBASE, and SCOPUS from 1966 to June 2023 following PRISM guidelines was done. Seventy-nine papers were selected: 39 on rheumatoid arthritis; 16 on systemic lupus erythematosus; 14 on scleroderma; 6 on Sjogren’s syndrome; 1 on mixed connective tissue disease; and 3 that approached more than one CTD. Most of them showed that hearing loss (HL) in connective tissue disease patients was higher than in controls, mainly of the sensorineural type and at high frequencies. Associations with clinical features and autoantibodies profile of underlying conditions varied widely among the results. In conclusion, sensorineural hearing loss is common in individuals with CTD, and it is essential to be aware of this complication in order to establish an effective treatment.