ABSTRACT
Aim: To describe hearing loss in patients with spondyloepiphyseal dysplasia congenita and tarda. Methodology: A literature review of the National Library of Medicine's online database on hearing loss in patients with spondyloepiphyseal dysplasia congenita and tarda was performed. Results: Four articles were identified that reported hearing loss in subjects with spondyloepiphyseal dysplasia congenita and tarda. Including this study, a total of fourteen patients with hearing loss are reported. Eight patients with sensorineural loss and two patients with mixed hearing loss were identified. The type of hearing loss is unknown in 4 cases. Conclusion: Serial audiograms are recommended early in life in individuals with spondyloepiphyseal dysplasia congenita and when clinically indicated in patients with spondyloepiphyseal dysplasia tarda.