Which syndrome is characterized by hearing loss occurring in 74% of affected individuals?

Crouzon Syndrome is the condition characterized by hearing loss in approximately 74% of affected individuals. This syndrome is a genetic disorder resulting from mutations in the FGFR2 or FGFR3 genes, leading to craniosynostosis, which is the premature fusion of skull bones. The malformation of the skull and face associated with Crouzon Syndrome can lead to conductive hearing loss, often due to middle ear abnormalities.

Hearing loss occurs in a significant portion of patients with Crouzon Syndrome, dating from early childhood and frequently requiring ongoing audiological surveillance and potential interventions, such as hearing aids or surgical options.

The other syndromes listed do not show hearing loss with the same prevalence or are associated with different primary manifestations. For instance, Pfeiffer Syndrome primarily affects skull shape due to craniosynostosis but has a lower association with hearing loss. Apert Syndrome, while also impacting craniosynostosis and facial structure, does not have the same emphasis on hearing impairment. Similarly, Carpenter Syndrome has its specific set of features, and although hearing loss can occur, it is not as common or pronounced as in Crouzon Syndrome.