Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely.
Szymko-Bennett et al. (2001) found that the overall hearing loss in Type I Stickler Syndrome is generally mild and is not significantly progressive.
Patients with Apert syndrome have a high occurrence of middle ear disease, otitis media and conductive hearing loss (Perterson-Fazone et al., 2001).
Conductive hearing loss is frequently seen in this population due to almost constant middle ear disease (Gould et al., 1982).
Furthermore, inner ear anomalies have been described in Apert syndrome, such as dilatation of the vestibule, dysplastic semicircular canals and cochlear malformations (Zhou G et al. Otol Neurotol.
Literature has suggested that persons with Crouzon syndrome typically have conductive hearing loss caused by middle ear effusion (or fluid in the middle ear) and perforation to ossicular fixation (ossicles), intratympanic bony masses (tympanic membrane), ossicular anomalies and closure of the oval window.
In addition to external malformations, individuals with VCFS are more vulnerable to otitis media because of the presence of a cleft or other form of velopharyngeal inadequacy.
In addition to ear malformations, a conductive hearing loss can be present, typically ranging from mild to severe.
Individuals with Nager syndrome typically have the malformations of the auricle, external auditory canal and middle ear, including the ossicles.