Head and Neck Imaging

lacrimo-auriculo-dento-digital syndrome

Also known as Levy-Hollister syndrome. The syndrome is characterized by nasolacrimal duct obstruction (with hypolasia of the lacrimal puncta), cup-shaped ears with conductive hearing loss and sensorineural hearing loss , mild hypodontia with enamel dysplasia, and digital malformations. Narrowing of the external ear is present.
CT-imaging of the temporal bone shows various middle ear and inner ear malformations, including malformations of the ossicular chain , narrowing or absence of the oval window, hypoplasia of the cochlea with modiolar deficiency (see modiolus anomalies ).

RH