Inner ear dysplasia in sporadic lacrimo-auriculo-dento-digital syndrome. A case report and review of the literature.
The lacrimo-auriculo-dento-digital (LADD) syndrome is a rare inherited disorder affecting the lacrimal, auricular, salivary, and osseous system. Only very limited data on LADD associated petrous bone lesions are available up to now. We describe a young girl suffering from the LADD syndrome with sensorineural hearing loss and inner ear dysplasia. This case suggests, that inner ear dysplasia might be a more common feature of the LADD syndrome than has been assumed so far.
Meuschel-Wehner
Stephanie
S
Department of Otorhinolaryngology, Charité, Campus Mitte, Humboldt University, Schumannstrasse 20/21, D-10098 Berlin, Germany.
Klingebiel
Randolf
R
Werbs
Mechthild
M
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000293:Adolescent; D001299:Audiometry; D001842:Bone and Bones / Q000002:abnormalities; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging; D004825:Epiglottis / Q000002:abnormalities; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis; D006801:Humans; D021621:Imaging, Three-Dimensional; D007765:Lacrimal Apparatus / Q000002:abnormalities; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014071:Tooth Abnormalities