Vestibular pathology presenting as behavioral problems in a child with Down Syndrome: a case report

Here we present a case of superior semicircular canal dehiscence (SSCD) in a non-verbal 17-year-old male patient with trisomy 21 that presented with behavioral problems, instead of the more typical symptoms of episodic noise-induced vertigo. SSCD syndrome is characterized by sound-, or pressure-induced vertigo. Patients often complain of vertigo or oscillopsia evoked by loud noises, which may be either external sounds or those elicited by the patients themselves when they are speaking. In cases of SSCD, auditory testing will typically reveal a conductive hearing loss on the affected side while high-resolution temporal bone computed-tomography will demonstrate bony dehiscence overlying the ipsilateral superior semicircular canal. Here we present a report of SSCD in a patient with Down Syndrome who was without speech and reacted to noise stimulation with behavioral outbursts. In this case, simple modifications, such as noise avoidance and hearing protection were able to effect positive changes in behavior and improve social interactions. This case underscores the importance of a broad differential diagnosis, and the need to consider alternate presentations, in children with Down Syndrome or any child who is otherwise unable to express himself.