<unk>  is a type of<unk>  loss in which the root cause lies in the vestibulocochlear nerve (Cranial nerve VIII), the inner ear, or central processing centers of the brain. 
The Weber test, in which a tuning fork is touched to the head, localizes to the normal ear in people with this condition.
The Rinne test, which tests air conduction vs. bone conduction is positive (normal), though both bone and air conduction are reduced equally.
Sensorineural hearing loss
Bilateral vestibulopathy
Moon facies
Coarse facial features
Xerophthalmia
Ankyloglossia
Mandibular prominence
Parotid gland enlargement