Eye-ear overlap syndromes

Timothy C. Hain, MD, • Most recent update: July 19, 2020

There are just a few diseases that affect the eye and ear together. These are mainly autoimmune inner ear diseases, as one needs a condition that can attack two relatively unconnected sensory organs. These diseases are often recognized by ophthalmologists, but another pathway to diagnosis may be through otoneurology practices that have access to eye specialists (such as Chicago Dizziness and Hearing).

Cogan syndrome causes mainly hearing symptoms, but can also cause dizziness and eye disease. There is generally a brief episode of inflammatory eye disease (interstitial keratitis) followed by bilateral audiovestibular symptoms. The interstitial keratitis usually occurs with sudden onset and is characterized by photophobia, lacrimation, and eye pain. The vestibuloauditory dysfunction is usually bilateral, presenting with tinnitus, sensorineural hearing loss, and acute episodes of vertigo. In Cogan syndrome, fibrous tissue may obliterate the labyrinth.

Susac syndrome (microangiopathy of ear, brain and eye) is a extremely rare disorder in which deafness, reduced vision, and encephalopathy may all present simultaneously. The encephalopathy manifests with headache, confusion, memory loss, behavioral changes, dysarthria and occasional mutism. The hearing loss is usually bilateral and accompanied by tinnitus and vertigo. According to Susac, it is generally a low-frequency sensorineural loss, resembling that of Meniere's disease. The reduced vision is caused by retinal artery occlusions. MRI images, which are invariably abnormal, reveal multifocal white matter lesions, including the corpus callosum (Susac et al, 2004). The lesions of the corpus callosum in Susacs are typically medial and centrally located (snow-balling) compared to lesions seen in MS that are more often seen on the undersurface of the corpus callosum at the septal interface (Susac, 2004). Treatment is with immunosuppresants (Clement et al, 2003).

Vogt-Koyanagi-Harada syndrome (VKH), is another autoimmune disorder combining uveitis, vitiligo (patches of depigmented skin), poliosis (white eyebrows), and sometimes inner ear symptoms. It is mainly recognized by the uveitis, and has some overlap with Cogan's syndrome (which also has eye inflammation), and Susac (which has eye findings as well). VKH has some overlap with sympathetic ophthalmia (another autoimmune eye disease, triggered by eye trauma). If one sees the symptom of poliosis accompanyng uveitis and inner ear symptoms, , one should think VKH.