Clinical Pearls & Morning Reports
Published February 9, 2022
Conditions associated with both inner ear and ocular involvement and with systemic symptoms fall under three main categories: systemic vasculitides and rheumatologic diseases, autoinflammatory conditions, and infections. Read the NEJM Case Records of the Massachusetts General Hospital here.
Q: Can Lyme disease cause both hearing loss and ocular symptoms?
A: Lyme disease, which is caused by Borrelia burgdorferi, is a great masquerader. It is endemic in the Northeast region of the United States, and can cause constitutional symptoms such as myalgias, weight loss, and fatigue. It can also cause sensorineural hearing loss and vertigo, as well as uveitis and episcleritis.
Q: Is tuberculosis also among the conditions that can result in both hearing loss and eye symptoms?
A: Tuberculosis can be associated with numerous eye symptoms, ranging from lid to retinal symptoms. It can also involve the middle ear and mastoid sinus by passing through the eustachian tube. Tuberculosis can result in clinically significant destruction, including multiple tympanic membrane perforations and middle-ear inflammation, which can lead to hearing loss and dizziness.
A: Neurosyphilis can cause progressive bilateral sensorineural hearing loss and vertigo; histopathological studies have shown involvement of the temporal bone and its marrow spaces, the incus, and the region of the endolymphatic sac, which can lead to endolymphatic hydrops. Neurosyphilis is also a well-known cause of episcleritis, keratitis, and uveitis. Patients with relapsing polychondritis can have both ocular inflammation and inner ear dysfunction. Patients with Behçet’s syndrome can present with anterior and posterior uveitis, hearing loss, and tinnitus. Susac’s syndrome causes both ocular inflammation and sensorineural hearing loss. Paraneoplastic syndromes, which are due to an altered immune response to an underlying cancer, can also cause inner ear and eye deficits. These syndromes occur in 8% of patients with cancer and are most frequently associated with lung, breast, hematologic, medullary thyroid, gynecologic, and prostate cancers.
A: Cogan’s syndrome causes hearing loss, dizziness, and bilateral eye findings that were originally referred to as “nonsyphilitic keratitis.” The eye findings include bilateral ocular inflammation, photophobia, and eye pain and may involve interstitial keratitis, episcleritis, scleritis, and uveitis. The onset of hearing loss, tinnitus, and vertigo is typically sudden and occurs within 3 to 4 months after the eye findings develop. Temporal bone studies have shown osteoneogenesis of the cochlea. Constitutional symptoms such as fever, fatigue, weight loss, myalgias, and arthralgias occur within 2 months after the onset of the illness. The erythrocyte sedimentation rate and C-reactive protein level are elevated. Cogan’s syndrome may have cardiovascular manifestations, such as aortitis. The incidence is increased in persons with HLA-B17, HLA-A9, HLABw35, and HLA-Cw4. Anti-HSP70 antibodies have been reported to be present in 92.5% of patients with Cogan’s syndrome and have also been found in patients with autoimmune inner ear disease and rapidly progressive sensorineural hearing loss.