3.10 Otologic Manifestations of Systemic Diseases
Key Features
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Many systemic diseases may affect the vestibulocochlear system.
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Otologic manifestations can be the presenting signs of systemic disease.
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A high index of suspicion is required when evaluating otologic complaints.
A diverse group of systemic diseases affect the ear and produce otologic complaints. These conditions can be broadly characterized as infectious/granulomatous, autoimmune, neoplastic, metabolic, disorders of bone, and immunodeficiencies. Otologic manifestations can be part of the disease progression or the initial findings that can herald a diagnosis. A high index of suspicion is required, therefore, when investigating a patient′s otologic complaints.
Infectious/Granulomatous Processes
Tuberculosis
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Mycobacterium tuberculosis via blood vessels, lymphatic vessels, or direct through the auditory (eustachian) tube
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Chronic otorrhea, tympanic membrane (TM) perforations, granulation in middle ear, bone sequestra
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Diagnosis: acid-fast stain of exudates, culture (negative in 70%), polymerase chain reaction
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Treatment: standard antituberculosis therapies, surgery for refractory cases
Syphilis
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Spirochetal infection, Treponema pallidum, congenital and acquired forms
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Ossicular/temporal bone osteitis in latent syphilis
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Hearing loss abrupt, bilateral, and progressive in secondary/tertiary syphilis
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Granulomatous lesions (gummas) affect middle ear and cause TM perforation
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Treatment: benzathine penicillin 2.4 million units every week for 6 to 12 weeks, prednisone 60 mg every other day for 3 to 6 months, tapered slowly
Lyme Disease
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Spirochetal infection, Borrelia burgdorferi, transmitted by ticks
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Facial paralysis (especially bilateral), tinnitus, sensorineural hearing loss (SNHL), otalgia, vertigo
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Treatment: doxycycline 100 mg orally every day for 14 to 21 days
Mumps
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Paramyxovirus, an RNA virus
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Unilateral, high-frequency hearing loss, tinnitus. Hearing loss is usually permanent, vestibular symptoms resolve over weeks.
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Treatment: nonspecific; steroids may be of some benefit.
Measles (Rubeola)
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Paramyxovirus, an RNA virus
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Bilateral, high-frequency SNHL occurs acutely with onset of rash.
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50% of patients have improvement in hearing loss.
Sarcoidosis
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Multisystem disorder characterized by noncaseating granulomas
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SNHL, vestibular dysfunction, facial nerve paralysis
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Uveoparotid fever (Heerfordt′s syndrome): facial paralysis, parotitis, uveitis, pyrexia
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Treatment: nonspecific, corticosteroids, cytotoxic agents
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