Clinical Manifestations

The clinical manifestations of AIED are a consequence of injury to the delicate inner ear structures. As the inner ear is injured, patients sustain fluctuating or rapidly progressing sensorineural hearing loss, tinnitus, aural fullness, and vertigo attacks. Patients often complain of a decrease in hearing acuity or difficulty in understanding words. They may also have difficulty with balance.

The pattern of sensorineural hearing loss can vary, but the most common finding is a loss across all frequencies, or a flat pattern. Low frequency sensorineural hearing loss is also quite common. The hearing loss may be unilateral or bilateral. When the presentation is unilateral, symptoms often become bilateral over the course of days to weeks. Sometimes, however, there is a delay of many months to years before the contralateral ear becomes involved, and, in rare cases, the loss can be unilateral throughout the entire course of the illness.

Patients also note aural fullness, which may fluctuate along with the hearing loss. Tinnitus is a common complaint in patients with AIED. Depending on the frequencies involved in the hearing loss, patients will often complain of a constant tone or ringing at the frequencies lost. This is often described as an ocean roar, but is a nonspecific finding. The tinnitus is nonpulsatile. Relapses of the disease are often heralded by the return of loud roaring tinnitus, which precedes the hearing loss or vestibular symptoms and indicates the need for resumption of high-dose immunosuppressive therapy.

Acute episodes of vertigo are variably present in the disorder. The attacks usually last for several hours and are associated with severe nausea and vomiting. In between attacks, patients often complain of unsteadiness and have poor balance.

If the AIED is concurrent with a systemic autoimmune disorder (secondary AIED), patients will exhibit manifestations attributable to that disorder. Some of the more common systemic autoimmune disorders associated with AIED are listed in Table 2.

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