The prognosis for patients with AIED is quite variable. Part of this variability can be explained by the diverse nature of the disorder. Autoimmune injury in general can be from direct antibody injury toward specific epitopes, from immune complex deposits, or from cellmediated attacks by T cells. Depending on the cause of the autoimmune process and the speed with which it is treated, the damage to the cochlea and vestibule can be reversible or permanent. Cogan's syndrome, for example, carries a poor prognosis leading to bilateral profound hearing loss in greater than 60% of cases, even with aggressive treatment (see
Chapter 6 for further discussion of Cogan's syndrome). However, primary AIED rarely progresses to such an endpoint and can usually be stabilized or improved with prolonged high-dose corticosteroid treatment. This might come at the expense of one dead ear, however.
Complications of AIED include deafness as well as oscillopsia. Cochlear implants are an excellent choice for rehabilitation of patients with AIED who have progressed to unaidable profound bilateral hearing loss. As the autoimmune process damages the inner ear, cochlear nerve function remains intact, allowing for excellent results upon cochlear implantation. There have been several patients with AIED in whom implants have been performed whose function deteriorates, but with the use of immunosuppressives, it has shown improvement. Whether this is due to continuing damage to the neuronal population is speculative but highly probable. Unfortunately, oscillopsia resulting from bilateral vestibular loss remains a much more difficult problem for rehabilitation. Currently, there are no vestibular implants clinically available. The recommended treatment is aggressive physical therapy to reinforce the visual and proprioceptive senses to accommodate for the loss of vestibular input. Fortunately, this remains a rare endpoint for most patients with AIED.
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