Many treatments have been advocated for sudden sensorineural hearing loss, which is presumed to be of viral (or rarely vascular) origin. The diagnosis of "autoimmune" inner-ear disease is still predicated by a documented response to corticosteroid administration (20). Treatment strategies have varied between reports, but, in general, a dose of prednisone, 40 to 60 mg in the adult for two weeks, is an appropriate therapeutic trial. Substantial improvement on this dosage regimen would then warrant a slow taper of steroids over several months with close observation for recurrence. Even prompt administration of these doses results in a significant improvement in only approximately 40% to 50% of cases. Antiviral agents, vasodilators, antiplatelet agents, and anticoagulants have no proven role in the management of this entity.
There are several circumstances that warrant special mention. Patients with Ménière's disease and certain forms of genetic hearing loss may manifest spontaneous fluctuations in hearing. In these situations, the administration of steroids at the onset of the hearing loss may result in a false impression that the subsequent spontaneous improvement in hearing was actually the result of steroid administration. Thus, in patients in whom there is question of whether the hearing is actually responding to steroids, it is often prudent to withhold the administration of prednisone for two weeks, to see if there is spontaneous resolution of the hearing loss. If the hearing loss does not resolve during that time frame, then administration of steroids would be warranted and a positive therapeutic response would support the diagnosis of "autoimmune" inner-ear disease.
Patients who show a definite therapeutic response to steroids, but relapse when weaned from these drugs, pose another therapeutic dilemma. To date, an efficacious prednisone-sparing regimen has not been developed. Numerous drugs including methotrexate, azothiaprine, and etanercept have been shown to be ineffective in maintaining patients' hearing. While cyclophosphamide, given as an oral regimen or in monthly intravenous boluses, has traditionally been used in this patient group, its efficacy has not been definitively established. In addition, it carries specific, undesirable complications. In patients who cannot be maintained in remission without chronic use of prednisone, a reasonable alternative is to discontinue treatment, allow the disease to take a natural course, and then utilize a cochlear implant. These patients typically perform extremely well with cochlear implants and can avoid the complications of prolonged use of potentially toxic medications.
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