A total of twelve patients with a relatively uncommon form of progressive sensorineural deafness (autoimmune inner-ear disease) were treated orally with 1 mg/kg azathioprine, once daily, and with 30 mg prednisolone, every other day, for 4 weeks. Statistically significant increases in the ability to hear pure tones or in discrimination on audiometry took place in 10/12 patients. This condition was initially described as 'sensorineural hearing loss', but it is now clear that the term 'autoimmune inner-ear disease' is more appropriate since the vestibular compartment as well as the cochlear compartment is involved. This relatively uncommon disease is one of the few forms of sensorineural deafness that can be successfully treated.

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