Current clinical and pathological features of round window diseases.

The round window niche and membrane can be involved in clinical problems including perilymphatic fistulas, sensorineural hearing loss in otitis media, and a variety of others. The background of these problems is documented by a review of the literature and recent experimental evidence on round window grafting and tracer studies. Clinical pathological correlates including recent clinical experience are discussed for perilymphatic fistulas (sudden deafness, chronic progressive fluctuating sensorineural hearing loss, and association with Meniere's disease), sensorineural hearing loss in acute and chronic otitis media, and findings in round window membrane including absent round window reflex in cases of exploratory tympanotomy and otosclerosis. In addition, a new syndrome termed perilymphatic hypertension is described as seen in a subset of patients with sudden sensorineural hearing loss.