Dual or multiple pathologic entities simultaneously affecting the same anatomical region presents a blinding challenge to the clinician for an early and precise diagnosis. The co-existence of acoustic neuroma and otosclerosis in seven patients is analyzed with detailed histories and clinical commentary. The diagnosis of otosclerosis preceeded that of neuroma in all cases. Any unilateral shift in hearing, whether sudden or progressive, is unusual and should provoke suspicion of active retrofenestral (labyrinthine) otosclerosis, perilymph fistula in post stapedectomy cases, labyrinthine ischemia, or neuroma. The literature on these subjects has, therefore, been reviewed and critically discussed in relation to each of the case reports. The association of nasopharyngeal irradiation with acoustic neuroma in Case 4 prompted a search of the literature and summaries of the three previously reported cases of cervical neurofibroma associated with regional irradiation are included. The eighth case is a fascinating corollary to the theme — a woman with von Recklinghausen's disease who has stapedial otosclerosis. Her life has been threatened with malignant degeneration in a peripheral neuroma. Malignant degeneration of neuromas in von Recklinghausen's disease has been reviewed but no report of malignant degeneration in an acoustic neuroma could be found.
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