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Heightened physician awareness of the sociologic and psychologic trauma related to deafness coupled with an increased emphasis on early diagnosis has prompted the employment of sophisticated diagnostic studies in an attempt at identification of an acoustic neuroma as a cause of hearing loss. Because profound bilateral hearing impairment is a catastrophic event, the management and care of an individual with an eighth nerve lesion in an only hearing ear remains a significant challenge for both patient and physician. In this article we will summarize our experience with this unique patient population through case presentations that will include diagnostic testing and intraoperative and perioperative findings. The literature regarding diagnosis and management will be reviewed. Recommendations for options available in the care of these patients will be discussed.