Unilateral progressive sensorineural hearing loss, tinnitus, and unsteadiness are the usual initial symptoms of acoustic neuroma. Of the last 100 consecutive cases of acoustic neuroma detected at our Centre, 14 had atypical symptoms, Five patients manifested sudden hearing loss; one of these had complete recovery. Three patients reported long-standing unilateral hearing loss, ranging from 10 to 20 years. Six patients had normal hearing, one of whom was diagnosed incidently when the investigations were performed for contralateral glomus tumor. A second patient, a young woman, experienced weakness of lower limbs. The remaining four patients had only subjective symptoms of hearing loss or tinnitus. Acoustic tumors could have been overlooked easily in these patients. It is important to have a high index of suspicion in all cases of sudden hearing loss, asymmetric sensorineural loss of any duration, subjective sensation of hearing loss, and tinnitus. It is mandatory to investigate these cases with auditory brainstem responses, any abnormality of which makes it necessary to perform magnetic resonance imaging with gadolinium.
Labyrinthine destruction by direct cholesteatoma invasion has always been considered a serious threat to the inner ear function. A number of reports in the literature have cited both patients who had preservation of hearing despite widespread erosion of the labyrinth by cholesteatoma and patients who had retained auditory function despite surgical removal of the matrix from the labyrinth. In most cases the vestibular portion of the inner ear was invaded but cases of cochlear involvement have been described as well. Twelve cases with pre-operative auditory function preservation despite extensive labyrinthine destruction treated at our Institution are reported. Seven cases retained cochlear function post-operatively. Possible explanations of this occurrence and implications of related with hearing preservation in the presence of widespread inner ear destruction by chronic inflammatory disease are discussed.
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