Spontaneous musical auditory perceptions in patients who develop abrupt bilateral sensorineural hearing loss. An uninhibition syndrome?

Conclusions: Spontaneous musical auditory perceptions commonly occur in patients who develop abrupt bilateral sensorineural hearing loss. The findings in both subjects who were image tested while having these perceptions are suggestive of a biological substrate for this process and of a central locus for auditory memory seemingly located in and around area 39 of Brodmann. When an individual has abrupt bilateral sensorineural hearing loss, stored musical memory can be released and this person can have musical perceptions without an external source. It is likely that an abrupt bilateral loss of inner ear function might uninhibit neuronal groups storing auditory memory.

Objectives: The objectives of this study were as follows. (1) To determine if spontaneous musical auditory perceptions occur in patients who develop abrupt bilateral severe sensorineural hearing loss (not necessarily simultaneously). (2) To determine if there is a biological substrate to the process of recalling previous auditory perceptions. (3) To compare these findings with our normal databases of unstimulated and pure tone-stimulated volunteers. (4) To establish a hypothesis for the mechanisms of these occurrences.

Patients And Methods: Thirty two patients who had had abrupt bilateral severe sensorineural hearing loss (the interval between the losses of both ears could have been years) were contacted. They were asked if they had ever had the sensation of having musical auditory perceptions without external auditory stimuli. Two of these patients were image tested with single photon computerized emission tomography (neuroSPECT) while they were having these perceptions.

Results: All of the 32 subjects had musical auditory perceptions following their hearing loss in the second ear or when hearing loss in both ears occurred simultaneously. The two patients who were image tested with neuroSPECT had similar findings. There was a statistically significant increase in perfusion in area 39 of Brodmann, more intense on the right side, with increased perfusion also in both frontal lobes at the middle gyrus, with bilateral hypoperfusion in area 38 of Brodmann. These findings are similar to those observed in normal volunteers stimulated with pure tones.