Cochlear Implantation for Sensorineural Hearing Loss Related to Cochlear Aqueduct Obstruction by a Jugular Foramen...

Cochlear implantation (CI) may be used as a viable method for restoring hearing in patients with sensorineural hearing loss (SNHL) caused by jugular foramen tumors.

42-year-old female presenting with sudden-onset SNHL, pulsatile tinnitus, and otalgia, with a PTA of 119 dB and AzBio sentence score of 0% in quiet. Brain MRI identified a 22 mm tumor within the left jugular foramen and hypoglossal canal involving the cochlear aqueduct. Patient underwent stereotactic radiosurgery (SRS), with the tumor receiving 15 Gy in a single fraction, followed by a left CI with full electrode insertion without resistance. Main outcome measures included audiometric outcomes pre- and post-CI, including PTA and AzBio sentence scores in quiet, and MRI imaging of the tumor pre- and post-SRS.

Upon activation of CI, PTA had improved to 42 dB with AzBio sentence score of 50% in quiet within 1 month after activation. One-year follow-up revealed PTA of 38 dB and AzBio sentence score of 29% in quiet. Datalogging at 1 month after CI activation revealed 11.4 h per day of CI use at 1 month and 5.5 h per day at 1 year after CI activation. Patient reported that aural rehabilitation exercises were stopped during this time period. Post-SRS imaging revealed minimal growth of the jugular foramen tumor.

This report demonstrates that CI for asymmetric sensorineural hearing loss or single-sided deafness related to cochlear aqueduct obstruction from jugular foramen pathology yields outcomes similar to conventional CI recipients. Imaging surveillance of the tumor remains feasible for tumors treated with SRS. This case report provides valuable insight and offers guidance for surgeons and patients facing a similar clinical scenario.

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