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Updated: Aug 4, 2025
Cochlear Implant Surgery and Electrically-evoked Auditory Brainstem Response Recordings in C57BL/6 Mice
Published on: January 9, 2019
Cochlear Implants in Deaf Patients with Novel TMPRSS3 Gene Mutation
Objective:
To investigate the effect of cochlear implants in deaf patients with TMPRSS3 gene pathogenic variations.
Methods:
Variations of deafness genes were detected in 2 patients with profound hearing loss. Both received unilateral cochlear implantation. Hearing and speech abilities were evaluated and analyzed before and 3 and 6 months after surgery. The analysis included post-surgery evaluation of auditory behavior (Categories of Performance [CAP]) and Speech Intelligibility Rating (SIR).
Results:
In the 2 patients, 3 pathogenic single nucleotide variations (SNVs) of TMPRSS3 gene and a large deletion in 21q22.3 were detected. The CAP and SIR grades increased with the recovery time.
Conclusion:
Cochlear implants have a good effect in patients with TMPRSS3 gene mutation deafness. Preoperative gene testing has a certain reference significance for the prognosis in patients with the deafness gene mutation.
