Cochlear Implant Outcomes in Patients With TMTC2 -Associated Sensorineural Hearing Loss and Auditory Neuropathy/Auditory Dys-Synchrony.
Otol Neurotol
; 45(6): e478-e482, 2024 Jul 01.
Article
em En
| MEDLINE
| ID: mdl-38769084
ABSTRACT
OBJECTIVE:
To investigate the outcomes of cochlear implantation in patients with TMTC2 -associated sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony. PATIENTS Adult and pediatric cochlear implant (CI) patients followed in an academic center who tested positive for TMTC2 genetic variant rs35725509. INTERVENTION Cochlear implantation. MAIN OUTCOMEMEASURES:
Speech perception scores in quiet.RESULTS:
Ten CI patients were identified with TMTC2 variant rs35725509 out of 157 patients who underwent genetic testing (i.e., 6.3% of patients tested). All demonstrated progressive, bilateral hearing loss with severe-to-profound audiometric thresholds preoperatively. Pre-CI and 1-year post-CI speech recognition percent correct scores were compared. Post-CI speech perception (mean 61.0%, standard deviation 31.4%) was significantly higher than pre-CI speech perception (mean 21.0%, standard deviation 27.0%) ( p = 0.002). Individually, 9 of the 10 subjects experienced significant improvements in speech perception pre- to post-CI ( p < 0.05). Electrically evoked compound action potential measures were available for five patients, and all showed normal electrically evoked compound action potential thresholds.CONCLUSION:
Patients with TMTC2 -associated sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony have significantly improved speech perception outcomes with cochlear implantation and should be considered candidates for this intervention if there are no other contraindications.
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Percepção da Fala
/
Implantes Cocleares
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Implante Coclear
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Perda Auditiva Central
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Perda Auditiva Neurossensorial
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Otol Neurotol
Assunto da revista:
NEUROLOGIA
/
OTORRINOLARINGOLOGIA
Ano de publicação:
2024
Tipo de documento:
Article