Abstract: Objectives: To assess the prevalence and clinical characterization of variants in the TECTA gene among individuals with bilateral sensorineural hearing loss of unknown etiology in northern Spain. Methods: A 6-year (2018-2024) observational, prospective, and descriptive study was conducted on patients with bilateral sensorineural hearing loss at a tertiary hospital. Next generation sequencing using a gene panel for sensorineural hearing loss was performed to detect pathogenic, likely pathogenic, or variants of unknown significance in the TECTA gene. Results: Among 326 patients, pathogenic or likely pathogenic TECTA variants were found in 7 patients (2.14%), including c.3107G>A (n = 6) and c.5383+6T>A (n = 1). Variants of unknown significance were found in 8 patients (2.45%). About 14 of 15 probands had a family history of hearing loss with autosomal dominant inheritance. Eight relatives with confirmed pathogenic variants were also included, totalling 23 cases. Six patients with pathogenic variants and 3 with variants of unknown significance had moderate mid-frequency hearing loss, while others had severe high-frequency loss. Hearing loss was typically progressive, ranging from congenital onset to the fifth decade. Most were treated with hearing aids; none required cochlear implants. Conclusions: TECTA gene variants are relatively common in this population, with c.3107G>A being the most frequent. The typical phenotype is slowly progressive, mid-to-high frequency sensorineural hearing loss, often starting in childhood and usually requiring hearing aids fitting with good results in improving speech intelligibility.

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