The American Academy of Otolaryngology-Head and Neck Surgery considers genetic testing to be indicated and medically necessary for the evaluation and management of unilateral and bilateral sensorineural and mixed hearing loss in children and select adults. Comprehensive hearing-loss gene panel testing is now widely available and provides the highest diagnostic yield of any etiologic testing method in evaluation of the cause of hearing loss in these groups. A genetic diagnosis of hearing loss affects clinical management and improves patient care by providing prognostic information for possible progression of hearing loss, identification of syndromic associations and their associated comorbid conditions that require additional specialty evaluation, allowing enrollment in clinical trials, and possibly improving the likelihood of hearing loss treatment. Clinicians may order and manage genetic testing for hearing loss and consider referral to a medical geneticist or genetic counselor as needed.
Published: May 2026
References:
- Shearer, A. Eliot, Michael S. Hildebrand, Amanda M. Odell, and Richard JH Smith. “Genetic hearing loss overview.” GeneReviews®[Internet](2025).
- Shearer, A. Eliot. “Genetic testing for pediatric sensorineural hearing loss in the era of gene therapy.” Current Opinion in Otolaryngology & Head and Neck Surgery5 (2024): 352-356.
- Brodie, Kara D., et al. “Impact of genetic testing on hearing interventions.” The Laryngoscope8 (2023): 1982-1986.
- Li, Marilyn M., et al. “Clinical evaluation and etiologic diagnosis of hearing loss: A clinical practice resource of the American College of Medical Genetics and Genomics (ACMG).” Genetics in Medicine7 (2022): 1392-1406.
- Yamamoto, Nobuko, et al. “Comprehensive gene panel testing for hearing loss in children: Understanding factors influencing diagnostic yield.” The Journal of Pediatrics262 (2023): 113620.
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