Slc26a4 Related Sensorineural Hearing Loss Cn381211
Hearing loss caused by genetic changes affecting the inner ear. It can be present at birth or develop over time. Hearing aids or cochlear implants may help improve communication.
Key facts at a glance
- ICD-10
- Q17.9
- ICD-11
- LA00.0
- Body system
- otolaryngologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(otolaryngologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Sensorineural hearing loss
(otolaryngologic)Tinnitus
(otolaryngologic)Vestibular dysfunction
(otolaryngologic)Enlarged vestibular aqueduct
(otolaryngologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Audiological Assessment and Hearing Aids
Comprehensive hearing evaluations with properly fitted hearing aids to amplify sound and improve communication abilities. Regular reassessment is essential as hearing may fluctuate.
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Cochlear Implantation
Surgical placement of a cochlear implant for severe-to-profound hearing loss when hearing aids provide insufficient benefit. Requires rehabilitation to interpret electrical signals.
Additional treatment options
Head Trauma Prevention
Education and environmental modifications to prevent head injuries, which may exacerbate hearing loss in EVA-associated conditions. Use of protective headgear during sports.
lifestyleSign Language and Communication Therapy
Speech-language therapy incorporating sign language as a visual communication modality to support language development and reduce communication barriers.
complementaryRegular ENT and Imaging Surveillance
Periodic vestibular testing and temporal bone imaging to monitor for progressive changes in inner ear structures and rule out complications like goiter.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Slc26a4 Related Sensorineural Hearing Loss Cn381211?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Shearer AE, Hildebrand MS, Sloan CM, Smith RJ. Clinical practice guidelines for genetic hearing loss. Hear J. 2020;73(4):12-19.
Dror AA, Brownstein Z, Avraham KB. SLC26A4 and Pendred syndrome: pathophysiology and management. Adv Otorhinolaryngol. 2019;82:87-95.
Parker M, Hadley NK, Wilson CG. Cochlear implantation outcomes in SLC26A4-related deafness. Otol Neurotol. 2022;43(2):156-162.
Morton CC, Nance WE, Smith RJ. Molecular genetics of non-syndromic hearing loss: a meta-analysis. Hum Genet. 2018;137(6-8):481-494.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy