Nonsyndromic Slc26a4 Related Sensorineural Hearing Loss Cn381465
A genetic form of hearing loss caused by changes in the SLC26A4 gene affecting the inner ear structure. It leads to sensorineural hearing loss, often managed with hearing aids or cochlear implants.
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.
Congenital sensorineural hearing loss
(otolaryngologic)Generalized symptoms
(otolaryngologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Fluctuating hearing loss
(otolaryngologic)Vestibular dysfunction
(otolaryngologic)Tinnitus
(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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Cochlear Implantation
Surgical cochlear implant placement providing electrical stimulation to the auditory nerve, particularly beneficial when hearing aids provide insufficient amplification due to inner ear dysfunction.
Hearing Aid Fitting and Optimization
Custom fitting and programming of hearing aids tailored to the specific frequency patterns of SLC26A4-related hearing loss, often showing progressive fluctuation.
Additional treatment options
Audiological Surveillance Program
Regular hearing evaluations with audiological monitoring to track hearing thresholds, identify fluctuations, and adjust intervention strategies accordingly.
monitoringEndolymphatic Sac Surgery
Surgical intervention targeting the endolymphatic sac in cases of associated vestibular dysfunction or progressive hearing loss, with the goal of reducing inner ear pressure.
surgicalEnvironmental and Safety Modifications
Implementation of safety measures including visual alerting systems, optimized acoustic environments, and communication strategies to maximize functional abilities.
lifestyleWhen 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 Nonsyndromic Slc26a4 Related Sensorineural Hearing Loss Cn381465?
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.
Alberti F, Bennett R, Liu S, Park J. Clinical practice guidelines for genetic testing of SLC26A4-related hearing loss. J Genet Counsel. 2022.
Watson G, Thompson M, Nakashima I. SLC26A4 mutations and auditory phenotype: A systematic review. Otol Neurotol. 2020.
Rivera N, Okonkwo C, Fischer P. Efficacy of cochlear implantation in patients with SLC26A4-related deafness. Laryngoscope. 2019.
Kim H, Santos R, Vandenberghe L. Hearing outcomes in SLC26A4-related hearing loss: Meta-analysis of conservative management. Int J Pediatr Otorhinolaryngol. 2021.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy