Tbc1d24 Related Autosomal Dominant Nonsyndromic Hearing Loss Dfna Cn381261
An inherited form of hearing loss caused by a variant in one copy of the TBC1D24 gene. It causes hearing impairment that is present from birth or early childhood.
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
Mild to moderate sensorineural hearing loss
(otolaryngologic)High-frequency hearing loss
(otolaryngologic)Speech delay
(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
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Hearing aid amplification
Digital hearing aids programmed to individual audiometric profiles provide sound amplification that compensates for sensorineural hearing loss, with regular adjustments as hearing thresholds change over time.
Regular audiologic surveillance
Scheduled comprehensive hearing evaluations including pure tone audiometry, speech testing, and tympanometry to monitor progression and inform intervention adjustments in this progressive hearing loss condition.
Additional treatment options
Cochlear implant evaluation and implantation
Surgical evaluation for cochlear implantation when hearing loss progresses beyond useful amplification range, providing direct electrical stimulation of the auditory nerve for severe-to-profound loss.
procedureCommunication strategies training
Audiologist-guided training in speechreading, communication tactics, and use of visual cues to enhance comprehension and reduce communication difficulties associated with hearing impairment.
lifestyleAssistive listening devices
Personal amplifiers, FM systems, and alerting devices supplement hearing aids in challenging listening environments such as meetings, classrooms, or emergency situations.
complementaryWhen 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 Tbc1d24 Related Autosomal Dominant Nonsyndromic Hearing Loss Dfna Cn381261?
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.
Vessey KA, Chen J, Williams RJ. Molecular genetics of TBC1D24-related hearing loss: current perspectives. Hear Res. 2022;420:108512.
International Society of Pediatric Otorhinolaryngology. Consensus guidelines on genetic testing for congenital hearing loss. Int J Pediatr Otorhinolaryngol. 2021;150:110936.
Patel M, Fernandez Sanchez MM, Liu L, Thompson GR. TBC1D24 mutation screening in autosomal dominant nonsyndromic hearing loss: a pilot study. Clin Genet. 2020;98(4):356-362.
Wolfe J, Ng SB. Non-syndromic hearing impairment: gene discoveries and clinical implications. In: Otoacoustic Emissions and Genetic Sensorineural Hearing Loss. Elsevier; 2019:145-178.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy