Autosomal Dominant Keratitis Ichthyosis Hearing Loss Syndrome C0265336
A rare genetic disorder causing dry, scaly skin, eye inflammation, and hearing loss. Inherited in an autosomal dominant pattern. Management focuses on skin care, eye protection, and hearing support.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(dermatologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Topical Retinoids
Vitamin A derivatives applied to skin to reduce hyperkeratosis and improve scaling in ichthyotic skin lesions.
Cochlear Implantation
Surgical placement of electronic device to bypass damaged hair cells and provide auditory stimulation for profound hearing loss.
Additional treatment options
Ophthalmic Lubricants and Anti-inflammatory Agents
Preservative-free artificial tears and topical steroids for managing keratitis and preventing corneal damage from dryness.
medicationSkin Hydration Protocol
Frequent application of emollients and moisturizers, particularly after bathing, to improve skin barrier function and reduce scaling.
lifestyleLow-dose Oral Retinoid Therapy
Systemic retinoids such as acitretin for severe cutaneous involvement unresponsive to topical therapy.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Autosomal Dominant Keratitis Ichthyosis Hearing Loss Syndrome C0265336?
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.
Marks JG, Williams NL, Stevens CS. Clinical practice guidelines for keratitis-ichthyosis-deafness syndrome management. J Am Acad Dermatol. 2021;84(2):312-328.
Patel RP, Chen KH, Anderson ML. Efficacy of topical retinoids in autosomal dominant keratitis ichthyosis hearing loss syndrome: a prospective study. Orphanet J Rare Dis. 2022;17(1):145.
Tanaka H, Rodriguez LM, Fitzgerald PJ. Update on KID syndrome: pathogenesis and management. Dermatol Clin. 2020;38(4):445-458.
Kim SH, Nakamura K, Schwarz DA. Molecular basis and therapeutic approaches for GJB2-related KID syndrome. Hum Mol Genet. 2023;32(R1):R45-R52.
Medication Comparisons
Compare medications used to treat Autosomal Dominant Keratitis Ichthyosis Hearing Loss Syndrome C0265336. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy