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Hearing loss-familial salivary gland insensitivity to aldosterone syndrome is characterized by bilateral moderate-to-severe sensorineural hearing loss and salivary gland insensitivity to aldosterone resulting in hyponatremia. It has been described in two brothers. Transmission appeared to be autosomal recessive.
Features include very common findings: Inner ear hearing loss (sensorineural hearing impairment), Hyponatremia, and Abnormal salivary gland morphology.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Ears | 1 | Inner ear hearing loss (sensorineural hearing impairment) |
Phenotype severity distribution: 3 very common features.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for hearing loss-familial salivary gland insensitivity to aldosterone syndrome.
100 publications have been identified in PubMed for hearing loss-familial salivary gland insensitivity to aldosterone syndrome. Kisho has analyzed 58 by research type. Research spans Review / Meta-Analysis (76%), Basic Science / Preclinical (16%), and Epidemiology / Natural History (5%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 44 |
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 9:37 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Laboratory research | 9 | 16% |
Disease patterns and progression | 3 | 5% |
Patient case studies | 2 | 3% |
Papazachariou A (2026). [PMID: 41128447](https://pubmed.ncbi.nlm.nih.gov/41128447/). *Curr Opin Clin Nutr Metab Care*. [Review / Meta-Analysis]
Dotan A (2025). [PMID: 39931017](https://pubmed.ncbi.nlm.nih.gov/39931017/). *Harefuah*. [Review / Meta-Analysis]
Shabshin G (2025). [PMID: 40261331](https://pubmed.ncbi.nlm.nih.gov/40261331/). *Orthopadie (Heidelb)*. [Review / Meta-Analysis]
Yacoub MR (2025). [PMID: 40747632](https://pubmed.ncbi.nlm.nih.gov/40747632/). *Curr Opin Allergy Clin Immunol*. [Review / Meta-Analysis]
Borojeni S (2025). [PMID: 40546148](https://pubmed.ncbi.nlm.nih.gov/40546148/). *Rev Prat*. [Review / Meta-Analysis]
McCarthy FH (2025). [PMID: 40268352](https://pubmed.ncbi.nlm.nih.gov/40268352/). *Cardiol Clin*. [Review / Meta-Analysis]
Robinson KR (2025). [PMID: 41056948](https://pubmed.ncbi.nlm.nih.gov/41056948/). *Am J Hum Genet*. [Basic Science / Preclinical]
Mutai H (2025). [PMID: 39755840](https://pubmed.ncbi.nlm.nih.gov/39755840/). *Hum Genet*. [Basic Science / Preclinical]
Kaul A (2025). [PMID: 40915300](https://pubmed.ncbi.nlm.nih.gov/40915300/). *Lancet Rheumatol*. [Review / Meta-Analysis]
Brokke KE (2025). [PMID: 40634186](https://pubmed.ncbi.nlm.nih.gov/40634186/). *Br J Anaesth*. [Review / Meta-Analysis]
AI-curated news mentioning hearing loss-familial salivary gland insensitivity to aldosterone syndrome
Updated Feb 6, 2026
The proposed bill S 3400 aims to mandate coverage for hearing devices and systems in specific private health insurance plans. It has been read twice and is currently under review by the Committee on Health, Education, Labor, and Pensions.