Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Book

Syndromic Sensorineural Hearing Loss

Andrew E. Sutton  1 , Julie Goldman  2
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
Affiliations
Free Books & Documents
Book

Syndromic Sensorineural Hearing Loss

Andrew E. Sutton et al.
Free Books & Documents

Excerpt

Congenital hearing loss affects approximately 1 to 3 infants per 1,000 live births. About 70% of these children have nonsyndromic hearing loss, which occurs without any associated medical conditions. The remaining 30% experience syndromic hearing loss, which is linked to other medical anomalies. Among children with genetic causes of hearing loss, around 20% will show additional symptoms alongside their hearing impairment.

All children suspected of having hearing loss should undergo comprehensive evaluations that consider their gestational, perinatal, postnatal, and family histories. Clinical signs of syndromic sensorineural hearing loss (SNHL) can vary significantly and may include abnormalities found during ear and eye examinations, as well as assessments of the cardiac, integumentary, dental, chromosomal, neurological, skeletal, renal, endocrine, or craniofacial systems. Diagnostic tests and imaging should be customized for each case.

Treatment options for hearing loss may include medical interventions for middle ear diseases, amplification devices, surgical correction of external and middle ear deformities, or cochlear implants. This activity focuses on syndromic SNHL, which can arise from biochemical, metabolic, vascular, hematologic, or endocrine dysfunctions, as well as issues affecting the vestibulocochlear nerve.

PubMed Disclaimer

Conflict of interest statement

Disclosure: Andrew Sutton declares no relevant financial relationships with ineligible companies.

Disclosure: Julie Goldman declares no relevant financial relationships with ineligible companies.

References

    1. Plum A, Winterhager E, Pesch J, Lautermann J, Hallas G, Rosentreter B, Traub O, Herberhold C, Willecke K. Connexin31-deficiency in mice causes transient placental dysmorphogenesis but does not impair hearing and skin differentiation. Dev Biol. 2001 Mar 15;231(2):334-47. - PubMed
    1. Tekin M, Arnos KS, Pandya A. Advances in hereditary deafness. Lancet. 2001 Sep 29;358(9287):1082-90. - PubMed
    1. Rivolta MN, Holley MC. Cell lines in inner ear research. J Neurobiol. 2002 Nov 05;53(2):306-18. - PubMed
    1. Ahmed S, Sheraz S, Malik SA, Ahmed NR, Malik SA, Farooq S, Raheem A, Basheer F, Nayyar ZA, Fazal-e-Malik Frequency Of Congenital Hearing Loss In Neonates. J Ayub Med Coll Abbottabad. 2018 Apr-Jun;30(2):234-236. - PubMed
    1. Mehra S, Eavey RD, Keamy DG. The epidemiology of hearing impairment in the United States: newborns, children, and adolescents. Otolaryngol Head Neck Surg. 2009 Apr;140(4):461-72. - PubMed

Publication types

LinkOut - more resources