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HomeOtolaryngology and Facial Plastic SurgerySyndromic Sensorineural Hearing Loss

Syndromic Sensorineural Hearing Loss

Practice Essentials

Sensorineural hearing loss (SNHL) has many different presentations, ranging in severity from mild to profound, including low- and high-pitch patterns. Genetic hearing loss may appear as an isolated finding or as part of a syndrome. About 70% of genetic hearing loss is nonsyndromic, and about 30% is syndromic.

Signs and symptoms

The following histories should be obtained:





Clinical findings suggestive of syndromes associated with hearing loss include the following:

Ear examination findings

Eye examination findings

Integumentary examination findings

Cardiac findings

Renal findings

Dental findings

Endocrine/metabolic findings

Chromosomal abnormalities

Neurologic abnormalities

Skeletal examination findings

Craniofacial abnormalities


A routine series of laboratory tests is not recommended. Studies, selected on an individualized basis, may include the following:

Genetic testing

Complete blood count count with differential

Serum chemistries

Blood sugar

Blood urea nitrogen and creatinine

Thyroid function studies


Fluorescent treponemal antibody absorption (FTA-ABS)

Specific immunoglobulin M (IgM) assays

Autoimmune panel

Imaging studies to be considered include the following:

Computed tomography

Magnetic resonance imaging

Renal ultrasonography

Other tests that may be helpful include the following:

Techniques for identifying and assessing hearing impairment (eg, audiometry and tympanometry)




Medical care may include the following:

Treatment of any middle ear disease


Assistive listening devices and personal systems

Surgical care may include the following:

Surgical management of external and middle ear deformities

Cochlear implantation

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