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Syndromic hearing loss means that hearing impairment is associated with other conditions. Up to 30% of hereditary hearing impairments are syndromic.1 There are currently over 400 known syndromes that include hearing loss and affect various other systems of the body, including the kidneys, the eyes, and the heart. 

Syndromic hearing loss is inherited in one of the following patterns: 

  1. Autosomal recessive   
  2. Autosomal dominant  
  3. X-linked  

Autosomal recessive inheritance patterns in which the mutated gene is inherited from each parent and the child expresses the syndrome.    

Autosomal dominant inheritance patterns in which the mutated gene is inherited from only one parent and the child expresses the syndrome. 

X-linked inheritance patterns in which the mutated gene on the X chromosome is passed from the mother to a son and the son has the syndrome. 

The characteristics of syndromic hearing loss can vary from person to person, even within the same family. Hearing loss can affect one ear (unilateral) or both (bilateral), range from mild to profound, and involve the outer, middle, and / or inner ear, depending on the severity of the syndrome and any other health problems. The hearing loss may also be stable or progressive, meaning that it may change over time. 

Some of the more commonly known syndromes that present with hearing loss include: 

  • Alport 
  • Branchio-Oto-Renal (BOR) 
  • Charcot Marie Tooth 
  • Pendred 
  • Goldenhar  
  • Jervell and Lange-Neilsen 
  • Stickler 
  • Usher 
  • Turner 
  • Waardenburg 
  • Treacher Collins

Role of an Audiologist

Audiologists identify, diagnose, and provide treatment options for patients with hearing loss and dizziness. They work closely with physicians and speech-language pathologists and are an important part of the management team. 

 If you or someone in your family has been diagnosed with a hearing loss and you have concerns regarding a syndromic hearing loss talk to your doctor or Audiologist. Find an Audiologist near you to set up an appointment.

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References

1 Communications. “Genes and Hearing Loss.” American Academy of Otolaryngology-Head and Neck Surgery, 3 Jan. 2018, www.entnet.org/content/genes-and-hearing-loss.

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