Is Hearing Loss Hereditary?

Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, it can also be acquired at birth or hereditary. Congenital hearing loss is a very common condition and affects about 3 in every 1000 babies. There are many genetic causes that can lead to hearing impairment both mild and severe. The problematic genes can be passed to the children from one or both parents, who may or may not have hearing impairment, and manifest actively or the child may only be a carrier and thus hearing loss may occur in the next generation. About 60% of deafness in newborns is due to inherited genetic issues.

Chromosomes are the parts of cells that carry hereditary information and genes that are defective may be passed on from generation to generation. Hearing loss whose origin comes from such defective genes, can be syndromic or non-syndromic. Depending on the specific type, genetic hearing loss can be classified in 4 groups of hearing impairment - autosomal dominant (usually one parent has hearing loss and passes the gene to the child), autosomal recessive (both parents have normal hearing but carry a recessive gene), X-linked and mitochondrial.

Syndromic hearing loss

Up to 30% of all hereditary hearing impairment is syndromic. This type of hearing loss occurs with additional genetic problems represented in many different medical conditions. There are about 400 known syndromes that include hearing difficulties as part of all genetic abnormalities that occur. One such condition is the Waardenburg syndrome, which represents with a sensorineural hearing loss as well as an abnormal pigmentation of the eyes, hair and skin. Other common syndromes with the participation of autosomal dominant hearing loss are the Branchiootorenal syndrome, the Stickler syndrome and Neurofibromatosis 2 .

In the group of autosomal recessive hearing impairment falls the Usher syndrome, which comprises hearing, visual and vestibular problems. Other common autosomal recessive syndromes are the Pendred syndrome, Jervell and Lange-Nielsen syndrome, Biotinidase deficiency and Refsum disease.

X-linked hearing loss usually affects males and the defective gene is carried by the mother. Syndromes that are connected to this type of abnormality are the Alport syndrome and the Mohr-Tranebjaerg syndrome.

Non-syndromic hearing loss

About 70% of inherited hearing impairment is non-syndromic and hearing loss is the only trait that characterizes it. The greatest portion, around 80%, is due to autosomal recessive inheritance. The two main non-syndromic conditions that cause hearing loss are otosclerosis and DPHL (dominant progressive hearing loss). Otosclerosis usually occurs when the person is between 20 and 50 years of age. It manifests with a growth in the middle ear leading to a progressive conductive hearing loss. The DPHL and prebycusis (hearing loss connected to aging) are the most widespread forms of sensorineural progressive hearing loss. While prebycusis is common for adults over 60 and is a cumulative result of lifestyle, exposure to noise and natural aging, the dominant progressive hearing loss usually occurs early in childhood and may cause balance problems as the organ affected in the condition is the inner ear, host to the vestibular apparatus.

Related Articles

Does Medicare Cover Hearing Aids? (Updated 2020)

01 December, 2020 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

What's the Difference between Behind The Ear and In The Ear Hearing Aids?

02 October, 2020 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Buying Hearing Aids: 20 Things You Should Know

24 September, 2020 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Protect Your Hearing During Yard Work

22 April, 2020 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

What you need to know about Itchy Ears

30 January, 2020 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

How to Pop your Ears Safely and other Ear Popping problems

12 November, 2019 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Black Friday Deals From Hearing Direct 2018

20 November, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Do Ear Wax Removal Candles Work?

13 November, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

5 Reasons You May Have Clogged Ear

09 November, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Devices for the Hard of Hearing in the Home and Workplace

05 November, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Hearing Aid Insurance

20 October, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Tips For Unblocking Your Ears

08 October, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Hearing Aid Manufacturers

05 October, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Human Hearing Range

07 September, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Ménière's Suitable Hearing Aids

17 August, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Is Wearing Ear Plugs At Night A Good Idea?

14 August, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...

Waterproof Hearing Aids: What You Need To Know

13 August, 2018 | Gary Hill
Hearing loss is mainly a result of aging, continuous noise exposure, medications, underlying conditions, or traumas. However, ...