Deafness

Common Name(s)

Deafness, Hearing loss, Hearing impairment

Hearing loss is considered to be anything from slight to profound impairment in the ability of a person to detect frequencies and intensities of sound. It can be acquired during life (due to injury, infection, illness, exposure to very loud noises or aging) or present at birth (congenital). It can be inherited. Hearing loss can be isolated (no other symptoms) or part of a syndrome. It can be progressive (gets worse over time) or stable. Hearing loss can occur in one or both ears, and affect different areas of the ears. There are three main types of hearing loss: conductive hearing loss, sensorineural hearing loss, and mixed hearing loss.

Deafness is the most profound form of hearing loss, where an individual can hear very little. In children, deafness is defined in children whose hearing loss is greater than 90 dB, but deafness can also occur as one ages or as part of a genetic condition such as Usher’s syndrome, illness such as Meniere’s disease, or any of the other acquired causes.

Symptoms of hearing loss or deafness include delayed development of speech or unclear speech, playing music loudly or having the volume on the television turned up, and consistent need to ask for others to repeat what they said during conversation. Diagnosis depends on the age of the individual but may include a range of tests to determine the cause. Treatment depends on the cause of the hearing loss and the type of hearing loss (conductive versus sensorineural versus mixed). If you or a family member has been diagnosed with hearing loss or deafness, talk to your doctor and audiologist about the most current treatment options. Support groups are a great source of information and will help connect you with others affected by hearing loss. If the hearing loss is part of a syndrome or is inherited, talking with a genetic counselor will help you understand more about the way it may run in your family.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Deafness" for support, advocacy or research.

Usher Syndrome Coalition

The Usher Syndrome Coalition's mission is to raise awareness and accelerate research for the most common cause of combined deafness and blindness. The Coalition also provides information and support to individuals and families affected by Usher syndrome.

Last Updated: 31 Oct 2014

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BEGINNINGS For Parents of Children Who Are Deaf or Hard of Hearing, Inc.

BEGINNINGS provides emotional support and access to unbiased information to families with deaf or hard of hearing children, age birth through 21. BEGINNINGS provides an impartial approach to meeting the diverse needs of these families and the professionals who serve them. These services are also available to deaf parents who have hearing children. The mission of BEGINNINGS is to inform and empower parents as they make decisions about their child. In addition, BEGINNINGS is also committed to providing technical assistance to professionals who work with these families.

Last Updated: 27 Jul 2015

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Hands & Voices

Hands & Voices is dedicated to supporting families with children who are Deaf or Hard of Hearing without a bias around communication modes or methodology. We're a parent-driven, non-profit organization providing families with the resources, networks, and information they need to improve communication access and educational outcomes for their children. Our outreach activities, parent/professional collaboration, and advocacy efforts are focused on enabling Deaf and Hard-of-Hearing children to reach their highest potential.

Last Updated: 26 Mar 2013

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Hearing Loss Association of America

The mission of HLAA is to open the world of communication to people with hearing loss by providing information, education, support and advocacy.

Last Updated: 27 Jul 2015

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Oklahoma Family Network

To Provide emotional, informational and navigational support to those who have special health care needs or a disability, their families and the professionals who serve them. OFN promotes family/professional partnerships and family-centered care, assuring services are offered as recipients prefer..

Last Updated: 15 Apr 2013

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Zellweger Baby Support Network

This organization was started by a small group of parents whose lives have been affected in some way by a rare disorder. Our gift, in honor of our children, was to start this site, so we can help families and to raise awareness of these rare disorders. If your child has been diagnosed with Zellweger syndrome, a peroxisomal disorder or another rare disorder, we are here to help you. You are not alone! Our mission is to promote, advance, and improve awareness of Zellweger syndrome and other peroxisomal disorders, to assist, support, and aid, financially or otherwise, individuals and families affected by Zellweger syndrome. We will help you learn more about these disorders. We will provide emotional support, and we can help you to create a journal site for your child so that family and friends can be kept up to date on your child. We will pray with you and for you as you journey through life with your special angel. We know this is a hard time for you; you want answers, informa

Last Updated: 20 Feb 2013

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General Support Organizations

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How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Deafness" for support, advocacy or research.

Usher Syndrome Coalition

The Usher Syndrome Coalition's mission is to raise awareness and accelerate research for the most common cause of combined deafness and blindness. The Coalition also provides information and support to individuals and families affected by Usher syndrome.

http://www.usher-syndrome.org

Last Updated: 31 Oct 2014

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BEGINNINGS For Parents of Children Who Are Deaf or Hard of Hearing, Inc.

BEGINNINGS provides emotional support and access to unbiased information to families with deaf or hard of hearing children, age birth through 21. BEGINNINGS provides an impartial approach to meeting the diverse needs of these families and the professionals who serve them. These services are also available to deaf parents who have hearing children. The mission of BEGINNINGS is to inform and empower parents as they make decisions about their child. In addition, BEGINNINGS is also committed to providing technical assistance to professionals who work with these families.

http://www.ncbegin.org

Last Updated: 27 Jul 2015

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Hands & Voices

Hands & Voices is dedicated to supporting families with children who are Deaf or Hard of Hearing without a bias around communication modes or methodology. We're a parent-driven, non-profit organization providing families with the resources, networks, and information they need to improve communication access and educational outcomes for their children. Our outreach activities, parent/professional collaboration, and advocacy efforts are focused on enabling Deaf and Hard-of-Hearing children to reach their highest potential.

http://www.handsandvoices.org

Last Updated: 26 Mar 2013

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Hearing Loss Association of America

The mission of HLAA is to open the world of communication to people with hearing loss by providing information, education, support and advocacy.

http://www.hearingloss.org

Last Updated: 27 Jul 2015

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Oklahoma Family Network

To Provide emotional, informational and navigational support to those who have special health care needs or a disability, their families and the professionals who serve them. OFN promotes family/professional partnerships and family-centered care, assuring services are offered as recipients prefer..

http://www.oklahomafamilynetwork.org

Last Updated: 15 Apr 2013

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Zellweger Baby Support Network

This organization was started by a small group of parents whose lives have been affected in some way by a rare disorder. Our gift, in honor of our children, was to start this site, so we can help families and to raise awareness of these rare disorders. If your child has been diagnosed with Zellweger syndrome, a peroxisomal disorder or another rare disorder, we are here to help you. You are not alone! Our mission is to promote, advance, and improve awareness of Zellweger syndrome and other peroxisomal disorders, to assist, support, and aid, financially or otherwise, individuals and families affected by Zellweger syndrome. We will help you learn more about these disorders. We will provide emotional support, and we can help you to create a journal site for your child so that family and friends can be kept up to date on your child. We will pray with you and for you as you journey through life with your special angel. We know this is a hard time for you; you want answers, informa

http://www.zbsn.org

Last Updated: 20 Feb 2013

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General Support Organizations

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General Resources

Usher Syndrome Registry

The Usher Syndrome Registry bridges the gap between researchers and patients. Building the registry is a fundamental step towards quickening the pace of discovery and ensuring access to patients as more clinical trials are launched.

Updated 31 Oct 2014

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Usher Syndrome Conference Calls

The Coalition holds monthly conference calls in which leading experts talk about the latest news and research impacting families with Usher Syndrome. Live captioning is provided. Transcripts and past presentations can be found on our website.

Updated 31 Oct 2014

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Blogs

Usher Syndrome Blog

The Usher Syndrome Blog touches on a lot of issues that Usher families face.

Updated 31 Oct 2014

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Deafness" returned 819 free, full-text research articles on human participants. First 3 results:

Pigmentary retinopathy, rod-cone dysfunction and sensorineural deafness associated with a rare mitochondrial tRNA(Lys) (m.8340G>A) gene variant.
 

Author(s): Jaidip S Gill, Steven A Hardy, Emma L Blakely, Sila Hopton, Andrea H Nemeth, Carl Fratter, Joanna Poulton, Robert W Taylor, Susan M Downes

Journal: Br J Ophthalmol. 2017 Sep;101(9):1298-1302.

 

The rare mitochondrial DNA (mtDNA) variant m.8340G>A has been previously reported in the literature in a single, sporadic case of mitochondrial myopathy. In this report, we aim to investigate the case of a 39-year-old male patient with sensorineural deafness who presented to the eye ...

Last Updated: 21 Jul 2017

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Familial clustering of congenital deafness, patent ductus arteriosus, Eisenmenger complex, and differential cyanosis: A case report.
 

Author(s): Ting-Wei Lin, Chih-Wei Tseng, Chi-Yao Huang, Kuo-Yang Wang, Kae-Woei Liang

Journal: Medicine (Baltimore). 2017 Jun;96(24):e7105.

 

Few studies had reported syndromes that include patent ductus arteriosus (PDA) with Eisenmenger syndrome and congenital deafness clustered in male siblings without facial, skeletal, or mental abnormalities.

Last Updated: 14 Jun 2017

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A novel pore-region mutation, c.887G > A (p.G296D) in KCNQ4, causing hearing loss in a Chinese family with autosomal dominant non-syndromic deafness 2.
 

Author(s): Bangqing Huang, Yanping Liu, Xue Gao, Jincao Xu, Pu Dai, Qingwen Zhu, Yongyi Yuan

Journal:

 

Hereditary non-syndromic hearing loss is the most common inherited sensory defect in humans. The KCNQ4 channel belongs to a family of potassium ion channels that play crucial roles in physiology and disease. Mutations in KCNQ4 underlie deafness non-syndromic autosomal dominant 2, ...

Last Updated: 25 Mar 2017

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Deafness" returned 56 free, full-text review articles on human participants. First 3 results:

Autosomal Recessive Keratoderma-Ichthyosis-Deafness (ARKID) Syndrome Is Caused by VPS33B Mutations Affecting Rab Protein Interaction and Collagen Modification.
 

Author(s): Robert Gruber, Clare Rogerson, Christian Windpassinger, Blerida Banushi, Anna Straatman-Iwanowska, Joanna Hanley, Federico Forneris, Robert Strohal, Peter Ulz, Debra Crumrine, Gopinathan K Menon, Stefan Blunder, Matthias Schmuth, Thomas Müller, Holly Smith, Kevin Mills, Peter Kroisel, Andreas R Janecke, Paul Gissen

Journal: J. Invest. Dermatol.. 2017 Apr;137(4):845-854.

 

In this paper, we report three patients with severe palmoplantar keratoderma associated with ichthyosis and sensorineural deafness. Biallelic mutations were found in VPS33B, encoding VPS33B, a Sec1/Munc18 family protein that interacts with Rab11a and Rab25 proteins and is involved ...

Last Updated: 26 Dec 2016

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Body Perception and Action Following Deafness.
 

Author(s): M S Houde, S P Landry, S Pagé, M Maheu, F Champoux

Journal: Neural Plast.. 2016 ;2016():5260671.

 

The effect of deafness on sensory abilities has been the topic of extensive investigation over the past decades. These investigations have mostly focused on visual capacities. We are only now starting to investigate how the deaf experience their own bodies and body-related abilities. ...

Last Updated: 16 Feb 2016

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From Hyperactive Connexin26 Hemichannels to Impairments in Epidermal Calcium Gradient and Permeability Barrier in the Keratitis-Ichthyosis-Deafness Syndrome.
 

Author(s): Isaac E García, Felicitas Bosen, Paula Mujica, Amaury Pupo, Carolina Flores-Muñoz, Oscar Jara, Carlos González, Klaus Willecke, Agustín D Martínez

Journal: J. Invest. Dermatol.. 2016 Mar;136(3):574-83.

 

The keratitis-ichthyosis-deafness (KID) syndrome is characterized by corneal, skin, and hearing abnormalities. KID has been linked to heterozygous dominant missense mutations in the GJB2 and GJB6 genes, encoding connexin26 and 30, respectively. In vitro evidence indicates that KID ...

Last Updated: 23 Feb 2016

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Symptoms, Diagnosis, and Treatment

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Cochlear Implantation for Treatment of Single-sided Deafness
 

Status: Recruiting

Condition Summary: Total Unilateral Deafness; Unilateral Partial Deafness

 

Last Updated: 17 Aug 2017

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Last Updated: 31 Aug 2016

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Cochlear Implantation in Adults With Asymmetric Hearing Loss Clinical Trial
 

Status: Recruiting

Condition Summary: Hearing Loss

 

Last Updated: 27 Sep 2017

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