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 792 free, full-text research articles on human participants. First 3 results:

Novel domain-specific POU3F4 mutations are associated with X-linked deafness: examples from different populations.
 

Author(s): Guney Bademci, Akeem Lasisi, Kemal O Yariz, Paola Montenegro, Ibis Menendez, Rodrigo Vinueza, Rosario Paredes, Germania Moreta, Asli Subasioglu, Susan Blanton, Suat Fitoz, Armagan Incesulu, Levent Sennaroglu, Mustafa Tekin

Journal:

 

Mutations in the POU3F4 gene cause X-linked deafness type 3 (DFN3), which is characterized by inner ear anomalies.

Last Updated: 1 May 2015

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Mutations Impairing GSK3-Mediated MAF Phosphorylation Cause Cataract, Deafness, Intellectual Disability, Seizures, and a Down Syndrome-like Facies.
 

Author(s): Marcello Niceta, Emilia Stellacci, Karen W Gripp, Giuseppe Zampino, Maria Kousi, Massimiliano Anselmi, Alice Traversa, Andrea Ciolfi, Deborah Stabley, Alessandro Bruselles, Viviana Caputo, Serena Cecchetti, Sabrina Prudente, Maria T Fiorenza, Carla Boitani, Nicole Philip, Dmitriy Niyazov, Chiara Leoni, Takaya Nakane, Kim Keppler-Noreuil, Stephen R Braddock, Gabriele Gillessen-Kaesbach, Antonio Palleschi, Philippe M Campeau, Brendan H L Lee, Celio Pouponnot, Lorenzo Stella, Gianfranco Bocchinfuso, Nicholas Katsanis, Katia Sol-Church, Marco Tartaglia

Journal: Am. J. Hum. Genet.. 2015 May;96(5):816-25.

 

Transcription factors operate in developmental processes to mediate inductive events and cell competence, and perturbation of their function or regulation can dramatically affect morphogenesis, organogenesis, and growth. We report that a narrow spectrum of amino-acid substitutions ...

Last Updated: 11 May 2015

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Cortical cross-modal plasticity following deafness measured using functional near-infrared spectroscopy.
 

Author(s): Rebecca S Dewey, Douglas E H Hartley

Journal: Hear. Res.. 2015 Jul;325():55-63.

 

Evidence from functional neuroimaging studies suggests that the auditory cortex can become more responsive to visual and somatosensory stimulation following deafness, and that this occurs predominately in the right hemisphere. Extensive cross-modal plasticity in prospective cochlear ...

Last Updated: 9 May 2015

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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 50 free, full-text review articles on human participants. First 3 results:

Cochlear implantation and single-sided deafness.
 

Author(s): Joshua Tokita, Camille Dunn, Marlan R Hansen

Journal: Curr Opin Otolaryngol Head Neck Surg. 2014 Oct;22(5):353-8.

 

Recently, more patients with single-sided deafness (SSD) have been undergoing cochlear implantation. We review recent studies and case reports to provide an overview of the efficacy of cochlear implants to rehabilitate patients with SSD with regards to sound localization, speech discrimination, ...

Last Updated: 4 Sep 2014

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Developmental and cross-modal plasticity in deafness: evidence from the P1 and N1 event related potentials in cochlear implanted children.
 

Author(s): Anu Sharma, Julia Campbell, Garrett Cardon

Journal: Int J Psychophysiol. 2015 Feb;95(2):135-44.

 

Cortical development is dependent on extrinsic stimulation. As such, sensory deprivation, as in congenital deafness, can dramatically alter functional connectivity and growth in the auditory system. Cochlear implants ameliorate deprivation-induced delays in maturation by directly ...

Last Updated: 2 Mar 2015

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Gene therapy for deafness.
 

Author(s): D C Kohrman, Y Raphael

Journal: Gene Ther.. 2013 Dec;20(12):1119-23.

 

Hearing loss is the most common sensory deficit in humans and can result from genetic, environmental or combined etiologies that prevent normal function of the cochlea, the peripheral sensory organ. Recent advances in understanding the genetic pathways that are critical for the development ...

Last Updated: 5 Dec 2013

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Outcomes In Children With Developmental Delay And Deafness
 

Status: Recruiting

Condition Summary: Deafness; Hearing Loss

 

Last Updated: 7 Dec 2015

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Cochlear Implantation for Treatment of Single-sided Deafness
 

Status: Recruiting

Condition Summary: Total Unilateral Deafness; Unilateral Partial Deafness

 

Last Updated: 6 Jan 2016

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Implantation of the HiRes90K™ Advantage Cochlear Implant With HiFocus™ Mid-Scala and Development of a Combined Electric and Acoustic Stimulation Technology in Adults With Partial Deafness
 

Status: Recruiting

Condition Summary: Hearing Loss; Partial Deafness; Hearing Disorders; Ear Diseases; Otorhinolaryngologic Diseases

 

Last Updated: 9 Oct 2015

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