Auditory neuropathy spectrum disorder

Common Name(s)

Auditory neuropathy spectrum disorder

Auditory neuropathy spectrum disorder (ANSD) is a rare disorder in which hearing loss occurs due to a lack of normal middle ear reflexes and absence of brain stem response to auditory signals. This means that the auditory nerves are not working, but interestingly, hair cells in the ear are normal. Because hair cells are often normal, ANSD does not always show up on initial newborn hearing tests. 40% of ANSD cases are genetic. Symptoms include difficulty hearing noise, fluctuation in hearing ability, and low levels of speech perception. There is currently no medical treatment for ANSD, but cochlear implants and hearing aids may be used to improve hearing ability.

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Auditory neuropathy spectrum disorder" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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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 "Auditory neuropathy spectrum disorder" returned 26 free, full-text research articles on human participants. First 3 results:

Evaluation of Hearing Handicap in Adults with Auditory Neuropathy Spectrum Disorder.
 

Author(s): Prashanth Prabhu

Journal: J Int Adv Otol. 2017 Aug;13(2):226-229.

 

The present study attempted to evaluate hearing handicap in adults with auditory neuropathy spectrum disorder (ANSD). The study also attempted to determine if gender, pure tone average, speech identification scores (SIS), and reported duration of hearing loss could predict the hearing ...

Last Updated: 31 Dec 1969

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Speech Perception in Quiet and in Noise Condition in Individuals with Auditory Neuropathy Spectrum Disorder.
 

Author(s): Kumari Apeksha, Ajith U Kumar

Journal: J Int Adv Otol. 2017 Apr;13(1):83-87.

 

The study investigated the effect of noise on syllable perception in individuals with Auditory Neuropathy Spectrum Disorder (ANSD) and compared that with the normal hearing individuals.

Last Updated: 31 Dec 1969

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Novel compound heterozygous mutations in the OTOF Gene identified by whole-exome sequencing in auditory neuropathy spectrum disorder.
 

Author(s): Fengzhu Tang, Dengke Ma, Yulan Wang, Yuecai Qiu, Fei Liu, Qingqing Wang, Qiutian Lu, Min Shi, Liang Xu, Min Liu, Jianping Liang

Journal:

 

Many hearing-loss diseases are demonstrated to have Mendelian inheritance caused by mutations in single gene. However, many deaf individuals have diseases that remain genetically unexplained. Auditory neuropathy is a sensorineural deafness in which sounds are able to be transferred ...

Last Updated: 31 Dec 1969

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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 "Auditory neuropathy spectrum disorder" returned 4 free, full-text review articles on human participants. First 3 results:

Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder.
 

Author(s): Guilherme Machado de Carvalho, Priscila Ramos, Castilho Arthur, Alexandre GuimarĂ£es, Edi Sartorato

Journal: J Int Adv Otol. 2016 Apr;12(1):8-15.

 

To describe the performance and results of CIs (cochlear implant) in patients with AN (auditory neuropathy) and to present a medical literature review.

Last Updated: 31 Dec 1969

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Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
 

Author(s): Anu Sharma, Garrett Cardon

Journal: Hear. Res.. 2015 Dec;330(Pt B):221-32.

 

Cortical development is dependent to a large extent on stimulus-driven input. Auditory Neuropathy Spectrum Disorder (ANSD) is a recently described form of hearing impairment where neural dys-synchrony is the predominant characteristic. Children with ANSD provide a unique platform ...

Last Updated: 31 Dec 1969

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Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review.
 

Author(s): Nayara Freitas Fernandes, Marina Morettin, Elisabete Honda Yamaguti, Orozimbo Alves Costa, Maria Cecilia Bevilacqua

Journal: Braz J Otorhinolaryngol. ;81(1):85-96.

 

Currently, there are no doubts about the benefits of cochlear implants for the development of children with severe or profound hearing loss. However, there is still no consensus among researchers and professionals regarding the benefits for the improvement of hearing skills in children ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.