Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder in which the ear detects sound but cannot transmit the information to the brain due to nerve damage or damage to the tiny hair cells within the cochlea.
It’s estimated that one to three children per 10,000 births are affected by ANSD, but it can affect people of all ages and may also occur later in life.1 ANSD may be present in one ear (unilateral auditory neuropathy spectrum disorder) or both ears (bilateral auditory neuropathy spectrum disorder).
Learn the signs, symptoms, and treatments for this disorder, which can affect not only hearing ability but also general quality of life.
The difference between ANSD and hearing loss is simple: ANSD results in a type of hearing loss, but not all cases of hearing loss are caused by ANSD. And not all cases of ANSD result in the same level of hearing loss; ANSD is a spectrum disorder, meaning the symptoms and severity can range from mild hearing loss to functional deafness, depending on the level of nerve or hair cell damage.
For some people, ANSD hearing loss can cause dyssynchrony, a mismatch between how sounds occur and how the brain processes them. Sounds may arrive at the brain at various intervals or sound like static, making it difficult to process information like speech or alarms.
It’s also important to note the difference between auditory neuropathy spectrum disorder vs. auditory processing disorder (APD) because they present similar symptoms but require different types of treatment. In both cases, the ears hear normally, but ANSD is caused by issues with the auditory nerve, and APD is related to the auditory pathways in the brain. A medical professional can provide an accurate diagnosis with proper testing.
In some cases, ANSD is inherited through genetics, but many cases are caused by other factors, including:
Not all ear nerve damage leads to ANSD. The disorder occurs when a specific pathway becomes damaged. Damage to the eighth cranial nerve (the auditory nerve that controls hearing) was originally identified as the cause, but the definition of ANSD was later expanded to include loss of inner hair cells or damage between the auditory nerve and the brain.1
In addition to general hearing loss, ANSD symptoms and signs include:
If you suspect a child, loved one, or yourself may have ANSD, see a medical provider for testing. A combination of otoacoustic emission (OAE) tests and auditory brainstem response (ABR) tests are a common way to test for ANSD. Doctors are trained to look for auditory neuropathy ABR test manifestations (signs). Auditory neuropathy is likely present if the ABR reading is absent or abnormal and the OAE reading is normal.
If your medical provider suspects ANSD, they may order additional tests like MRIs or genetic tests to determine the cause and appropriate treatment.
Never underestimate the importance of ear care, your hearing might depend on it.
ANSD is not curable, but certain treatments can help relieve symptoms and improve hearing. Depending on your symptoms and their severity, your doctor may recommend:
The best ANSD treatment plan varies from person to person, and there is no one-size-fits-all solution. In general, a multidisciplinary approach with a mix of hearing devices and therapy leads to the most favorable outcomes2.
Note: Miracle-Ear hearing care professionals (HCPs) cannot diagnose ANSD. Miracle-Ear hearing aids do not cure ANSD but may be part of an effective auditory neuropathy treatment plan. Talk to a medical provider about your options to make an informed decision.
1. “Auditory Neuropathy Spectrum Disorder.” NORD (National Organization for Rare Disorders), 18 Aug. 2021, rarediseases.org/rare-diseases/auditory-neuropathy-spectrum-disorder/.
2. De Siati, Romolo Daniele, et al. “Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports.” Journal of Clinical Medicine, vol. 9, no. 4, 1 Apr. 2020, p. 1074, www.mdpi.com/2077-0383/9/4/1074/htm, https://doi.org/10.3390/jcm9041074.
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