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As an overview, low frequency (or tone) sensorineural hearing loss (LF SNHL or LT SNHL) is unusual, and mainly reported in Meniere's disease as well as related conditions such as low CSF pressure.
Le Prell et al (2011) suggested that low frequency hearing loss was detected in 2.7% of normal college student's ears.
Oishi et al (2010) suggested that about half of patients with LTSNHL developed high or flat hearing loss within 10 years of onset. The new international criteria for Meniere's disease require observation of a low-tone SNHL. Gatland et al (1991) reported fluctuation in hearing at low frequencies with dialysis was common, and suggested there might be treatment induced changes in fluid or electrolyte composition of endolymph (e.g. Although OAE's are generally obliterated in patients with hearing loss > 30 dB, for any mechanism other than neural, this is not always the case in the low-tone SNHL associated with Meniere's disease.
The lack of universal correspondence between audios and OAE's in low-tone SNHL in at least some patients with Meniere's, suggests that the mechanism of hearing loss may not always be due to cochlear damage. Of course, in a different clinical context, one might wonder if the patient with the discrepency between OAE and audiogram might be pretending to have hearing loss. Papers are easy to publish about genetic hearing loss conditions, so the literature is somewhat oversupplied with these. Bespalova et al (2001) reported that low frequency SNHL may be associated with Wolfram syndrome, an autosomal recessive genetic disorder (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness).
Bramhall et al (2008) a reported that "the majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein). Eckert et al(2013) suggested that cerebral white matter hyperintensities predict low frequency hearing in older adults. Fukaya et al (1991) suggested that LFSN can be due to microinfarcts in the brainstem during surgery. Ito et al (1998) suggested that bilateral low-frequency SNHL suggested vertebrobasilar insufficiency. Yurtsever et al (2015) reported low and mid-frequency hearing loss in a single patient with Susac syndrome. There are an immense number of reports of LTSNHL after spinal anesthesia and related conditions with low CSF pressure. Fog et al (1990) reported low frequency hearing loss in patients undergoing spinal anesthesia where a larger needle was used. Waisted et al (1991) suggested that LFSNHL after spinal anesthesia was due to low perilymph pressure. Wang et al reported hearing reduction after prostate surgery with spinal anesthesia (1987).
We have encountered a patient, about 50 years old, with a low-tone sensorineural hearing loss that occured in the context of a herpes zoster infection (Ramsay-Hunt).
LTSNHL is generally viewed as either a variant of Meniere's disease or a variant of sudden hearing loss, and the same medications are used. Auditory Neuropathy was first identified in the 1980s when advanced testing procedures became available to measure the action of the cells in the cochlea. Speech understanding difficulties that are worse than can be predicted from other tests of hearing function. Other neuropathies that may affect coordination for activities like writing, running or talking.
The diagnosis of auditory neuropathy is based on a certain pattern of results from a number of different hearing tests. Depending upon what method is used for newborn hearing screening (OAEs or ABRs), some children with auditory neuropathy may pass an initial hearing screening conducted in the hospital. Audiologists vary in their opinions about using hearing aids for a child with auditory neuropathy. Previously, it was thought that children with auditory neuropathy would not be good cochlear implant candidates.
It is safe to say that no single teaching approach fits all children with auditory neuropathy.


Mild precipitously sloping to profound means that hearing loss is mild for the lower frequencies.
This kind of hearing loss is also called "Ski Slope Loss." Children with this kind of hearing loss can work just fine in quiet rooms. A child with this amount of hearing loss will need to use hearing aids as well as other assistive listening devices to hear what people are saying.
The majority of hearing aids sold today are canal hearing aids and in-the-ear hearing aids. A cochlear implant is a device that provides direct electrical stimulation to the auditory nerve.
In sensorineural hearing loss where there is damage to the tiny hair cells in the cochlea, sound cannot reach the auditory nerve. With a cochlear implant, the damaged hair cells are bypassed and the auditory nerve is stimulated directly.
Thorough evaluation is conducted before a patient receives the implant, to decide whether they’re a candidate.
The speech processor is a computer that analyzes and digitizes the sound signals and sends them to a transmitter worn on the head just behind the ear. The movement of the ossicles in the middle ear cause movement of the fluid, which causes the hair cell to bend resulting in the stimulation of the auditory nerve. A hearing aid is an electronic assistive device which is most often worn behind or in the ear.
Although a hearing aid may not provide its wearer with completely normal hearing, it should be able to provide considerable benefit in overcoming the effects of a hearing loss.
To us, this seems a little overly stringent, compared to the earlier 1995 AAO criteria that just required any kind of hearing loss, but criteria are better than no criteria. That being said, genetic conditions should not be your first thought when you encounter a patient with LTSNHL. One would think that diabetes insipidus is so rare that hearing loss is probably not the main reason these individuals are seeking attention. These patients are extremely rare because there isn't much microsurgery done close to the brainstem. We are dubious about the conclusion of this paper because it is different than nearly every other paper in the literature. We are dubious that this has any applicability to humans and we have never seen a patient with lithium toxicity with any LFSNHL. We have observed this occasionally ourselves, but of course, the usual pattern is a conductive hyperacusis. We find this somewhat plausible as pregnancy and delivery might be associated with spinal anesthesia, with changes in collagen, and with gigantic changes in hormonal status. Auditory Brainstem Response (ABR) and Otoacoustic Emission (OAE) results are used to find this condition. Ongoing testing may show poorer than expected speech understanding and great difficulty hearing in noisy backgrounds. Now we know that some children benefit more from a cochlear implant than from hearing aids. He may also need special ear mold add-ons or changes, like the Libby Horn or the Continuous Flow Adapter (CFA) to make these sounds accessible to the child. If bone conduction thresholds are higher than air conduction thresholds, this could mean that something is not working right in the middle or outer ear. It picks up sounds - just like a hearing aid microphone does -- and sends them to the speech processor . Specially designed earmuffs that resemble earphones can protect your ears by bringing most loud sounds down to an acceptable level. Activities such as riding a snowmobile, hunting, and listening to extremely loud music for long periods of time can damage your ears. In public, such as in a restaurant or at a social gathering, choose a place to talk that's away from noisy areas.


Hearing devices, such as TV-listening systems or telephone-amplifying devices, can help you hear better while decreasing other noises around you.
These devices alert you to a particular sound (such as the doorbell, a  ringing telephone, or a baby monitor) by using louder sounds, lights, or vibrations to get your attention.
Television closed-captioning makes it easier to watch television by showing the words at the bottom of the screen so that you can read them. Due to recent developments in digital technology and advanced electronic design, today hearing aids can be so small that they are virtually invisible in the ear canal. There is a large literature about genetic causes, mainly in Wolfram's syndrome ( diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), probably because it is relatively easier to publish articles about genetic problems. He defined LTSNH as that the sum of the three low frequencies (125, 250, 500) was more than 100 dB, and the sum of the 3 high frequencies (2, 4, 8) was less than 60 dB. In other words, Wolfram's is probably not diagnosed mainly by ENT's or audiologists, but rather by renal medicine doctors, as DI is exceedingly rare, while LTSNHL is not so rare in hearing tests. That being said, it is very rare to encounter any substantial change in hearing after pregnancy. With this type of hearing disorder, the inner ear or cochlea seems to receive sounds normally. The electrodes pick up responses from the hearing nerve and estimate how the cochlea and hearing nerve are responding to sound. Parents and physicians may not suspect hearing loss right away if an infant’s response to sound changes from day to day. Children with some types of genetically inherited auditory neuropathy may benefit more from cochlear implants than children with other causes of the disorder.
A team can help parents observe their child and determine what techniques bring about the best responses. So you may want to get your child tested for how his hearing loss will affect him in school. Foam, pre-formed, or custom-molded earplugs made of plastic or rubber also can effectively protect your ears from damaging noise. Wearing hearing protectors or taking breaks from the noise during loud recreational activities can protect your ears.
Roughly speaking, the frequency patterns of hearing loss are divided up into: Low-frequency, mid-frequency, high-frequency, notches, and flat.
However, signals leaving the cochlea may be disorganized or the hearing nerve itself may not process sound normally. Children with auditory neuropathy may also have hearing responses that fluctuate or worsen over time. Knowing you've lost some hearing means you're in a position to take steps to prevent further hearing loss. Turning down the volume when listening to music can help you avoid damage to your hearing. Hearing aids must be set carefully to prevent damage to the parts of the ear that receive sounds normally. Sometimes, a child with auditory neuropathy has great difficulty understanding what is heard.
Teachers and other professionals can help families to decide whether or not an approach is working. The most striking finding with auditory neuropathy is that Otoacoustic Emissions (OAEs) are normal.
It appears that only a small percentage of people with hearing loss have auditory neuropathy.




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