Older children and adults often state their inability to hear and describe whether they suffer from mild, moderate or severe hearing loss, whether only one or both ears are affected. In case of bilateral CHL, the severity of symptoms may differ between both ears. Hearing impairment may be the predominant or even the only symptom experienced, but in some cases, other complaints are reported. A feeling of fullness or pressure in the ear and otalgia are commonly noted. In detail, the following symptoms may be observed in patients presenting with impacted cerumen, otitis media and otosclerosis:

• The vast majority of patients having impacted cerumen does not report any complaints. CHL may be very mild and thus may not interfere with everyday life. Only in more severe cases, patients suffer from otalgia, pruritus, tinnitus and possibly dizziness.
• Otitis media is typically associated with fever, pain, otorrhea and CHL.
• Otosclerosis usually manifests in form of progressive CHL. Tinnitus, dizziness and vertigo may be reported [7].

Neonates and infants don't typically provide such detailed anamnestic data. Here, parents may observe their child to be stubborn, not to interact with their surroundings. Pre-lingual CHL, i.e., hearing impairment that manifests before the acquisition of language and speech takes place, may cause the child to vocalize little and to show delays in cognitive development. Otalgia is often expressed by frequent touching of the ears, but may also lead to continuous indisposition and restlessness.

• Inflammation

  This is a case of conductive hearing loss with the normal tympanic membrane due to fibrosis caused by potential inflammation of the long process of the incus. High resolution CT was a useful tool for the detection of ossicular disorder. [ncbi.nlm.nih.gov]

  Perforations (holes) of the eardrum, middle ear infection or inflammation, otosclerosis, and trauma are potential sources of conductive hearing loss. This type of hearing loss may be surgically correctable. [umm.edu]

  Corticosteroids may also be used to reduce cochlea hair cell swelling and inflammation after exposure to loud noise. [hearingloss.org]

  Some of the causes of conductive hearing loss include: A build-up of earwax in the outer ear stopping sound getting to the ear drum Ear infection or inflammation in the outer ear (Otitis Externa) or middle ear (Otitis Media) A build-up of fluid in the [amplifon.com]

• Surgical Procedure

  The patients gained normal hearing levels using a simple surgical procedure. [ncbi.nlm.nih.gov]

  If the vertigo is not medically controlled, then various surgical procedures are used to eliminate the vertigo. [hearingloss.org]

  Holes in the drum can be repaired by a surgical procedure called tympanoplasty. If the hole was the only cause of hearing loss, surgical closure should improve hearing. [luriechildrens.org]

• Weakness

  You have new headaches, weakness, or numbness anywhere on your body. Decreased hearing; Deafness; Loss of hearing; Conductive hearing loss; Sensorineural hearing loss; Presbycusis Arts HA. Sensorineural hearing loss in adults. [nlm.nih.gov]

  There also seems to be a weak association with increased serum calcium (de Carvalho et al, 2006). [dizziness-and-balance.com]

  The 5th or 7th cranial nerve or both are often affected by tumors that involve the 8th nerve, so loss of facial sensation and weak jaw clench (5th) and hemifacial weakness and taste abnormalities (7th) point to a lesion in that area. [merckmanuals.com]

• Short Stature

  She has short stature, microcephaly, conductive hearing loss, skin mastocytosis and microtia. [ncbi.nlm.nih.gov]

• High Pitched Voice

  high-pitched voices and sounds such as birds singing. [signiausa.com]

• Tinnitus

  The aberrant ICA was treated, and the pulsatile tinnitus disappeared and hearing recovered after the surgery. This case suggests that surgery is practical to relieve troublesome tinnitus and hearing loss in appropriate cases with aberrant ICA. [ncbi.nlm.nih.gov]

  Can You Prevent Tinnitus Links Between Hearing Loss And Tinnitus Tinnitus Causes Tips For Dealing With Tinnitus About What Makes Us Different Contact Us Dr Hilary Bio Free 5 Star Service Aftercare Aftercare Roadmap Free Lifetime Hearing Care Service [hiddenhearing.co.uk]

  Do you also have tinnitus? Tinnitus (noises you can hear that are not caused by sounds in the outside world) can make it much harder to hear with any given level of hearing loss. [hearinglink.org]

  Learn More About Topic Tinnitus Over 50 million Americans have experienced tinnitus, or ringing in ears, which is the perception of sound without an external source being present. [entnet.org]

• Hearing Impairment

  hearing loss partial or complete loss of the sense of hearing ; called also deafness. The number of hearing impaired individuals has steadily increased over the past few decades. [medical-dictionary.thefreedictionary.com]

  These results suggest that developmental CHL impairs both within- and across-frequency processing and provide behavioral evidence that CNS mechanisms are affected by a peripheral hearing impairment. [ncbi.nlm.nih.gov]

  Mechanical or functional impairment of any of those organs may lead to hearing impairment, hearing loss and eventually deafness. [symptoma.com]

  There are two different types of hearing impairments, conductive hearing loss and sensorineural hearing loss. A third type is a combination of the two, called a mixed hearing loss. [dallashear.com]

• Sudden Hearing Loss

  Sudden hearing loss may also occur over a few hours or two to three days. Sudden hearing loss should always be evaluated immediately to rule out serious underlying medical problems as well as lessen the risk of temporary deafness becoming permanent. [deafwebsites.com]

  Sudden hearing loss If you have a sudden hearing loss in one or both ears, it's important to see an ear, nose and throat specialist urgently so you can benefit from treatment straight away. [actiononhearingloss.org.uk]

  This type of hearing loss may be sudden or slowly progressive. [umm.edu]

  Roadmap Free Lifetime Hearing Care Service When To Get A Hearing Check Up Wellness Programme The Connection Between Dementia And Hearing Loss The link between Stroke and Sudden Hearing Loss The Link Between Your Heart And Your Hearing Health The Links [hiddenhearing.co.uk]

• Hearing Problem

  Nevertheless, craniomandibular origins are frequently overlooked in the medical profession as possible causes for hearing problems. The following case illustrates this point. [ncbi.nlm.nih.gov]

  See your provider for any other hearing loss. Call your provider if: Hearing problems interfere with your lifestyle. Hearing problems do not go away or become worse. The hearing is worse in one ear than the other. [nlm.nih.gov]

  About a third of adults older than 65—and half of those older than 85—have some hearing problem. It is the third most common chronic disease among older adults. Hearing loss is more than an inconvenience. [healthinaging.org]

  Hearing Loss Genetic Hearing Loss Noise Induced Hearing Loss Bilateral Hearing Loss Unilateral Hearing Loss Conductive Hearing Loss High Frequency Hearing Loss Low Frequency Hearing Loss Sensorineural Hearing Loss Hearing Problems Fungal Ear Infections [hiddenhearing.co.uk]

• Rinne's Test Positive

  Rinne test, which tests air conduction vs. bone conduction is positive, because both bone and air conduction are reduced equally. less common Bing and Schwabach variants of the Rinne test. absolute bone conduction (ABC) test. Table 1. [en.wikipedia.org]

• Confusion

  We know that if you have hearing loss or if someone close to you is affected, the feeling can be overwhelming and maybe a little confusing. [healthyhearing.com]

  Therefore, learning ASL may aid the development of English language skills rather than confuse them. [emedicine.medscape.com]

  People with hearing loss may misunderstand a question and give an apparently bizarre answer, leading others to believe they are confused. [merckmanuals.com]

  NOMENCLATURE The nomenclature used in the literature to describe hearing losses caused by third-window lesions is confusing. [ncbi.nlm.nih.gov]

Besides anamnesis and physical examination, otoscopy and audiologic tests are usually carried out first. With regards to the latter, functional tests don't necessarily allow for a precise localization of the cause of hearing impairment. Some tests do require active participation of the patient and are therefore not suitable for neonates and infants. Auditory brainstem response and otoacoustic emissions can be measured in such patients, though [8]. In fact, they are commonly applied in newborn hearing screenings. Pure-tone and speech audiometry are reserved for elder children and adults. This also applies to tuning fork tests like Weber test and Rinne test that may be indicated to distinguish CHL from SNHL.

Because in CHL patients, acoustic wave propagation through the outer or middle ear is disturbed, chances are good that an otoscopic examination of the external auditory canal and the tympanic membrane yield pathologic findings. While a tentative diagnosis of impacted cerumen may be confirmed this way, pneumatic otoscopy and tympanometry are often required in otitis media patients [9]. Otosclerosis affects the ossicles in the middle ear and tympanometry and diagnostic imaging (computed tomography) have to be performed in order to detect a decrease in ossicle mobility and alterations in bone structure, respectively. Exploratory tympanotomy may also be considered. If these measures don't allow for a reliable diagnosis, additional techniques have to be applied. Serological tests are helpful to detect underlying infectious diseases. Neoplasms are usually diagnosed by means of magnetic resonance imaging.

If feasible, causal therapy should be provided.

• In case of impacted cerumen sufficiently severe to induce hearing loss, manual removal of the accumulated earwax may be required. This task is facilitated if the affected ear is irrigated with saline solution or ceruminolytics shortly before. Some cases may be resolved by mere application of ceruminolytics [10].
• Otitis media patients should be treated with antimicrobials and anti-inflammatory drugs. Additional measures like myringotomy and drainage of accumulated fluids may be necessary to reduce middle ear pressure and to diminish pathogen loads.

To date, causal treatment is not available for patients suffering from otosclerosis. Here, hearing aids may be applied to improve sound perception. In severe cases, non-functional ossicles have to be replaced with the corresponding protheses. Stapedectomy is commonly performed. Similarly, surgical interventions are necessary to reconstruct parts of the auditory system if a child is born with developmental defects causing CHL.

Prognosis depends on the underlying disease. Reconstructive surgery is often necessary to treat patients suffering from developmental defects and congenital CHL. In case of otitis media or impacted cerumen, the outcome is generally very good because causal therapy is available. This does not apply to otosclerosis patients and many affected individual have to undergo surgery in order to regain their ability to hear. Few pathologies associated with acquired CHL have an unfavorable prognosis, e.g., malignant otitis externa.

CHL may be congenital or acquired, whereby the latter is much more common than the former.

Congenital CHL is the result of developmental defects like malformation of one or more ossicles and fixed stapes [2]. Furthermore, microtia, aural atresia and more complex craniosynostosis may be the cause of CHL in neonates. Congenital rubella syndrome and hearing loss due to prenatal infection with measles virus, mumps virus or other pathogens rather induce congenital SNHL.

CHL may be acquired at any age and hearing impairment is often claimed by patients suffering from otitis externa, impacted cerumen, exostosis of the external auditory canal, otitis media (especially if an effusion is present) or tympanic membrane perforation. Also, traumatic lesions of the external or middle ear may hinder sound wave propagation. Temporal bone fractures may cause CHL, SNHL or mixed hearing loss [3]. Otosclerosis is an acquired disease of as of yet unknown origin. Besides impacted cerumen, it is the most common cause of CHL in adults. Here, both progressive limitation of the mobility of the stapes as well as resorption and formation of the bony labyrinth in the inner ear hinder ossicle vibration and pressure wave propagation through perilymph and endolymph. Tumors like cholesteatoma, osteoma or paraganglioma may also lead to CHL. Of note, congenital cholesteatoma have repeatedly been described. Such tumors are rarely diagnosed in pediatric patients, but the presence of cholesteatoma in individuals without a medical history of otorhinolaryngologistic diseases led to the hypothesis of them having an embryologic origin [4].

Although incidence and prevalence of CHL are lower than of SNHL, the former is still a common condition. According to a study recently conducted in Australia, prevalence of CHL among neonates averages 0.3% [5]. Interestingly, significant differences have been observed between indigenous and non-indigenous Australians, with the former being affected about twice as often as the latter (prevalence of CHL among infants 35% vs. 18%). These findings imply racial differences that may also exist with regards to ethnicities in other continents. It has to be noted that gender and racial predilection as well as age distribution of CHL may vary depending on the underlying disease, but, in general, women and men, patients of all races and ages may suffer from CHL.

Sound waves pass auricles, external auditory canal, tympanic membrane, ossicles and oval window, before reaching the inner ear and the organ of Corti. Here, mechanical stimuli are converted into electrical impulses which are conducted to higher brain centers. As per definition, CHL affects the passage of acoustic waves through outer and middle ear, whereas SNHL is diagnosed if cochlear signal conversion, conduction via the vestibulocochlear nerve or processing in the auditory cortex are disturbed. Accordingly, CHL generally results from the inability of acoustic waves to pass a mechanical obstacle blocking any of the aforementioned anatomical structures or impeding wave propagation. In contrast, SNHL may be provoked by mechanical or functional anomalies, e.g., by neuronal death after cerebral ischemia.

In detail, CHL may be caused by:

• Mechanical obstruction of the external acoustic meatus. A very common pathology leading to this condition is impacted cerumen. Here, accumulation of earwax due to a narrow auditory canal, an altered composition of cerumen, or futile attempts to remove it interfere with sound wave propagation to the tympanic membrane.
• Lesions of the tympanic membrane. Under physiological conditions, acoustic waves reach the tympanic membrane, it vibrates, and transmits those vibrations to the ossicles malleus, incus, and stapes. In case of tympanic membrane perforation or myringitis, this function cannot be carried out [6].
• Impairment of mechanical conduction through the middle ear. One of the most common causes of CHL is otitis media with effusion, most frequently diagnosed in children. In contrast, otosclerosis is typically diagnosed in adults. It is caused by abnormal bone remodeling.

Depending on their pathogenesis, developmental defects may be prevented by complying with vaccination plans, or by avoiding possible sources of infection. It is generally recommendable to keep healthy during pregnancy and to refrain from consumption of alcohol and drugs.

With regards to acquired CHL, the following measures may be helpful to prevent hearing loss:

• Refraining from attempts to remove cerumen with unsuited tools.
• Wearing ear plugs while swimming.
• Provision of adequate care for patients affected by upper respiratory infections.

The auditory system consists of outer, middle and inner ear as well as the vestibulocochlear nerve (corresponding to the eighth cranial nerve) and processing brain structures. Mechanical or functional impairment of any of those organs may lead to hearing impairment, hearing loss and eventually deafness. Depending on the precise site of lesion, different forms of hearing loss are distinguished [1]:

• Conductive hearing loss (CHL) affects outer and/or middle ear. Acoustic waves correspond to changes in air pressure and propagate in a wave-like manner. They are captured by the auricles, pass the external acoustic meatus, the tympanic membrane and are transmitted in form of vibrations towards the inner ear by malleus, incus and stapes. Pathologies interfering with these processes cause CHL.
• Sensorineural hearing loss (SNHL) is provoked by anomalies upstream of the inner ear and cochlea. In the cochlea, pressure waves are converted into electrical impulses by sensory hair cells. Electric impulses are conducted to the auditory cortex in form of action potentials, pass the cochlear nerve, medulla and pons.
• Mixed hearing loss is diagnosed if patients suffer from both CHL and SNHL.
• Auditory processing disorders are entities in which sound wave conduction and cochlear generation of electric impulses are unaltered, while acoustic stimuli cannot be associated with other sensory impressions.

Clinical presentation, therapy and prognosis of CHL depend on the underlying disease. In most cases, CHL is acquired, develops progressively, but may be treated with medication or surgery. The outcome is often better than in SNHL patients, because CHL is rarely associated with irreversible nerve damage.

Hearing loss refers to a decreased ability to recognize sounds and speech, and this condition may result from a wide variety of diseases. Depending on the precise site of tissue damage, various types of hearing loss are distinguished: If an illness affects outer or middle ear, a patient may develop conductive hearing loss (CHL). This term is based on the fact that acoustic waves, which correspond to propagating alterations in air pressure, are conduced through these organs until they reach the cochlea in the inner ear. In contrast, patients whose cochlea is unable to convert pressure into electrical signals, or who present with problems transmitting those signals to the brain, are diagnosed with sensorineural hearing loss.

CHL may be congenital, i.e., present at birth, or, more commonly, acquired. Developmental defects associated with malformations of auricles, external auditory canal or ossicles are possible causes of congenital CHL, and they may be triggered by infectious diseases like rubella. The most frequent forms of acquired CHL are impacted cerumen, otitis media and otosclerosis. In case of the former, accumulated cerumen blocks the auditory canal and does impede sound waves to reach the tympanic membrane and the middle ear. In order for pressure waves to pass the middle ear, vibrations of the tympanic membrane have to be transmitted to the ossicles, which are located behind it. If a patient suffers from otitis media, the mobility of tympanic membrane and ossicles may be reduced, and consequently, sound perception may be impaired. Otosclerosis is typically diagnosed in adults and affects the ossicles in the middle ear.

CHL patients may experience symptoms besides hearing loss, e.g., a feeling of fullness or pressure in the ear and pain, but these largely depend on the underlying disease. Similarly, treatment and prognosis differ according to diagnosis. In general, drug therapy, reconstructive surgery as well as hearing aids may be considered possible therapeutic options.

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