Keratosis obturans is a relatively uncommon ear disease, where a dense plug of keratin, formed by abnormal accumulation of desquamated skin, in sheet like layers (lamellae), in the bony (deeper) part of the external auditory canal. [1] It is clinically diagnosed when removal of the debris shows silvery white peripheral matrix and causes excruciating pain. [2] [3]
Keratosis obturans was first properly described by Wreden of St. Petersburg in 1874, who differentiated this condition from impacted wax. [2] [4] Peipergedes and Behnke were the first to define the distinctions between the two. [2]
The most common symptoms are hearing loss and severe pain secondary to the accumulation of keratin in the ear canal. [5] Keratosis obturans has been classified into four grades depending on the severity of symptoms: [2]
Grade | Feature |
---|---|
Grade I | Mild pain ± ear block with the presence of accumulated keratin enveloped by a tightly adherent matrix; no discernible expansion of external canal |
Grade II | Moderate to severe pain ± conductive deafness; presence of accumulated keratin enveloped by a tightly adherent matrix with mild expansion of the bony canal in the presence of keratosis obturans. |
Grade III | Moderate to severe pain ± conductive deafness; presence of accumulated keratin enveloped by a tightly adherent matrix with expanded bony canal with granulation tissue at the osteo-cartilaginous junction. |
Grade IV | Presence of accumulated keratin enveloped by a tightly adherent matrix (grade III) with exposure of the mastoid air cells with/without facial nerve involvement |
The diagnosis of keratosis obturans is clinical. Differentiation between keratosis obturans and impacted wax is difficult at first presentation. It is diagnosed only when attempted removal of the substance causes excruciating pain, and shows silvery white peripheral matrix. When the matrix is peeled, new capillaries that were formed within the matrix rupture, resulting in bleeding. It is possible that these new capillaries are formed as a result of inflammation of the surrounding bony canal. [2]
Canaloplasty, where the ear canal is widened using grafts, was first proposed as the treatment for keratois obturans. However, with the migration of keratin within the canal, any amount of widening could not restore the migration of skin. Reconstruction of the bony canal with cartilage graft from temporalis fascia has showed some results. [6] But firstly preferred is cleaning with 1 percent acetic acid.
Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. Cholesteatomas are not cancerous as the name may suggest, but can cause significant problems because of their erosive and expansile properties. This can result in the destruction of the bones of the middle ear (ossicles), as well as growth through the base of the skull into the brain. They often become infected and can result in chronically draining ears. Treatment almost always consists of surgical removal.
Otitis media is a group of inflammatory diseases of the middle ear. One of the two main types is acute otitis media (AOM), an infection of rapid onset that usually presents with ear pain. In young children this may result in pulling at the ear, increased crying, and poor sleep. Decreased eating and a fever may also be present. The other main type is otitis media with effusion (OME), typically not associated with symptoms, although occasionally a feeling of fullness is described; it is defined as the presence of non-infectious fluid in the middle ear which may persist for weeks or months often after an episode of acute otitis media. Chronic suppurative otitis media (CSOM) is middle ear inflammation that results in a perforated tympanic membrane with discharge from the ear for more than six weeks. It may be a complication of acute otitis media. Pain is rarely present. All three types of otitis media may be associated with hearing loss. If children with hearing loss due to OME do not learn sign language, it may affect their ability to learn.
Earwax, also known by the medical term cerumen, is a waxy substance secreted in the ear canal of humans and other mammals. Earwax can be many colors, including brown, orange, red, yellowish, and gray. Earwax protects the skin of the human ear canal, assists in cleaning and lubrication, and provides protection against bacteria, fungi, particulate matter, and water.
An ear is the organ that enables hearing and body balance using the vestibular system. In mammals, the ear is usually described as having three parts: the outer ear, the middle ear and the inner ear. The outer ear consists of the pinna and the ear canal. Since the outer ear is the only visible portion of the ear in most animals, the word "ear" often refers to the external part alone. The middle ear includes the tympanic cavity and the three ossicles. The inner ear sits in the bony labyrinth, and contains structures which are key to several senses: the semicircular canals, which enable balance and eye tracking when moving; the utricle and saccule, which enable balance when stationary; and the cochlea, which enables hearing. The ear is a self cleaning organ through its relationship with earwax and the ear canals. The ears of vertebrates are placed somewhat symmetrically on either side of the head, an arrangement that aids sound localization.
Conductive hearing loss (CHL) occurs when there is a problem transferring sound waves anywhere along the pathway through the outer ear, tympanic membrane (eardrum), or middle ear (ossicles). If a conductive hearing loss occurs in conjunction with a sensorineural hearing loss, it is referred to as a mixed hearing loss. Depending upon the severity and nature of the conductive loss, this type of hearing impairment can often be treated with surgical intervention or pharmaceuticals to partially or, in some cases, fully restore hearing acuity to within normal range. However, cases of permanent or chronic conductive hearing loss may require other treatment modalities such as hearing aid devices to improve detection of sound and speech perception.
Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve. The pathway of the facial nerve is long and relatively convoluted, so there are a number of causes that may result in facial nerve paralysis. The most common is Bell's palsy, a disease of unknown cause that may only be diagnosed by exclusion of identifiable serious causes.
The ear canal is a pathway running from the outer ear to the middle ear. The adult human ear canal extends from the pinna to the eardrum and is about 2.5 centimetres (1 in) in length and 0.7 centimetres (0.3 in) in diameter.
Ear pain, also known as earache or otalgia, is pain in the ear. Primary ear pain is pain that originates from the ear. Secondary ear pain is a type of referred pain, meaning that the source of the pain differs from the location where the pain is felt.
Otitis is a general term for inflammation in ear or ear infection, inner ear infection, middle ear infection of the ear, in both humans and other animals. When infection is present, it may be viral or bacterial. When inflammation is present due to fluid build up in the middle ear and infection is not present it is considered Otitis media with effusion. It is subdivided into the following:
Surfer's ear is the common name for an exostosis or abnormal bone growth within the ear canal. They are otherwise benign hyperplasias (growths) of the tympanic bone thought to be caused by frequent cold-water exposure. Cases are often asymptomatic. Surfer's ear is not the same as swimmer's ear, although infection can result as a side effect.
Keratosis Pharyngis is a medical condition where keratin grows on the surface of the pharynx, that is the part of the throat at the back of the mouth. Keratin is a protein that normally occurs as the main component of hair and nails. It is characterized by the presence of whitish-yellow dots on the pharyngeal wall, tonsils or lingual tonsils. They are firmly adherent and cannot be wiped off. The surrounding region does not show any sign or inflammation or any other symptoms that make affect the rest of the body.
Ear drops are a form of topical medication for the ears used to treat infection, inflammation, impacted ear wax and local anesthesia. They are commonly used for short-term treatment and can be purchased with or without a prescription. Before using ear drops, refer to the package insert or consult a health professional for the amount of drops to use and the duration of treatment.
A perforated earhole is a prick in the eardrum. It can be caused by infection, trauma, overpressure, inappropriate ear clearing, and changes in middle ear pressure. An otoscope can be used to view the eardrum to diagnose a perforation. Perforations may heal naturally or require surgery.
In human anatomy, Prussak's space is the small middle ear recess, bordered laterally by the flaccid part of Shrapnell's membrane, superiorly by the scutum and lateral malleal ligament, inferiorly by the lateral process of the malleus, and medially by the neck of the malleus. From the neck of the malleus, the anterior malleolar fold and the anterior ligament arise, demarcating Prussak's space anteriorly. Ventilation of Prussak's space is only possible posteriorly above the posterior malleus fold.
Hearing loss with craniofacial syndromes is a common occurrence. Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely.
Otitis externa, also called swimmer's ear, is inflammation of the ear canal. It often presents with ear pain, swelling of the ear canal, and occasionally decreased hearing. Typically there is pain with movement of the outer ear. A high fever is typically not present except in severe cases.
Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal. This tumor is rare, with several names used in the past. Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), and ceruminous mucoepidermoid carcinoma.
An otic polyp is a benign proliferation of chronic inflammatory cells associated with granulation tissue, in response to a longstanding inflammatory process of the middle ear.
Endoscopic ear surgery (EES) is a minimally invasive alternative to traditional ear surgery and is defined as the use of the rigid endoscope, as opposed to a surgical microscope, to visualize the middle and inner ear during otologic surgery. During endoscopic ear surgery the surgeon holds the endoscope in one hand while working in the ear with the other. To allow this kind of single-handed surgery, different surgical instruments have to be used. Endoscopic visualization has improved due to high-definition video imaging and wide-field endoscopy, and being less invasive, EES is gaining importance as an adjunct to microscopic ear surgery.
Collaural fistula or cervico-aural fistula is a type of fistula whose openings are at external auditory canal and the neck, usually in the upper part of anterior border of sternocleidomastoid muscle. It occurs at birth because the defect is in the embryological branchial cleft. It is rare, and accounts for 8% of the branchial cleft anomalies, which is why it is sometimes misdiagnosed.