Symptoms & signs :

• audioanalgesia : reduction or abolition of the perception of pain by listening through a head set to recorded music to which a background of “white noise” has been added.
• hyperacusis / hyperacousia / hyperacusia / hyperakusis / acoustic or auditory hyperesthesia : exceptionally acute hearing, the hearing threshold being unusually low. It may or may not be accompanied by pain

Aetiology : peripheral facial (VII) nerve paralysis
• deafness / hearing loss

Epidemiology : permanent childhood hearing impairment (PCHI) is a congenital defect that affects 112 per 100,000 children worldwide.
Grading :
• partial deafness : hypoacusis / hypacusia / hypacusis / acoustic or auditory hypoesthesia slightly diminished auditory sensitivity, with hearing threshold levels above the normal limit so that the impairment is measurable in dB
• unilateral
• bilateral
• total deafness
• unilateral : anakusis / anacusis / anacousia / hemianacusia
• bilateral (cophosis)
• deaf-mutism : the absence both of the sense of hearing and of the faculty of speech
• prelingual deafness : deafness acquired before the development of speech, leading to mutism
• postlingual deafness : deafness acquired after the development of speech, leading to partial mutism only
Aetiology :
• conductive / conduction / transmissive hearing loss : mechanical blockage in the auditory system

Aetiology :
• chronic hearing loss
• pinna
• external auditory meatus (wax plug)
• atresia auris
• middle ear cavity
• otosclerosis
• deformity / malformation
• sudden hearing loss
• acoustic trauma deafness : noise-induced hearing loss caused by a single loud noise such as blast injury
• boilermakers' deafness : noise-induced hearing loss in boilermakers
• rupture of round window
• fracture of stapes
• fracture of otic capsule
• blow up of annular ligament of stapes followed by perilymphatic fistula
• perceptive / sensorineural / transmission hearing loss

Epidemiology : approximately 1.2 children per 1,000, or 40,000 children, will be diagnosed with moderate to profound inner ear hearing loss each year in the USA. 80% of the patients with hearing loss remain undiagnosed after their initial assessment based on standard diagnostic tests
Aetiology : while there are as many as 150 genes suspected of being linked to inner ear hearing loss, at this point, the gene chip targets 13 key genes.
• sensory hearing loss (cochlear deafness)
• presbycusis / presbiacusia : a progressive, bilaterally symmetric perceptive hearing loss occurring with age.
• sudden hearing loss
• sudden idiopathic hearing loss
• sudden secondary hearing loss

Aetiology :
• viruses
• mumps virus
• HHV-3 / VZV
• measles virus
• rubella virus
• Influenzaviruses
• bacteria
• tertiary syphilis (Treponema pallidum subsp.pallidum)
• suppurative labyrinthitis
• vascular (vascular deafness)
• dysmetabolic angiopathy in diabetes mellitus and renal failure
• occlusion of the internal auditory artery by spasm or thrombosis
• cochlear artery
• cochleovestibular artery
• fibrinolytic drugs
• stellate ganglion block
• barotrauma
• aerembolism during hyperbaric therapy
• allergic
• autoimmune
• panarteritis nodosa
• rheumatoid arthritis (RA)
• Wegener granulomatosis
• ototoxic : aminoglycosides (toxic deafness / ototoxic hearing loss)
Symptoms : unilateral hearing loss often associated with transient vertigo and persistent tinnitus
Treatment  must be instituted within 48 hours to be most effective:
• mandatory bed rest of 7-10 days.
• donnatal or tincture Belladonna q.i.d.
• nicotinic acid flushing q.i.d.
• histamine vasodilation using 2.75 mg of histamine in 200 mL of 5% dextrose in water I.V. at a rate to cause flushing, but not cause headache or significant drop in blood pressure.
• dextran, 500 cc per day (not with histamine)

systemic steroids, such as prednisone 60 mg daily x 7 days, tapered to zero over another 7 days.
Laboratory examinations : hearing threshold and speech testing are done at regular intervals, initially q.o.d., then at longer intervals in the ensuing weeks and months.
Prognosis : most patients have some residual hearing loss.
Therapy : gene therapy
• neural hearing loss (retrocochlear or central deafness)

Aetiology :
• midbrain (midbrain deafness : deafness dependent on injury of the fillet tract of the tegmentum)
• multiple sclerosis
• diffuse encephalitis
• meningomyelitis
• pons glioma
• psychogenous hearing loss
• eigth cranial nerve (nerve deafness) : acoustic neurinoma
• cortical auditory areas (cortical deafness)
• syphilitic meningitis
• tubercular meningitis
• cystic arachnoiditis
• mixed hearing loss is simply a combination of conductive and sensorineural components.
Laboratory examinations : Bilateral permanent childhood hearing impairment that is moderate, severe, or profound affects 1 in 750 children and is present at birth in > 80% of affected children^{ref1, ref2, ref3}. Such impairments are associated with impaired language acquisition, learning, and speech development^{ref1, ref2, ref3} (Allen TE. Patterns of academic achievement among hearing impaired students: 1974 and 1983. In: Schildroth AN, Karchmer AM, eds. Deaf children in America. San Diego, Calif.: College-Hill Press, 1986:161-206). Currently, screening for bilateral permanent childhood hearing impairment, with the use of transiently evoked otoacoustic emissions and automated measurement of auditory brain-stem responses, is recommended for all infants before the age of 3 months in the USA^ref, the UK (Quality standards in paediatric audiology. Vol. 1: guidelines for the early identification of hearing impairment. London: National Deaf Children's Society, 1994:1-10), and Europe^ref. The value of these recommendations is supported by studies showing that enrollment in an intervention program by nine months of age, as compared with later intervention, is associated with improvements in the verbal ability quotient by as much as 19 points^ref (equivalent to 0.5 to 0.6 SD) and that birth during periods in which universal hearing screening of newborns was in place is associated with a similar benefit^ref. The U.S. Preventive Services Task Force, however, has rated the quality of evidence linking early treatment or birth during periods with universal newborn hearing screening with improved language function as fair or poor^ref. In a previous controlled trial in the Wessex region of southern England, universal newborn screening increased the rate of early referral (i.e., before 6 months of age) for audiologic assessment of babies with bilateral permanent childhood hearing impairment, defined as a hearing loss of at least 40 dB hearing level (HL), on 2 assessments at least 12 months apart^{ref1, ref2}. Early detection of childhood hearing impairment was associated with higher scores for language but not for speech in midchildhood^ref
• if air conduction and bone conduction overlap => perceptive deafness
• if air conduction < bone conduction (air-bone gap) => transmissive deafness
• for simulated deafness
• Gault test : the patient's good ear is closed and a sound is made near the supposed bad ear; winking on the tested side indicates hearing.
• Erhard's test : a test for detecting simulated deafness
Grading :
• light deafness : 20 < HL < 40 dB
• intermediate deafness :  40 < HL < 60 dB
• severe deafness : 60 < HL < 80 dB
• deep deafness : HL > 90 dB
The average HL or pure-tone average (PTA) should agree with the SRT by + or -5 dB in the greatest proportion of hearing loss types. This relationship is very often used as an internal reliability check between pure-tone and speech test results. Someone attempting to feign a hearing loss generally would have a much better SRT than would be predicted from the pure-tone average. A hearing aid generally would not be considered for a patient, unless the loss had reached the "moderate" classification in the better ear.
Speech discrimination scores are more qualitative in their interpretation than are SRTs. The general concept is that in sensorineural hearing loss, the speech discrimination score will be directly proportional to the degree of system damage (cochlear hair cells or neural fibers). There is no quantitative way to predict speech discrimination ability from the pure-tone audiogram. Most persons with sensorineural losses have greater difficulty with speech discrimination in noise than in quiet. This is because the noise further reduces their ability to hear high-frequency consonants which carry the preponderance of speech information. Also, these patients may be very much annoyed by loud sounds (because of the recruitment phenomenon) and make less than ideal candidates for hearing aid use. Grading :
• > 80% on the W-1 lists are considered good
• 60-70% : fair
• < 60% : poor
The Deaf (with a capital 'D') are a tight-knit community. They view deafness not as a medical condition to be cured, but as a cultural identity to be celebrated. It's a view that the hearing find difficult to understand. But Kate Nelson, an actor with the Australian Theatre of the Deaf (ATOD) in Sydney is ideally placed to try to explain it. While some are born into the Deaf community, Nelson was born into a hearing family and has only come to Deaf culture relatively late in life. Nelson was born profoundly deaf, meaning that she could hear some sounds, but not enough to understand speech. She was brought up completely in the hearing world -- she wears hearing aids, can speak clearly and lip reads. She did not discover sign language until she was 21. Now in her 30s, Nelson's hearing is deteriorating further, and she finds herself increasingly identifying with the Deaf community. Sign language is much more than creating hand shapes, she points out. It incorporates facial expressions, posture and other body movements. When Nelson switches from speaking to signing, she becomes more animated, with her whole face and body conveying meaning and feeling. In the future, Nelson wants to explore more deaf issues, such as the desire of some deaf people to have deaf children^ref
Therapy :
• hearing aids
• aural rehabilitation comrises methods of amplification (provision of hearing aids and instruction in their use), and the maximisation of communication skills
• amplification
• provision of hearing aid
• instruction and counselling in its use
• maximisation of communication skills
• lip reading
• "learning to listen"
• speech conservation
• utilising visual clues
Patients must be counselled to understand that the aid does not allow them to hear normally but will enable them to have less difficulty understanding others. This will not happen overnight and patients need to be aware that it will take some time for them to get used to certain patterns of sound made louder. Where available, group orientation programmes have proved useful in increasing the benefits obtained by new hearing aid users. In addition to being supplied with hearing aids patients also need to be taught how to improve their communication skills. This may involve speech reading, learning to listen (listening is an active process unlike hearing), tuition to maintain good quality speech, and instruction on how to utilise visual clues such as facial and body gestures. Speech and language therapists have a significant role in the teaching of speech conservation and lip reading classes are taught by audiologists or at adult education centres.
• environmental aids :
• sound enhancement
• telecommunications
• television enhancement
• signal alerting
To distinguish words and sounds hearing aid users need the primary signal to be significantly louder than the background noise. This is feasible if the speaker is close and background noise is at a minimum. At home older people can ask visitors to come closer and switch off the television. However in restaurants, theatres, and noisy wards where the doctor may stand at the foot of the bed the listening environment can be very taxing. It is important for patients and doctors to realise that turning up the volume may make matters worse. Assistive listening devices or environmental aids are invaluable in such circumstances. These fall into 4 categories. An example of sound enhancement technology, a voice amplifier, was used in the study of Fook et al.. With sound enhancement technology the signal (such as speech from an individual or sound from a television) is transmitted directly to the ear of the individual via hardwire, radiotransmission, or infrared. The problems of environmental noise and distance are thus avoided. The voice amplifier or communicator is a an example of a hardwired system. These are relatively inexpensive and are ideal for use when being interviewed by professionals in a ward environment. Radiosystems may be used when speaker and listener are in different rooms, and the receiver may be incorporated into a behind-the-ear aid. Infrared systems are most suited for transmission from media such as televisions and stereo systems (as in concert halls). Built in amplifiers can be used to help those with hearing impairment use the telephone. Even despite these measures some elderly people still have difficulty discriminating speech over the telephone. The boom in home computers and email (not the sole province of the young!) has been a blessing in such circumstances. Telecaptioning, where dialogue is displayed across the bottom of the television screen, is another helpful visual adaptation, while a vibrating pillow, which notifies someone it is time to get up, or flashing doorbells are examples of a signal alerting devices. Audiologists who specialise in environmental aids can provide advice to individuals and institutions about all such devices, and they can be supplied to individuals through social services departments.
Web resources : Royal National Institute for Deaf People (RNID)
• otalgia / earache / otodynia : pain in the ear
• otalgia intermittens : otalgia of an intermittent type
• reflex otalgia / otoneuralgia : referred pain in the ear
• auricular pavillon is innervated by
• large auricular nerve (from cervical plexus) : neuralgia
• small occipital nerve (from CII-CIII roots)
• auriculotemporal nerve (from V (trigeminal) cranial nerve) :
• neck tumors
• TMJ dysfunction syndrome
• Eagle's syndrome
• oral cavity diseases (dental diseases (otalgia dentalis), oral cancers)
• external auditory meatus : auricular nerve (from X (vagus) cranial nerve) :
• laryngeal cancers
• hypopharyngeal cancers
• middle ear :
• Wrisberg's intermediate nerve (from VII (facial) cranial nerve) :
• tabes dorsalis (tabetic otalgia)
• Ramsay-Hunt syndrome / geniculate neuralgia or otalgia / herpes zoster auricularis or oticus (HZO) / otic neuralgia
• auriculotemporal nerve (from V (trigeminal) cranial nerve) :
• neck tumors
• TMJ dysfunction syndrome
• Eagle's syndrome
• oral cavity diseases (dental diseases (otalgia dentalis), oral cancers)
• Jacobson's tympanic nerve (from IX (glossopharyngeal) cranial nerve) :
• tonsillar abscess or neoplasms
• diseases of retromolar trigonus, tongue base, pharyngoepiglottal fold
• laryngeal cancers
• inner ear :
• diplacusia / diplacusis / double disharmonic hearing : the perception of a single auditory stimulus as 2 sounds, as a result of cochlear pathology
• binaural diplacusis : different perception of a single auditory stimulus by the 2 ears
• disharmonic diplacusis : a form of diplacusis in which a given pure tone is heard differently in the two ears.
• echo diplacusis : a form in which a sound of brief duration is heard in the one ear a fraction of a second later than in the other ear.
• monaural diplacusis / diplacusis monauralis : a form in which a pure tone is heard in the same ear as a split tone of 2 frequencies
• pseudotinnitus : sensation of pulsation or murmur sensed when lying at bed and/or lying the ear over the cushion. It may cause alarm in patients with panic disorder
• tinnitus (aurium) / acuphen : a noise in the ears, such as ringing, buzzing, roaring, or clicking
• tinnitus cerebri : tinnitus experienced as being inside the head rather than in an ear.
• objective tinnitus : a rare type of tinnitus that is audible to others, such as to an examiner with a stethoscope
• clicking tinnitus : a form of objective tinnitus in which the patient hears a clicking sound; it occurs with serous otitis media.
• Leudet's tinnitus : a form of objective tinnitus in which the patient hears a crackling sound, produced by involuntary contraction of an internal muscle, coinciding with a tic of fibers of the mandibular division of the trigeminal nerve.
• vascular tinnitus in cassa tympani
• clonus due to tension of malleus
• subjective tinnitus : the usual type of tinnitus, in which the sound cannot be heard by an examiner or measured by objective instruments
• nonvibratory tinnitus : produced by biochemical changes in the nerve mechanism of hearing.
• vibratory tinnitus : caused by transmission to the cochlea of vibrations from adjacent tissues or organs, most often from blood in vascular malformations. Occasionally the vibrations are loud enough to be heard by an examiner.
• pulsatile tinnitus : sound is rhythmic and synchronous with the heartbeat
Tonality :
• high tonality
• low tonality
Lasting :
• permanent
• intermittent
• relapsing
Aetiology :
• otogenous tinnitus
• outer ear
• wax plug
• exostosis of meatus acusticus externus
• middle ear
• purulent acute otitis media
• tympanosclerosis
• facial nerve palsy
• hemotympanum
• stapedium spasm
• tensor tympani spasm
• stapes artery persistence
• tympanic paraganglioma
• inner ear (99%)
• sudden hearing loss
• trauma and fractures of rocca petrosa
• otosclerosis
• acoustic trauma
• acute
• chronic (associated with hypoacusia)
• Meniére disease (associated with hypoacusia and vertigo)
• Meniére-like syndromes
• auricular zone
• labyrinthine capsule osteodystrophies
• retrocochlear (acoustic nerve)
• acoustic neurinoma
• ponto-cerebellar cystic arachnoiditis
• toxic or infectious acoustic neuritis (viral meningitides)
• diseases of bulbo-protuberantial region
• multiple sclerosis
• cancers
• cancers of cortical acoustic area
• angioma : sensation of murmur or pulsation even out from bed => auscultation at the level of eyeball or techa cranica
• extraotogenous tinnitus
• drugs
• quinine
• aminoglycosides
• acetylsalicylic acid
• loop diuretics
• cisplatin
• chloroform
• toxics
• aniline
• benzene
• CO
• phosphorus
• lead
• arsenic
• mercury
• sulfur
• nicotine
• cocaine
• caffeine
• ethanol
• diabetes mellitus
• gout
• liver failure
• renal failure
• dysthyroidism
• vasculopathies (associated with hypoacusia)
• systemic arterial hypertension
• systemic arterial hypotension
• arteriosclerosis
• anemias
• Neri-Barré-Liéou syndrome
• acoustic hallucinations
• dysequilibrium : any derangement of the sense of equilibrium
• dizziness / giddiness : a disturbed sense of relationship to space; a sensation of unsteadiness with a feeling of movement within the head.
• height vertigo : dizziness (not a true vertigo) felt on looking down from a high location.
• apoplectic or tenebric vertigo / scotodinia : dizziness with blurring of vision and headache
• pseudovertigo : any dizziness or other form of lightheadedness that resembles vertigo but does not involve a sense of rotation

Aetiology :
• hyperventilation (reproducible)
• panic disorder
• orthostatic systemic arterial hypotension
• never associated to nystagmus
• vertigo : an illusory sense that either
• objective vertigo : the objects seen by the patient seem to be moving around him
• subjective, rotar, rotatory or systematic vertigo : the patient has a sensation of turning round and round
• encephalic vertigo : a sensation of movement of tissues within the skull, as of the brain turning over and over.
• Lermoyez's syndrome : tinnitus and hearing loss preceding an attack of vertigo and then subsiding after the vertigo has become established
It is rotatory (differential diagnosis from lipothymia and obnubilation), has acute-onset and may last hours. The difference between objective and subjective has no prognostic meaning
Aetiology :
• central vertigo : vertigo due to disease of the CNS
• cerebral or organic vertigo : vertigo resulting from a brain lesion, such as a cerebellar infarct
• angiopathic or arteriosclerotic vertigo : vertigo due to arteriosclerosis of cerebral vessels, particularly the vertebral or basilar arteries (vertebrobasilar insufficiency)
• endemic paralytic vertigo : vestibular neuronitis.
• epidemic vertigo

Aetiology : associated with either an URT infection (in the autumn) or gastroenteritis (in mid-January)
Symptoms & signs : acute onset of severe dizziness, nausea and vomiting, a slight fever, headache, and asthenia, with a duration of several weeks to months.
Laboratory examinations : caloric and audiological tests usually are normal, but spinal fluid may show some lymphocytic cells.
Treatment : antivertiginous and antinausea drugs such as Dramamine, Vontrol, Torecan, and Tigan.
Prognosis : recovery is usual
• epileptic vertigo : vertigo that accompanies vertiginous epilepsy, usually as part of an aura
• primary, peripheral or vestibular vertigo : vertigo due to disturbances of the vestibular system
• labyrinthine vertigo / recurrent aural vertigo / Meniere's disease
• laryngeal vertigo / cough or tussive syncope
• toxic vertigo : vertigo as a result of ototoxicity
• gastric or stomachal vertigo / vertigo ab stomacho laeso : a form associated with disease or disorder of the stomach
• alternobaric or pressure vertigo : a transient vertigo sometimes affecting those such as caisson workers and members of airplane crews who are subjected to large, rapid variations in barometric pressure
• ocular vertigo : a form due to eye disease, especially to paralysis of or lack of balance in the eye muscles, uncorrected myopia, diplopia
• pilot's vertigo / spatial disorientation : a condition in which a pilot or other air crew member is unable to determine accurately his spatial attitude in relation to the surface of the earth; it occurs only in conditions of poor visibility or when vision is otherwise restricted and results from vestibular illusions
• Tullio's phenomenon : vertigo induced by high-intensity sounds.
• mechanical vertigo : vertigo due to long-continued turning or vibration of the body
• kinetosis : any disorder caused by unaccustomed motion, characterized by nausea and vomiting, anorexia, headache, malaise, salivation, drowsiness, lethargy, and cold sweats
• riders' vertigo / motion sickness : sickness caused by motion experienced in any kind of travel, such as ...
• mal de mer / sea sickness : caused by the motion of a ship
• train sickness : produced by the motion of trains
• car sickness : produced by the motion of automobiles or other vehicles
• aerial, air or aviation sickness : due to change in air pressure and to the movements experienced in an airplane
• space adaptation syndrome / space sickness : a form of motion sickness occurring in a weightless environment during space flight. It is probably caused by conflicting signals concerning motion from the otolith (whose proper function depends on the presence of gravity) and the visual system (which affects the autonomic nervous system)
Therapy : scopolamine
• somatosensorial vertigo : due to peripheral neuropathies associated to diseases of the vestibular apparatus
• posttraumatic vertigo : vertigo following some injury, such as fracture of the temporal bone, whiplash (cervical vertigo), or lesions of the cerebral cortex or cerebellum.
• residual vertigo : 1. vertigo in the aftermath of some disease process.  2. vertigo associated with motion, resulting from hypofunction or absence of vestibular sensory or neural elements
• essential vertigo : vertigo whose cause is unknown.
• positional vertigo / postural vertigo : vertigo associated with a specific position of the head in space or changes in the position of the head in space
• benign positional or postural vertigo / benign paroxysmal positional vertigo (BPPV)

Aetiology :
• cupulolithiasis that causes exaggerated movement of the endolymph
• mal de debarquement syndrome is an uncommon illness affeting travelers after debarkation. It is characterized by an unsteadiness and lurching., without dizziness, which may not resolve for months or years. It is a diagnosis of exclusion, based on a characteristic history, and paucity of findings on neurologic and ENT clinical examination, although in some cases positional nystagmus has been detected during electronystagmography. There are references in the literature as well to a similar and more common entity, which typically lasts a matter of hours after debarkation. Some have suggested the use of the term persistent mal de debarquement syndrome to differentiate the two. The great majority of sufferes are women, and the age range is primarily from the fourth decade into the eighth, with a few younger patients. The duration of the unsteadiness varies from 4 weeks to 5 years

Therapy : medications used to treat dizziness or seasickness are typically of no help. Benzodiazepines have been reported to afford a degree of relief in some patients. Vestibular rehabilitation has been suggested as a useful treatment. Further cruises should be avoided once the illness has first manifested itself, even if that first experience is of short duration.
Symptoms & signs : onset of nystagmus and vertigo occur when the head moves to a certain position such as with one ear down, and relieved by returning to an upright position. There usually is a latent period of several seconds, and the nystagmus fatigues with repeated testing. Most cases have normal calorics and audiological examinations. Symptoms abate in about 8 weeks, but they may recur or even last for years.
Laboratory examinations : Hallpike's maneuver (the examiner turns the head of the seated patient to one side and pulls the patient backwards into a supine position with the head hanging over the edge of the examining table; the patient then looks straight ahead and the examiner observes for positional nystagmus, which is indicative of benign positional vertigo.
Therapy :
• avoidance of the position that creates the nystagmus and vertigo
• liberatory maneuvers
• Lempert or Baloh "barbecue" maneuver in BPPV of the horizontal semicircular canal
• Vannucchi "forced position" maneuver in elderly or overweight patients and in those with reduced mobility
• Semont maneuver, moving the patient from a seated position to a position on the right or left side, depending the pathology and type of vertigo (geotropic/apogeotropic)
• disabling positional vertigo : constant positional vertigo or dysequilibrium and nausea and vomiting in the upright position, without hearing disturbance or loss of vestibular function.
• horizontal vertigo : positional vertigo experienced when a person lies down.
• vertical vertigo : positional vertigo experienced when a person is in an upright position.
• lateral vertigo : vertigo caused by rapidly passing a row of similar objects, such as a fence or a series of pillars.
Symptoms & signs :  it is associated with a sense of lateral pull (due to connections to vestibulospinal nuclei), consciousness of movements and head position (due to connections to thalamocerebral nuclei), associated to nystagmus (due to connections to nuclei of III, IV, ad VI cranial nerves), nausea and vomiting, pallor, salivation, and sweating (due to connections to dorsal vagal nucleus tractus solitarii), eventually deagness, tinnitus or neurological deficiencies
• paralyzing vertigo : vertigo so severe that the patient is afraid to move
• nocturnal vertigo : a sensation of falling occurring as the subject is going to sleep.
• paroxysmal vertigo : vertigo occurring in sudden, brief attacks
• benign paroxysmal positional vertigo (BPPV)
• benign paroxysmal vertigo of childhood : a form of paroxysmal vertigo occurring in young children otherwise in good health; sudden attacks are accompanied by pallor, sweating, and immobility and less often by nausea and vomiting and nystagmus.
Timing : status vertiginosus : prolonged vertigo
• single episode :
• current
• in the past : labyrinthitis (in absence of neurological signs or deafness with tinnitus)
• recurrent :
• benign (Meniere's disease, neuronitis, BPPV) : mixed nystagmus not inhibited by fixation of sight, more intense; strong and latent (40 seconds) response to Hallpike maneuver, with mixed and not repeatable nystagmus
• malignant (vestibular epilepsy) : pure nystagmus, mild and repeatable response to Hallpike maneuvers, with immediate and pure nystagmus
Prognosis :
• physiological : poor coordination (kinetosis, altitude vertigo, eye vertigo), head extension
• pathological :
• injuries of sensory apparatus : labyrinthitis, Meniere's disease, BPPV
• injuries of afferent pathways : acoustic neurinoma, meningitis, aneurysms, gangliar or vestibular neuronitis (VZV), recurrent benign neuronitis
• central injury (pons and cerebellum) : vascular injury, demyelination, neoplasms, trauma, other neurological lesions
Differential diagnosis with other lightheadedness : orthostatic systemic arterial hypotension, walking disorders (peripheral neuropathies, myelopathies, cerebellar ataxia, Parkinson's disease combined with visual deficiencies); Krishaber's disease / cerebrocardiac syndrome : a syndrome characterized by tachycardia, insomnia, lightheadedness or vertigo, hyperesthesia, and a feeling of emptiness in the head
Differential diagnosis between central and peripheral nystagmus is made on the basis of : kind of nystagmus, intensity (more severe in peripheral nystagmus, auditory disturbances (deafness, tinnitus : if it is not Meniere disease, it is probably acoustic neurinoma), occurrence of other neurological symptoms (e.g. lack of abdominal reflexes => CNS, motor disorders => vestibular nuclei)
Therapy : antivertiginous drugs and antiemetic drugs
• aural, labyrinthine or vestibular nystagmus
• otorrhea : a discharge from the ear
• mucous otorrhea due to tympanic membrane perforation
• serous otorrhoea due to outer ear eczema
• purulent otorrhea / otopyorrhea due to furunculus, acute otitis media, cholesteatoma
• hematic otorrhea / otorrhage due to ...
• perforation of tympanic membrane
• fracture of rocca petrosa with perforation of tympanic membrane
• chronic otitis media
• mixed otorrhea
• serohematic otorrhea in bullous-hemorrhagic myringitis
• cerebrospinal fluid otorrhea / otoliquorrhea : escape of CSF through the external auditory meatus due to fracture or other pathology of the temporal bone leading to CSF fistula.
• otohematoma due to fracture of rocca petrosa without perforation of tympanic membrane
• sensation of fullness or pressure in the ears

• acoumetry
• vocal acoumetry
• aphonous voice
• conversation voice
• high tones
• low tones
• diapason acoumetry (pure tones)
• Rinne test : a hearing test performed, with the opposite ear masked, with tuning forks of 256, 512, and 1024 Hz by alternately placing the stem of the vibrating fork on the mastoid process and 1/2 inch from the external auditory meatus until it is no longer heard at one of these positions. When air conduction is greater than bone conduction (positive Rinne test), it indicates normal hearing or sensorineural hearing loss. When bone conduction is greater than air conduction (negative Rinne test), it indicates conductive hearing loss
• Weber acoumetry : placed on forehead, symmetric or asymmetric hearing
• Schwabach's test : a hearing test performed, with the opposite ear masked, with tuning forks of 256, 512, 1024, and 2048 Hz, alternately placing the stem of the vibrating fork on the mastoid process of the patient and that of the examiner (whose hearing should be normal) until it is no longer heard by one of them. The result is expressed as “Schwabach prolonged” if heard longer by the patient (indicative of conduction deafness), as “Schwabach shortened or diminished” if heard longer by the examiner (indicative of sensorineural deafness), and as “Schwabach normal” if heard for the same time by both.
• Bing test : a vibrating tuning fork is held to the mastoid process and the auditory meatus is alternately occluded and left open: changes in loudness (positive Bing) are perceived by the normal ear and in sensorineural deafness, but in conduction deafness no difference is perceived (negative Bing).
• audiometry
• basic hearing tests
• the ability to hear a vibrating tuning fork (Weber test) may help the doctor to determine if hearing loss is transmissive (due to a middle ear problem) or neurosensorial (due to nerve damage) in origin
• audiometers => audiogram
• manual, self-recording
• microprocessor controlled
• pure-tone audiometry / liminar tonal audiometry : the patient is asked to respond whenever he hears a pure tone, increasing or decreasing intensity of the signal. The lowest intensity at which the patient responds at a particular frequency is called the hearing level (HL). HLs are determined at
• octave frequencies 250, 500, 1000, 2000, 4000 and 8000 Hz
• half-octave frequencies of 3000 and 6000 Hz.
Each continuous tone is presented for a period not exceeding 1". Intermittent (pulse) tones are also frequently used, especially in patients where tinnitus is present. There will be several tone presentations at a particular frequency before the HL is recorded on the audiogram. Masking noise generated within the audiometer is used when one ear needs to be isolated from the other in order to get a correct threshold measurement for the test ear
• broad-frequency band noise is most efficient for masking pure-tones
• narrow-frequency band noise
In a situation where one ear of the patient is "dead", incorrect information would be obtained for the nonfunctional ear if masking were not used for the good ear. By air conduction measurement, the nonfunctional ear would yield HL's around 50 to 60 dB. This is due to a phenomenon called "crossover." Even though the signal is presented at the nonfunctional ear, it is heard by the good ear primarily by direct energy transmission through the head from the vibrating earphone cushion. The head creates about a 50 to 60 dB "barrier" between ears. If proper masking noise is applied to the good ear in the case mentioned, then a correct determination of a profound hearing loss would be made. Masking of the contralateral ear is done more frequently in BC than in AC. This is because interaural attenuation, while about 50-60 dB for AC, is practically nonexistent (0-5 dB) for BC. In the previous example of the "dead" ear, a BC measurement without proper contralateral masking would have shown normal BC hearing in the nonfunctional ear due to the low (0-5 dB) crossover levels. In general, pure-tone HLs are determined for both ...
• air conduction (AC) (earphones)
• left ear = x (if masked square)
• right ear = o (if masked triangle with upward apex)
• bone conduction (BC) (electromechanical vibrator). HLs are determined at 250, 500, 1000 and 2000 Hz (Weber), Gellé, Bing and occlusion index. Since it requires more energy to drive a mechanical vibrator than an earphone, the maximum hearing loss that can be measured for BC is less than for AC, (e.g., 70 dB for BC and 110 for AC). Care should be taken to place the vibrator on the mastoid without contacting the pinna. This is to ensure that responses at low frequencies are auditory and not tactile in nature.
• left ear = < (if masked triangle with leftward apex)
• right ear = > (if masked triangle with rightward apex)
• speech audiometry
• speech reception threshold (SRT) : the amplitude at which the patient can repeat back approximately 50% of the 2-syllable words presented to him. There are 6 word lists, each list being a different scrambling of the same 36 words. The most widely used form is CID Auditory Test W-1
• phonetically-balanced (PB) score / PB Max : the percentage of 50 single-syllable words the patient can correctly repeat back is determined. When these word lists (24 lists with 50 words each, and 200 words in the corpus) were developed in the late 1940's, it was believed that the phonemes in each 50-word list had to have the same proportionate frequency of occurrence as that in everyday English, in order for the test to be valid. This was later shown to be unnecessary, but the terminology "PB" still remains today. The relationship between word discrimination and amplitude (SPL) is called articulation curve or performance intensity (PI) function. The PB words, the most widely used form being CID Auditory Test W-22, are presented at a level of 40 dB above the SRT in routine use. Since this represents a supra-threshold presentation, masking noise is almost always used in the contralateral ear. It is at this amplitude or sensation level (SL) that most patients would achieve maximum performance. However, there are instances where this is not the case. So, ideally, a performance intensity function would be generated by presenting the monosyllabic word lists at a variety of sensation levels. A phenomenon called roll-over is  is characterized by a wornsening of discrimination as loudness is increased. This finding is characteristic of retrocochlear disorders (e.g., acoustic neuroma) and to a lesser extent Meniere's syndrome.
Often speech discrimination testing is done in a noise background. A variety of word lists and test formats are used for this purpose. The basic concept behind this is to provide a more realistic environment in the measurement of speech discrimination. The signal to noise ratio (S/N) is expressed in dB, and represents the number of dB the average signal (speech in this case) is above or below the level of the noise.
• threshold tone decay test (TDT) is a pure-tone, supra-threshold test usually done at 4,000 Hz first, and, if positive, the test frequency is dropped by octaves until 500 Hz is tested. The tone is presented at 5 dB sensation level (SL) for 1 minute. If the patient can hear the tone for the entire period at the same level, the test is negative. If the level of the tone has to be raised by 20 or more dB above the starting level, the test is positive. The TDT is a measure of auditory adaptation and is considered a screening test for retrocochlear pathology. If the test is positive, other, more detailed, tests would be done in order to help establish the reason for the abnormal adaptation and the site of the lesion. The suprathreshold adaptation test (STAT) is also frequently used. The test is positive if a high level (e.g., 100 dB) tone cannot be heard over a 60" period.
• advanced hearing tests
• short increment sensitivity index (SISI) : tones of 1- to 5-decibel increments in intensity and lasting 0.5 second are superimposed on a continuous (carrier) tone of the same frequency at random intervals, the carrier tone being 20 decibels above the speech reception threshold. Pure-tones (250, 500, 1000, 2000, 4000 or 6000 Hz) are presented at 25 dB SL. The result is expressed in terms of % correct identification out of 20, 1-dB increments, added to a reference pure-tone level. A high % correct response is indicative of a cochlear pathology as only patients with cochlear damage can detect these increments
• alternate binaural loudness balance (ABLB) test is 1 of 2 direct tests of a phenomenon called recruitment. Recruitment is an abnormal growth of loudness in which soft sounds are not heard while loud sounds are perceived to be as loud as in a normal ear. The presence of recruitment narrows the dynamic range of hearing significantly and is characteristic of a cochlear (sensory) pathology. In order to do this test, it is necessary for hearing to be within normal limits in the contralateral ear at the same frequency at which the test is being done in the poorer ear.
• Bekesy audiometry is an advanced site-of-lesion test and is a special form of the more routine, self-recording audiometry procedure. The patient is asked to track his pure-tone threshold by means of a response button, first for a pulsing tone and then for a continuous tone. Either a discrete frequency or continuous frequency tracing can be generated. The audiograms are traced on the same graph. The audiogram is then categorized according to the relationship between the pulsed and continuous tracings. There are 5 recognized types of Bekesy audiograms
• type I Bekesy tracing
• type II Bekesy tracing
• type III Bekesy tracing
• type IV Bekesy tracing
• type V Bekesy tracing in patients attempting to feign a hearing loss. It has essentially a type IV configuration, except that the continuous and pulsed tracings are reversed (i.e., hearing for the continuous tone is shown as being better than hearing for the pulsed tone). Perceptually, the loudness of a supra-threshold tone is greater for a continuous signal than for a pulsed signal. What the patient is actually doing is tracing an equal loudness contour for each type of signal. It should be noted that this test can be done with some of the self-recording screening audiometers in the fleet. All that is necessary is to be able to select either a pulsed or continuous tone on the instrument. In a clinical setting, the LOT test, mentioned previously, would be done and would in most cases accentuate the dB separation between the 2 tracings.
Bekesy testing is much more effective in identifying sensory problems than it is in identifying retrocochlear problems.
• Bekesy comfortable loudness technique (BCL)
• reverse Bekesy tracings
• lengthened off time (LOT) test is also used where malingering is suspected. This is basically a Bekesy test with the period between pulses lengthened and unequal to the duration of the pulse itself, (e.g., 800 ms off and 200 ms on). This temporal pattern magnifies the difference between the pulsed and continuous tracings, making the identification of possible malingering easier.
• electrophysiological noninvasive measures of auditory function that involve computer averaging of the auditory system's electrical response to clicks or tone pips used in cases of functional (nonorganic) hearing loss or psychogenic problems (noncollaborative babies)
• electro-cochleography (EChocG)
• brainstem auditory evoked potentials (BAEP)
• electroencephaloaudiometry (EEA)
• sensitized speech tests are tests in which the auditory stimulus is speech that has been altered, either in the amplitude, temporal, or frequency domain. They are used when a central auditory disorder is suspected. "Central" is defined as a site of lesion somewhere in the brainstem or cortical auditory areas. Pure-tone tests are not sufficiently complex in nature to identify these lesions. In general, as the site of lesion proceeds centrally in the auditory system, the tests to identify it need to become more and more complex in structure. The flight surgeon's contact with this type of test information would be quite rare in the active duty population. It would more likely occur in the retired or dependent groups.
• binaural distorted speech tests : tests of the capacity of the CNS to coordinate 2 incoming speech patterns, each of which is incomplete.
• monaural loudness balance (MLB) test : a test to determine recruitment in bilateral sensorineural deafness; the loudness sensation at impaired frequencies is compared with that at normal frequencies
• impedance audiometry / impedancemetry measures various mechanical aspects of the middle ear. A probe tip is inserted into the test ear and an airtight seal is obtained before testing begins. A standard earphone is placed over the contralateral ear. There are 3 holes in the probe tip :
• to introduce a pure tone with n = 220 Hz into the space created between the tip of the probe and the tympanic membrane
• to a microphone which measures the SPL in the space
• to introduce air into the space (either negative or positive pressure relative to normal atmospheric pressure can be achieved)
4 general measurements are obtained using the impedance audiometer
• Metz test / acoustic reflex test : measurement of the acoustic reflex threshold by testing for contraction of the stapedial muscle in response to sound; used to differentiate between conductive and sensorineural deafness and to diagnose acoustic neuroma. Stapedial reflex thresholds - ipsilateral and contralateral - are determined by introducing an acoustic stimulus at various amplitudes through the earphone. When the amplitude is high enough (75 dB above the threshold for that frequence for pure tones (usually 500, 1000 or 4,000 Hz) : maybe the device cannot produce such SPL), the stapedius will contract. Since this is a consensual reflex that stiffens both tympanic membranes, it can be monitored on the opposite side as a change on the compliance meter indicating lowered compliance (i.e., higher impedance). It lacks in otosclerosis, tympanosclerosis, and facial nerve palsy before stapedial ramus.
• static compliance is calculated from the difference in volume between the resting compliance measurement and the measure taken with 200 MMW (equivalent) pressure - the inverse of impedance and expressed in cc or mL
• tympanogram : a dynamic plot of compliance (inversely related to acoustic impedance) as a function of externally applied pressure (from -400 to +200 mmH₂O to avoid mechanical damage) automatically generated by the machine and recorded on an associated plotter. This indirectly measures pressure in middle ear as compliance is maximal when P_{middle ear} = P_applied (expressed in MMW or daPa)
• type A (normal) tympanogram : bell-shaped with peak = 0.7 mL at P = P_atm.
• type B tympanogram : bell-shaped with peak = 0.4 mL at P = P_atm (seen in tympanosclerosis and secretory otitis media)
• type C tympanogram : line with peak = 0.2 mL at P = - 400 mmH₂O, due to Eustachian tube dysfunction
• type D tympanogram : enlarged and flattened curve with peak = 0.2 mL at P = - 200 mmH₂O
• type E tympanogram : curve with peak = 0.5 mL at P = - 300 mmH₂O
• type F tympanogram : curve with peak = 0.7 mL at P = + 100 mmH₂O
• type G tympanogram :curve with first peak = 0.7 mL at P = -75 mmH₂O, incisure = 0.3 mL at P = P_atm, and second peak = 0.9 mL at P = + 50 mmH₂O
• type H tympanogram : bell-shaped with peak = 0.95 mL at P = P_atm (seen in interruption of ossicle chain)
Exam may be painful if patient has otitis externa and makes no sense if he has perforation of tympanic membrane
• Andersson test / reflex decay test :
• alternate binaural loudness balance (ABLB) test : comparison of the intensity levels at which a given pure tone sounds equally loud to the normal ear and the ear with hearing loss; done to determine recruitment with unilateral sensorineural loss.
• alternate loudness balance test : a hearing test done with pure tones that compares the loudness perceived in one ear with that perceived in the other, with the frequency kept constant.
In addition to defining middle ear problems, impedance audiometry can yield useful information in helping to identify the following conditions :
• acoustic neuroma, through the stapedius reflex decay test
• site of facial nerve palsy
• Eustachian tube status
• fistula
• functional hearing loss, volitionally or psychogenically based
• recruitment, comparing stapedial reflex thresholds to the auditory thresholds
• vestibulometry
• revealed vestibular nystagmus
• latent nystagmus : nystagmus which occurs only when one eye is covered.
• positional nystagmus : that which occurs, or is altered in form or intensity, on slow assumption of certain positions of the head without practicing head rotations

Signs : Cawthorne, Dix, and Hallpike method : with the patient's eyes in the straight ahead position, the patient is rapidly placed supine with the head hanging over the edge of the table, and the eyes are observed for 60". The patient is then raised up and then returned to the hyperextended position with the head in one direction, again for 60". The procedure is repeated in the opposite direction. The position should be held until the nystagmus subsides
• permanent positional nystagmus : it persists for > 60" and has central origin
• barbiturates : positional nystagmus
• [ethanol]_plasma > 0.02% : positional alcohol nystagmus (PAN)
• PAN I begins about 30' after ingestion, as the blood alcohol peaks, and lasts approximately 3 and a half hours. The nystagmus is always in the direction of the gaze or toward the position of the head, for examble, a right-beating nystagmus appears with right gaze, head turned to the right side or if the right of the patient's head is down in the lateral position. There is a gradual diminution after the peak and an intermediate period of about 1.7 hr in which there is no nystagmus.
• PAN II begins approximately 5 hours after the initial ingestion, and the nystagmus is in the opposite direction of the gaze or lateral head position and persists for several hours after the blood alcohol level has disappeared. PAN II is greatest when the "hangover" symptoms are greatest.
• paroxysmal positional nystagmus : canalolithiasis in posterior > horizontal > anterior canals
• benign paroxysmal positional vertigo (BPPV)
• cervical nystagmus
• provoked vestibular nystagmus
• rotatory nystagmus : even in presence of disease, the 2 labyrynths are symmetric and answers are overlapped up to a = 15°/s²
• head- shaking test (20 passive head oscillations in 10") under Frenzel eyeglasses => uncompensated system with unknown aetiology
• rotating seat have upper limit for a = 18°/s², only 1 or 2 measures are meaningful
• caloric nystagmus : rotatory nystagmus induced by irrigating the ears with 250-300 mL of warm or cold water for 40" (Hallpike-Fitzgerald technique) or air when patient has head inclined downward by 30° to place the lateral semicircular canals (the most used in vivo) on horizontal plane (Brunig position)
• caloric test / Búrúny's test, sign, or symptom / nystagmus test (for ocular and vestibular functioning) : irrigation of the normal ear with warm water produces rotatory nystagmus (caloric nystagmus) toward the irrigated side; irrigation with cold water produces similar nystagmus away from that side
• cold water (30°C) => ampullifugal current => inhibitory current for 120"
• warm water (44°C) => ampullipetal current => excitatory current for 100"
The fast component of nystagmus is directed toward the prevalent labyrinth.
• labyrinthine prevalence (LP) = (warm water_{right ear} + cold water_{right ear}) - (warm water_{left ear} + cold water_{left ear})
• directional prevalence (DP) = (warm water_{right ear} + cold water_{left ear}) - (warm water_{right ear} + cold water_{left ear})
• total system reflectivity (TR) = warm water_{right ear} + cold water_{right ear} + warm water_{left ear} + cold water_{left ear}
Normal values are within 1s from average (i.e. ±15% for LP and ±18% for DP)
• galvanic or electrical nystagmus : a vestibular nystagmus caused by electrical stimulation of the labyrinth of the inner ear
• optokinetic nystagmus
• pendular nystagmus
• pneumatic nystagmus
• harmonic acceleration test (for vestibulo-ocular reflex): rotation of a patient seated in a chair in complete darkness, with monitoring of eye movements; with normal vestibulo-ocular reflexes the eyes will undergo rotatory nystagmus to the same degree in both eyes in the direction opposite to that of the rotation.
• Hitzig test (for vestibular apparatus): the positive electrode of a galvanic current is applied just in front of the ear being examined while the negative electrode is held in the patient's hand, the patient standing with feet together and eyes closed. A current of 5 mA causes a leaning toward the positive pole in normal persons
• stabilometry
• static stabilometry
• dynamic stabilometry

• panotitis : an inflammation of all the parts or structures of the ear
• diseases of outer ear
• congenital malformations of the outer ear
• coloboma :
• fistula auris congenita / congenital preauricular fistula :  an epidermis-lined tract communicating with an ear pit on the skin; it results from imperfect fusion of the first and second branchial arches in formation of the auricle
• atresia auris / aural atresia : obstruction of the external acoustic meatus; it may be either congenital or acquired through trauma or disease
• anotus / anotia : congenital absence of the external ear(s)
• microtia : gross hypoplasia or aplasia of the auricle (pinna) of the ear, with a blind or absent external acoustic meatus
• polyotia : the condition of having more than 2 ears.
• triotus : an individual with a supernumerary ear
• melotia : a developmental anomaly characterized by displacement of the ear onto the cheek
• macrotia : abnormal enlargement of the pinna of the ear.

Aetiology : fragile X syndrome
Aetiology : thalidomide during pregnancy
• otohematoma / auricular hematoma / hematoma auris : hematoma of the perichondrium of the auricle, most often on the superior lateral surface, resulting in a hematoma.

Aetiology : trauma
Therapy :
• in the early stages, aspiration of the blood using sterile technique with a large 14-gauge needle. A pressure dressing is then applied.
• for large, chronic, or recurrent hematomas, incision and drainage are recommended. The entire ear is disinfected and anesthetized, a large curving incision is made through the skin of the scaphoid fossa following the curvature of the helix. The hematoma is then evacuated using sterile technique. In some chronic or recurrent cases, instead of blood there is only xanthochromic fluid. Some surgeons advise curettement of the cyst walls. A thin rubber drain is inserted the length of the hematoma sac and then withdrawn over the next 2-3 days. Fine nylon or silk interrupted sutures about 1 cm apart are used for closure of the incision, and a pressure dressing is applied. Through and through monofilament sutures tied over soft sponges for direct pressure are also effective.
Prognosis : left untreated, the slow absorption of blood, loss of nourishment to the cartilage, and infection may lead to a deformed auricle or "cauliflower ear".
• lacerations

Therapy : avoid excessive debridement, approximate the cartilage with perichondrial sutures on both sides, use white silk or cotton for buried sutures on the thin lateral surface, and use good splinting with a pressure dressing. Even though a portion of the ear may look nonviable, it is usually best to clean, approximate, splint, and then wait for demarkation before final debridement. Through and through sutures tied lightly over cotton rolls, etc., may be used for splinting as well as the pressure dressing. Exposed cartilage or subcutaneous tissue should be covered with fine mesh gauze impregnated with an antibiotic ointment. Adequate antibiotic coverage is strongly recommended.
• foreign bodies in the external canal. Anything that will fit has been found in the external auditory canal. Some of the foreign bodies are inert and cause no problems or symptoms, but most commonly they produce a canal blockage with a mild decrease in hearing, itching, infection, and drainage or even a cough, mediated through pressure on the twig of the tenth cranial nerve (Arnold's nerve). Removal of round objects is most difficult, and it is best accomplished with a fine, blunt, right angle hook that can be inserted past and behind the object. Special cup or serrate jaw forceps can also be used. Hard, sharp, and large objects should be softened, if possible, and removed with care, protecting the canal from trauma and bleeding. Compressed strips of Gelfoam^® may be tried on sharp corners. Friable or adherent material may require loosening or dissolution before removal. Debrox^® (carbamide peroxide) works well in most cases. A fine stream of water, 2% acetic acid in water, or alcohol is used for irrigation and directed under direct vision and controlled pressure. Hydroscopic objects such as corn or peas may swell if saturated with water, therefore, alcohol irrigation or forcep/hook removal is recommended. Live insects should be killed rapidly by flooding of the canal with ether, alcohol, or oil and then removed with forceps. After the object has been removed, the canal should be suction-cleaned or wiped dry and eardrops or ointments applied for treatment of any possible tissue trauma or infection.
• hypertrichosis pinnae auris / hairy ears : hypertrichosis involving the pinna of the ear; it may be a Y-linked or an autosomal dominant trait
• outer ear eczema
• psoriasis
• humidity
Therapy : H₃BO₃ 2%, boric alcohol
• outer ear erysipela
• outer ear cancers are the same as for other skin areas
• chondrodermatitis nodularis chronica helicis / Winkler's disease : a small, painful, scaly, nodular lesion on the helix of the ear, colored skin color, grayish, or translucent; multiple lesions may occur along the rim of the ear. Seen usually in middle-aged men and more often on the right ear
• keratosis obturans : obstruction of the external auditory meatus by a mass of desquamated epithelium and cerumen
• otitis externa / external otitis

Aetiology : maceration of the skin from water or other fluid drainage and trauma, often self-inflicted, when trying to scratch or clean wax from the canal. Infection may take place in cartilage, hairs and sebaceous and ceruminous glands
• acute otitis externa (AOE) / swimmer's ear / tank ear
• HHV-3 / VZV (Ramsay-Hunt syndrome / geniculate neuralgia or otalgia / herpes zoster auricularis or oticus (HZO) / otic neuralgia)
• bacterial AOE
• circumscribed AOEs / furuncular otitis externa
• Staphylococcus aureus (7.8%)
• Staphylococcus epidermidis (9.1%)
• Microbacterium otitidis(6.6%)
• Microbacterium alconae(2.9%)
• Staphylococcus caprae(2.6%)
• Staphylococcus auricularis(2%)
• Enterococcus faecalis(1.9%)
• Enterobacter cloacae(1.6%)
• Staphylococcus capitis subsp. urealyticus(1.4%)
• Staphylococcus haemolyticus(1.3%)
• Streptococcus pyogenes
• diffuse otitis externa :  involving a relatively wide area, without formation of a furuncle
• malignant AOE / necrotizing AOE : Pseudomonas aeruginosa (38%) : progressive, necrotizing, frequently fatal infection of the external auditory canal and base of the skull; it affects chiefly elderly diabetes mellitus and immunocompromised patients.
• fungal AOE / otomycosis / Hong Kong, hot weather, Singapore, or tropical ear (< 5% all AOEs)

Aetiology : commonly associated with long-term use of antibiotic drops and wet debris in the canal.
• Aspergillus niger (otomycosis aspergillina)
• Candida spp.
• Phoma glomerata
• otomucormycosis : mucormycosis affecting the ear
Therapy :
• when the canal is not inflamed and the infection is mostly a saprophytic fungal growth, the cerumen, debris, and fungal growth are suctioned out followed by flushing of the canal with alcohol and thorough drying
• when the canal is inflamed, gentle, meticulous suction is the first step in treatment.
• Mycostatin or Fungizone cream is applied for Candida infections
• Aspergillus species may require treatment with 2% gentian violet in 55% alcohol, or 1% thymol solution in ethyl alcohol, or perhaps best of all, a 25% m-cresyl acetate (Cresatin) solution.
In chronic otorrhea, the underlying pathology must be controlled, or the fungus will return.
Symptoms & signs :
• no hypoderma => undistendable skin => otalgia spontaneous (=> dysphagia) or evoked by pressure over tragus, traction of lobus or movement of TMJ
• swelling, fever, trismus, irradiated otalgia (tonsillitis)
• transmissive hypoacusia
• circumscribed furuncle in bacterial AOEs.
• chronic otitis externa

Aetiology :
• perichondritis (inflammation of the perichondrium) and chondritis of the auricle

Aetiology :
• outer ear eczema
• furunculosis
• otohematoma
• relapsing polychondritis
Symptoms : painful, hot cellulitis of the pinna with brawny edema
Therapy : systemic antibiotic therapy and warm, wet compresses, repeated cleaning of the wound. If chondritis develops, the infected area must be opened and drained with excision of infected cartilage.
• diabetes mellitus
• recurrent traumas
• strict meatus externus
• otobiosis

Aetiology :
• Otobius lagophilus
• Otobius megnini
• otomyiasis : infestation of the ear by larvae
Symptoms & signs : otorrhage, purulent otorrhea / otopyorrhea
• otitis desquamativa : otitis externa in which there are overdevelopment and desquamation of the cutaneous epithelium.
The fissures of Santorini in the floor of the external meatus cartilage may allow for spread of infection into the soft tissues of the periauricular region.
Therapy :
• application of dry heat to the external ear
• topical or systemic (in diffuse swelling or cellulitis) antibiotics
• of the furuncle points, the top should be removed by suction or needle to allow for drainage.  the entire canal should be gently suctioned and cleared of visible debris, and the inflamed tissue should be wiped with antibiotic drops or 2% acetic acid solution. Carbamide peroxide (Debrox^®) may be needed if there is hard or thick debris. Blind suction and wiping of diffusely swollen canals should be very gentle with attention to the direction of the canal and distance to the tympanic membrane (2.5 cm). A wet cotton canal wick about 3/4 to 1 inch long or the commercial wicks (i.e., Pope Oto-wick^®) are recommended for use in all cases with diffuse swelling of the canal. Burrow's solution (1:10) is an excellent astringent-type medication, but any of the antibiotic-cortisone aqueous otic preparations can be used on the wick. The cotton wick should be large enough to be snug and in contact with the inflamed tissues. It is kept wet and removed after 24 hours. Antibiotic drops themselves create debris, so the canal should be cleaned and a new wick inserted daily until the swelling has markedly resolved. Patients with adenopathy and cellulitis should be treated with systemic antibiotics, and the pain can be controlled with a strong anodyne
• ear dressing to splint, protect, and absorb drainage from the ear with maximum comfort to the patient. It must also resist movement or displacement. The bandage material most commonly used is a supportive pad behind the ear, a fluff dressing of loose gauze or mechanic's waste, and a support covering of the dressing with material like Kling^® or Kerlix^® elastic or stretch gauze. First, 2-3 4 x 4-inch pads are folded together in half, and then a "C" shape is cut out of the center that will fit behind and around the ear. Next, the entire ear is covered with 2-3 inches of fluff dressing or mechanic's waste. If splinting of the pinna contours is important, as in lacerations, this can be accomplished by careful insertion of ointment-impregnated cotton in the grooves of the scaphoid fossa, canal meatus, and concha. The external bandage of an elastic or stretch gauze usually begins on the forehead and is always wrapped from the front to the back of the ear. To keep the dressing out of the patient's eyes, 2 pieces of umbilical tape or thin gauze are laid vertically on both sides of the forehead. The stretch gauze is wrapped first across the center of the fluff, across the lower occiput, above the opposite ear, and then repeated below and above the first wrap, resulting in a football helmet like appearance. The forehead tapes are now tied and tape strips applied to hold the gauze in position, using intermittent applications about 6 inches in length.
• tympanocervical abscess : one arising in the tympanum and extending to the neck
• tympanomastoiditis : inflammation of the tympanic cavity and the pneumatic cells of the mastoid process
• tympanomastoid abscess : an abscess of the tympanum and mastoid
• ear lobe crease : a diagonal crease in the ear lobe associated with aging; when present in younger persons it may be a sign of coronary artery disease (CAD).
• myringitis (rarely accompanying acute otitis externa, commonly accompanying acute otitis media)
• acute myringitis

Aetiology :
• Mycoplasma pneumoniae
• bullous-hemorrhagic myringitis / myringitis bullosa haemorrhagica

Aetiology :
• Influenzavirus
• Streptococcus pneumoniae
Symptoms & signs : bullae even in outer and middle ear => transmissive hypoacusia; pain disappears at rupture of bullae => serohematic otorrhea
Therapy : antibacterial to prevent bacterial overinfection, H₂O₂
• granulomatous myringitis
• myringomycosis : otomycosis of the membrana tympani
‡
• tympanosclerosis : the presence of masses of hard, dense connective tissue around the auditory ossicles.

Laboratory examinations : otoscopy
• diseases of middle ear
• Eustachian tube dysfunction
• acute dysfunction

Aetiology :
• nasopharyngitis
• nasopharyngeal cancers (unilateral)
• infectious (paranasal) sinusitis
• catarrhal otitis media
• allergy
• chronic dysfunction

Aetiology :
• anatomic or mechanical tube occlusion
• more common in babies due to
• improper blowing of the nose
• shorter tube
• smaller volume and higher cartilage density
• lower elastin
• smaller tube angle (10° instead of 45°)
• supine feeding => regurgitation occlude tube
Self-limiting within age 10.
• adenoid hypertrophy
• adenoiditis
• flogosis of pharyngeal end of Eustachian tube
• nasopharyngeal cancers near torus tubarius (monolateral dysfunction), usually associated to laterocervical lymphadenopathy
• eustachian tube mucosal melanoma
• deficit of opening
• floppy tube
• uneffective opening mechanisms
• uneffective tensor veli palatini
• uneffective levator veli palatini
Pathogenesis : mucosa of cassa tympani and mastoid absorb O₂ => pressure negativization => retraction of tympanic membrane => transmissive hypoacusia and trasudate => muciparous metaplasia => mucus => blood
Symptoms & signs : fullness in the ear, mild intermittent discomfort or pain, and hypoacusia (not referred by babies; evident as attention deficit, dyslalia, unless neurosensorial problems are associated), autophonia, acuphens, loud tonality, relapsing otalgia, vertigo. tubotympanic disease : inflammatory disease of the middle ear resulting from eustachian tube dysfunction and decreased pressure in the tympanic cavity.
Laboratory examinations :
• otoscopy shows some with either a normal appearance or slight hyperemia of the vascular strip. The short process of the malleus is prominent or foreshortened, and the malleus may angle more posteriorly than usual (horizontal). In chronic cases, there is a "dimple" or retraction of the pars flaccida.
• type C tympanogram
Complications :
• tympanosclerosis
• adhesive otitis media /  tympanic membrane atelectasis / atelectatic otitis media
• cholesteatoma
Therapy :
• antibiotics
• vaccines
• antihistamines
• mucolytics
• myringotomy / myringostomy / tympanotomy / tympanostomy
• tubaric catheterism
• steroids
• adenoidectomy
• ventilation tube (temporaneous prosthesis) and tympanocentesis
• patulous eustachian tube : abnormal patency of the tube^ref

Aetiology : associated with pregnancy, rapid weight loss, mucosal atrophy (e.g., that due to atrophic rhinitis or occurring after radiotherapy), or muscular dysfunction. In many instances, however, a satisfactory explanation cannot be found
Pathogenesis : this anomaly permits the movement of air into and out of the middle ear by means of the eustachian tube during nasal breathing. The tympanic membrane moves outward when the patient exhaled, owing to the transmission of positive nasopharyngeal air pressure (relative to the atmospheric pressure) to the middle ear.
Symptoms & signs : when the patient inhales, the tympanic membrane moves inward because of the opposite mechanism. Adopting a recumbent position relieved the symptoms because of increased venous stasis and passive compression of the eustachian tube.
Therapy : insertion of a grommet into the right tympanic membrane, which led to the resolution of his symptoms.
• direct trauma to middle ear

Aetiology : the increase in air or fluid pressure in the ear canal caused by a slap to the side of the head, falling off water skis, improper water entry during a dive, ear blocks while flying, ear squeeze during SCUBA, or improper irrigation of the ear canal.
Pathogenesis : rupture of the tympanic membrane, laceration of the canal, and occasionally, ossicular disarticulation or subluxation of the stapes.
Symptoms : bleeding, often marked tinnitus, and occasional vertigo and hearing loss, depending on the degree and location of the injury. Infection could result when foreign material, especially water, is forced into the middle ear through a ruptured tympanic membrane.
Treatment : suction clearing, oral antibiotic coverage for 5-7 days, and a base line audiogram. Clean, small traumatic perforations usually heal within 3 weeks, but the patient must avoid any significant barometric pressure changes as the perforation nears closure, and at no time should water or other fluids be allowed in the ear. Never use ear drops unless a true infection with purulent drainage develops and then use only the suspension preparations.
• perilymph or perilymphatic fistula : rupture of the round window with leakage of perilymph into the middle ear, causing sensorineural deafness; it usually results from trauma such as barotrauma or from an erosive disease process.
• barotitis media / baro-otitis media / otitic barotrauma / aerotitis media / aviation otitis / aviator's ear / middle ear barotrauma (90%)

Aetiology : as one ascends to altitude, the outside pressure decreases, and the greater middle ear pressure forces open the "flutter valve" pharyngeal end of the Eustachian tube every 400 to 500 feet to about 35,000 feet, and then every 100 feet thereafter. During descent, the collapsed, closed, pharyngeal end of the Eustachian tube prevents air from entering the tube. The increasing relative negative pressure in the middle ear further holds the soft tissues together, and muscular (active) opening of the Eustachian tube must be accomplished before the differential pressure reaches 80-90 mmHg. Once this magnitude of differential pressure is established, muscular action cannot overcome the suction effect on the closed Eustachian tube, and the tube is said to be "locked"
Symptoms & signs : nausea and vomiting, vertigo, rupture of the tympanic membrane, shock or syncope, retraction of the tympanic membrane with the classic backward displacement of the malleus, a prominent short process, and anterior and posterior folds, hyperemia or hemorrhages in the tympanic membrane, serous and bloody fluid visible behind the membrane.
Treatment :
• equalization of pressure
• Valsalva maneuver : the nose and mouth are closed and the vocal cords opened. Air pressure is then forced into the nose and nasopharynx forcing open the Eustachian tube and increasing the pressure in the middle ear space. This can be observed as a bulging of the tympanic membrane, especially in the posterior superior quadrant. One of the best methods to prevent vocal cord closure is to instruct the patient or aircrewman to close his nose with his fingers and then attempt to blow his fingers off his nose, causing the nose to bulge from the pressure. The buildup of pressure should be < 1.5" to prevent the venous congestion that reduces the efficiency of the Eustachian tube function.
• Toynbee maneuver : close the nose and swallow => small, quick retraction movement
• Politzer method : politzerization is the mechanical inflation of the middle ear with a source of pressure, either an air pump or rubber bag, with a one-way valve. If no gauge is present, the starting pressure should just be sufficient to blow off a lightly applied finger. When a pressure gauge is available, initial attempts should be done with < 10 psi. To seal and deliver the pressure into the nose, an olive tip of metal, hard rubber, or glass is the most efficient. This tip may be attached to an atomizer if smoke or mist is desired for diagnostic or therapeutic reasons.The first attempt at politzerization should be done by inserting the olive tip into a nostril, getting a good seal but not striking the vestibule or septal walls. The opposite naris is occluded, and the patient is instructed to repeat K-K-K-K-K, loudly and sharply, as a one second burst of air is delivered. A characteristic soft palate flutter sound is heard if the procedure is performed correctly. If no results are obtained with this technique, the patient is instructed to swallow, and as the thyroid notch raises up, air pressure is again applied in the nose. For people who have trouble with a dry swallow, a sip of water may be given. In the low-pressure chamber, this method is most often used to get maximum opening of the Eustachian tube. It must be remembered that with the water technique, prolonged or high pressure might cause damage to the tympanic membrane with even a remote possibility of damage to the round window membrane and inner ear
• persistent serous fluid may be removed by needle aspiration, but thick mucoid or organized blood must be removed by myringotomy if it has not cleared after 2-3 weeks of intensive therapy
• relief of pain
• prevention or treatment of infections in the ear, Eustachian tube, or nasopharynx : antibiotics are used only when infection is present in the URT or develops during treatment.
• otitis media (10%)
• acute otitis media

Aetiology :
• purulent otitis media / suppurative otitis media :
• 90% : direct extensions of infectious rhinitis or nasopharyngitis, frequently set up by improper blowing of the nose.
• Streptococcus pneumoniae
• Streptococcus pyogenes
• Streptococcus agalactiae
• Staphylococcus aureus
• Staphylococcus epidermidis
• Moraxella catarrhalis
• Haemophilus influenzae
• Chlamydia trachomatis
• 10% : direct extensions of outer ear infections when the tympanic membrane is broken
Symptoms & signs : deep, sometimes throbbing otalgia exacerbated by Valsalva maneuver or swallowing, fever, and a mild to moderate hearing loss develop. Some people occasionally may have dizziness, nausea and vomiting
Therapy :
• penicillin for 7-10 days
• antihistamine decongestant or plain decongestant medication by mouth
• analgesics
• catarrhal otitis media / secretory otitis media / serous otitis media produces blockage of the Eustachian tube and middle ear mucosa inflammation, without bacterial invasion.
• mucoid otitis media : serous otitis media in which the secretion is particularly viscous
Symptoms & signs : fullness or plugged feeling in the ear and may feel as if fluid is present. There is hyperemia of the vascular strip and annulus. There is usually little or no hearing loss or tympanic membrane bulging
Therapy :
• antihistamine decongestant or plain decongestant medication by mouth
• analgesics
• otitis desquamativa : overdevelopment and desquamation of the mucous epithelium.
Laboratory examinations : tympanic membrane hyperemia (malleus hand and epitympanic area) and slight bulging, especially in the pars flaccida => bulging and inflammation distort or obscure the normal landmarks on the tympanic membrane => blanching => perforation (pain is usually decreased, but drainage may be inadequate)
Treatment : if perforation appears to be imminent, it is wise to do a myringotomy to assure adequate drainage and clear perforation that heals more rapidly. If the tympanic membrane ruptures spontaneously, suction cleaning should be done, and if the drainage area is inadequate, consideration should be given to enlarging it by myringotomy. The draining ear should be cleaned frequently to prevent chronic complications. Topical medication is only used in large perforations or when an external otitis is present or develops from the drainage.
• chronic otitis media
• closed tympanic membrane
• congenital cholesteatoma / dermoid cyst
• secretory chronic otitis media
• chronic catarrhal otitis media
• otitis media with effusion
• catarrhotubaric otitis media
• catarrhal tubotympanitis
• sero-mucous otitis media

‡
• glue ear : a chronic condition marked by a collection of fluid of high viscosity in the middle ear, due to obstruction of the eustachian tube with or without tympanic membrane atelectasis
• marginal (>> nonmarginal) perforation of tympanic membrane
• simple chronic otitis media : exudate
• granulomatous chronic otitis media : exudate, sometimes osteitis
• cholesteatoma tympani : a type of cholesteatoma usually associated with chronic infection of the middle ear, formed of the outer desquamating layers of stratified squamous epithelium which has extended inward and upward to line the tympanum, epitympanum, and antrum.

Pathogenesis :
• immigration theory
• retraction theory
• papillary proliferation theory
• metaplasia theory
Epithelial invasion from external auditory canal => capsule of multilayered squamous keratinized epithelium => desquamation => cholesteatoma => exudate => cell proliferation (positive feedback) ; osteitis
Symptoms & signs : loss of Politzer light cone at otoscopy due to retraction of tympanic membrane
Therapy : surgery
• chronic perforation of the tympanic membrane

Symptoms & signs : small perforations cause minimal hearing loss
Complications :
• cholesteatoma
• mastoiditis
Therapy :
• small, dry, central perforations may be closed by cauterizing the edge of the perforation with trichloroacetic acid. It can be left open or one may elect to place a small patch made from cigarette paper or other thin paper over the perforation. Usually the patch is moistened in antibiotic drops before application.
• large perforations with a dry middle ear may be closed by a tissue graft if the Eustachian tube is functioning. Testing of this function is fairly accurate by tympanography. Poor or absent Eustachian tube function gives surgery a decreased chance for success.
If the ossicles show fixation or if there is considerable scarring with adhesions, hearing might decrease somewhat further even though the perforation is closed, as a result of the poorer transmission of sound and the cancellation effect of sound striking both oval and round windows at the same time. A perforation, per se, which allows for equalization of pressure between the middle ear and the atmosphere does not affect flying. Sudden cold or hot air or water and loud noise may cause vertigo more easily in the perforated ear. Of course, water in a perforated ear usually leads to infection and drainage. The principles of treatment are meticulous cleaning of the canal perforation and middle ear, removal of granulation tissue, and control of the infection with both systemic and topical antibiotics solutions introduced into the middle ear. One technique is to fill the canal with the solution and gently compress the tragus into the meatus while swallowing. If the otorrhea is not too heavy, antibiotic powders may be insufflated, or the older powder preparations, such as Sulzberger's 1% iodine and 1% boric acid, are often effective. For thick drainage and debris, it may be necessary to irrigate with a 1.5-2% acetic acid solution. The area should be suctioned clean and dry before using the antibiotic drops or powders to increase their effectiveness.
• Brown's sign : blanching of the tympanic membrane and of the area behind it, seen with pneumatic pressure on the membrane; it indicates presence of a vascular tumor or other lesion in the middle ear.
• mastoiditis : inflammation of the mastoid antrum and cells, sometimes as a result of otitis media.
• Bezold's mastoiditis : a form in which the pus has escaped and formed tracts into the neck

Complication : von Bezold's abscess is an abscess in the neck in which pus tracts have formed deep to the superior portion of the sternocleidomastoid muscle and along the posterior belly of the digastric muscle
• coalescent mastoiditis : a form in which the bony partitions between the air cells erode so that air cells coalesce into large cavities; further erosion of the temporal bone runs the risk of intracranial abscess.
• sclerosing mastoiditis : mastoiditis attended with hardening and condensation of the bone.
• silent mastoiditis : a progressive destructive mastoiditis with mild systemic and local manifestations.
Symptoms & signs :
• Bezold's sign : an inflammatory swelling below the apex of the mastoid process
• Granger's sign : if in the radiograph of an infant < 2 years old, the anterior wall of the lateral sinus is visible, extensive destruction of the mastoid is indicated.
• Bethea's sign or method : when the examiner, standing behind the patient, places his fingertips on the upper surfaces of corresponding ribs high in the patient's axillae, unilateral impairment of chest expansion is indicated by less respiratory movement of the ribs on the side affected
Complication : mastoid or intramastoid abscess : an abscess within the mastoid process and the air cells => mastoid empyema : suppurative inflammation of the mucous lining of the cavities of the mastoid process
• diseases of inner ear
• inner ear trauma : temporal bone fractures
• longitudinal or middle fossa fracture that parallels the long axis of the petrous pyramid is usually due to forces applied to the temporoparietal region. The middle ear is always damaged. The tympanic membrane is torn and bleeds. The labyrinthine capsule is usually spared, as is the facial nerve. Longitudinal temporal bone fractures are 4 times more frequent than the transverse variety.
• transverse or posterior fossa fractures usually result from forces applied to the occipital or occipitomastoid region. There is essentially a fracture of the labyrinth that spares the middle ear. There may be hemotympanum, but rarely rupture of the tympanic membrane. Usually, there is both cochlear and vestibular function loss, and the facial nerve is damaged in the internal auditory meatus or horizontal portion. Only sterile ear instruments should be used for examination, and dry ear precautions must be taken.
Treatment : cranial checks, prophylactic antibiotics, and a complete neurological evaluation. A baseline audiogram is valuable if the patient's condition permits.
• aerotitis interna / barotitis interna / baro-otitis interna / inner ear barotrauma

Aetiology : overly aggressive use of the Valsalva maneuver to clear what the patient thought was an ear block. In reality, the problem was an over-inflated middle ear and distended tympanic membrane, which gives a similar blocked feeling, but usually has no pain
Symptoms & signs : when the round window membrane ruptures, there may be variable degrees of tinnitus and persistent or positional vertigo, often with nausea and vomiting
Laboratory examinations : calorics are usually diminished on the involved side, and a sensorineural hearing loss, often across the board, is present with poor discrimination of words
Prognosis : most complete recoveries have had repairs within 48 hours
• otosclerosis

Aetiology :
• hereditary (50%)
• autosomal dominant inheritance with incomplete penetrance
• polygenic inheritance
• sporadic (50%)
Pathogenesis : primitive osteodystrophy of labyrinthine capsula => bone demineralization => deposition of new bone tissue (Schwartze's sign : pink blush behind the tympanic membrane because of hyperemia of the mucous membrane around the promontory at otoscopy) => cartilagineous remnants in labyrinth => otospongiosis of the bony labyrinth, especially adjacent to the footplate of the stapes; it may cause stapedio-ovalar ankylosis resulting in conductive hearing loss. Cochlear otosclerosis may also develop, resulting in sensorineural hearing loss. Endochochlear => periosteal => endoosteal.
Symptoms & signs : asymptomatic => monolateral conductive hypoacusia =toxic enzymes diffuse to cochlea via perilymph=> mixed hypoacusia (75%) => progressive bilateral hypoacusia => Willis paracusia; rarely vestibular disorders
Laboratory examinations : type B tympanogram . Bezold's triad : prolonged bone conduction (negative Rinne test) and lessened perception of low tones
Therapy :
• NaF (side effect : nephrotoxicity)
• bisphosphonates
• stapedioplasty
• cochlear implants
• ankylosis of the stapes by post-inflammatory osteogenesis (syndrome of Coyle Shea Jr) is a pathologic entity still not well known, often confounded with otosclerosis. If surgical treatment is the same as for otosclerosis (stapedectomy, platinectomy, veinous graft and piston), the prognosis is not the same and a great carefulness is necessary, as well in operatory indications as in operatory procedure itself^ref.
• labyrinthoses
• congenital labyrinthoses
• chromosomal aberrations
• cri du chat syndrome
• trisomy 21 / Down syndrome / mongolism
• trisomy 13 syndrome / Patau syndrome
• trisomy 18 syndrome / Edwards syndrome
• foetal erythroblastosis due to Rh incompatibility
• infectious forms :
• rubella virus (Gregg's triad)
• mumps virus
• HHV-3 / VZV
• HHV-5 / CMV
• influenzavirus
• HBV
• measles virus
• Toxoplasma gondii
• toxics
• thalidomide
• maternal diabetes mellitus
• hereditary forms
• simple forms : autosomal recessive deafness
• Scheibe's deafness : congenital deafness due to partial aplasia of the saccule and cochlear duct (Scheibe's aplasia), Corti's ganglion hypoplasia, ectasia of cochlear canal
• Michel's deafness : congenital deafness due to total lack of development of the inner ear (Michel's aplasia) and sometimes even rocca petrosa
• Mondini-Alexander deafness : development only of a single curved tube representing the cochlea, and similar immaturity of the vestibular canals; a high-frequency hearing loss results
• Bing-Siebenmann deafness : bony labyrinth well formed but membranous part and particularly the sense organ poorly developed. This type is often associated with retinitis pigmentosa
• Siebenmann deafness : changes mainly in middle ear and often due to thyroid hormone deficiency
• associated forms
• morphodysplastic forms
• Jervell and Lange-Nielsen syndrome (JLNS)
• Klein-Waardenbourg syndrome
• congenital deafness and onychodystrophy / Feinmesser-Zelig or Door syndrome
• histodystrophic forms : Marfan syndrome
• abiotrophic forms :
• oculocutaneous albinism (OCA)
• Usher syndrome
• Cockayne syndrome
• Alport syndrome
• Refsum disease
• metabolic forms
• Pfaundler-Hurler disease (MPS IH)
• mucopolysaccharidosis type IV (MPS IV) / Morquio syndrome
• Pendred syndrome
• acquired forms
• neonatal forms
• prematurity
• postmaturity
• anoxia
• dystocic delivery
• post-natal forms
• toxic
• exogenous
• thalidomide
• maternal diabetes mellitus
• ototoxic substances
• endogenous substances
• diabetes mellitus
• renal failure
• liver failure
• senile involutive
• labyrinthopathies
• labyrinthitis / otitis interna
• cochlear labyrinthitis
• infectious
• isolated serous labyrinthitis

Aetiology : viruses
• mumps virus
• Influenzaviruses
• HHV-5 / CMV
• HHV-3 / VZV
• post-vaccinial
Prognosis : there is usually return of labyrinthine function over weeks or months. With fistulas, there is often a permanent nerve-type hearing loss, and some patients have chronic positional vertigo.
• acute suppurative, bacterial or purulent labyrinthitis : labyrinthitis in which pus enters the labyrinth

Aetiology : bacteria
• tympanogenic labyrinthitis : acute suppurative labyrinthitis that results from invasion from the tympanic cavity through a fistula.
• hematogenic labyrinthitis : acute suppurative labyrinthitis that results from invasion by bacteria from septicemia
• Corynebacterium diphtheriae
• Salmonella typhi
• Treponema pallidum subsp. pallidum
• meningogenic labyrinthitis : acute suppurative labyrinthitis that results from invasion of meningitis through an erosion of the temporal bone.
Symptoms & signs : severe and often permanent vertigo and sensorineural hearing loss
Complications such as meningitis or brain abscess lead to toxic symptoms of headache, malaise, and fever.
Therapy : antibiotics and surgery
Prognosis : some small mortality can be expected even with treatment. For those who recover, there is usually no recovery of the cochlear or vestibular responses, and 3-5 weeks are required for compensation. It is often impossible to be sure of complete eradication of disease, and there is questionable compensation for loss of hearing and labyrinthine function and occasional residual ataxia.
Aetiology :
• acute otitis media
• cholesteatoma
• ear surgery (no nystagmus)
Symptoms & signs : vestibular symptoms (spontaneous nystagmus to the opposite ear, nausea and vomiting, true vertigo, ataxia, past-pointing) usually preceding the cochlear depression (loss of hearing) by a few hours to several days
Laboratory examinations : in patients with cholesteatoma, if exerting pressure and then suction using a pneumo-otoscope produces nystagmus and vertigo, a labyrinthine fistula is present
• vascular or neuritic => Citelli sudden hearing loss or acute cochlear lock (continue, fluctuating, partial, pantonal)
• acute serous, sterile or toxic labyrinthitis : a type caused by chemical or toxic irritants that invade the labyrinth, usually from the middle ear
• circumscribed labyrinthitis / labyrinthine fistula / perilabyrinthitis : acute serous labyrinthitis in a discrete area, due to erosion of the bony wall of a semicircular canal with exposure of the membranous labyrinth
Aetiology :
• acute febrile
• pneumonia
• cholecystitis
• allergy
• extreme fatigue
• toxic or chemical substances
• overindulgence in food or alcohol
• aminoglycosides
• idiopathic
Therapy : antivertiginous drugs and bed rest
Prognosis : usually there is recovery from vertigo in 3-6 weeks
• cochleo-vestibular
• angioneurotic : Ménière's disease / autoimmune inner ear disease (AIED)
• angioneurotic, vascular, toxic, ... : Ménière-like syndromes (subcontinuous vertigo with neither nausea nor vomiting, rarely hearing loss)

Aetiology :
• toxics
• exogenous
• streptomycin
• salicylates
• quinine
• tobacco
• food poisoning
• endogenous
• gastrointestinal diseases
• liver failure
• renal failure
• systemic arterial hypotension
• systemic arterial hypertension
• arteriosclerosis
• arteritis
• cervical arthrosis
• hyperthyroidism
• menopause
• vestibular neuritis
• orthosympathetic-neurosympathetic dysequilibrium
• nervous : oto-neuro-vestibular syndromes
• vestibular
• vestibular neuronitis
• Gerlier's disease : an acute disease seen in farm workers and stablemen, characterized by vertigo, ptosis, and motor disorders; it is probably a form of vestibular neuronitis
Aetiology : antecedent or concomitant infection in the URT, maxillary sinuses, or teeth
Symptoms & signs : sudden, debilitating vertigo, nausea and vomiting, and spontaneous nystagmus. The cochlea is spared, but one or both of the labyrinths have abnormal calorics. Vestibular symptoms decrease somewhat after a few hours, but they remain fairly severe for the first week, slowly decreasing over the next 4-8 weeks. About 70% of these patients have permanent, decreased caloric function.
Therapy : supportive and reeducation
Prognosis : self-limiting disease, recurrent attacks even as long as 4 years after the initial attack.
• vascular of mechanical : BPPV
• mechanical : kinetosis
Symptoms & signs : inflammation of the labyrinth; it may be accompanied by sensorineural hearing loss or vertigo.
• Hennebert's sign : rotatory nystagmus when positive or negative pressure is applied to the tympanic membrane, indicative of labyrinthitis with leakage of perilymph; positive pressure (air compression) causes nystagmus toward the affected side, and negative pressure (air rarefaction) causes nystagmus away from the affected side.
• osteodystrophic labyrinthopathies
• circumscribed to labyrinthine capsule :
• otosclerosis
• labyrinthitis ossificans : abnormal ossification in the labyrinth after a trauma or an infection with inflammation
• diffuse to other skeletal districts :
• endemic cretinism
• thyroid dysgenesis
• fibrocystic bone disease
• osteogenesis imperfecta congenita
• osteopetrosis
• osteitis deformans / Paget's disease of bone (pagetoid deafness)
• traumatic labyrinthopathies
• head trauma
• labyrinthine syndrome due to fracture of rocca petrosa
• labyrinthine syndrome without fracture of rocca petrosa
• concussion of the labyrinth : trauma to the labyrinth, usually from a blow on or explosion near the ear, resulting in tinnitus and deafness.
• labyrinthine contusion
• acoustic trauma
• acute acoustic trauma
• chronic acoustic trauma
• cerebellopontine angle (CPA) tumors
• benign CPA tumors (> 99%)
• acoustic neurinoma (80%)
• epidermoid
• cystic arachnoiditis
• cerebellopontine angle meningioma
• malignant CPA tumors (<1%)
• primary or secondary tumors of the
• temporal bone
• CNS
• leptomeningeal melanoma
• sarcoma
Symptoms & signs : cerebellopontine angle syndrome : a syndrome caused by a tumor of the cerebellopontine angle or an acoustic tumor, characterized by hearing loss, subjective noises, ipsilateral cerebellar ataxia, and eventually ipsilateral abducens (VI) nerve palsy and facial (VII) nerve paralysis, accompanied by intracranial hypertension
Symptoms (labyrinthine symptoms) & signs :
• Babinski-Weil test : the patient, with eyes shut, walks forward and backward 10 times. A person with labyrinthine disease deviates from the straight path, bending to one side when walking forward and to the other side when walking backward
• Mittelmeyer's test : the patient tries to march in place with eyes closed; in vestibular disorder he will turn to the side ipsilateral to vestibular loss, or contralateral to vestibular excitation
• Hitzig test (for vestibular apparatus): the positive electrode of a galvanic current is applied just in front of the ear being examined while the negative electrode is held in the patient's hand, the patient standing with feet together and eyes closed. A current of 5 mA causes a leaning toward the positive pole in normal persons.
• Búrúny's symptom : in disturbances of equilibrium of the vestibular apparatus, the direction of the fall is influenced by changing the position of the patient's head.
Complications : Voltolini's disease : acute, painful inflammation of the inner ear followed by meningitis with deafness and unconsciousness

• Noise at National Center for Environental Health (NCEH), CDC
• Hearing loss at National Center on Birth Defects and Developmental Disabilities (NCBDDD), CDC
• Early Hearing Detection and Intervention Program (EHDP) at CDC
• Screening guidelines

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