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Welcome to Ed's Pathology Notes, placed here originally for the convenience of medical students at my school. You need to check the accuracy of any information, from any source, against other credible sources. I cannot diagnose or treat over the web, I cannot comment on the health care you have already received, and these notes cannot substitute for your own doctor's care. I am good at helping people find resources and answers. If you need me, send me an E-mail at scalpel_blade@yahoo.com Your confidentiality is completely respected. No texting or chat messages, please. Ordinary e-mails are welcome.
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I'm still doing my best to answer everybody. Sometimes I get backlogged, sometimes my E-mail crashes, and sometimes my literature search software crashes. If you've not heard from me in a week, post me again. I send my most challenging questions to the medical student pathology interest group, minus the name, but with your E-mail where you can receive a reply.
Numbers in {curly braces} are from the magnificent Slice of Life videodisk. No medical student should be without access to this wonderful resource.
I am presently adding clickable links to images in these notes. Let me know about good online sources in addition to these:
pathology.org -- my cyberfriends, great for current news and browsing for the general public
EnjoyPath -- a great resource for everyone, from beginning medical students to pathologists with years of experience
Medmark Pathology -- massive listing of pathology sites
Estimating the Time of Death -- computer program right on a webpage
Pathology Field Guide -- recognizing anatomic lesions, no pictures
Freely have you received, freely give. -- Matthew 10:8. My site receives an enormous amount of traffic, and I'm still handling dozens of requests for information weekly, all as a public service.
Pathology's modern founder, Rudolf Virchow M.D., left a legacy of realism and social conscience for the discipline. I am a mainstream Christian, a man of science, and a proponent of common sense and common kindness. I am an outspoken enemy of all the make-believe and bunk that interfere with peoples' health, reasonable freedom, and happiness. I talk and write straight, and without apology.
Throughout these notes, I am speaking only for myself, and not for any employer, organization, or associate.
Special thanks to my friend and colleague, Charles Wheeler M.D., pathologist and former Kansas City mayor. Thanks also to the real Patch Adams M.D., who wrote me encouragement when we were both beginning our unusual medical careers.
If you're a private individual who's enjoyed this site, and want to say, "Thank you, Ed!", then what I'd like best is a contribution to the Episcopalian home for abandoned, neglected, and abused kids in Nevada:
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Especially if you're looking for information on a disease with a name that you know, here are a couple of great places for you to go right now and use Medline, which will allow you to find every relevant current scientific publication. You owe it to yourself to learn to use this invaluable internet resource. Not only will you find some information immediately, but you'll have references to journal articles that you can obtain by interlibrary loan, plus the names of the world's foremost experts and their institutions.
Alternative (complementary) medicine has made real progress since my generally-unfavorable 1983 review. If you are interested in complementary medicine, then I would urge you to visit my new Alternative Medicine page. If you are looking for something on complementary medicine, please go first to the American Association of Naturopathic Physicians. And for your enjoyment... here are some of my old pathology exams for medical school undergraduates.
I cannot examine every claim that my correspondents
share with me. Sometimes the independent thinkers
prove to be correct, and paradigms shift as a result.
You also know that extraordinary claims require
extraordinary evidence. When a discovery proves to
square with the observable world, scientists make
reputations by confirming it, and corporations
are soon making profits from it. When a
decades-old claim by a "persecuted genius"
finds no acceptance from mainstream science,
it probably failed some basic experimental tests designed
to eliminate self-deception. If you ask me about
something like this, I will simply invite you to
do some tests yourself, perhaps as a high-school
science project. Who knows? Perhaps
it'll be you who makes the next great discovery!
Our world is full of people who have found peace, fulfillment, and friendship
by suspending their own reasoning and
simply accepting a single authority that seems wise and good.
I've learned that they leave the movements when, and only when, they
discover they have been maliciously deceived.
In the meantime, nothing that I can say or do will
convince such people that I am a decent human being. I no longer
answer my crank mail.
This site is my hobby, and I do not accept donations, though I appreciate those who have offered to help.
During the eighteen years my site has been online, it's proved to be one of the most popular of all internet sites for undergraduate physician and allied-health education. It is so well-known that I'm not worried about borrowers. I never refuse requests from colleagues for permission to adapt or duplicate it for their own courses... and many do. So, fellow-teachers, help yourselves. Don't sell it for a profit, don't use it for a bad purpose, and at some time in your course, mention me as author and William Carey as my institution. Drop me a note about your successes. And special thanks to everyone who's helped and encouraged me, and especially the people at William Carey for making it still possible, and my teaching assistants over the years.
Whatever you're looking for on the web, I hope you find it, here or elsewhere. Health and friendship!
Describe in some detail the etiology, pathogenesis, contributing factors, and major subtypes of the following conditions:
otitis externa
otitis media
Describe the etiology and pathogenesis of each of the following curious conditions:
cauliflower ear
frostbite
cholesteatoma
otosclerosis
presbycusis
Define vertigo, and distinguish the following causes in terms of their pathogenesis and symptomatology:
Ménière's disease
vestibular neuronitis (viral labyrinthitis)
Báràny's benign positional vertigo
Define, describe, and cite etiologic and aggravating factors for conduction and sensorineural deafness. Distinguish the expected benefit of a conventional hearing aid in these two conditions.
Give the prevalence of prevocational deafness, and the two names of the manual language preferred by the deaf in communicating with each other in the U.S.
KCUMB Students
"Big Robbins" -- Head & Neck
Lectures follow Textbook
QUIZBANK
Eye & Ear #'s 43-52
Eye and Ear
|
INTRODUCING THE EAR
Diseases of the ear are seldom studied by anatomic or clinical pathologists. We process smears and cultures (usually from external otitis), and handle surgical specimens of squamous cell carcinomas, less common tumors, and tiny stapedectomy specimens. Usually we do not examine the petrous temporal bones at autopsy even from hearing-impaired patients, except to look for middle ear hemorrhage (as in suspected drowning).
This is in sharp contrast to the importance of ear disease. Every year, one million children in the U.S. have tympanostomy tubes placed, and 600,000 have tonsils and adenoids removed for prophylaxis of recurrent otitis media. (The adenoids are likely to contain bacterial biofilms that keep the process going: Ann. Otool. 122: 109, 2013). Before the antibiotic era, middle ear infections commonly spread to the brain and caused death (Jonathan Swift, Oscar Wilde).
Around 3% of U.S. workers have become hearing impaired, and somewhere between 20% and 60% of people over age 65 have become hard of hearing. Most often these problems are sensorineural, and therefore cannot really be corrected by prescribing a hearing aid. Remember to rule out hearing loss before you decide that a young person is "retarded" or an older person is "demented".
Certain favorite drugs (notably the aminoglycoside antibiotics) predictably damage your patients' hearing, often irreversibly (Otol. Clin. N.A. 17(4): 761, 1984); in parts of the developing world in which antibiotics are handed out to sick people like candy, hearing loss from aminoglycosides is a major problem (Br. Med. J. 313: 648, 1996). There's a mutation in the mitochondrial genome that will cause an aminoglycoside to deafen you -- we now check kids with cancer for it (Arch. Dis. Child. 95: 153, 2010). Ethacrynic acid, probably the world's best diuretic, caused so much deafness that is has gone out of use. For the electron microscopy of cisplatin cochlear pathology, see J. Laryngol. Otol. 122: 1151, 2008.
Vertigo due to disease of the membranous labyrinth is not as common as hearing loss but can be even more disabling.
Pretty much anything, anywhere in the outer, middle, or inner ear, can be Wegener's. Don't miss it. J. Laryngol. 108: 144, 1994.
AURICLE (PINNA)
The dermis over most of the external surface of the ear flap is continuous with the perichondrium. A hematoma that separates the skin and cartilage of the outer ear is likely to cause ischemic necrosis of the cartilage, then organize and produce a cauliflower ear (best known, but by no means confined to, boxers and rugby players.) Auricular hematomas should be aspirated by a physician.
Infections ("perichondritis") are secondary to trauma. Classically a problem with athletes, today's fashion for piercing the ear through the cartilage has perichondritis much more common. Perichondritis is often caused by the hard-to-kill Pseudomonas aeruginosa. Whatever microbe is involved, it is protected from phagocytosis by the avascular cartilage, which makes perichondritis especially hard to treat.
* Leave the diagnosis of "angiolymphoid hyperplasia with eosinophilia", which produces nodules on the skin around the pinna, to us.
CHONDRODERMATITIS NODULARIS HELICIS is a patch of necrosis over a cartilage on the pinna, usually on the upper portion on the side on which the patient sleeps.
A mainstay of treatment is a strap-on foam donut to protect the ear during sleep time.
Since cartilage is involved, the surgery is a bit more tricky than elsewhere.
Occasionally, the problem is systemic vasculitis, with antigen-antibody complexes precipitating in this cold spot.
RELAPSING POLYCHONDRITIS (* systemic chondromalacia, etc.) is an illness in which various cartilages become inflamed and eventually turn into scar tissue which contracts. In the pinna, it is striking, painful, and looks terrible. It can ruin the nasal septum, a vertebral disk, or one or more joint cartilages, and in the trachea and bronchi (and for some reason even the heart valve annuli and aorta) it can kill.
Antibodies and/or sensitized T-cells against type II collagen are common (Mayo Clin. Proc. 77: 971, 2002, others). Novel therapy: Am. J. Med. Sci. 324: 101, 2002.
Today, patients seem to respond well to anti-TNF agents (Rheumatology 46: 1738, 2009).
Common non-neoplastic masses in the auricle include Darwin's tubercle (what's that?), tophi, congenital capillary hemangiomas, epidermoid inclusion cysts (behind the pinna or in the earlobe), preauricular cysts (an embryologic defect in the skin just in front of the ear canal), and keloids (from ear-piercing) are all common.
Actinic keratoses, squamous cell carcinomas, and basal cell carcinomas, are all common on top of the pinna.
Calcification of the pinna indicates Addison's disease (most famously; there are other causes). You will have to decide for yourself whether ear lobe creases mean coronary artery atherosclerosis.
Frostbite results when cold and sharp ice crystals damage the small blood vessels. This can lead to permanent nerve damage and even necrosis.
EXTERNAL AUDITORY CANAL
The ear canal is hard to keep dry and gets picked. "Ordinarily the canal cleans itself", with cerumen and keratin flowing outward very slowly due to the movements of chewing. This really doesn't work very well, and the external canals can get grungy.
CERUMEN IMPACTION is common and annoying, interferes with hearing, "and can cause tinnitus and vertigo". Even if earwax is not impacted, adults try to remove it from their ears and those of their children. Q-tips pack cerumen, making it less likely to come out naturally and creating a good culture medium underneath. You will learn the right ways to clean ears on rotations.
EXTERNAL OTITIS is a generic term for inflammatory disorders (infections, contact dermatitis, seborrhea, etc., etc.) of the external auditory canal.
Remember that the external auditory canal is very sensitive. Patients present with itching and pain, and later with a discharge. (If you see nothing with your otoscope, check the throat too; mild inflammation around the eustachian tube will be felt in the ear.)
INFECTED HAIRS are usually due to staphylococcus and are very painful.
OTITIS EXTERNA ("swimmer's ear"), extremely common, results from swelling of wet keratin (as in dish-pan hands) which plugs the outlets of the little hair follicles and invites infection. Pseudomonas organisms are the usual offenders; think also of candida in very hot weather.
FUNGAL OTITIS (aspergillus, candida) and ACTINOMYCOSIS can produce chronic, itchy infections.
CONTACT DERMATITIS results from ear drops, hair sprays, atopic dermatitis, or picking. SEBORRHEA (oily, scaly skin) of the canal and auricle is of unknown etiology. Both of these problems are familiar from general dermatology.
MALIGNANT EXTERNAL OTITIS is a pseudomonas infection that affects diabetics and spreads into the skull Am. Fam. Phys. 309: 2003.
KERATOSIS OBLITERANS ("canal cholesteatoma") is a mysterious condition in which keratin keeps accumulating in the canal and plugging it.
During your career, you will see many interesting FOREIGN BODIES in ear canals. Insects get stuck in the cerumen and make a person miserable. Beans swell up due to fluid in the canal, and are especially hard to remove.
TRAUMATIC PERFORATION OF THE EARDRUM results from diving, water skiing, fights, and instrumentation. This presents a problem for otolaryngologists and attorneys.
Tumors of the external auditory canal are rare.
EXOSTOSES ("surfer's ear"; bony overgrowths) sometimes occur in children with other birth defects and the resulting hearing impairment does not help the child's development. (Check for this in "profoundly retarded Downs' syndrome kids"). Acquired exostoses are most famous for occurring in people who swim/surf in cold water; it's clearly true (Otolaryngology 126: 499, 2002) and that tells me they're hyperplasias rather than real neoplasms. The histopathology is successive laying-down of bone layers just under the periosteum (Laryngoscope 108: 195, 1998). Treating them: J. Laryng. Ot. 108: 106, 1994.
ADENOMAS of cerumen-producing cells or sweat glands occur rarely.
Remember that a tiny fracture of the skull can produce cerebrospinal fluid otorrhea. Think of this whenever there is a discharge "from the external ear canal".
* Ramsay-Hunt is zoster causing Bell's palsy; look for vesicles and ask about pain in the ear canal.
* Older people's pinnas really are relatively bigger. For some reason, cartilage never stops growing. Somebody finally measures... Br. Med. J. 311: 1668, 1995.
MIDDLE EAR
Most middle ear problems result from problems opening and closing the eustachian tube. Many factors -- big tonsils, upper respiratory infections, allergy, cleft palate, deviated septum, paralysis of part of the palate, or just being a small kid -- can interfere with proper function.
AEROTITIS MEDIA is the familiar pain caused by barotrauma, as when one's ears fail to pop while changing altitude in an airplane. Recurrent episodes can lead to middle ear deafness.
ACUTE CATARRHAL OTITIS MEDIA means tissue fluid in the ear in the absence of obvious infection.
Many of these children require tonsillectomy-adenoidectomy and/or myringotomy with placement of tympanostomy drains ("tubes").
CHRONIC CATARRHAL OTITIS MEDIA ("glue ear") is a permanent case of mucous otitis media, which usually leads to deafness.
FAILURE OF THE EUSTACHIAN TUBE TO CLOSE results in hearing one's own respirations and voice in the ear.
OTITIS MEDIA, infection of the middle ear, is common and troublesome. Update Lancet 363: 465, 2004.
VIRAL OTITIS MEDIA is the full feeling in the ear that often accompanies a viral "cold". It may become quite painful, and is hard to tell from bacterial otitis media.
ACUTE BACTERIAL OTITIS MEDIA (the common earache) is a among the most common complaints for which people seek medical attention.
This is primarily a pediatric disease. Most patients have their first attack between 6 and 36 months of age.
Poor eustachian tube function prevents proper drainage from the ear, and when infection supervenes, the resulting inflammatory edema further interferes with drainage.
The usual agents are Streptococcus viridans and pneumoniae, Hemophilus influenzae, Staphylococcus epidermidis and aureus, and Branhamella (a troublesome, penicillin-resistant organism).
Acute bacterial otitis media is very painful, and inflammation and bulging of the drum are obvious on examination. Response to antibiotics is generally good, but some patients may require myringotomy for immediate relief.
Of course, this does not correct the underlying eustachian tube dysfunction. Today, children get tympanostomy "tubes" inserted into their eardrums to provide drainage; these are removed after several years, when the eustachian tubes are working better.
The dreaded complications of acute otitis media -- rupture of the tympanic membrane, and spread to the cochlea, labyrinth, mastoids, meninges, and the brain -- are seldom seen today. However, endotoxin occasionally produces permanent hearing and/or labyrinthine damage (J. Laryng. Ot. 108: 310, 1994).
Of course, untreated common otitis media causes a great deal of unnecessary suffering in the poor nations. Giving vitamin A may help prevent some of the problems in hard-to-reach communities (BMJ 344: d7962, 2012).
The rare ACUTE NECROTIZING OTITIS MEDIA is usually due to beta-hemolytic streptococci and results in necrosis of the bones.
* Xylitol chewing gum, which is already very popular in Scandinavia and which is a powerful cavity-preventer (un-stickies the oral cavity) seems to prevent otitis media as well (Br. Med. J. 313: 1180, 1996, the famous case in which optional participation in a grammar school kids' science-project was blown up as a grave violation of the participants' human rights).
CHRONIC SUPPURATIVE OTITIS MEDIA is a longstanding, smoldering bacterial infection that can eventually destroy the middle ear.
CHOLESTEATOMA is a fancy name for a mass of keratin (like an epidermoid cyst) in the middle ear. It can be congenital (surgeon's nightmare Arch. Otol. 138: 280, 2012), acquired from perforation of the eardrum, or develop from chronic suppurative otitis media. The common theme is a fold in the tympanic membrane, forming a closed cavity in which skin keratin accumulates. This is a major surgical problem.
The major true tumor of the middle ear is the GLOMUS JUGULARE TUMOR, which forms in the "glomus jugulare", a miniature version of the carotid body. The tumor recapitulates the normal structure. Patients complain first of pulsatile tinnitus; hearing loss develops as the tumor slowly enlarges. Surgery is challenging.
OTOSCLEROSIS
In this very common disease, new bony and fibrous tissue forms in the ear. One older white person in 200 is seriously hearing-impaired as a result, and many more are found to be affected at autopsy if one looks.
Calcification and ossification of the annular ligament of the stapes prevents transmission of sound impulses at the oval window. Surgery, with insertion of a plastic stapes or simply incision of the hardened area, helps some of these patients.
If new bone formation distorts the bony portion of the cochlea, sensorineural deafness results.
Otosclerosis is a major cause of acquired hearing loss, though fortunately it is seldom severe. It is also blamed for much of the tinnitus that troubles the elderly.
At least nine genes, all of variable expressivity, are known so far (Am. J. Hum. Genet. 84: 328, 2009). Most patients are Caucasians.
COCHLEA AND AUDITORY NERVE
We seldom examine cochleas at autopsy, but there have been a few studies of congenital and acquired sensorineural deafness. In most cases, the cochlear hair cells are damaged or missing.
Viral infections, notably congenital rubella, congenital or acquired CMV, Lyme disease, post-natal mumps, and Lassa fever can cause permanent deafness.
PRESBYCUSIS, the sensorineural hearing impairment that comes with advancing age, involves degeneration and loss of high-frequency hair cells. Some families are much more severely affected than others. The same changes occur in young people exposed to very loud sounds (industrial noise, rock music).
* High-power cochlear pathologists distinguish four types: SENSORY (abrupt loss of high tones), STRIAL (loss of tones more uniformly), NEURAL (loss of word discrimination), and COCHLEAR CONDUCTIVE (the only type without anatomic pathology). This is deep stuff; read about it in Ann. Ot. Rhin. Lar. 102: 1, 1993.
Cerebellopontine angle tumors (schwannomas, meningiomas) are common, usually lie on the eighth nerve, and produce cranial nerve palsies.
Autoimmunity is implicated in cases of sudden "idiopathic" deafness, but the autoantibodies remain elusive (Acta Oto-Laryngol. 121: 28, 2001). Keep listening.
Also remember Paget's disease of bone can compress the auditory nerves. (Notice the size and shape of Beethoven's head -- it has been suggested that he went deaf from Paget's disease.)
Cochlear implants: Classic work Nature 352: 236, 1991; update Ped. Clin. N.A. 50: 341, 2003; the younger you get the implant, the better you learn to understand and use language (JAMA 303: 1498, 2010; Arch. Otol. 121: 73, 2012).
Tinnitus (i.e., "ringing" and other odd noises in the ear) is complicated: Mayo Clin. Proc. 66: 614, 1991.
* Tune deafness ("tin ear"; you have trouble picking up off-notes in familiar tunes played poorly) seems to be hereditary: Science 291: 1879 & 1969, 2001. Around 5% of folks are affected. The most severe form is "tone deafness", in wich a person cannot tell which of two musical notes is higher -- this is a handicap only in music appreciation, and these people can speak and appreciate speech inflections normally. The anti-instrumentalist movement was driven by a famous preacher who was famously tone-deaf, but the story's more complicated.
MEMBRANOUS LABYRINTH AND VESTIBULAR NERVE
Deaf, giddy, odious to my friends,
Now all my consolation ends...
-- Jonathan Swift
Several distinct diseases affect the balance system, but these seldom are examined by pathologists. Problems here cause vertigo, the distinct sensation of spinning. From my review of the literature, there's been only one serious study by pathologists that's found anything; this was an inflamed endolymphatic sac (Ann. Otol. 106: 190, 1997).
MÉNIÈRE'S DISEASE is a poorly-understood illness featuring attacks of vertigo with hearing loss and tinnitus.
VESTIBULAR NEURONITIS (the old "labyrinthitis") is viral involvement of "the labyrinth" (actually, its first connection in the brainstem). Usually this follows a more conventional viral illness (remember Lyme disease too). Patients can walk again in only a few weeks, and finally recover, often with minor residual damage. (* This is how I got my stagger.)
BÁRÁNY'S BENIGN POSITIONAL VERTIGO is a frightening condition that results (??) from an otolith breaking loose from the utricle. When the patient's head tilts into a certain position, the little rock settles on the sensor, triggering a spectacular attack. The diagnosis is easy and reassurance is all that is required (Mayo Clin. Proc. 66: 596, 1991). Review NEJM 370: 1138, 2014.
DEAFNESS AND HEARING IMPAIRMENT
One child in 1000 is born deaf. One person in 500 is prevocationally deaf or hard-of-hearing, and about half of us will have at least some hearing impairment as we grow older. (This is a major quality-of-life problem in the elderly. See Ann. Int. Med. 113: 188, 1990). Conduction deafness (i.e., disease of the external and middle ear) causes loss of hearing at all frequencies, and is best treated with a hearing aid. Sensorineural deafness (i.e., disease of the inner ear or auditory nerve) often (not always) interferes more with ability to hear higher frequencies, and sounds are greatly distorted. Mere amplification of sounds does not correct the defect, so the older hearing aids were less helpful. Central deafness, due to disease in the brain, is very rare, since the hearing pathways are so diffuse.
The most common known cause of congenital deafness in today's older adults is rubella, though more than half of cases are "idiopathic". Many genetic syndromes produce profound sensorineural deafness.
The most common mutation (around 40% of all non-syndromic congenital deafness) is connexin 26. On the hair cells of the cochlea, abnormalities of this protein are extremely common in familial and sporadic congenital deafness: Lancet 351: 394, 1998; NEJM 339: 1585, 1998; Lancet 353: 1298, 1999; Lancet 358: 1082, 2001; Int. J. Ped. Oto. 68: 995, 2004; cochlear implants early usually work pretty well. This is especially common because deaf people tend to marry one another: Lancet 356: 500, 2000.
* There are lots more. Progressive adult deafness from a mutation in a transcription factor: Science 279: 1950, 1998). DFNA10 mutations produce a progressive deafness beginning in childhood (Ann. Otol. 110: 861, 2001). Usher syndrome, the commonest cause of combined blindness-deafness, with nerve deafness and retinitis pigmentosa both, is caused at least in some case by defective myosin VII-A, which anchors to the actin cytoskeleton and moves things around inside cells: Nature 374: 60, 1995; physics and a bunch more Proc. Nat. Acad. Sci. 93: 3232, 1996. At least one Waardenburg locus, a dominant that imparts variable deafness, different-colored eyes, and a white forelock, has been cloned -- Nature 355: 635 & 637, 1992; Am. J. Hum. Genet. 52: 455, 1993, stay tuned for more, homologous to drosophila homeobox. The major genes for deafness (around 70) were discovered in the 1990's: Nat. Med. 4: 1245, 1998; Lancet 358: 1082, 2001. Today, a congenitally deaf or hard-of-hearing person can expect to have the gene identified, and carriers sought among relatives (Ped. Clin. N.A. 50: 315, 2003).
Severe deafness may be acquired from meningitis, trauma, or a variety of less common problems, including aminoglycoside antibiotics.
Until recently, ideologies dominated education of the deaf in our country. Most educators emphasized "teaching normal speech and lip-reading" in the education of deaf children, often forbidding the sign language that the deaf community actually uses to communicate. This was a crime. Even after years of practice, "reading lips" is very difficult. (The Ameslan/ASL sign for "lip-reading" is a combination of the signs for "mouth" and "difficult/impossible".) And attempts by the prelingually deaf to speak usually result in ridicule.
In some places, truly deaf (not just hard-of-hearing) children are still "mainstreamed" in regular classes for hearing children. Again, this is done for reasons that are in part political and ideological. Deaf kids in a Nicaraguan school where this is practiced actually are developing a sign language from scratch so they could communicate with each other (Science 293: 1758, 2001). "Mainstreaming" is back in style today and is supposed to get badly hearing-impaired children socialized into mainstream society. In the United States, this is not working well; hearing-impaired girls do well with hearing friends only if they don't mind being alone, while hearing-impaired boys don't make hearing friends at all unless they're really good at sports (Child Care Health Dev. 29(6): 511, 2003). Deaf students in the UK feel disadvantaged compared to hearing peers if they are mainstreamed, but complain that the deaf-only classes are very poorly taught (J. Deaf Stud. 15: 358, 2010).
During the late 20th century, the hearing community began to notice Ameslan (American Sign Language) that the deaf use (Science 247: 1127, 1989). It is fascinating, and is as demanding to learn as any other foreign language. The best interpreters are hearing children who grew up with one or two deaf parents. Do not confuse Ameslan with finger-spelling, in which the words of English are spelled out using hand positions. The Soviet Union taught all deaf and hearing children to finger-spell (the Russian alphabet, of course), in the hopes of making communication easier. |
Many of the deaf like to consider theirs a separate culture. However, in communities where there are more deaf than usual (old-time Martha's Vineyard, birthplace of American Sign Language, was one example), deafness has not been considered a handicap because "everybody speaks sign language". How physicians communicate with the deaf: JAMA 273: 227, 1995. Cochlear implants have enabled many deaf children to hear. If the child is to understand spoken language, you want to place the implant while the chid is very young, and talk to the child (Lancet 356: 466, 2000). It usually works pretty well, though not always, and not surprisingly, the data now confirms it improves the quality of life and is a money-saver (Ped. Clin. N.A. 50: 341, 2003; update on language acquisition JAMA 303: 1498, 2010; these kids ultimately achieve in school and at work at the same levels as everybody else Arch. Otol. 136: 366, 2010). And have them learn to play a musical instrument (Pediatrics 125: e793, 2010). Probably because cochlear implants are available, most states have passed laws mandating hearing checks for newborns.
* The value getting bilateral implants early -- there seems to be a critical time for the brain to develop the superior hearing discernment that stereo hearing offers: Arch. Otol. 138: 134, 2012; Brain 136: 1609, 2013.
* Cochlear implantation for babies
used to be "extremely controversial" because of multiculturalism.
The politically-correct attitude was that
minority-group identity was more important
than a child's ability to enjoy music, learn to read and write,
talk to most of the people he/she meets, use an ordinary telephone,
or do most kinds of jobs. In the late 1990's
the discussion became extremely heated and bitter.
Someone more cynical than myself would also suspect deaf parents who
want their kid to remain deaf want more of the kid's time and affection
for themselves rather than for friends and co-workers who can hear.
(Remember the truism, "Child abuse begins with the parents' frustrated
desire for the child's affection?")
All about "deaf culture values" from the most
vocal opponent of cochlear implants: Otolaryngology 119: 297, 1998.
Proponents (Hastings Center Report 28(4): 6, 1998) pointed out that
separate
equally-valid culture or not, the deaf are the beneficiaries
of expensive services (interpreters, special schools, special communications
equipment) that the much-maligned mainstream society works very hard to
provide.
It seems to me that (right or wrong) legislatures should balk at
expensive entitlements for disabled people who
refuse to accept effective treatment -- or especially, who demand that
their children remain disabled for life.
Also a movie "The Sound and the Fury", 2001 (lots of angry deafness militants).
As recently as 2003, writers actually celebrated the fact
that parents had a "choice" about
letting their child hear or not
(Arch. Ped. Adol. Med. 157: 162, 2003).
Well, times changed. Even ethicists who use terms like "ethnocide"
(Monash Bioethics Review 21: 29, 2002, i.e., allowing cochlear implants
to babies to enable them to hear is in some respects like Hitler's mass-murder of the Jewish
people and other minorities) consider the surgery mandated on moral and ethical
grounds.
More importantly,
today the vast majority of parents of kids with
cochlear implants agree they wish their kid had gotten it earlier
(Arch. Oto.
130:
673, 2004; good update from Gallaudet, of all places).
Today, really good screening of all
newborns for bad hearing is now a mandate for pediatricians,
to get the implants placed as early as possible (Arch. Otol. 137: 230, 2011;
Arch. Dis. Child. 96: 62, 2011).
A few multiculturalists overseas still complain that treating a baby
in a timely way "denies [the child] access to all possibilities including
that of a Deaf Life" (J. Deaf Studies 11: 102, 2006) -- but let's think;
not treating the baby denies him/her access to the entire mainstream culture,
including almost all the satisfying and productive jobs,
talking to 99.5% of the rest of the human race,
and the enjoyment of music.
Anyway, the cochlear implant kids are now teenagers, enjoying
friendship with members of the hearing community, learning like
other kids, and dancing to popular music. They are unanimously
pleased (J. Deaf Stud. 12: 303, 2007).
And nowaday pre-lingually deaf teens who were denied cochlear implants
as babies because of "multiculturalism" etc. are getting them -- and
outcomes are pretty good (Arch. Ped. 166: 35, 2012;
Ann Otol. 122: 222, 2012).
* The term is "designer disability." Britain is where the controversy over deliberate in-vitro conception of a deaf
child rather than a hearing child was played out (Lancet 359: 1315, 2002;
warning: ugly reading in your lecturer's opinion.) "Ethicists" lined up on both
sides.
When a politically-correct member of the British parliament tried to insert language
into a law about child health care that would allow parents
to choose a deaf embryo over a hearing embryo for reasons of multiculturalism,
one of Britain's deaf medical students lambasted him (BMJ 336: 1148, 2008).
The British
removed references of deafness
to please deaf-community militants but still made it every bit as illegal.
For ongoing complaints from some ethicists, see "eroding parent-physician trust" (!; Lancet 371: 1663, 2008.)
{04577} internal auditory canal, normal
{13128} tympanic membrane, normal
{13168} stapes, normal and abnormal
{15077} cochlea, inner ear section
{15078} cochlea, inner ear section
{15079} cochlea, normal
{15079} cochlea, normal
{15080} cochlea, normal
{15081} organ of corti, normal
{15082} organ of corti, normal
{16288} cochlea, normal
{16289} cochlea, normal
{16292} cochlea, normal
{16293} cochlea, normal
{16301} vestibulocochlear nerve, normal
{16320} cochlea, normal
{16322} cochlea and semicircular canal, normal
{16338} cochlea, normal
{16339} cochlea, normal
{16340} cochlea, normal
{16341} cochlea, normal
{16350} cochlea, normal
{16360} oval window, normal
{16361} oval window, normal
{20714} cochlea, normal
{44877} tympanic membrane, normal
BIBLIOGRAPHY / FURTHER READING
I urge anyone interested in learning more about this topic in pathology to consult these standard textbooks.
In my notes, the most helpful current journal references are embedded in the text. Students using these during lecture strongly prefer this. And because the site is constantly being updated, numbered endnotes would be unmanageable. What's available online, and for whom, is always changing. Most public libraries will be happy to help you get an article that you need. Good luck on your own searches, and again, if there is any way in which I can help you, please contact me at scalpel_blade@yahoo.com. No texting or chat messages, please. Ordinary e-mails are welcome. Health and friendship!
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