Sensory - Hearing Abnormalities: Difference between revisions

From Embryology
No edit summary
No edit summary
Line 1: Line 1:
[[File:Hearing_cartoon.jpg|right|400px]]
==Introduction==
==Introduction==
[[File:Newborn_hearing_test.jpg|thumb|Newborn hearing test]]
How and why do things go wrong in development? Developing of hearing requires a complex origin, organisation, and timecourse means that abnormal development of any one system can impact upon the development of hearing. There are many different abnormalities of hearing development that can result in hearing loss and can broadly be divided into either conductive or sensorineural loss. These abnormalities can have genetic, environmental or unknown origins. In addition, abnormalities of the external ear (position and structure) is used as a clinical diagnostic tool for developmental abnormalities in other systems.
In Australia, there is now an early postnatal screening of neonatal hearing as part of a NSW State Wide Infant Screening Hearing (SWISH) Program using [[A#Automated Auditory Brainstem Response|Automated Auditory Brainstem Response]] (AABR).
:{{Template:Hearing Links}}  | [http://embryology.med.unsw.edu.au/Notes/ear.htm original page]


How and why do things go wrong in development?


:{{Template:Abnormality Links}}
:{{Template:Abnormality Links}}


==Some Recent Findings==
==Some Recent Findings==
* '''Review - The etiology of otosclerosis'''<ref><pubmed>20513039</pubmed></ref> "Otosclerosis is a common form of hearing loss characterized by abnormal bone remodeling in the otic capsule. It is a complex genetic disease, caused by a combination of genetic and environmental factors. During the past decade, several attempts have been made to identify factors for otosclerosis."


==References==


==Inner Ear Abnormalities==
Common cavity, severe cochlear hypoplasia
===Cholesteatoma===
Epithelium trapped within skull base in development, erosion of bones: temporal bone, middle ear, mastoid
==Middle Ear Abnormalities==
Rare and can be part of first arch syndrome.
Fixation of the middle ear ossicles Malleus, Incus and Stapes
==Outer Ear Abnormalities==
[[File:Microtia.jpg|thumb|Microtia]]
[[File:Preauricular sinus.jpg|thumb|Preauricular sinus]]
Several genetic effects and syndromes, Environmental Effects
* Microtia - abnormally small external ear
* Preauricular sinus - occurs in 0.25% births, bilateral (hereditary) 25-50%, unilateral (mainly the left), duct runs inward can extend into the parotid gland, Postnatally sites for infection
Fetal Alcohol Syndrome
[[File:FASface.jpg|thumb|Fetal Alcohol Syndrome Face]]
* Postion- Lower or uneven height, "railroad track” appearance, curve at top part of outer ear is under-developed, folded over parallel to curve beneath
== Congenital Deafness ==
'''Sensorineural''' - cochlear or central auditory pathway
'''Conductive '''- disease of outer and middle ear
===Sensorineural===
Cochlear or central auditory pathway
* Hereditary
* recessive- severe
* dominant- mild
** can be associated with abnormal pigmentation (hair and irises)
* Acquired
** rubella (German measles), maternal infection during 2nd month of pregnancy, vaccination of young girls
** cytomegalovirus <ref><pubmed>20500943</pubmed></ref>
** streptomycin
** antibiotic
** thalidomide
===Conductive===
Disease of outer and middle ear
* produced by otitis media with effusion, is widespread in young children.
* temporary blockage of outer or middle ear
==Newborn Hearing Screening==
[[File:Newborn_hearing_test.jpg|thumb|Newborn hearing test]]
In Australia, there is now an early postnatal screening of neonatal hearing as part of a NSW State Wide Infant Screening Hearing (SWISH) Program using [[A#Automated Auditory Brainstem Response|Automated Auditory Brainstem Response]] (AABR).
* Very low birthweight infants and universal newborn hearing screening in a developing country<ref><pubmed>20450464</pubmed></ref>
'''Links:''' [http://www.health.nsw.gov.au/initiatives/swish/index.asp NSW Statewide Infant Screening - Hearing (SWISH) Program]
==Bionic Ear==
The "Cochlear Implant" was pioneered in development by Professor Graeme Clark (1960s, Australia).<ref><pubmed>18816421</pubmed>[http://www.rehab.research.va.gov/jour/08/45/5/Clark.html JRRD]</ref> It consists of an array of electrodes implanted within cochlea,  that directly electrically stimulate the auditory nerve fibres.
* Young children with cochlear implants compared with children with normal hearing.<ref><pubmed>20452685</pubmed></ref>
== References ==
<references/>
<references/>
===Online Textbooks===
* '''Clinical Methods''' [http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=cm.chapter.1949 63. Cranial Nerves IX and X: The Glossopharyngeal and Vagus Nerves] | [http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=cm.chapter.3847 The Tongue] | [http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=cm.chapter.3777 126. The Ear and Auditory System] | [http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=cm.chapter.3627#3654 An Overview of the Head and Neck - Ears and Hearing] | [http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=cm.chapter.3897 Audiometry]
* '''Health Services/Technology Assessment Text (HSTAT)''' Bethesda (MD): National Library of Medicine (US), 2003 Oct. [http://www.ncbi.nlm.nih.gov:80/books/bv.fcgi?db=Books&rid=hstat1a.section.25014#25029 Developmental Disorders Associated with Failure to Thrive]
* '''Search Bookshelf'''  [http://www.ncbi.nlm.nih.gov/sites/entrez?db=Books&cmd=search&term=hearing%20development hearing development]


===Reviews===
===Reviews===
* The International Journal of Developmental Biology [http://www.ijdb.ehu.es/web/contents.php?vol=51&issue=6-7 Vol. 51 Nos. 6/7 (2007) Ear Development]


===Articles===
===Articles===
Line 18: Line 99:


'''Search Pubmed:''' [http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=search&term=Hearing%20Development%20Abnormalities Abnormalities]
'''Search Pubmed:''' [http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=search&term=Hearing%20Development%20Abnormalities Abnormalities]
==Additional Images==
<gallery>
File:Inner_ear_haircells.jpg|Inner ear hair cells
File:External ear anatomy.jpg|External ear anatomy
File:Preauricular_tag_01.jpg|Preauricular tag
File:Preauricular_tag_02.jpg|Preauricular tag
</gallery>
==External Links==
* Embryo Images - [http://www.med.unc.edu/embryo_images/unit-ear/ear_htms/eartoc.htm Hearing]
* NIDCD - [http://www.nidcd.nih.gov/health/balance/balance_disorders.asp Balance Disorders]
* NSW Health - [http://www.health.nsw.gov.au/initiatives/swish/index.asp NSW Statewide Infant Screening - Hearing (SWISH) Program]
* American Academy of Audiology - [http://www.audiology.org/Pages/default.aspx  American Academy of Audiology] | [http://www.audiology.org/news/Pages/JudyGravel.aspx In Memoriam: Judy Gravel]




Revision as of 10:17, 5 June 2010

Hearing cartoon.jpg

Introduction

Newborn hearing test

How and why do things go wrong in development? Developing of hearing requires a complex origin, organisation, and timecourse means that abnormal development of any one system can impact upon the development of hearing. There are many different abnormalities of hearing development that can result in hearing loss and can broadly be divided into either conductive or sensorineural loss. These abnormalities can have genetic, environmental or unknown origins. In addition, abnormalities of the external ear (position and structure) is used as a clinical diagnostic tool for developmental abnormalities in other systems.


In Australia, there is now an early postnatal screening of neonatal hearing as part of a NSW State Wide Infant Screening Hearing (SWISH) Program using Automated Auditory Brainstem Response (AABR).

Hearing Links: Introduction | inner ear | middle ear | outer ear | balance | placode | hearing neural | Science Lecture | Lecture Movie | Medicine Lecture | Stage 22 | hearing abnormalities | hearing test | sensory | Student project

  Categories: Hearing | Outer Ear | Middle Ear | Inner Ear | Balance

Historic Embryology - Hearing 
Historic Embryology: 1880 Platypus cochlea | 1892 Vertebrate Ear | 1902 Development of Hearing | 1906 Membranous Labyrinth | 1910 Auditory Nerve | 1913 Tectorial Membrane | 1918 Human Embryo Otic Capsule | 1918 Cochlea | 1918 Grays Anatomy | 1922 Human Auricle | 1922 Otic Primordia | 1931 Internal Ear Scalae | 1932 Otic Capsule 1 | 1933 Otic Capsule 2 | 1936 Otic Capsule 3 | 1933 Endolymphatic Sac | 1934 Otic Vesicle | 1934 Membranous Labyrinth | 1934 External Ear | 1938 Stapes - 7 to 21 weeks | 1938 Stapes - Term to Adult | 1940 Stapes | 1942 Stapes - Embryo 6.7 to 50 mm | 1943 Stapes - Fetus 75 to 150 mm | 1946 Aquaductus cochleae and periotic (perilymphatic) duct | 1946 aquaeductus cochleae | 1948 Fissula ante fenestram | 1948 Stapes - Fetus 160 mm to term | 1959 Auditory Ossicles | 1963 Human Otocyst | Historic Disclaimer

| original page


Abnormality Links: abnormal development | abnormal genetic | abnormal environmental | Unknown | teratogens | ectopic pregnancy | cardiovascular abnormalities | coelom abnormalities | endocrine abnormalities | gastrointestinal abnormalities | genital abnormalities | head abnormalities | integumentary abnormalities | musculoskeletal abnormalities | limb abnormalities | neural abnormalities | neural crest abnormalities | placenta abnormalities | renal abnormalities | respiratory abnormalities | hearing abnormalities | vision abnormalities | twinning | Developmental Origins of Health and Disease |  ICD-11
Historic Embryology  
1915 Congenital Cardiac Disease | 1917 Frequency of Anomalies in Human Embryos | 1920 Hydatiform Degeneration Tubal Pregnancy | 1921 Anencephalic Embryo | 1921 Rat and Man | 1966 Congenital Malformations

Some Recent Findings

  • Review - The etiology of otosclerosis[1] "Otosclerosis is a common form of hearing loss characterized by abnormal bone remodeling in the otic capsule. It is a complex genetic disease, caused by a combination of genetic and environmental factors. During the past decade, several attempts have been made to identify factors for otosclerosis."


Inner Ear Abnormalities

Common cavity, severe cochlear hypoplasia

Cholesteatoma

Epithelium trapped within skull base in development, erosion of bones: temporal bone, middle ear, mastoid

Middle Ear Abnormalities

Rare and can be part of first arch syndrome.

Fixation of the middle ear ossicles Malleus, Incus and Stapes

Outer Ear Abnormalities

Microtia
Preauricular sinus

Several genetic effects and syndromes, Environmental Effects

  • Microtia - abnormally small external ear
  • Preauricular sinus - occurs in 0.25% births, bilateral (hereditary) 25-50%, unilateral (mainly the left), duct runs inward can extend into the parotid gland, Postnatally sites for infection

Fetal Alcohol Syndrome

Fetal Alcohol Syndrome Face
  • Postion- Lower or uneven height, "railroad track” appearance, curve at top part of outer ear is under-developed, folded over parallel to curve beneath

Congenital Deafness

Sensorineural - cochlear or central auditory pathway Conductive - disease of outer and middle ear

Sensorineural

Cochlear or central auditory pathway

  • Hereditary
  • recessive- severe
  • dominant- mild
    • can be associated with abnormal pigmentation (hair and irises)
  • Acquired
    • rubella (German measles), maternal infection during 2nd month of pregnancy, vaccination of young girls
    • cytomegalovirus [2]
    • streptomycin
    • antibiotic
    • thalidomide

Conductive

Disease of outer and middle ear

  • produced by otitis media with effusion, is widespread in young children.
  • temporary blockage of outer or middle ear

Newborn Hearing Screening

Newborn hearing test

In Australia, there is now an early postnatal screening of neonatal hearing as part of a NSW State Wide Infant Screening Hearing (SWISH) Program using Automated Auditory Brainstem Response (AABR).

  • Very low birthweight infants and universal newborn hearing screening in a developing country[3]

Links: NSW Statewide Infant Screening - Hearing (SWISH) Program

Bionic Ear

The "Cochlear Implant" was pioneered in development by Professor Graeme Clark (1960s, Australia).[4] It consists of an array of electrodes implanted within cochlea, that directly electrically stimulate the auditory nerve fibres.

  • Young children with cochlear implants compared with children with normal hearing.[5]

References

  1. ↑ <pubmed>20513039</pubmed>
  2. ↑ <pubmed>20500943</pubmed>
  3. ↑ <pubmed>20450464</pubmed>
  4. ↑ <pubmed>18816421</pubmed>JRRD
  5. ↑ <pubmed>20452685</pubmed>

Online Textbooks

Reviews

Articles

Search Pubmed

Search Pubmed: Abnormalities


Additional Images

  • Inner ear hair cells

    Inner ear hair cells

  • External ear anatomy

    External ear anatomy

  • Preauricular tag

    Preauricular tag

  • Preauricular tag

    Preauricular tag

External Links


Glossary Links

Glossary: A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z | Numbers | Symbols | Term Link

Cite this page: Hill, M.A. (2024, May 1) Embryology Sensory - Hearing Abnormalities. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Sensory_-_Hearing_Abnormalities

What Links Here?
© Dr Mark Hill 2024, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G
Retrieved from ‘https://embryology.med.unsw.edu.au/embryology/index.php?title=Sensory_-_Hearing_Abnormalities&oldid=25138’
Powered by MediaWiki