Inner ear aplasia, also referred to as michel aplasia, is due to failure of development of the otic placode occurring. Inner ear aplasia, also referred to as michel aplasia, is due to failure of development of the otic placode occurring before the third week of gestation 2, 3. Furthermore, as some of these patients may need ear surgery, 2 the anomalous course of the seventh nerve may result in the morbidity of iatrogenic trauma to facial nerve. Michel aplasia, also known as complete labyrinthine aplasia cla, is a congenital abnormality of the inner ear and is characterized by bilateral absence of differentiated inner ear structures with resultant anacusis.
The prospective immunogenicity surveillance registry prims was conducted to estimate the. This article is within the scope of wikiproject medicine, which recommends that medicinerelated articles follow the manual of style for medicinerelated articles and that biomedical information in any article use highquality medical sources. Cla, also called michel anomaly or michel aplasia, is a rare. Michel in 1863 in the autopsy report of an 11yearold deaf and mute boy who died in the childrens hospital of strasbourg. Michel inner ear deformity is a rare developmental anomaly refers to the total aplasia of the inner ear. A child born with this condition has either a short or absent radius bone in one or both of his or her arms.
Complete labyrinthine aplasia cla, michel anomaly is a. Pdf we sought better understanding about the facial nerve anatomy in the rare inner ear michel anomaly to help better define this aplasia and prevent. Aplasia definition and meaning collins english dictionary. This is not to be confused with the word, atrophy, which means that something develops and then wastes away. It is caused by a defect in a developmental process. Michel aplasia is thought to result from failure of development of the otic placode. Pdf what is expected of the facial nerve in michel aplasia.
It would include the aplasia cutis congenital wherein there is a congenital absence of skin. Pure red blood cell aplasia, acquired, usually presents as a severe condition. Complete labyrinthine aplasia cla, also referred to as michel aplasia, is a severe congenital anomaly of the inner ear, defined by the complete absence of inner ear structures. It is characterized by the bilateral absence of differentiated inner ear structures and results in complete deafness. What is expected of the facial nerve in michel aplasia. It most commonly affects the scalp, but any location of the body can be affected.
Aplasia of a single cell line in the bone marrow is less common than are the trilineage aplasias described earlier, and it may be congenital or acquired. As the name suggests, this anomaly was first reported by michel in the autopsy report of a 12yearold boy. This is a very rare condition and usually affects adults. Jun 26, 2019 cochlear hypoplasia and common cavity and cochlear aplasia. Complete labyrinthine aplasia cla, michel anomaly is a severe anomaly of the ear defined by total absence of inner ear structures. Frieden classified acc in 1986 into 9 groups on the basis of location of the lesions and associated congenital anomalies. Michel aplasia, also known as complete labyrinthine aplasia cla, is a congenital abnormality of the inner ear and is characterized by bilateral absence of differentiated inner ear structures with resultant anacusis it should not be confused with michel dysplasia pathology. Pdf radiological findings of michel aplasia researchgate. Absent 8th nerves on both sides and right 7th nerve. It is defined by a bilateral absence of differentiated inner ear structures 1, and persons who have this deformity have anacusis. Magill department of otolaryngology and maxillofacial surgery room b226 956 court avenue 38163 memphis tn usa department of radiology college of medicine, the university of tennessee, the health science center room b226 956 court avenue 38163. Michel dysplasia michel dysplasia kavanagh, k magill, h.
Michel aplasia is a rare developmental anomaly refers to the total aplasia of the inner ear. Jul 01, 2019 cochlear hypoplasia and common cavity and cochlear aplasia. Additional and relevant useful information for pure red cell aplasia. Radial aplasia is a congenital defect which affects the formation of the radius bone in the arm. It is defined by a bilateral absence of differentiated inner ear structures, and persons who have this deformity have anacusis. Michel aplasia can be seen in girls or boys and it does not have any significant gender difference 68. Magill department of otolaryngology and maxillofacial surgery room b226 956 court avenue 38163 memphis tn usa department of radiology college of medicine, the university of tennessee, the health science center room b226 956 court avenue 38163 memphis tn usa abstract a case is. Please visit the project page for details or ask questions at wikipedia talk. Aplastic anemia is the failure of the body to produce blood cells. It is characterized by an absence of red cell precursors reticulocytes in the marrow and a low red blood cell count. However, if it is properly managed from the early stages, it can be maintained and. Facial nerve, michel aplasia, inner ear anomaly in 1863, michel reported a congenitally deaf 11yearold boy, from whose autopsy a bilateral absence of inner ear structures was diagnosed.
Michel aplasia definition of michel aplasia by medical. Congenital inner ear abnormality is the most common cause of neurosensory hearing loss. Cochlea, vestibule and semicircular canals do not exist. Aplasia cutis congenita is a rare disorder characterized by congenital absence of skin. Aplasia comes from a greek word a which means not and plasis meaning molding. Michel aplasia that is the most severe and rare type in these classifications occurs due to the nonformation of otic placodes in the beginning of the development. Absence of inner ear structures on both sides complete labyrnithine aplasia. We sought better understanding about the facial nerve anatomy in the rare inner ear michel anomaly to help better define this aplasia and prevent. Mondini malformation is a historical term used to described incomplete partition abnormalities michel deformity, cochlear aplasia and cochlear hypoplasia.
A case of bilateral a1 anomaly was recorded as a component of bilateral a16 anomaly bilateral total labyrinthine aplasia or michel deformity. There are some reports of this rare condition showing up in various degrees, ranging from total aplasia of petrous to more subtle defects of michel dysplasia. Michel foucault fue una figura importante en dos oleadas sucesivas del pensamiento frances del siglo xx. Michel aplasia in association with microdontia and malformation of the external ear microtia type i defines the lamm syndrome labyrinthine aplasia, microtia, and microdontia. Aplasia medical definition merriamwebster medical dictionary. Constituye una contraindicacion figura 2 aplasia laberintica completa. However, a case of unilateral michel type aplasia has recently been reported. Aplasia cutis congenita is a condition in which there is congenital present from birth absence of skin, with or without the absence of underlying structures such as bone. Michel aplasia is a rare condition, first described by p.
However, a case of unilateral micheltype aplasia has. Michel aplasia, also known as complete labyrinthine aplasia cla, is a congenital abnormality of the inner ear. Michel aplasia, first described in 1863 1 is a very rare malformation with complete bony and membranous aplasia of the inner ear. Diamondblackfan anemia is an autosomal recessive cause of pure red cell aplasia that is often associated with other abnormalities. Michel aplasia should not be confused with michel dysplasia. Sep 01, 2019 cochlear hypoplasia and common cavity and cochlear aplasia. Sep 17, 2016 aplasia cutis congenita is a condition in which there is congenital present from birth absence of skin, with or without the absence of underlying structures such as bone. Knowing the facial nerve direction in these patients will help to better define this anomaly and may eventually result in classification of michel. Chief of staff, automatism, highball, impressionism, jackpotplasia is a combining form with the meaning growth, cellular multiplication, of the kind specified by the initial element. Michel aplasia a congenital abnormality of the inner ear characterised by bilateral absence of inner ear structures, attributed to a failure of development of the otic placode. The scalp is the most commonly involved area with lesser involvement of trunk and extremities.
In 1863, michel reported a congenitally deaf 11yearold boy, from whose autopsy a bilateral absence of inner ear structures was diagnosed. Pdf incidence of erythropoietin antibodymediated pure. While most people with aplasia cutis congenita have no other abnormalities, some people have congenital. Congenital abnormalities of the inner ear is the most common cause of. As the name suggests, this anomaly was first reported by michel in the autopsy report of a 12yearold boy with a history of. However, if it is properly managed from the early stages, it can be maintained and prevented from developing into its chronic form. Jan 06, 2019 additional and relevant useful information for pure red cell aplasia. The radius is the lateral bone thumb side which connects the humerus of the upper arm to the wrist via articulation with the carpal bones. Aug 08, 2019 cochlear hypoplasia and common cavity and cochlear aplasia. Michel aplasia clearly differs from michel dysplasia, in. Other words that entered english at around the same time include.
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