This report highlights the case of a patient with mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus. Recently, the american journal of otology published an english translation by gordon j. Mondini dysplasia has been associated with thalidomide and rubella embryopathies as well as a number of syndromes an incomplete partition is called the mondini dysplasia or malformation. The hearing loss is associated with inner ear abnormalities, which can be readily identified with appropriate imaging procedures, ranging from an isolated enlarged vestibular aqueduct eva to a typical cochlear malformation known as mondini dysplasia. We reported two cases of recurrent meningitis and both of them had mondini dysplasia, which provides a link between the brain and inner ear and is associated with cerebrospinal fluid, otorrhearhinorrhea, hearing impairment, and recurrent meningitis. Patients with complex of enlarged vestibular aqueduct, mondini dysplasia, large vestibule, and semicircular canal dysplasia emvs complex demonstrated a significantly higher incidence of fluctuating hearing loss 93% and a better hearing level compared with those with other malformations. The early diagnosis of the mondini dysplasia by means of electrocochleography and temporal bone polytomography, and the prevention of further hearing loss through the endolymphatic subarachnoid shunt operation, brought new data on the followup of patients. Caroosorio e, espinoojeda a, guevaramaldonado l, herreacastro jc. Mondini dysplasia, also known as mondini malformation and mondini defect, is an abnormality of the inner ear that is associated with sensorineural hearing loss. Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. To change the order of your pdfs, drag and drop the files as you want. Cochlear anomalies classification radiology reference. Four major patterns of congenital inner ear malformation have been described.
Outcomes in cochlear implantation in incomplete partition type ii mondini dysplasia seung hyo choi2, kwang sun lee1 1 department of otolaryngology, asan medical center, university of ulsan college of medicine, seoul, korea 2 department of otolaryngology,jeju national university hospital, jeju national university, college of medicine, jeju, korea. Peripheral vestibular pathology in mondini dysplasia. Outcomes in cochlear implantation in incomplete partition. Mondini dysplasia presenting as otorrhea without meningitis.
A new classification for cochleovestibular malformations. Children with mondini dysplasia are predisposed to developing a spontaneous cerebrospinal fluid csf leak and recurrent meningitis. M ore than 200 years ago, temporal bone dissection by carlo mondini revealed wide vestibular aqueducts in patients with cochlear dysplasia mondinis dysplasia. Mondini dysplasia genetic and rare diseases information center. Usher syndrome can be classified into 3 different types on the basis of clinical findings. Therefore, it is more accurate and useful for clinical purposes to classify these malformations in descending order of severity as follows. Miyamoto et al performed cochlear implantation on a child with mondini inner ear malformation and encountered a profuse flow of.
In our patient, despite having a congenital malformation of the inner ear, mondini dysplasia, curiously, the meningoencephalic infections did not begin until 10. If you have problems viewing pdf files, download the latest version of adobe reader. The mondini dysplasia from early diagnosis to cochlear implant. Michel deformity, cochlear aplasia, common cavity, ip.
What is a mondini and what difference does a name make. Common clinical features of children with enlarged. The deafness is associated with an abnormality of the bony labyrinth. Mondini dysplasia genetic and rare diseases information. Mondini dysplasia is a congenital malformation of the inner ear.
Mondini dysplasia is a rare malformation of the inner ear commonly associated with loss of hearing and vestibular function. You can merge pdfs or a mix of pdf documents and other files. The inner ear as seen from the back of the head, left side left image. Merge pdf files combine pdfs in the order you want with the easiest pdf merger available. Pdf mondini dysplasia presenting as otorrhea without meningitis. Diagnosis is based on the ct images and the rm images. Pdf the laura multichannel cochlear implant in a true.
Mondini dysplasia, described in 1791 by carlo mondini, is one of the most commonly diagnosed inner ear malformations. There is a recent article, entitled recurrent bacterial. Tray sealing technology, platform technology and filling systems. Mondini dysplasia associated to recurrent bacterial meningitisa clinical and imaging correlation. It was first described in 1791 by carlo mondini after dissecting the inner ear of an 8yearold deaf boy.
Mondini dysplasia an overview sciencedirect topics. Pdf mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. This deformity was first described in 1791 by mondini after examining the inner ear of a deaf boy. Cochlear implantation in mondini dysplasia request pdf. Cochlear anomalies are a variety of congenital anomalies which, depending on the exact time at which an insult occurs during embryogenesis, may have different manifestations.
Terminology is often used imprecisely leading to confusion not only among clinicians, but also in the literature. The mondini malformation is a rare congenital inner. We compared those numbers in our mondini dysplasia group versus our control group. Click, drag, and drop to reorder files or press delete to remove any content you dont want. Mondini malformation is a historical term used to described incomplete partition abnormalities michel deformity, cochlear aplasia and cochlear hypoplasia. Mondini dysplasia is a malformation of the inner ear regrouping two different anomalies. Mondini dysplasia is a type of inner ear malformation that is present at birth congenital. Utility of magnetic resonance cisternography with intrathecal gadolinium in detection of cerebrospinal fluid fistula associated with mondini dysplasia in a patient with recurrent meningitis. Mondini deformity represents a later malformation, so the amount of dysplasia is much less than in type ii. Mondini dysplasia is an inner ear malformation thought to be caused by the arrest of neural tube development.
Using mondini dysplasia as an inclusive term would not have permitted differentiation between these two subgroups. The frequency of these disorders mainly comes from temporal bone autopsies. Patients with mondini deformity often have loss of lowfrequency hearing and vertigo. Rearrange individual pages or entire files in the desired order. The mondini dysplasia can occur in cases of pendred syndrome and branchiootorenal syndrome and in other syndromes, but can occur in nonsyndromic deafness.
In 1978, valvassori and clemis were the first to use imaging to identify 50 patients with large vestibular aqueducts lvas. Case report recurrent bacterial meningitis in a child with. Fisher, md, phd maslah saul md professor of neurology director, stanford epilepsy center in 2005, the ilae released a conceptual definition of seizures and epilepsy, followed by an operational practical definition in 2014. Cochlear disorders are often all classified as mondini deformities but more accurately mondini dysplasia is the result of absence of the distal 1 and 12 turns of the cochlea i. Red indicates serious or lifethreatening conditions blue indicates common conditions history prenatal or perinatal infection. Sensorineural hearing loss and mondini dysplasia caused by. Mondini malformation discussion cochlear disorders are often all classified as mondini deformities but more accurately mondini dysplasia is the result of absence of the distal 1 and 12 turns of the cochlea i. Mondini deformity refers to the absence of the apical modiolus and interscalar septum, resulting in an incomplete partitioning of the cochlea together with an enlarged vestibular aqueduct eva and dilated vestibule. Computed tomography showed inner ear malformations in both males, with enlarged internal auditory meatus and mondini dysplasia. Mondini dysplasia, also known as mondini malformation and mondini defect, is an abnormality. A perchlorate discharge test documents delayed organification of iodine by the thyroid. Mondini dysplasia is characterized by a short flat cochlea, large vestibule, wide, small or missing semicircular canals, and immature sensorineural structures.
Individuals with mondini dysplasia have one and a half coils of the cochlea instead of. Eg, mondini dysplasia, usher syndrome, joubert syndrome, schiebe deformity, enlarged vestibular aqueduct syndrome evaluation of dizziness in children and adolescents, table 1 uptodate, 2012. Sensorineural hearing loss and mondini dysplasia caused by a. In mondini dysplasia, the apical turn of the cochlea fails to form, and patients are profoundly deaf at birth. Mondini dysplasia usually occurs sporadically as an isolated abnormality occurring in only one individual in a family with no other abnormalities but it can be associated with a variety of syndromes including klippel feil syndrome, pendred syndrome, digeorge syndrome, wildervanck syndrome, fountain syndrome, johansonblizzard syndrome, and some chromosomal trisomies. Otic lesions and congenital hypothyroidism in the developing chick. Apaydin et al, in most cases an omim database link to the main type of the genetic disorder is provided. The mutated pendred syndrome gene pds slc26a4 is a member of the solute carrier protein 26 anion transporter family, and the gene product pendrin is a. The mondini dysplasiafrom early diagnosis to cochlear. What links here related changes upload file special pages permanent link page. In eva, vestibular dysfunction may be present and the hearing loss is variable.
Closeup view of the inner ear comparing a normal center image and enlarged right image. Click add files and select the files you want to include in your pdf. The loss of type i and type ii hair cells in the cristae of the superior, lateral, and posterior semicircular canals, as well as in the saccular and utricular macula, was significantly higher in our mondini dysplasia group than in our control group. The fistula associated with mondini dysplasia usually presents with rhinorrhea if the tympanic membrane is intact, as was the case of our patient. Individuals with mondini dysplasia have one and a half coils of the cochlea instead of the normal two coils. The laura multichannel cochlear implant in a true mondini dysplasia.
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