Mondini dysplasia--late complications. 1985

D P Mitchell, and A M Rubin

Mondini's deformity is the second most common malformation seen in congenital genetic deafness. It is characterized by bony and membranous abnormalities of the inner ear, with a wide range of morphological and functional abnormalities. The importance of the Mondini malformation is that the patient is at an increased risk of developing meningitis or bilateral total hearing loss (or both) at an early age. Six cases are presented which illustrate the various sequelae of the Mondini malformation including meningitis, vertigo, and auditory deterioration. Decompression of the endolymphatic sac was undertaken in three patients. Emphasis is placed on the danger of head trauma, even minor, in these patients. The necessity of early diagnosis with adequate patient counseling is stressed.

UI MeSH Term Description Entries
D007758 Ear, Inner The essential part of the hearing organ consists of two labyrinthine compartments: the bony labyrinthine and the membranous labyrinth. The bony labyrinth is a complex of three interconnecting cavities or spaces (COCHLEA; VESTIBULAR LABYRINTH; and SEMICIRCULAR CANALS) in the TEMPORAL BONE. Within the bony labyrinth lies the membranous labyrinth which is a complex of sacs and tubules (COCHLEAR DUCT; SACCULE AND UTRICLE; and SEMICIRCULAR DUCTS) forming a continuous space enclosed by EPITHELIUM and connective tissue. These spaces are filled with LABYRINTHINE FLUIDS of various compositions. Labyrinth,Bony Labyrinth,Ear, Internal,Inner Ear,Membranous Labyrinth,Bony Labyrinths,Ears, Inner,Ears, Internal,Inner Ears,Internal Ear,Internal Ears,Labyrinth, Bony,Labyrinth, Membranous,Labyrinths,Labyrinths, Bony,Labyrinths, Membranous,Membranous Labyrinths
D008297 Male Males
D008575 Meniere Disease A disease of the inner ear (LABYRINTH) that is characterized by fluctuating SENSORINEURAL HEARING LOSS; TINNITUS; episodic VERTIGO; and aural fullness. It is the most common form of endolymphatic hydrops. Meniere's Disease,Meniere's Syndrome,Vertigo, Aural,Auditory Vertigo,Aural Vertigo,Ménière Disease,Ménière's Disease,Ménière's Vertigo,Otogenic Vertigo,Auditory Vertigos,Disease, Meniere,Disease, Meniere's,Disease, Ménière,Disease, Ménière's,Diseases, Ménière,Diseases, Ménière's,Meniere Syndrome,Menieres Disease,Menieres Syndrome,Ménière Diseases,Ménière Vertigo,Ménière's Diseases,Ménière's Vertigos,Ménières Disease,Ménières Vertigo,Otogenic Vertigos,Syndrome, Meniere's,Vertigo, Auditory,Vertigo, Ménière's,Vertigo, Otogenic,Vertigos, Auditory,Vertigos, Ménière's,Vertigos, Otogenic
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003638 Deafness A general term for the complete loss of the ability to hear from both ears. Deafness Permanent,Hearing Loss Permanent,Prelingual Deafness,Deaf Mutism,Deaf-Mutism,Deafness, Acquired,Hearing Loss, Complete,Hearing Loss, Extreme,Acquired Deafness,Complete Hearing Loss,Deafness, Prelingual,Extreme Hearing Loss,Permanent, Deafness,Permanent, Hearing Loss,Permanents, Deafness
D005260 Female Females
D006259 Craniocerebral Trauma Traumatic injuries involving the cranium and intracranial structures (i.e., BRAIN; CRANIAL NERVES; MENINGES; and other structures). Injuries may be classified by whether or not the skull is penetrated (i.e., penetrating vs. nonpenetrating) or whether there is an associated hemorrhage. Frontal Region Trauma,Head Injuries,Head Trauma,Occipital Region Trauma,Parietal Region Trauma,Temporal Region Trauma,Craniocerebral Injuries,Crushing Skull Injury,Forehead Trauma,Head Injuries, Multiple,Head Injury, Minor,Head Injury, Open,Head Injury, Superficial,Injuries, Craniocerebral,Injuries, Head,Multiple Head Injuries,Occipital Trauma,Open Head Injury,Superficial Head Injury,Trauma, Head,Craniocerebral Injury,Craniocerebral Traumas,Crushing Skull Injuries,Forehead Traumas,Frontal Region Traumas,Head Injuries, Minor,Head Injuries, Open,Head Injuries, Superficial,Head Injury,Head Injury, Multiple,Head Traumas,Injuries, Minor Head,Injuries, Multiple Head,Injuries, Open Head,Injuries, Superficial Head,Injury, Craniocerebral,Injury, Head,Injury, Minor Head,Injury, Multiple Head,Injury, Open Head,Injury, Superficial Head,Minor Head Injuries,Minor Head Injury,Multiple Head Injury,Occipital Region Traumas,Occipital Traumas,Open Head Injuries,Parietal Region Traumas,Region Trauma, Frontal,Region Trauma, Occipital,Region Trauma, Parietal,Region Traumas, Frontal,Region Traumas, Occipital,Region Traumas, Parietal,Skull Injuries, Crushing,Skull Injury, Crushing,Superficial Head Injuries,Temporal Region Traumas,Trauma, Craniocerebral,Trauma, Forehead,Trauma, Frontal Region,Trauma, Occipital,Trauma, Occipital Region,Trauma, Parietal Region,Trauma, Temporal Region,Traumas, Craniocerebral,Traumas, Forehead,Traumas, Frontal Region,Traumas, Head,Traumas, Occipital,Traumas, Occipital Region,Traumas, Parietal Region,Traumas, Temporal Region
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths

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