Book contents
- Frontmatter
- Dedication
- Contents
- Preface
- Case 1 Dense basilar artery sign
- Case 2 Global anoxic brain injury
- Case 3 Acute infarction
- Case 4 Vertebral artery dissection
- Case 5 Subacute infarct
- Case 6 Subarachnoid hemorrhage
- Case 7 Intracranial aneurysms
- Case 8 Giant aneurysms
- Case 9 Acute intracerebral hematoma
- Case 10 Cerebral amyloid angiopathy
- Case 11 Primary CNS vasculitis
- Case 12 Reversible cerebral vasoconstriction syndrome
- Case 13 Moyamoya disease/syndrome
- Case 14 Cortical venous thrombosis
- Case 15 Developmental venous anomalies
- Case 16 Dural arteriovenous fistula
- Case 17 Cavernous malformation
- Case 18 Tumefactive demyelinating lesion
- Case 19 Acute disseminated encephalomyelitis
- Case 20 Progressive multifocal leukoencephalopathy
- Case 21 Osmotic myelinolysis
- Case 22 Neurosarcoidosis
- Case 23 Posterior fossa masses in children
- Case 24 Low-grade glioma
- Case 25 Diffuse intrinsic pontine glioma
- Case 26 Pseudoprogression of GBM
- Case 27 Pseudoresponse in treatment of GBM
- Case 28 Low-grade oligodendroglioma
- Case 29 Primary CNS lymphoma
- Case 30 Pineal region tumors
- Case 31 Intraventricular masses
- Case 32 Colloid cyst
- Case 33 Primary intraosseous meningioma
- Case 34 Suprasellar meningioma
- Case 35 Pituitary macroadenoma
- Case 36 Brain abscess
- Case 37 Neurocysticercosis
- Case 38 Tuberculosis
- Case 39 Creutzfeldt–Jakob disease
- Case 40 Herpes encephalitis
- Case 41 Wernicke’s encephalopathy
- Case 42 Hypertrophic olivary degeneration
- Case 43 Adrenoleukodystrophy
- Case 44 Mild traumatic brain injury
- Case 45 Isodense subdural hematoma
- Case 46 Posterior reversible encephalopathy syndrome
- Case 47 Late-onset adult hydrocephalus secondary to aqueductal stenosis
- Case 48 Intracranial hypotension
- Case 49 Idiopathic intracranial hypertension
- Case 50 Rathke’s cleft cyst
- Case 51 FLAIR sulcal hyperintensity secondary to general anesthesia
- Case 52 Virchow–Robin spaces
- Case 53 Arachnoid granulations
- Case 54 Benign external hydrocephalus
- Case 55 Pitfalls in CTA
- Case 56 Asymmetric pneumatization of the anterior clinoid process
- Case 57 Fibrous dysplasia of skull base
- Case 58 Sphenoid bone pseudolesion
- Case 59 Clival lesions
- Case 60 Perineural spread
- Case 61 Cochlear dysplasia
- Case 62 Labyrinthitis ossificans
- Case 63 Superior semicircular canal dehiscence
- Case 64 Fluid entrapment in the petrous apex cells
- Case 65 Acquired cholesteatoma
- Case 66 Malignant otitis externa
- Case 67 Temporal bone fractures
- Case 68 Allergic fungal sinusitis
- Case 69 Invasive fungal sinusitis
- Case 70 Spontaneous CSF leaks and sphenoid cephaloceles
- Case 71 Juvenile nasal angiofibroma
- Case 72 Idiopathic orbital pseudotumor
- Case 73 Optic neuritis
- Case 74 Intraparotid lymph nodes
- Case 75 Benign mixed tumor
- Case 76 First branchial cleft cyst
- Case 77 Nasopharyngeal cysts
- Case 78 Cystic nodal metastasis
- Case 79 Low-flow vascular malformations
- Case 80 Parapharyngeal masses
- Case 81 Third branchial apparatus anomaly
- Case 82 Parathyroid adenoma
- Case 83 String sign
- Case 84 Carotid artery dissection
- Case 85 Traumatic arterial injury
- Case 86 Craniovertebral junction injuries
- Case 87 Odontoid fractures
- Case 88 Vertebral compression fractures
- Case 89 Sacral insufficiency fracture
- Case 90 Paget’s disease of the spine
- Case 91 Renal osteodystrophy
- Case 92 Calcific tendinitis of the longus colli
- Case 93 T2 hyperintense disc herniation
- Case 94 Disc herniation and cord compression
- Case 95 Postoperative disc herniation versus postsurgical scarring
- Case 96 Degenerative endplate alterations
- Case 97 Spinal dysraphism
- Case 98 Tethered spinal cord
- Case 99 Chiari I malformation
- Case 100 Spinal vascular malformations
- Case 101 Cord compression
- Case 102 Demyelinating/inflammatory spinal cord lesion
- Case 103 Subacute combined degeneration
- Case 104 Intradural cyst
- Case 105 Spinal CSF leaks
- Case 106 Leptomeningeal drop metastases
- Index
- References
Case 61 - Cochlear dysplasia
Published online by Cambridge University Press: 18 December 2013
- Frontmatter
- Dedication
- Contents
- Preface
- Case 1 Dense basilar artery sign
- Case 2 Global anoxic brain injury
- Case 3 Acute infarction
- Case 4 Vertebral artery dissection
- Case 5 Subacute infarct
- Case 6 Subarachnoid hemorrhage
- Case 7 Intracranial aneurysms
- Case 8 Giant aneurysms
- Case 9 Acute intracerebral hematoma
- Case 10 Cerebral amyloid angiopathy
- Case 11 Primary CNS vasculitis
- Case 12 Reversible cerebral vasoconstriction syndrome
- Case 13 Moyamoya disease/syndrome
- Case 14 Cortical venous thrombosis
- Case 15 Developmental venous anomalies
- Case 16 Dural arteriovenous fistula
- Case 17 Cavernous malformation
- Case 18 Tumefactive demyelinating lesion
- Case 19 Acute disseminated encephalomyelitis
- Case 20 Progressive multifocal leukoencephalopathy
- Case 21 Osmotic myelinolysis
- Case 22 Neurosarcoidosis
- Case 23 Posterior fossa masses in children
- Case 24 Low-grade glioma
- Case 25 Diffuse intrinsic pontine glioma
- Case 26 Pseudoprogression of GBM
- Case 27 Pseudoresponse in treatment of GBM
- Case 28 Low-grade oligodendroglioma
- Case 29 Primary CNS lymphoma
- Case 30 Pineal region tumors
- Case 31 Intraventricular masses
- Case 32 Colloid cyst
- Case 33 Primary intraosseous meningioma
- Case 34 Suprasellar meningioma
- Case 35 Pituitary macroadenoma
- Case 36 Brain abscess
- Case 37 Neurocysticercosis
- Case 38 Tuberculosis
- Case 39 Creutzfeldt–Jakob disease
- Case 40 Herpes encephalitis
- Case 41 Wernicke’s encephalopathy
- Case 42 Hypertrophic olivary degeneration
- Case 43 Adrenoleukodystrophy
- Case 44 Mild traumatic brain injury
- Case 45 Isodense subdural hematoma
- Case 46 Posterior reversible encephalopathy syndrome
- Case 47 Late-onset adult hydrocephalus secondary to aqueductal stenosis
- Case 48 Intracranial hypotension
- Case 49 Idiopathic intracranial hypertension
- Case 50 Rathke’s cleft cyst
- Case 51 FLAIR sulcal hyperintensity secondary to general anesthesia
- Case 52 Virchow–Robin spaces
- Case 53 Arachnoid granulations
- Case 54 Benign external hydrocephalus
- Case 55 Pitfalls in CTA
- Case 56 Asymmetric pneumatization of the anterior clinoid process
- Case 57 Fibrous dysplasia of skull base
- Case 58 Sphenoid bone pseudolesion
- Case 59 Clival lesions
- Case 60 Perineural spread
- Case 61 Cochlear dysplasia
- Case 62 Labyrinthitis ossificans
- Case 63 Superior semicircular canal dehiscence
- Case 64 Fluid entrapment in the petrous apex cells
- Case 65 Acquired cholesteatoma
- Case 66 Malignant otitis externa
- Case 67 Temporal bone fractures
- Case 68 Allergic fungal sinusitis
- Case 69 Invasive fungal sinusitis
- Case 70 Spontaneous CSF leaks and sphenoid cephaloceles
- Case 71 Juvenile nasal angiofibroma
- Case 72 Idiopathic orbital pseudotumor
- Case 73 Optic neuritis
- Case 74 Intraparotid lymph nodes
- Case 75 Benign mixed tumor
- Case 76 First branchial cleft cyst
- Case 77 Nasopharyngeal cysts
- Case 78 Cystic nodal metastasis
- Case 79 Low-flow vascular malformations
- Case 80 Parapharyngeal masses
- Case 81 Third branchial apparatus anomaly
- Case 82 Parathyroid adenoma
- Case 83 String sign
- Case 84 Carotid artery dissection
- Case 85 Traumatic arterial injury
- Case 86 Craniovertebral junction injuries
- Case 87 Odontoid fractures
- Case 88 Vertebral compression fractures
- Case 89 Sacral insufficiency fracture
- Case 90 Paget’s disease of the spine
- Case 91 Renal osteodystrophy
- Case 92 Calcific tendinitis of the longus colli
- Case 93 T2 hyperintense disc herniation
- Case 94 Disc herniation and cord compression
- Case 95 Postoperative disc herniation versus postsurgical scarring
- Case 96 Degenerative endplate alterations
- Case 97 Spinal dysraphism
- Case 98 Tethered spinal cord
- Case 99 Chiari I malformation
- Case 100 Spinal vascular malformations
- Case 101 Cord compression
- Case 102 Demyelinating/inflammatory spinal cord lesion
- Case 103 Subacute combined degeneration
- Case 104 Intradural cyst
- Case 105 Spinal CSF leaks
- Case 106 Leptomeningeal drop metastases
- Index
- References
Summary
Imaging description
The spectrum of congenital cochlear and vestibular anomalies is very wide, ranging from complete absence of the inner ear structures to minimal dysplastic chances that are difficult to visualize. Essentially all of these present with bilateral congenital sensorineural hearing loss, however [1].
The most severe variety is known as labyrinthine aplasia (Michel’s anomaly), where there is no cochlea, vestibule, or semicircular canal development at all. Common cavity is lesser in severity and refers to a single “cystic” space that represents a combination of dysplastic vestibule and cochlea that does not closely resemble either [2]. When the developmental arrest occurs around week 7 of gestation the cochlea is better formed and may present as incomplete partition type I (IP-I) (Fig. 61.1), where there is incomplete development of all turns of the cochlea, with lack of modiolus. When there is normal differentiation of the basilar turn of the cochlea with incomplete partition between middle and apical cochlear turns and a large vestibular aqueduct (LVA), it is known as incomplete partition type II (IP-II) anomaly (Figs. 61.2, 61.3). This is also known as the classic Mondini type of anomaly, although in the literature the term Mondini is commonly used, erroneously, to refer to a wide spectrum of inner ear anomalies [3]. An even milder form of cochlear dysplasia is due to incomplete development of the apical turn of the cochlea, which occurs at week 8 of gestation (Fig. 61.4). It results in modiolus deficiency with mild asymmetry of the scalar vestibuli and scala tympani. It is often associated with LVA.
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- Pearls and Pitfalls in Head and Neck and NeuroimagingVariants and Other Difficult Diagnoses, pp. 285 - 288Publisher: Cambridge University PressPrint publication year: 2013