EURORAD ESR

Case 12603

Brothers with X-linked congenital hearing loss

Author(s)
McGarry P, Turner J.

Ulster Hospital,
Upper Newtonards Road,
Dundonald, N. Ireland, BT16 1RH.
 
Patient
male, 255 year(s)
 
 
  • Figure 1
    Patient 1 - axial CT

    Patient 1 - axial CT showing bulbous left-sided IAC (white arrow)

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 2
    Patient 1 - axial CT

    Patient 1 - axial CT showing bulbous right-sided IAC (white arrow)

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 3
    Patient 1 - axial CT

    Patient 1 - axial CT showing a mildly enlarged vestibular aqueduct on the right side (white arrow) and abnormal configuration of the vestibule (grey arrow).

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 4
    Patient 1 - coronal CT

    Patient 1 - coronal CT showing similar bilateral abnormalities of bulbous IAC with lack of separation from basal turn of cochlea (white arrows) and abscence of bony modiolus (grey arrows).

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 5
    Patient 2 - axial CT

    Patient 2 - axial CT showing bulbous IAC bilaterally (white arrows).

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 6
    Patient 2 - axial CT

    Patient 2 - axial CT showing hypoplasia of the basal turn of the right cochlea which cannot be separated from the IAC (white arrow). Abscence of the bony modiolus (grey arrow).

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 7
    Patient 2 - axial CT

    Patient 2 - axial CT showing hypoplasia of basal turn of left cochlea which cannot be seperated from IAC (white arrow). Absence of bony modiolus (grey arrow).

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 8
    Patient 1 - axial CISS MRI

    Patient 1 - axial CISS MRI showing bulbous right IAC with lack of separation from basal turn of cochlea (white arrow). Note prominent vestibular aqueduct (grey arrow), which is larger in diameter than lateral...

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 9
    Patient 1 - axial CISS MRI

    Patient 1 - axial CISS MRI showing bulbous left IAC with lack of separation from basal turn of cochlea (white arrow). Note prominent vestibular aqueduct (grey arrow), similar in diameter to the lateral semicircular...

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 10
    Patient 2 - axial CISS MRI

    Patient 2 - axial CISS MRI showing bulbous IAC with lack of separation from the basal turn of the cochlea (right).

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 11
    Patient 2 - axial CISS MRI

    Patient 2 - axial CISS MRI showing bulbous IAC with lack of separation from the basal turn of the cochlea (left). Note the normal left vestibular aqueduct.

     
    Area of Interest: Ear / Nose / Throat; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
Patient 1 - axial CT showing bulbous left-sided IAC (white arrow)
 
Patient 1 - axial CT showing bulbous right-sided IAC (white arrow)
 
Patient 1 - axial CT showing a mildly enlarged vestibular aqueduct on the right side (white arrow) and abnormal configuration of the vestibule (grey arrow).
 
Patient 1 - coronal CT showing similar bilateral abnormalities of bulbous IAC with lack of separation from basal turn of cochlea (white arrows) and abscence of bony modiolus (grey arrows).
 
Patient 2 - axial CT showing bulbous IAC bilaterally (white arrows).
 
Patient 2 - axial CT showing hypoplasia of the basal turn of the right cochlea which cannot be separated from the IAC (white arrow). Abscence of the bony modiolus (grey arrow).
 
Patient 2 - axial CT showing hypoplasia of basal turn of left cochlea which cannot be seperated from IAC (white arrow). Absence of bony modiolus (grey arrow).
 
Patient 1 - axial CISS MRI showing bulbous right IAC with lack of separation from basal turn of cochlea (white arrow). Note prominent vestibular aqueduct (grey arrow), which is larger in diameter than lateral semicircular canal.
 
Patient 1 - axial CISS MRI showing bulbous left IAC with lack of separation from basal turn of cochlea (white arrow). Note prominent vestibular aqueduct (grey arrow), similar in diameter to the lateral semicircular canal.
 
Patient 2 - axial CISS MRI showing bulbous IAC with lack of separation from the basal turn of the cochlea (right).
 
Patient 2 - axial CISS MRI showing bulbous IAC with lack of separation from the basal turn of the cochlea (left). Note the normal left vestibular aqueduct.
 
 
 
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