Appropriate Imaging Modality for the Etiologic Diagnosis of Congenital Single-Sided Deafness in Children

Abstract

We aimed to compare the diagnostic yield between temporal bone computed tomography (TBCT) and internal auditory canal MRI (IAC MRI) for the etiologic diagnosis of children with congenital single-sided deafness (SSD) and the evaluation of cochlear implant (CI) candidacy. In the original cohort, 24 subjects with congenital SSD were enrolled and underwent both TBCT and IAC MRI. We recruited an additional 22 consecutive infants with congenital SSD (the supplementary cohort) and evaluated in particular the cochlear nerve (CN) integrity using IAC MRI. Cochlear nerve deficiency (CND) was classified as ‘absent’, ‘small’, and ‘indeterminate’ via mutual comparison between optical and parameters based on the MRI results. The most common etiologies were CND in the original cohort (19 out of 24). Notably, accurate evaluations of CN status (‘small CN’ = 2, ‘indeterminate CN’ = 2), inner ear malformations, and brain abnormalities were possible only with MRI. The ‘indeterminate CN’ tended to be more frequently detected in SSD ears than in unaffected ears. MRI appeared to be more accurate than TBCT in a meticulous differentiation of CN, which is crucial for the selection of appropriate CI candidacy among congenital SSD children. Additionally, we introduced the novel concept of ‘indeterminate CN’, of which the causal relationship with SSD awaits confirmation.

Cochlear Implantation in Children with Single‐Sided Deafness

“…Since cochlear nerve deficiency is estimated to occur in up to 50% of cases of severe‐to‐profound unilateral congenital hearing loss, careful review of the preoperative MRI is critical 2,37 . An MRI demonstrates greater diagnostic yield for cochlear nerve deficiency because it is able to assess for the presence of the cochlear nerve itself 38 . In contrast, a temporal bone CT scan alone does not rule out cochlear nerve deficiency because its diagnosis is inferred by the identification of a narrow bony cochlear nerve canal (BCNC), which may not always be present 39 .…”

“…39 Though a narrow BCNC has a relatively high positive predictive value for cochlear nerve deficiency, it cannot be a reliable predictor for abnormal cochlear nerve status due to its low negative predictive value, especially in the setting of a hypoplastic cochlear nerve. 38,39 This underscores the importance for obtaining an MRI in the evaluation of congenital SSD, particularly if CI is being considered.…”

“…2,37 An MRI demonstrates greater diagnostic yield for cochlear nerve deficiency because it is able to assess for the presence of the cochlear nerve itself. 38 In contrast, a temporal bone CT scan alone does not rule out cochlear nerve deficiency because its diagnosis is inferred by the identification of a narrow bony cochlear nerve canal (BCNC), which may not always be present. 39 Though a narrow BCNC has a relatively high positive predictive value for cochlear nerve deficiency, it cannot be a reliable predictor for abnormal cochlear nerve status due to its low negative predictive value, especially in the setting of a hypoplastic cochlear nerve.…”

Deregulation of Protein Phosphatase 2A inhibitor SET is Associated with Malignant Progression in Breast Cancer

“…Our results are consistent with previous reports showing that knockdown of SET with siRNA significantly reduces tumour growth of MDA-MB-231, MDA-MB-436, and MDA-MB-468 in xenograft experiments in non-obese diabetic/SCID/γ-chain null mice 15 . Furthermore, SET overexpression is associated with worse recurrence-free survival and it has been shown to reduce tamoxifen-induced anti-tumour effect in patients with primary breast cancer 26 . High SET protein levels are also associated with poor prognosis of metastatic colon cancer, non-small-cell lung cancer, pancreatic cancer, CML, and AML [16][17][18][19]27 .…”

Simpler and effective radiological evaluations for modiolar proximity of a slim modiolar cochlear implant electrode

“…High-resolution temporal bone CT scans were performed on the day after the surgery, particularly in adult cochlear implant recipients, whenever available. Axial images were obtained with 0.4 mm slice thickness using 120 kV, 64 × 0.6 mm collimation, 1-s rotation, pitch factor of 0.85, and 205 mAs, in accordance with the age of the subjects by 256-channel multi-detector computed tomography (SOMATOM Force, Siemens Healthineers, Forchheim, Germany) 30 . All data were reconstructed using two sets of ultra-high resolution kernels with different resolution indices (Uh59, resolution index of 8.3 line-pair (lp)/cm; Ur81, 19.7 lp/cm).…”