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2 results

  • Chapter 4 - Aplasia

  • Jon van der Walt, Attilio Orazi, Daniel A. Arber, University of Chicago
  • Book:
    Diagnostic Bone Marrow Haematopathology
    Published online:
    12 November 2020
    Print publication:
    21 January 2021, pp 42-63

  • Summary

    Aplasia is a pathologic term that is broadly defined as the absence or near-absence of one or more haematopoietic lineages in the bone marrow (BM). Clinically, BM aplasia affecting more than one lineage is referred to as aplastic anaemia (AA), despite the fact that this group of disorders often results in pancytopaenia rather than anaemia alone. Cytopaenias can be seen in a number of different conditions, and new-onset pancytopaenia in children and adults requires an extensive work-up, including a BM core biopsy (BMB) for confirmation of haematopoietic aplasia/hypoplasia and exclusion of an infiltrative marrow process or fibrosis. Bone marrow aplasia develops as a result of injury to multipotent haematopoietic stem cells, which can occur in the context of constitutional (primary aplasia) or acquired (secondary aplasia) disorders (Table 4.1). This chapter will discuss the diagnostic criteria and pathophysiology of specific disorders presenting with aplasia and demonstrate an algorithmic approach to the diagnostic evaluation of patients presenting with this common and non-specific finding (Table 4.2).

  • Unilateral auditory neuropathy spectrum disorder: retrocochlear lesion in disguise?

  • A Mohammadi, P Walker, K Gardner-Berry
  • Journal:
    The Journal of Laryngology & Otology / Volume 129 / Issue S1 / January 2015
    Published online by Cambridge University Press:
    18 November 2014, pp. S38-S44
    Print publication:
    January 2015
  • Objective:

    To investigate whether the aetiology for hearing impairment in neonates with unilateral auditory neuropathy spectrum disorder could be explained by structural abnormalities such as cochlear nerve aplasia, a cerebellopontine angle tumour or another identifiable lesion.

    Methods:

    In this prospective case series, 17 neonates were diagnosed with unilateral auditory neuropathy spectrum disorder on electrophysiological testing. Diagnostic audiology testing, including auditory brainstem response testing, was supplemented with computed tomography and/or magnetic resonance imaging.

    Results:

    Ten of the neonates (59 per cent) showed evidence for cochlear nerve aplasia. Of the remaining seven, four were shown to have another abnormality of the temporal bone on imaging. Only three neonates (18 per cent) were not diagnosed with cochlear nerve aplasia or another lesion. Three computed tomography scans were reported as normal, but subsequent magnetic resonance imaging revealed cochlear nerve aplasia.

    Conclusion:

    Auditory neuropathy spectrum disorder as a unilateral condition mandates further investigation for a definitive diagnosis. This series demonstrates that most neonates with unilateral auditory neuropathy spectrum disorder had pathology as visualised on computed tomography and/or magnetic resonance imaging scans. Magnetic resonance imaging is an appropriate first-line imaging modality.