Making the diagnosis

Diagnosis & tests

No single test makes the diagnosis. It is made by concordance — symptoms, physiology and imaging agreeing — because a dehiscence on a scan is common and easily over-called.

The audiometric signature

Trainee

The audiogram shows a low-frequency air–bone gap — but with supranormal bone conduction (thresholds better than 0 dB) and preserved acoustic reflexes, unlike true conductive loss.1

Audiogram — affected ear

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air (O)   bone (<)   gap shaded

Cervical-VEMP threshold

Affected ear70 dB
Normal ear90 dB

The affected ear responds at a lower threshold than the normal range (shaded) — the third window makes the saccule abnormally sound-sensitive. Ocular-VEMP amplitude is correspondingly raised.

VEMP — the physiologic test

VEMPs are the most useful physiologic test: the third window lowers the cervical-VEMP threshold and raises the ocular-VEMP amplitude on the affected side.2 See the VEMP chapter for technique and the full pattern library.

Imaging — confirm, don’t over-call

High-resolution temporal-bone CT confirms the defect, but axial slices over-call thin bone by partial-volume averaging. Reformat in the plane of the canal (Pöschl) and orthogonal to it (Stenvers):

dehiscence along the arccanal laid out as a ring
Pöschl plane. Parallel to the superior canal — lays the canal out as a ring so a roof defect is seen along its arc. The most sensitive single view.

For indications and protocols across vertigo, see Role of imaging.

Putting it together — the concordance check

Diagnosis requires the strands to agree. Note what happens with imaging alone: a dehiscence on CT without symptoms is an incidental finding, not the syndrome.3

Diagnostic-confidence checker

Switch on each strand that is present. Concordance of all three gives a confident diagnosis.

ResultCriteria not metWithout characteristic symptoms plus at least one objective supporting finding, SSCD is not established. Keep otosclerosis, Ménière's and perilymph fistula in the differential.

Key points

  • Low-frequency air–bone gap with supranormal bone conduction and PRESERVED reflexes — not true conductive loss.
  • VEMP: low cervical threshold, high ocular amplitude on the affected side — the key physiologic test.
  • Confirm on CT reformatted in the Pöschl and Stenvers planes; axial slices over-call thin bone.
  • Diagnose by concordance — imaging alone, without symptoms, is an incidental finding.