PMC10448821_01
Auditory tests at the first visit (A,B) and after the relapse of Kawasaki disease (C,D). Pure tone audiometry tests (A,C) and auditory steady-state response tests (B,D) were examined. At the first visit, the patient was unable to complete the left pure tone bone conduction audiometry due to his age (A). CT (E) and MRI (F) showed no significant findings including labyrinthine and ossifications in the cochlea.
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F1
The pure tone audiometry and auditory steady-state response (ASSR) test showed right-side moderate and left-side severe sensory hearing loss (Figures 1A,B). Finally, the patient developed severe hearing loss on the right side (Figures 1C,D). As preoperative evaluation by CT (Figure 1E) and MRI (Figure 1F) showed no ossification or fibrosis in the cochlea, a slim modiolar electrode was selected for the hearing preservation strategy.
Time course of the hearing level by pure tone audiometry. The average was calculated by the following formula: (T500 + T1,000 x 2 + T2,000)/4. T500, T1,000, and T2,000 indicate the level of air conduction threshold of 500, 1,000, and 2,000, respectively. Red line: right pure tone average, blue line: left pure tone average. Vertical axis: hearing level in decibels, horizontal axis: days since the onset of Kawasaki disease. Green arrowhead: timing of systemic steroid.
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F2
The time course of hearing level of both sides by pure tone audiometry is shown in Figure 2.
Left cochlear implantation. (A) The sheath of slim modiolar electrode was inserted into the round window. (B) After complete insertion of the electrode through the round window, the sheath was removed.
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F3
A shape memory electrode stored in the sheath (CI532, Cochlear Ltd.) was inserted through the round window, then the electrode was inserted along the inner wall of scala tympani to a sufficient depth without any resistance on the 1065th day after onset of KD (Figure 3).
Play audiometry (A) and speech audiometry (B) after cochlear implantation. Maximum speech discrimination score was evaluated by using a table of meaningless monosyllables of 67-S proposed by the Japan Audiological Society. Black triangle and light blue line indicate the results with cochlear implant. White triangle and red line indicate the results without cochlear implant.
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F4
One year after the surgery, a play audiometry test with the cochlear implant showed marked reduction in threshold at all frequencies compared with that without cochlear implant (Figure 4A). Speech audiometry showed 90% of maximum speech discrimination score with the cochlear implant, despite the results being completely indistinguishable without the cochlear implant (Figure 4B).