Objective

2

To investigate the effect of cochlear implantation on hearing and speech rehabilitation for a patient with suspected Alport syndrome.

Methods

2

The case data of patients before cochlear implantation

cochlear implantation surgery

postoperative cochlear mapping results and evaluation data were sorted out.

Results

2

The patient was admitted to the hospital with the main complaint of "finding the hearing loss of the child for more than

5 months". CT of temporal bone showed left otitis media and common cavity malformation of bilateral inner ears. Gene report results showed that a highly suspicious mutation was found:

NM_033380.3 (COL4A5): c.1331t>g (p.ile444ser)

. Five years after cochlear implantation

the impedance results of each electrode were within the normal range (

<

15 kΩ). The child could complete the electrical stimulation threshold test with the child behavior audiometry method. All 12 pairs of electrodes were open

without discomfort to loud sound. The child could distinguish Lin's six tones. After rehabilitation

the child could have daily conversation

but his pronunciation was not clear enough. The questionnaire result showed that MAIS scored 37 points

SIR scored 4 points and CAP scored 6 points. The hearing threshold was 30-45dB HL. The syllable test result was 80%

and sentence in quiet was 84%.

Conclusion

2

The patients with suspected Alport syndrome combined with common cavity can obtain a good intervention effect by cochlear implantation and mapping.