[CIS PIDD] [cis-pidd] XLA and cochlear implant

[CIS-PIDD]

Dear Guisela,

One of the (now adult) XLA patients I follow also had bilateral neurosensorial deafness, no mutations found in the DPP gene, he received bilateral cochlear implant with good success.

Best

Isabelle Meyts,
University Hospitals Leuven, Belgium

Van: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Verzonden: donderdag 9 maart 2017 7:21
Aan: CIS-PIDD <cis-pidd at lyris.dundee.net>
Onderwerp: [cis-pidd] XLA and cochlear implant

Dear colleagues.

I would truly appreciate your words of advise.

I am following a 11 year old boy, diagnosed with XLA (BTK mutation), who also presents with bilateral neurosensorial hypoacusia. I believe it is not secondary to infections or medication, I think it could be deafness-dystonia opctic neuronopathy syndrome. The patient is on IVIG and is generally doing well.

I have looked for information regarding the use of cochlear implants in these patients. The information is scarce and I have found only a few case reports, that suggest they usually don´t help.

I would like to know if you have experience with cochlear implants in XLA patients and if so, is it helpful? Would you reccommend to try it with my patient?

Thank you so much.

Best regards

Guisela Adriana Alva Lozada
Allergy and Immunology
Hospital Nacional Edgardo Rebagliati Martins
Lima Peru



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