Audiological Findings in Wolf Hirschhorn Syndrome: A Case Report

Year 2022, , 987 - 992, 30.12.2022

Asuman Küçüköner , Ömer Küçüköner

https://doi.org/10.47115/jshs.1183343

Abstract

Wolf-Hirschhorn syndrome (WHS) is a contiguous gene disorder clinically characterized by prenatal and postnatal growth deficiency, distinctive craniofacial features, mental retardation, and seizures. The disease is caused by partial loss of the distal part of the short arm of chromosome 4. Although hearing loss has been reported in individuals with WHS in the literature, sensorineural hearing loss is rare. In this article, it is aimed to give information about hearing loss and rehabilitation with a hearing aid in a patient with WHS who has sensorineural hearing loss.

Keywords

Wolf-Hirschhorn syndrome, Sensorineural hearing loss, Hearing aid, Auditory rehabilitation

WOLF HİRSCHHORN SENDROMUNDA ODYOLOJİK BULGULAR: OLGU SUNUMU

Abstract

Wolf-Hirschhorn sendromu (WHS), klinik olarak doğum öncesi ve doğum sonrası büyüme eksikliği, ayırt edici kraniyofasiyal özellikler, mental retardasyon ve nöbetlerle tanımlanan bitişik bir gen bozukluğudur. Hastalığa 4. kromozomun kısa kolunun distal kısmında oluşan kısmi kayıp neden olur. Literatürde WHS'li bireylerde işitme kaybı bildirilmiş olmasına rağmen, sensörinöral işitme kaybı nadirdir. Bu makalede sensörinöral işitme kaybı mevcut olan WHS’li hastanın işitme kaybı ve işitme cihazı ile rehabilitasyonu hakkında bilgi vermek amaçlanmıştır.

Keywords

Wolf-Hirschhorn sendromu, sensörinöral işitme kaybı, işitme cihazı, İşitsel rehabilitasyon

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