Low-Frequency Sensorineural Hearing Loss in Familial Hemophagocytic Lymphohistiocytosis Type 5
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Low-Frequency Sensorineural Hearing Loss in Familial Hemophagocytic Lymphohistiocytosis Type 5. / Bezdjian, Aren; Bruijnzeel, Hanneke; Pagel, Julia; Daniel, Sam J; Thomeer, Hans G X M.
In: ANN OTO RHINOL LARYN, Vol. 127, No. 6, 06.2018, p. 409-413.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Low-Frequency Sensorineural Hearing Loss in Familial Hemophagocytic Lymphohistiocytosis Type 5
AU - Bezdjian, Aren
AU - Bruijnzeel, Hanneke
AU - Pagel, Julia
AU - Daniel, Sam J
AU - Thomeer, Hans G X M
PY - 2018/6
Y1 - 2018/6
N2 - INTRODUCTION: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased.CASE PRESENTATION: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature. Management and Outcome: The progressive nature of FHL disorder may cause bilateral, low-frequency, irreversible sensorineural hearing loss. This type of hearing loss should be considered among the long-term sequelea presenting with FHL5.DISCUSSION: We recommend audiological evaluation at initial FHL5 diagnosis to assess for hearing functions. Follow-up in audiology should be part of the long-term monitoring of patients with FHL5 as hearing loss could develop long after diagnosis.
AB - INTRODUCTION: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased.CASE PRESENTATION: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature. Management and Outcome: The progressive nature of FHL disorder may cause bilateral, low-frequency, irreversible sensorineural hearing loss. This type of hearing loss should be considered among the long-term sequelea presenting with FHL5.DISCUSSION: We recommend audiological evaluation at initial FHL5 diagnosis to assess for hearing functions. Follow-up in audiology should be part of the long-term monitoring of patients with FHL5 as hearing loss could develop long after diagnosis.
KW - Adult
KW - Hearing Loss, Sensorineural/diagnosis
KW - Humans
KW - Lymphohistiocytosis, Hemophagocytic/complications
KW - Male
U2 - 10.1177/0003489418771714
DO - 10.1177/0003489418771714
M3 - SCORING: Journal article
C2 - 29776323
VL - 127
SP - 409
EP - 413
JO - ANN OTO RHINOL LARYN
JF - ANN OTO RHINOL LARYN
SN - 0003-4894
IS - 6
ER -