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Published on: July 6, 2013
Progressive hearing loss following acquired cytomegalovirus infection in an immunocompromised child
Ken Kato1, Hironao Otake, Mitsuhiko Tagaya
1Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine, 65, Tsurumai-cho, Showa-ku, Nagoya, Japan. katoken@med.nagoya-u.ac.jp
Insights
This case study highlights acquired cytomegalovirus (CMV) infection causing progressive hearing loss in a child with Langerhans cell histiocytosis (LCH). The findings suggest a link between CMV infection and sensorineural hearing loss in immunocompromised patients.
Area of Science:
- Pediatric Otolaryngology
- Infectious Diseases
- Hematology-Oncology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder.
- Umbilical cord blood transfusion is a treatment for severe LCH.
- Cytomegalovirus (CMV) infection is a common opportunistic infection in immunocompromised individuals.
Observation:
- A 5-month-old female diagnosed with LCH received an unrelated donor umbilical cord blood transfusion at 14 months.
- CMV infection was confirmed post-transfusion, indicating an acquired infection.
- Progressive mixed and sensorineural hearing loss was observed starting at age 7, advancing to profound bilateral hearing loss over 3 years.
Findings:
- Three-dimensional FLAIR MRI revealed inner ear high-intensity areas suggestive of bilateral labyrinthitis.
- The acquired CMV infection, occurring under iatrogenic immunodeficiency, is implicated as the cause of progressive sensorineural hearing loss.
- This case underscores the potential ototoxicity of CMV in immunocompromised pediatric patients.
Implications:
- Highlights the importance of audiological monitoring in immunocompromised children with LCH and post-transfusion CMV infection.
- Suggests CMV infection as a potential cause of progressive sensorineural hearing loss in this vulnerable population.
- Informs clinical practice regarding the management of hearing complications in pediatric LCH patients.
Abstract:
We report a rare case of progressive hearing loss after acquired CMV infection in a child with Langerhans cell histiocytosis (LCH). A 5-month-old female was diagnosed as having LCH. When she was 14 months old, she received an unrelated donor umbilical cord blood transfusion for the treatment of intractable LCH. CMV infection was confirmed after the blood transfusion. Because her own umbilical cord had no CMV, the CMV infection was not congenital. When she was 7 years old, mixed hearing loss was noted with bilateral otitis media with effusion. After that time, the sensorineural hearing loss progressed to bilateral profound hearing loss over 3 years. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging with gadolinium contrast enhancement revealed a high intensity area in the inner ear that suggested bilateral labyrinthitis. This case demonstrates the possibility that, under the immunodeficiency, the acquired CMV infection causes progressive sensorineural hearing loss.
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