A 15-Year-Old Boy With Facial Palsy and Progressive Hearing Loss

نویسندگان

  • Paola America Huaynate-Cuadrado
  • Janet M. Poulik
  • Jocelyn Yu Ang
چکیده

A 15-year-old previously healthy African American male developed peripheral facial palsy over the course of 10 days. His primary care physician prescribed steroids and acyclovir to which he initially showed improvement, which lasted for about a month. After the facial palsy was resolved, he started complaining of unilateral, intermittent headaches and progressive right-sided hearing loss that worsened over a month associated to dizziness. He was taken to the emergency department. On his initial admission, a brain magnetic resonance imaging (MRI) was performed, which showed right laby-rinthitis and bilateral neuritis of cranial nerves, dural enhancement consistent with meningitis, and partial opacification of the mastoid air cells with right side predominance (Figure 1A-C). A spinal tap was performed and revealed no evidence of bacterial or viral meningitis. The patient was admitted to the Infectious Disease service for a workup. His hearing loss had progressed to the left side as well and was observed to be mostly sensori-neural. Blood and cerebrospinal fluid (CSF) cultures for bacterial, fungal, mycobacterial, and viral microorganisms were performed; however, no growth was shown. Serology was reactive for Epstein-Barr virus and varicella zoster virus, showing a resolved infection and previous immunization, respectively. Mycoplasma IgM also was positive. He completed 5 days of azithromycin and 7 days of acyclovir followed by 7 days of valacyclovir. A rheu-matologic workup was performed showing the following: antinuclear antibody, weakly positive, with a 1:80 titer for both homogeneous and speckled pattern; anti-dsDNA antibody, negative; rheumatoid factor, 2.1 IU/mL (reference range = 0.0-6.0 IU/mL); extractable nuclear antigen screen, negative; angiotensin-converting enzyme (ACE), 106 Units/L (reference range = 18-101 Units/L). A high-resolution thorax computed tomography was normal. Full sequencing of the NOD2 gene was negative for the mutation associated with Blau's syndrome. The patient was started on systemic steroids and was discharged. He continued to follow with ENT and was not seen by other subspecialties. Over the course of 2 months, ENT continued with the steroids, but the hearing loss and the facial palsy worsened when a taper was attempted. Audiograms from this period showed profound bilateral sensorineural hearing loss, and because of the clinical progression the child was admitted to the Infectious Disease service. CSF and blood studies were repeated, and for the first time, fluid from the right and left middle ear was sent for serology and culture. All the cultures remained sterile. Polymerase chain reaction (PCR) for herpes simplex virus (HSV) 1 and …

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عنوان ژورنال:

دوره 3  شماره 

صفحات  -

تاریخ انتشار 2016