Study Outlines Rare Case of PWS Patient with Ear Canal and Bone Infection

A new study described a rare case of a young man with Prader-Willi syndrome (PWS) with an invasive infection of the ear canal and the skull base who was successfully treated with antibiotics.

The research, “Malignant otitis externa in a 21-year-old male patient with Prader-Willi syndrome,” was published in the journal SAGE Open Medical Case Reports.

Malignant otitis externa (MOE) affects the external auditory canal and temporal bone, and may have life-threatening complications. Patients with type 2 diabetes are most susceptible to MOE, but any type of immunosuppression setting can predispose an individual to the disease. The most frequent pathogen found in these patients is the bacteria Pseudomonas aeruginosa.

Now, a team in Brazil presented the case of a 21-year-old man with PWS, diabetes, and hypertension. He presented to the emergency department with fever, otalgia, and otorrhea — ear pain and discharge, two common initial complaints in people with MOE.

On admission, the patient had tachypnea (abnormally rapid breathing), increased respiratory effort, and marked left-sided otorrhea. Laboratory tests indicated diabetic ketoacidosis, which results from a faster-than-normal breaking down of fat into blood acids called ketones.

Computed tomography scans of the head and temporal bones — at the sides and base of the skull — revealed small areas of mastoid cell erosion in the left side, as well as significant discharge extending from the ear to soft tissues outside the cranium, in the left face, and in the cervical region.

A lumbar puncture excluded the spread of the infection into the central nervous system.

The patient was treated with an antimicrobial regimen, including vancomycin and meropenem. However, on the second day of the hospital stay, the patient’s condition deteriorated — his respiratory pattern worsened and he developed metabolic acidosis (excess production of acid). He was transferred to the intensive care unit (ICU). He required intubation and mechanical ventilation for six days.

The antibiotic regimen was changed to meropenem plus ciprofloxacin and dexamethasone on the fifth day of the initial treatment, because the patient no longer had a fever.

However, his condition worsened after being discharged from the ICU, with resurgence of fever, as well as cough with mucopurulent sputum, and a deteriorated respiratory pattern. Adding the antibiotic polymyxin B to the treatment plan for eight days to treat a hospital-acquired pneumonia eased these complications.

On day 21 of hospitalization, two imaging assessments showed alterations suggestive of infection in the left temporal bone. Fifteen days later, the patient showed eased ear and motor symptoms, as well as improved clinical and laboratory results. He was discharged in good condition, with a prescription for ciprofloxacin 750 mg and outpatient care.

One month later, the patient showed a pronounced decrease in active infection and inflammation in the left temporal region. Three months after discharge, the patient had no evidence of abnormalities. This led to discontinuation of the antibiotic treatment.

According to the team, to the best of its knowledge, “this is the first description of malignant otitis externa in a young patient with PWS.”

This case report draws the attention to “the importance of suspecting of MOE even in young patients, especially if risk factors for the disease are present, once early diagnosis can avoid or minimize life-threatening complications,” researchers said.