A woman in her forties with ear pain

A woman in her forties was referred to a maxillofacial surgery department by a dental specialist in oral surgery. The woman had a history of asthma and used an inhaler with asthma medication (ipratropium bromide 40 µg × 4, beclomethasone/formoterol 100/6 µg up to × 4 as needed) and a contraceptive pill. She did not smoke or use snus and did not drink alcohol. She reported that two months earlier she had a relatively rapid onset of left-sided ear fullness. She consulted her GP, who put her on sick leave and prescribed a course of antibiotics (phenoxymethylpenicillin 660 mg × 4 perorally for one week) due to suspected otitis media. Four days later, she experienced severe pain in the external auditory canal on the same side. Mild redness was observed in the ear canal, but there was no rash or other signs of external otitis, and C-reactive protein (CRP) was < 5 mg/L (reference range 0–4). She remained afebrile upon repeated examination by her GP.

Ciprofloxacin/fluocinolone acetonide ear drops (2 mg/mL) were tried twice a day for a week to treat redness in the ear canal, and decongestant phenylpropanolamine tablets (50 mg × 2 as needed) were prescribed for suspected sinusitis. Due to the lack of improvement, the patient returned to her GP four days later, who prescribed a combination nasal spray with antihistamine and corticosteroid (Dymista, 137 µg/50 µg × 2) and clindamycin capsules (150 mg × 4 for one week). However, this still did not alleviate the symptoms and the patient was subsequently referred to the Department of Otolaryngology. A CT of the head, sinuses and neck with intravenous contrast was performed, with normal findings except for possible pathology in the second molar in the upper left jaw (tooth 27). The patient was referred to a dental specialist in oral surgery for an assessment of dental status and the temporomandibular joint. The oral surgeon found no evidence of pathology in the teeth. A clinical examination of the temporomandibular joint was conducted, and for diagnostic purposes, local anaesthesia was administered to the left temporomandibular joint without relieving the pain.

The oral surgeon referred the patient to the maxillofacial surgery department. During consultation with the maxillofacial surgeon, the patient stated that the pain had gradually worsened and was affecting her sleep. She said that talking and swallowing triggered the pain and that it could last from minutes to several hours. No facial redness or swelling was observed. Upon examination, palpation tenderness was noted in the ear canal and numbness in the left nasal floor, along with reduced sensitivity in the posterior third of the tongue on the left side, and a burning sensation when touching the palate on the same side. No pain upon palpation of the styloid processes in the tonsil and posterior pharyngeal wall (which can be present in pain caused by elongated styloid processes, known as Eagle syndrome) (6).

The maxillofacial surgeon suspected glossopharyngeal neuralgia and referred the patient for MRI with intravenous contrast to assess whether there were signs of nerve compression from surrounding blood vessels. MRI (3D T2 turbo spin echo and fat-suppressed T1 after intravenous contrast) of the head and skull base revealed asymmetry around the glossopharyngeal nerve. The course of the nerve on the right side was unremarkable, while several small vascular structures were observed along the nerve on the left side at the cerebellopontine angle. These were interpreted as small pial veins (Figure 1). No arteries were observed in relation to the nerve or signs of denervation of the stylopharyngeus. A small, round contrast uptake was noted at the dura in the foramen magnum on the left side, prompting further examination with CT angiography of pre- and intracerebral vessels to rule out an aneurysm. CT showed normal pre- and intracerebral arteries and no aneurysmal dilation of the posterior inferior cerebellar artery. Bilateral elongated styloid processes were observed.

The patient was referred to a neurologist who initiated treatment with carbamazepine (200 mg × 2) for neuropathic pain, but this was discontinued due to adverse effects in the form of abdominal pain and constipation. The neurologist noted that the patient was now experiencing left-sided pain in the ear and throat throughout the day. The pain could improve for a few hours during the day, only to reoccur. The patient was still on sick leave due to the pain and was unable to perform her daily activities. Neurological examination confirmed sensory loss in the tongue and palate, and there was loss of pharyngeal reflex on the left side. Pregabalin (50 mg × 3) was prescribed.

Due to the intolerable pain, and in order to further investigate the condition, the patient was admitted to the neurology department two months after the initial assessment by the maxillofacial surgeon and three months after the onset of symptoms. At that time, she had been on pregabalin for three days with no improvement. A weight loss of 6.5 kg was reported since the onset of pain. Besides the impairments in the pharynx and palate, the neurological examination was normal. The patient underwent a chest CT, spinal puncture and blood tests to rule out neuroinflammatory and infectious causes, such as neurosarcoidosis and neuroborreliosis. All findings were normal, and her SARS-CoV-2 test was negative.

The patient was started on oxcarbazepine (titrated up to 300 mg × 2) for pain relief. She was also prescribed a 'cream mixture' consisting of 10 mL paracetamol syrup (24 mg/mL) and 10 mL of Xylocain gel (2 %) mixed with a little cream, which she could take 20–30 minutes before each meal. Eleven days after discharge, the patient contacted the neurology department again. She was distressed due to the worsening pain in her throat, palate and left ear, along with increasing problems with eating and drinking. The pain made it difficult for the patient to talk and use her mouth, and her swallowing was severely restricted.

The patient was recommended further titration of oxcarbazepine and an increase in the frequency of the cream mixture. Paracetamol 1 g × 4, etoricoxib 90 mg × 1 and amitriptyline 25 mg × 1 were also prescribed. The patient was referred for evaluation for decompression surgery due to glossopharyngeal neuralgia and was assessed by a neurosurgeon five months after the onset of symptoms. The condition was diagnosed as glossopharyngeal neuralgia with neuropathy. The clinical picture was deemed consistent with the MRI findings of arteries adjacent to the glossopharyngeal nerve on the left side, and the patient was referred for microvascular decompression surgery. Surgery took place six months after the onset of symptoms. Peroperatively, a large calibre vein was found along the entire course of the glossopharyngeal nerve on the left side, consistent with the MRI findings. The vein was separated and fixed away from the nerve. The postoperative period was uneventful, except for some nausea and dizziness. The patient was discharged to a local hospital after two days for further mobilisation. At a neurology check-up eleven weeks after surgery, the patient reported a significant improvement in pain, with shorter and less intense episodes than previously. One year after surgery, she experienced further improvement, and two years after surgery she continues to improve. She still sometimes experiences discomfort when exposed to the cold and when feeling tired. She underwent rehabilitation to normalise tongue and swallowing motor function, and her body weight has returned to normal (following a total weight loss of 10 kg up to the operation).