Geniculate Neuralgia: A Case Report and Systematic Review

Neurosurgery Open 2022;3(4):e00022.

Geniculate neuralgia (GN) is a rare disorder characterized by brief paroxysms of pain felt deeply in the auditory canal, like shock. The diagnosis of GN is essentially clinical and requiring the exclusion of other causes. The aim of this study was to report a case of surgical treatment of the disease and perform a systematic review of surgical treatment options.

CLINICAL PRESENTATION: A 62-year-old female patient has had severe, sharp pain in her left ear—like an ice pick—for the past 8 months, without any history of trauma or infection. She had triggers for pain, such as talking or swallowing. Imaging investigation showed a vessel touching VIIth and VIIIth nerves, possibly the anterior inferior cerebellar artery. After finding a clinical presentation compatible with GN, treatment with anticonvulsants, gabapentinoids, and opioids was attempted, but without success with such conservative treatments. Therefore, we opted for microvascular decompression and section of the nervus intermedius, which were performed without complications. Neurophysiological assessment was essential to monitor the long tracts and stimulate VIIth and VIIIth nerves to help identify the intermediate nerve. After the procedure, the patient was without pain, and after 12 months of follow-up, she remains without any pain.

CONCLUSION: Surgical treatment of GN might be beneficial when medical treatment has not worked. Cranial nerves neurophysiological monitoring is not routinely performed, and the identification is based on anatomy. A more comprehensive knowledge of this condition will help in the surgical treatment choice and in achieving better results.

Pain-free and pain-controlled survival after sectioning the nervus intermedius in nervus intermedius neuralgia: a single-institution review

J Neurosurg 131:352–359, 2019

Nervus intermedius neuralgia (NIN) or geniculate neuralgia is a rare facial pain condition consisting of sharp, lancinating pain deep in the ear and can occur alongside trigeminal neuralgia (TN). Studies on the clinical presentation, intraoperative findings, and ultimately postoperative outcomes are extremely limited. The aim of this study was to examine the clinical presentation and surgical findings, and determine pain-free survival after sectioning of the nervus intermedius (NI).

METHODS The authors conducted a retrospective chart review and survey of patients who were diagnosed with NIN at one institution and who underwent neurosurgical interventions. Pain-free survival was determined through chart review and phone interviews using a modified facial pain and quality of life questionnaire and represented as Kaplan-Meier curves.

RESULTS The authors found 15 patients with NIN who underwent microsurgical intervention performed by two sur- geons from 2002 to 2016 at a single institution. Fourteen of these patients underwent sectioning of the NI, and 8 of 14 had concomitant TN. Five patients had visible neurovascular compression (NVC) of the NI by the anterior inferior cerebellar artery in most cases where NVC was found. The most common postoperative complaints were dizziness and vertigo, diplopia, ear fullness, tinnitus, and temporary facial nerve palsy. Thirteen of the 14 patients reportedly experienced pain relief immediately after surgery. The mean length of follow-up was 6.41 years (range 8 months to 14.5 years). Overall recurrence of any pain was 42% (6 of 14), and 4 patients (isolated NIN that received NI sectioning alone) reported their pain was the same or worse than before surgery at longest follow-up. The median pain-free survival was 4.82 years ± 14.85 months. The median pain-controlled survival was 6.22 years ± 15.78 months.

CONCLUSIONS In this retrospective review, sectioning of the NI produced no major complications, such as permanent facial weakness or deafness, and was effective for patients when performed in addition to other procedures. After sectioning of the NI, patients experienced 4.8 years pain free and experienced 6.2 years of less pain than before surgery. Alone, sectioning of the NI was not effective. The pathophysiology of NIN is not entirely understood. It appears that neurovascular compression plays only a minor role in the syndrome and there is a high degree of overlap with TN.

Nervus intermedius and the surgical management of geniculate neuralgia

J Neurosurg 131:343–351, 2019

Geniculate neuralgia (GN) is an uncommon craniofacial pain syndrome attributable to nervus intermedius (NI) dysfunction. Diagnosis and treatment can be challenging, due to the complex nature of ear sensory innervation, resulting in clinical overlap with trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN).

METHODS A retrospective review of a prospective neurosurgical database at our institution was performed, 2000–2017, with a corresponding systematic literature review. Pain outcomes were dichotomized as unfavorable for unchanged/worsened symptoms versus favorable if improved/resolved. Eight formalin-fixed brains were examined to describe NI at the brainstem.

RESULTS Eleven patients were surgically treated for GN—9 primary, 2 reoperations. The median age was 48, 7 pa- tients were female, and the median follow-up was 11 months (range 3–143). Seven had ≥ 2 probable cranial neuralgias. NI was sectioned in 9 and treated via microvascular decompression (MVD) in 2. Five patients underwent simultaneous treatment for TN (4 MVD; 1 rhizotomy) and 5 for GPN (3 MVD; 2 rhizotomy). Eleven reported symptomatic improvement (100%); 8 initially reported complete resolution (73%). Pain outcomes at last contact were favorable in 8 (73%)—all among the 9 primary operations (89% vs 0%, p = 0.054). Six prior series reported outcomes in 111 patients.

CONCLUSIONS GN is rare, and diagnosis is confounded by symptomatic overlap with TN/GPN. Directed treatment of all possible neuralgias improved pain control in almost all primary operations. Repeat surgery seems a risk factor for an unfavorable outcome. NI is adherent to superomedial VIII at the brainstem; the intermediate/cisternal portion is optimal for visualization and sectioning.