Objective: To describe 3 cases of cervical dystonia with ear symptoms

Background: Dystonia is a movement disorder defined by sustained, patterned, sometimes tremulous postures with overflow movement. Non-motor symptoms are increasingly recognized (Albanese et al). Pain is common, particularly in cervical dystonia (Rosales et al; Comella et al). In a study of 167 patients with craniocervical dystonia, 62% reported pain (Poplawska-Domaszewicz et al). Pain typically involves posterior musculature with anterior radiation (Wang et al), but auricular involvement is rarely reported. We describe 3 cases of cervical dystonia with ear symptoms: 2 with otalgia and 1 with recurrent ear redness and pain consistent with red ear syndrome, all improved after botulinum toxin therapy.

Method: Clinical cases retrospectively collected from 2018-2025.

Results: Case 1: 53-year-old woman with severe right-sided neck pain radiating to the inner ear, right tilt/shift, and right shoulder elevation. MRI brain with internal auditory canal (IAC) was normal. Treated with 250U onabotulinum toxin, with 50U to the posterior auricular region, with symptom stabilization for ~4 years before loss to follow-up.

Case 2: 75-year-old man with right-sided neck pain beginning as posterior auricular pain spreading across the neck, ear fullness, posterior neck muscle hypertrophy, right tilt, and right shoulder elevation. MRI IAC was normal. Treated with up to 450U incobotulinum toxin, with 20U to the posterior auricular region, with symptom stabilization.

Case 3: 27-year-old woman with migraine presented with right head turn/tilt and dystonic tremor. She reported recurrent right ear redness and burning pain independent of migraine but associated with neck tightness. MRI brain was unremarkable. Treatment with 440U incobotulinum toxin improved dystonia and ear symptoms.

Conclusion: Cervical dystonia–related pain is a major contributor to disability. Pain may extend beyond typical posterior and anterior neck distributions to the posterior auricular or inner ear regions and, rarely, occur with red ear syndrome. Normal MRI IAC suggests symptoms are unlikely due to cochlear nerve pathology but may reflect abnormal central pain processing. Targeted toxin injections near auricular regions may reduce pain and improve quality of life. Larger case series are needed to better characterize this phenomenon. These cases highlight the importance of recognizing non-motor symptoms, including pain, in cervical dystonia.

References: 1. Albanese A, Di Giovanni M, Lalli S. European dystonia: diagnosis and management. J Neurol. 2019;26(1):5–17. doi:10.1111/ene.13762.
2. Rosales RL, Cuffe L, Regnault B, Trosch RM. Pain in cervical dystonia: mechanisms, assessment, and treatment. Expert Rev Neurother. 2021;21(10):1125–1134. doi:10.1080/14737175.2021.1984230.
3. Comella C, Bhatia K. An international survey of patients with cervical dystonia. J Neurol. 2015;262(4):837–848. doi:10.1007/s00415-014-7586-2.
4. Poplawska-Domaszewicz K, et al. Nonmotor symptoms of craniocervical dystonia: a multicentre international study using the Dystonia Non-Motor Symptoms Questionnaire. J Neural Transm. 2025.
5. Wang X, et al. Pain localization and response to botulinum toxin in cervical dystonia. Dystonia. 2025. doi:10.3389/dyst.2025.14652.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/auricular-symptoms-in-cervical-dystonia-a-case-series/