Tic-Like Repetitive Ear-Popping Behavior With Objective Eustachian Tube Opening in an Adult With Tourette Syndrome

Abstract

Tourette syndrome presents with diverse motor and vocal tics, yet involvement of Eustachian tube dynamics has not been previously described. We report the case of a 31-year-old woman who developed an unusual symptom she described as an “ear-popping tic,” characterized by a strong premonitory urge to release air from the ears and clicking sounds. Her long clinical history included childhood-onset tics, emotional instability, psychiatric hospitalizations, and fluctuating tic severity. At age 31, she reported repetitive ear-popping sensations accompanied by voluntary suppression to some extent. Ear, nose, and throat examination revealed normal tympanic membranes and hearing thresholds. Acoustic Eustachian tube function testing objectively demonstrated repetitive Eustachian tube opening independent of swallowing, a finding not previously documented in tic disorders. The patient described a strong urge to engage in ear-popping and partial suppressibility, although the temporal relationship between the auditory sensation and the urge was unclear. Differential diagnoses included voluntary ear-popping behavior, palatal myoclonus, middle ear myoclonus, and tensor tympani syndrome. The repetitive non-swallow-related tube opening was considered most consistent with tic-like contractions of the tensor veli palatini and levator veli palatini muscles, although voluntary components could not be fully excluded. One month later, she adopted lifestyle practices inspired by traditional East Asian medicine, leading to gradual psychological stabilization. The ear-popping tic became less intrusive, and all medications were eventually discontinued. This case highlights the importance of objective assessment in distinguishing tic-like phenomena from other causes of Eustachian tube opening.

INTRODUCTION

We report the case of a 31-year-old woman with adult Tourette syndrome, whose chief complaint was, as she termed it, a “tic of releasing air from the ears.” Frequent opening and closing of the Eustachian tube unrelated to swallowing was observed using Eustachian tube function testing, and patulous Eustachian tube was ruled out. Palatal myoclonus was considered in the differential diagnosis. This case involved an extremely rare phenomenon, which we considered could be described as “tic-like repetitive ear-popping.” According to Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition diagnostic criteria, the patient met the requirements for Tourette syndrome, including the presence of multiple motor tics and at least one vocal tic since childhood, symptom persistence for more than 1 year, onset before age 18, and the absence of another medical condition that could better explain the symptoms. The patient provided consent for the use of her clinical information, and the study was approved by our institutional ethics committee (SMNCC26-01).

CASE PRESENTATION

Family history

The patient grew up in a single-mother household following the divorce of her parents shortly after her birth. Her mother was usually otherwise occupied and often emotionally distant from the patient.

History of present illness

Age 8: The patient developed a tic characterized by straining of the abdomen and groaning “uu….” She experienced dyspnea and was evaluated by a child psychiatrist at a children’s hospital. The patient was then referred to a university hospital where pharmacotherapy was initiated.

Age 16: During high school, emotional instability emerged, leading to refusal to attend school.

Age 18: The patient entered a vocational school but was unable to adapt and stopped attending. Around this time, she became mentally unstable, exhibiting behavioral disturbances such as loud shouting, and was hospitalized twice in the Psychiatric Department of the university hospital.

Age 23: After discharge, the vocal tics gradually worsened.

Age 25: The patient visited our clinic. Medications were adjusted, psychological support was continued, and she sought employment while attending a vocational support facility for people with disabilities. She was able to work temporarily and had a period of stability.

Age 27: Brain MRI findings were normal.

Age 29: There was an increase in blinking and a tic involving breath-holding and groaning “uuu….” Work-related stress led to a depressive state, with agitation and suicidal ideation; she was seen by a psychiatrist, and risperidone was administered.

Age 30: The patient wandered outside at night with the intention to die after having ingested 60 tablets of valproate, which resulted in emergency transport and hospitalization in two psychiatric hospitals. Owing to a conflict with her mother, she began to live alone. The Wechsler Adult Intelligence Scale showed the following scores: a full IQ (78), verbal comprehension (64), perceptual organization (91), working memory (74), and processing speed (105). At this time, low-frequency sensorineural hearing loss was observed. Her medications included: clonazepam (1 mg), aripiprazole (3 mg, for the tics), sodium valproate (400 mg, for the epileptiform abnormalities indicated on electroencephalogram [EEG]), fluvoxamine (50 mg, for anxiety and obsessive-compulsiveness), and Yokukansan extract granules (Chinese medicine, 5 g).

Age 31: The patient began reporting hearing “shutter-like sounds,” “a tic of releasing air from the ears” and complained of a clear urge, “I want to pop my ears, release the air, let it out.” She also reported: “If I try to hold it back, I want to do it more, and I can control it to some extent.” The sounds were semi-rhythmic, between 0.5 and 2 seconds in duration, and continued throughout her waking hours. We suspected that this was due to the release of air from the ears. During neurological examination at our clinic, audible clicking sounds were heard from both ears. When asked whether these clicking sounds were tics, she responded that they were. We observed contraction of buccal muscles or a force exerted around the ears, but the exact mechanism could not be identified. The temporal relationship between the “shutter-like sounds” and the patient’s urge (“I want to pop my ears, release the air”) was unclear; however, she complained that there was a temporal relationship. Her symptoms resembled those of patulous Eustachian tube; therefore, she was referred to the university hospital’s Department of Otolaryngology.

ENT (ear, nose, and throat) findings

No abnormalities were identified in the tympanic membranes or external auditory canals. Pure-tone audiometry demonstrated normal hearing thresholds, and tympanometry revealed normal middle-ear function. Distortion Product Otoacoustic Emissions were also within normal limits. Acoustic Eustachian tube function testing did not demonstrate findings characteristic of patulous Eustachian tube. Instead, frequent opening and closing of the Eustachian tube, independent of swallowing, was recorded, which was interpreted as a tic phenomenon by the otolaryngologist (Fig. 1). This was considered to represent a tic involving repetitive ear-popping maneuvers. These findings indicated repetitive Eustachian tube opening independent of swallowing. This pattern was suggestive of tic-like activity; however, voluntary ear-popping maneuvers could not be excluded based on sonotubometry alone. Alternatively, given the possibility of repetitive voluntary ear-popping behavior, Eustachian tube dysfunction or soft-palate myoclonus were also considered in the differential diagnosis.

Fig. 1.

Sonotubometry findings. Acoustic measurements of Eustachian tube function in the right (upper panel) and left (lower panel) ears. Ch1 represents external auditory canal sound pressure (vertical axis), and Ch2 represents pharyngeal noise used as a swallowing marker. The horizontal axis indicates time, and 1.0 s/div denotes that each division corresponds to 1 second. Arrows on Ch2 indicate the time points at which the examiner instructed the patient to swallow. Swallowing-related events were identified by the presence of pharyngeal noise on Ch2. Non-swallow-related events were defined as increases in external auditory canal sound pressure on Ch1 occurring in the absence of pharyngeal noise on Ch2, indicating Eustachian tube opening unrelated to swallowing. “Duration” values shown on the figure represent automatically generated measurement intervals and do not indicate the duration of individual Eustachian tube openings, which were all less than 1 second and within the normal physiological range. Repetitive, non-swallow-related events correspond to involuntary eustachian tube opening associated with the patient’s tic-like events (downward arrow), whereas other peaks correspond to swallowing (upward arrow). Frequent tube opening unrelated to swallowing was observed bilaterally. CAL, calibration; S.P., sound pressure.

Clinical course

Following the ENT evaluation, the patient continued to complain of worsening ear sounds and marked discomfort. The patient exhibited significant body tension and generalized pain. Jaw pain intensified, and she began using a nighttime mouthpiece as a treatment for the temporomandibular joint disorder. One month later, the patient adopted several lifestyle modifications inspired by traditional East Asian practices. Although the causal relationship between her condition and these modifications is unclear, her psychological state gradually stabilized, and the tic-like repetitive ear-popping behavior became less distressing.

DISCUSSION

We interpreted this patient’s unusual symptom as tic-like repetitive ear-popping behavior, based on her long-standing history of tic symptoms, the presence of a strong premonitory urge, and partial suppressibility. Acoustic Eustachian tube function testing confirmed repetitive Eustachian tube opening independent of swallowing, providing objective physiological evidence of non-swallow-related tube opening. However, sonotubometry alone cannot distinguish voluntary ear-popping maneuvers from tic-like involuntary contractions. Although the patient reported an urge to pop her ears and partial suppressibility, we could not definitively confirm whether she was consciously performing ear-popping maneuvers during testing. Based on the clinical course and her tic history, tic-like repetitive ear-popping behavior was considered the most plausible explanation, although voluntary components could not be fully excluded.

Previous reports have described palatal tic [1] or palatal tremor-like phenomena associated with objective clicking sounds in patients with Tourette syndrome [2]. These cases suggested possible involvement of palatal or peritubal musculature. The present case differed in that repetitive Eustachian tube opening was objectively confirmed using acoustic Eustachian tube function testing, providing direct physiological evidence of non-swallow-related tube opening. The Eustachian tube is normally closed and opens transiently during swallowing or yawning to equalize pressure between the tympanic cavity and the nasopharynx [3]. The musculature involved in tube opening has been well described. The tensor veli palatini, innervated by the mandibular branch of the trigeminal nerve, applies tension to the soft palate, whereas the levator veli palatini, innervated by the pharyngeal plexus of the vagus nerve, elevates the soft palate and contributes to nasopharyngeal closure [4]. In this case, intermittent, nonswallow-related tube opening was considered most consistent with tic-like contractions of these muscles.

Sonotubometry is commonly used to evaluate Eustachian tube stenosis or patulous Eustachian tube [5,6]. Under normal swallowing, tube opening is accompanied by a decrease in external auditory canal pressure. By contrast, stenosis is characterized by a failure in pressure change during swallowing, whereas patulous Eustachian tube shows persistently elevated pressure. In the present case, pressure fluctuations occurred independently of swallowing, indicating Eustachian tube opening unrelated to normal physiological triggers.

Palatal myoclonus was considered in the differential diagnosis. Palatal myoclonus is characterized by rhythmic involuntary contractions of the soft palate and may be accompanied by objective tinnitus [7]. It is sometimes classified as a form of tremor, and tic-related mechanisms have been proposed, including suspected dopaminergic dysfunction in the basal ganglia [8]. Possible explanations for the patient’s clicking sounds include tinnitus associated with palatal myoclonus, muscular contraction sounds during soft palate elevation, or airflow-related sounds during Eustachian tube opening. Fiberoptic examination did not reveal palatal movements; therefore, a definitive diagnosis of palatal myoclonus could not be established.

Middle ear myoclonus and tensor tympani syndrome (TTS) [9] were also considered. These conditions involve involuntary contractions of the stapedius or tensor tympani muscles and may resemble facial tics. Contraction of the tensor tympani can secondarily open the Eustachian tube. Although the etiology of TTS remains unclear, psychological stress has been implicated.

The patient also experienced sudden sensorineural hearing loss, raising the possibility that she may have unconsciously attempted to open the Eustachian tube to regulate external auditory canal pressure. Such compensatory behavior could have contributed to the development of repetitive ear-popping actions.

Relevant differential diagnoses include habitual or compensatory ear-popping behavior, voluntary Valsalva-like maneuvers, palatal myoclonus or palatal tremor, middle ear myoclonus, TTS, and paroxysmal neurological phenomena, given the EEG abnormalities and valproate treatment. In addition, the patient’s low verbal comprehension score may have influenced how she described her internal sensations and urges. However, at neurological outpatient evaluation, neither habitual nor compensatory ear-popping behavior nor voluntary Valsalva-like maneuvers were observed. Although the patient showed EEG abnormalities, the overall clinical presentation was not suggestive of epilepsy. Therefore, these differential diagnoses were considered unlikely based on the findings at the time of examination.

Her psychological and physical state gradually stabilized after adopting lifestyle modifications inspired by traditional East Asian practices. Although the causal relationship is unclear, her symptom became less distressing, and pharmacotherapy was discontinued. Reduced attention to the symptom may itself have contributed to improvement, as diminished awareness is known to alleviate tic symptoms.

CONCLUSION

We reported a case of tic-like repetitive ear-popping behavior in an adult with Tourette syndrome, characterized by objective confirmation of non-swallow-related Eustachian tube opening using acoustic Eustachian tube function testing. Although the findings suggested tic-like contractions of the tensor veli palatini and levator veli palatini muscles, voluntary components could not be fully excluded, and several alternative diagnoses were considered. This case highlights the importance of objective physiological assessment and careful clinical correlation when evaluating atypical tic-like phenomena involving Eustachian tube dynamics.