By Marcello Cherchi, MD PhD
For patients
Exploding head syndrome (EHS) is a disorder in which, while you are just about to fall asleep, or just as you are about to wake up, you think you hear a loud sound that is short but frightening. This problem usually goes away on its own. If the problem persists, some people consider trying medication for it.
For clinicians
Overview
Exploding head syndrome (EHS) is a benign parasomnia consisting of a hypnogogic/hypnopompic auditory hallucination in which the patient experiences a very brief, loud sound, accompanied by fear, during the transition into or out of sleep. This can occur at any age, may be slightly more prevalent in females, does not have any clear risk factors, and generally occurs in isolation. Occasional abnormalities in electroencephalography and polysomnography have been described, though these are not needed to secure the diagnosis. Reassurance is generally sufficient for management, as the disease often remits spontaneously. Treatment with medications has been attempted, but none has been studied in randomized controlled trials.
Introduction
Sharpless (Sharpless 2014) states that the term “exploding head syndrome” (EHS) is usually credited to Pearce (Pearce 1988), but noted that the phenomenon was probably described at least as early as 1920 by Armstrong-Jones (Armstrong-Jones 1920) under the designation, “snapping of the brain.”
EHS is a parasomnia. Although the name implies a painful experience, this is actually a hypnogogic or hypnopompic auditory hallucination (Blom 2015) often accompanied by fright (Alkhateeb et al. 2023; Denis et al. 2019; Sharpless 2015). Patients generally experience this as an abrupt, disturbing sound while they are on the verge of transitioning from the waking state to the sleeping state, or from the sleeping state to the waking state.
Most of the literature on this topic consists of case reports and case series (Alkhateeb et al. 2023; Chakravarty 2008; Frese et al. 2014; Ganguly et al. 2013; Pearce 1989; Pirzada et al. 2020; Wang et al. 2019), and there are several reviews (Ceriani and Nahas 2018; Pearce 1988; Sharpless 2014).
Epidemiology
Aazh and colleagues (Aazh et al. 2023) reported that of 148 consecutive patients attending an audiology clinic for tinnitus/hyperacusis, 12 (8.1%) were diagnosed with EHS. The investigators found no clear correlation between the presence of EHS and demographic factors (age, gender), clinical features (tinnitus/hyperacusis distress) or comorbidities (anxiety, depression, sleep difficulties).
Denis and colleagues (Denis et al. 2019) conducted two studies on EHS. The first study analyzed 199 female undergraduate students and reported a lifetime prevalence of 37.19%, with 6.54% experiencing at least one episode per month. The second study included a sample of 1683 participants, age range 18 – 82 years (53.00% female), in which they found a lifetime prevalence of 29.59%, with monthly episodes occurring in 3.89% of participants.
Kirwan and Fortune (Kirwan and Fortune 2021) studied 135 adults and found a lifetime prevalence of EHS of 20.0%. They reported that patients with EHS experienced “more symptoms of anxiety and poorer sleep quality,” but found no correlation between the presence of EHS and demographic factors (age, gender) or other comorbidities (depressive symptoms).
Sharpless and colleagues (Sharpless et al. 2020) conducted a cross-sectional study of 3286 individuals with EHS and 2954 without EHS. Contrary to other studies, they found a slightly higher prevalence among women.
Pathophysiological mechanism of disease
The pathophysiological mechanism of EHS is unknown. Investigators have speculated about etiologies such as dissociative episodes (Feketeova et al. 2014), “cranial sensory shock” (Goadsby and Sharpless 2016; Puledda et al. 2021), migraine aura (Rossi et al. 2018) and others.
Clinical presentation
EHS presents as the perception of a loud sound, usually lasting less than a second (Khan and Slowik 2023), during the transition into or out of sleep, often accompanied by sensations of fear. Some descriptions state that it can be a multi-sensory phenomenon, with up to 27% of patients reporting associated visual abnormalities (Sharpless 2018).
Occasionally EHS is reported in conjunction with other parasomnias or other diseases. For example, Evans (Evans 2006) reported the case of a 26-year-old female who would experience EHS followed by sleep paralysis and a migraine headache. Hayreh (Hayreh 2020) described his own experience with EHS and noted that the episodes were associated with sick sinus syndrome.
Testing
Some studies have reported electroencephalographic abnormalities in EHS patients (Fotis Sakellariou et al. 2020).
Some studies have documented polysomnographic abnormalities in EHS patients (Nakayama et al. 2021; Sachs and Svanborg 1991).
Treatment
Reassurance is the initial management strategy for EHS (Green 2001), because after patients understand that this is not a life-threatening condition, the experience is less frightening and they often do not feel the need for additional intervention.
Lifestyle management strategies to reduce EHS are reviewed by Sharpless and colleagues (Sharpless et al. 2020). These include changes in alcohol intake, sleep position, sleep time, mindfulness and breathing techniques, and changing the number of pillows.
A variety of pharmacologic treatments have been reported, though none has been studied in a randomized controlled trial. These include:
- Amitriptyline (Pirzada et al. 2020)
- Clobazam (Gillis and Ng 2017)
- Clomipramine (Green 2001; Sachs and Svanborg 1991)
- Nifedipine (Jacome 2001)
- Topiramate (Palikh and Vaughn 2010)
Other attempted treatment modalities include single-pulse transcranial magnetic stimulation (Puledda et al. 2021).
Prognosis
EHS is generally considered a benign disease, and often the symptoms remint spontaneously (Sachs and Svanborg 1991).
Kitagawa and colleagues (Kitagawa et al. 2023) reported the appearance of EHS in a patient who subsequently developed mild cognitive impairment that progressed, and the patient eventually was diagnosed with Lewy body disease.
References
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Copyright © 2023, StatPearls Publishing LLC., Treasure Island (FL)
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